Experiences of mothers of infants with congenital heart disease before, during, and after complex cardiac surgery.

PubMed

Harvey, Kayla A; Kovalesky, Andrea; Woods, Ronald K; Loan, Lori A

2013-01-01

Experiences of mothers of infants undergoing complex heart surgery were explored to build evidence-based family-centered interventions. Congenital heart disease is the most frequent birth defect in the United States and is common worldwide. Eight mothers recalled through journal entries their experiences of the days before, during, and after their infant's surgery and shared advice for other mothers. Colaizzi's phenomenological method was utilized for data analysis. A validation survey of seven additional mothers from a support group occurred via email. Six themes were identified and validated: Feeling Intense Fluctuating Emotion; Navigating the Medical World; Dealing with the Unknown; Facing the Possibility of My Baby Dying, Finding Meaning and Spiritual Connection, and the umbrella theme of Mothering Through It All. Through a clearer understanding of experiences as described by mothers, health-care providers may gain insight as to how to better support mothers of infants undergoing heart surgery. Copyright © 2013 Elsevier Inc. All rights reserved.

Abnormal brain development in newborns with congenital heart disease.

PubMed

Miller, Steven P; McQuillen, Patrick S; Hamrick, Shannon; Xu, Duan; Glidden, David V; Charlton, Natalie; Karl, Tom; Azakie, Anthony; Ferriero, Donna M; Barkovich, A James; Vigneron, Daniel B

2007-11-08

Congenital heart disease in newborns is associated with global impairment in development. We characterized brain metabolism and microstructure, as measures of brain maturation, in newborns with congenital heart disease before they underwent heart surgery. We studied 41 term newborns with congenital heart disease--29 who had transposition of the great arteries and 12 who had single-ventricle physiology--with the use of magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and diffusion tensor imaging (DTI) before cardiac surgery. We calculated the ratio of N-acetylaspartate to choline (which increases with brain maturation), the ratio of lactate to choline (which decreases with maturation), average diffusivity (which decreases with maturation), and fractional anisotropy of white-matter tracts (which increases with maturation). We compared these findings with those in 16 control newborns of a similar gestational age. As compared with control newborns, those with congenital heart disease had a decrease of 10% in the ratio of N-acetylaspartate to choline (P=0.003), an increase of 28% in the ratio of lactate to choline (P=0.08), an increase of 4% in average diffusivity (P<0.001), and a decrease of 12% in white-matter fractional anisotropy (P<0.001). Preoperative brain injury, as seen on MRI, was not significantly associated with findings on MRS or DTI. White-matter injury was observed in 13 newborns with congenital heart disease (32%) and in no control newborns. Term newborns with congenital heart disease have widespread brain abnormalities before they undergo cardiac surgery. The imaging findings in such newborns are similar to those in premature newborns and may reflect abnormal brain development in utero. Copyright 2007 Massachusetts Medical Society.

Peripheral oxygen saturation, heart rate, and blood pressure during dental treatment of children with cyanotic congenital heart disease

PubMed Central

Dutra, Rosane Menezes Faria; Neves, Itamara Lucia Itagiba; Neves, Ricardo Simões; Atik, Edmar; de Paula Santos, Ubiratan

2014-01-01

OBJECTIVES: In this observational study, we evaluated the peripheral oxygen saturation (SpO2), heart rate, and blood pressure of children with cyanotic congenital heart disease who were undergoing dental extraction. METHODS: Forty-four patients between the ages of 6 and 12 years who underwent upper primary tooth extraction were included in the study. Of these, 20 patients were in the cyanotic congenital heart disease group and 24 were in the control group. RESULTS: Peripheral oxygen saturation, heart rate, and systolic blood pressure in the cyanotic congenital heart disease group varied quite significantly during the treatment protocol (p<0.05), with values of 80.5% (±7.6) to 82.8% (±7.8), 95.3 beats per minute (bpm) (±11.3) to 101.3 bpm (±9.8), and 93.6 mm Hg (±13,3) to 103.8 mm Hg (±12.7), respectively. The variations in the control group during the procedure were also significant. CONCLUSIONS: The changes observed during the study protocol, although statistically significant, were mild and lacked clinical relevance. The results indicate that dental treatment of children with cyanotic heart disease using a standardized protocol in decentralized offices without the support of a surgical center is safe. PMID:24838895

Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival.

PubMed

Alonso-Gonzalez, Rafael; Borgia, Francesco; Diller, Gerhard-Paul; Inuzuka, Ryo; Kempny, Aleksander; Martinez-Naharro, Ana; Tutarel, Oktay; Marino, Philip; Wustmann, Kerstin; Charalambides, Menelaos; Silva, Margarida; Swan, Lorna; Dimopoulos, Konstantinos; Gatzoulis, Michael A

2013-02-26

Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease. A total of 1188 patients with adult congenital heart disease (age, 33.1±13.1 years) undergoing lung function testing between 2000 and 2009 were included. Patients were classified according to the severity of lung dysfunction based on predicted values of forced vital capacity. Lung function was normal in 53% of patients with adult congenital heart disease, mildly impaired in 17%, and moderately to severely impaired in the remainder (30%). Moderate to severe impairment of lung function related to complexity of underlying cardiac defect, enlarged cardiothoracic ratio, previous thoracotomy/ies, body mass index, scoliosis, and diaphragm palsy. Over a median follow-up period of 6.7 years, 106 patients died. Moderate to severe impairment of lung function was an independent predictor of survival in this cohort. Patients with reduced force vital capacity of at least moderate severity had a 1.6-fold increased risk of death compared with patients with normal lung function (P=0.04). A reduced forced vital capacity is prevalent in patients with adult congenital heart disease; its severity relates to the complexity of the underlying heart defect, surgical history, and scoliosis. Moderate to severe impairment of lung function is an independent predictor of mortality in contemporary patients with adult congenital heart disease.

In defence of auscultation: a glorious future?

PubMed Central

Thompson, W Reid

2017-01-01

Auscultation of the heart using a simple stethoscope continues to be a central aspect of the cardiovascular examination despite declining proficiency and availability of competing technologies such as hand-held ultrasound. In the ears and mind of a trained cardiologist, heart sounds can provide important information to help screen for certain diseases such as valvar lesions and many congenital defects. Using emerging technology, auscultation is poised to undergo a transformation that will simultaneously improve the teaching and evaluation of this important clinical skill and create a new generation of smart stethoscopes, capable of assisting the clinician in quickly and confidently screening for heart disease. These developments have important implications for global health, screening of athletes and recognition of congenital heart disease. PMID:28243316

National practice patterns for management of adult congenital heart disease: operation by pediatric heart surgeons decreases in-hospital death.

PubMed

Karamlou, Tara; Diggs, Brian S; Person, Thomas; Ungerleider, Ross M; Welke, Karl F

2008-12-02

Surgery for grown-up (age > or = 18 years) patients with congenital heart disease (GUCH) is frequently performed by surgeons without specialization in pediatric heart surgery. We sought to define national practice patterns and to determine whether outcomes for GUCH patients are improved if they are treated by specialized pediatric heart surgeons (PHSs) compared with non-PHSs. We identified index cardiac procedures in patients with 12 congenital heart disease diagnostic groups using the Nationwide Inpatient Sample 1988 to 2003. PHSs were defined as surgeons whose annual practice volumes were made of >75% annual pediatric heart cases. GUCH operations were defined as operations within these 12 diagnoses occurring in patients > or =18 years of age. We identified 30,250 operations, yielding a national estimate of 152,277 +/- 7,875 operations. Of these, 111,816 +/- 7,456 (73%) were pediatric operations, and 40,461 +/- 1,365 (27%) were GUCH operations. PHSs performed 68% of pediatric operations in all diagnostic groups, whereas non-PHSs performed 95% of GUCH operations within the same diagnostic groups (P<0.0001). In-hospital death rates for GUCH patients operated on by PHSs were lower than death rates for GUCH patients operated on by non-PHSs (1.87% [95% CI, 0.62 to 3.13] versus 4.84% [95% CI, 4.30 to 5.38%]; P<0.0001). Survival advantage increased with increasing surgeon annual pediatric volume (P=0.0031). Pediatric patients within specific diagnostic groups are more likely to undergo operation by PHSs, whereas GUCH patients within the same diagnostic groups are more likely to undergo operation by non-PHSs. In-hospital death rates are lower for GUCH patients operated on by PHSs. GUCH patients should be encouraged to obtain surgical operation by PHS.

Identifying predictors of hospital readmission following congenital heart surgery through analysis of a multiinstitutional administrative Database.

PubMed

Smith, Andrew H; Doyle, Thomas P; Mettler, Bret A; Bichell, David P; Gay, James C

2015-01-01

Despite resource burdens associated with hospital readmission, there remains little multiinstitutional data available to identify children at risk for readmission following congenital heart surgery. Children undergoing congenital heart surgery and discharged home between January of 2011 and December 2012 were identified within the Pediatric Health Information System database, a multiinstitutional collection of clinical and administrative data. Patient discharges were assigned to derivation and validation cohorts for the purposes of predictive model design, with 17 871 discharges meeting inclusion criteria. Readmission within 30 days was noted following 956 (11%) of discharges within the derivation cohort (n = 9104), with a median time to readmission of 9 days (interquartile range [IQR] 5-18 days). Readmissions resulted in a rehospitalization length of stay of 4 days (IQR 2-8 days) and were associated with an intensive care unit (ICU) admission in 36% of cases. Independent perioperative predictors of readmission included Risk Adjustment in Congenital Heart Surgery score of 6 (odds ratio [OR] 2.6, 95% confidence interval [CI] 1.8-3.7, P < .001) and ICU length of stay of at least 7 days (OR 1.9 95% CI 1.6-2.2, P < .001). Demographic predictors included Hispanic ethnicity (OR 1.2, 95% CI 1.1-1.4, P = .014) and government payor status (OR 1.2, 95% CI 1.1-1.4, P = .007). Predictive model performance was modest among validation cohort (c statistic 0.68, 95% CI 0.66-0.69, P < .001). Readmissions following congenital heart surgery are common and associated with significant resource consumption. While we describe independent predictors that may identify patients at risk for readmission prior to hospital discharge, there likely remains other unreported factors that may contribute to readmission following congenital heart surgery. © 2014 Wiley Periodicals, Inc.

Utility of Magnetic Resonance Imaging in Cardiac Venous Anatomic Variants

DOE Office of Scientific and Technical Information (OSTI.GOV)

Eckart, Robert E.; Leitch, W. Shad; Shry, Eric A.

2003-06-15

The incidence of persistent left superior venacava (PLSVC) is approximately 0.5% in the general population; however,the coexistent absence of the right SVC has a reported incidence in tertiary centers of 0.1%. The vast majority of reports are limited to pediatric cardiology. Likewise, sinus of Valsalva aneurysm is a rare congenital anomaly, with a reported incidence of 0.1-3.5% of all congenital heart defects. We present a 71-year-old patient undergoing preoperative evaluation for incidental finding of aortic root aneurysm,and found to have all three in coexistence. Suggestive findings were demonstrated on cardiac catheterization and definitive diagnosis was made by magnetic resonance imaging.more » The use of MRI for the diagnosis of asymptomatic adult congenital heart disease will be reviewed.« less

The Impact of Critical Care Nursing Certification on Pediatric Patient Outcomes.

PubMed

Hickey, Patricia A; Gauvreau, Kimberlee; Porter, Courtney; Connor, Jean A

2018-06-14

To examine the relationship of nursing and unit characteristics including Critical Care Registered Nurse certification on patient complications for children undergoing congenital heart surgery in free-standing children's hospitals in the United States. A cross-sectional descriptive survey of nursing and organizational characteristics was sent electronically. Free-standing children's hospitals in the United States. Children undergoing congenital heart surgery (congenital cardiac cases). Nursing and unit characteristics from children's hospitals were obtained via electronic survey during years 2010 and 2014. Survey data from 2014 were linked with patient-level data age less than 18 years old using the Pediatric Health Information System for years 2013-2014. The Complication Screening Method of Congenital Heart Surgery was used to adjust for baseline patient differences. Among 23 of 43 free-standing children's hospitals (53%) in 2014, the median proportion of nurses with Bachelor of Science in Nursing preparation or higher at each site was 77% and with Critical Care Registered Nurse certification was 24%. Among 12,324 eligible congenital cardiac cases, the rate of complication was 34.4%. The odds of complications significantly decreased as the institutional percentage of nurses with a Bachelor of Science in Nursing increased (odds ratio, 0.83; p = 0.04). The odds of complications also significantly decreased as the institutional percentage of nurses with Critical Care Registered Nurse certification increased (odds ratio, 0.86; p = 0.02). Bachelor of Science in Nursing education and Critical Care Registered Nurse certification significantly impact pediatric patient outcomes. Recognition of nursing and organizational characteristics that are associated with improved pediatric patient outcomes is important to patients, their families, and society. Clinical leaders must commit to facilitating pathways for hiring candidates with Bachelor of Science in Nursing as a minimum to practice in PICUs and supporting a culture of professional certification.

Anesthesia for a Rare Case of Uncorrected Pentalogy of Fallot Undergoing Craniotomy and Drainage of Brain Abscess.

PubMed

Marulasiddappa, Vinay; Raghavavendra, B S

2015-07-01

Children with uncorrected cyanotic congenital heart diseases can present for non cardiac surgeries. They pose several challenges to the Anaesthesiologist, especially when they are posted for emergency surgery, due to the complex haemodynamic changes secondary to the heart disease. Pentalogy of Fallot (POF) is a rare form of congenital heart disease characterized by the association of Tetralogy of Fallot (TOF) with an atrial septal defect (ASD). TOF is the leading cause of intracardiac right to left shunt and is the commonest type of cyanotic congenital heart disease to cause a brain abscess. Children with POF presenting with brain abscess pose several challenges to the anaesthesiologist due to the altered haemodynamics and warrant a meticulous anaesthetic plan. There are very few case reports of Anaesthesia management of a child with Pentalogy of Fallot (POF) presenting for non cardiac surgery. We report the anaesthetic management of a rare case of a 5-year-old child with uncorrected POF, who presented to our Superspeciality hospital with a brain abscess and underwent an emergency craniotomy with drainage of the brain abscess successfully.

Effect of Obesity and Underweight Status on Perioperative Outcomes of Congenital Heart Operations in Children, Adolescents, and Young Adults: An Analysis of Data From the Society of Thoracic Surgeons Database.

PubMed

O'Byrne, Michael L; Kim, Sunghee; Hornik, Christoph P; Yerokun, Babatunde A; Matsouaka, Roland A; Jacobs, Jeffrey P; Jacobs, Marshall L; Jonas, Richard A

2017-08-22

Extreme body mass index (BMI; either very high or very low) has been associated with increased risk of adverse perioperative outcome in adults undergoing cardiac surgery. The effect of BMI on perioperative outcomes in congenital heart disease patients has not been evaluated. A multicenter retrospective cohort study was performed studying patients 10 to 35 years of age undergoing a congenital heart disease operation in the Society of Thoracic Surgeons Congenital Heart Surgery Database between January 1, 2010, and December 31, 2015. The primary outcomes were operative mortality and a composite outcome (1 or more of operative mortality, major adverse event, prolonged hospital length of stay, and wound infection/dehiscence). The associations between age- and sex-adjusted BMI percentiles and these outcomes were assessed, with adjustment for patient-level risk factors, with multivariate logistic regression. Of 18 337 patients (118 centers), 16% were obese, 15% were overweight, 53% were normal weight, 7% were underweight, and 9% were severely underweight. Observed risks of operative mortality ( P =0.04) and composite outcome ( P <0.0001) were higher in severely underweight and obese subjects. Severely underweight BMI was associated with increased unplanned cardiac operation and reoperation for bleeding. Obesity was associated with increased risk of wound infection. In multivariable analysis, the association between BMI and operative mortality was no longer significant. Obese (odds ratio, 1.28; P =0.008), severely underweight (odds ratio, 1.29; P <0.0001), and underweight (odds ratio, 1.39; P =0.002) subjects were associated with increased risk of composite outcome. Obesity and underweight BMI were associated with increased risk of composite adverse outcome independently of other risk factors. Further research is necessary to determine whether BMI represents a modifiable risk factor for perioperative outcome. © 2017 American Heart Association, Inc.

Modeling Major Adverse Outcomes of Pediatric and Adult Patients With Congenital Heart Disease Undergoing Cardiac Catheterization: Observations From the NCDR IMPACT Registry (National Cardiovascular Data Registry Improving Pediatric and Adult Congenital Treatment).

PubMed

Jayaram, Natalie; Spertus, John A; Kennedy, Kevin F; Vincent, Robert; Martin, Gerard R; Curtis, Jeptha P; Nykanen, David; Moore, Phillip M; Bergersen, Lisa

2017-11-21

Risk standardization for adverse events after congenital cardiac catheterization is needed to equitably compare patient outcomes among different hospitals as a foundation for quality improvement. The goal of this project was to develop a risk-standardization methodology to adjust for patient characteristics when comparing major adverse outcomes in the NCDR's (National Cardiovascular Data Registry) IMPACT Registry (Improving Pediatric and Adult Congenital Treatment). Between January 2011 and March 2014, 39 725 consecutive patients within IMPACT undergoing cardiac catheterization were identified. Given the heterogeneity of interventional procedures for congenital heart disease, new procedure-type risk categories were derived with empirical data and expert opinion, as were markers of hemodynamic vulnerability. A multivariable hierarchical logistic regression model to identify patient and procedural characteristics predictive of a major adverse event or death after cardiac catheterization was derived in 70% of the cohort and validated in the remaining 30%. The rate of major adverse event or death was 7.1% and 7.2% in the derivation and validation cohorts, respectively. Six procedure-type risk categories and 6 independent indicators of hemodynamic vulnerability were identified. The final risk adjustment model included procedure-type risk category, number of hemodynamic vulnerability indicators, renal insufficiency, single-ventricle physiology, and coagulation disorder. The model had good discrimination, with a C-statistic of 0.76 and 0.75 in the derivation and validation cohorts, respectively. Model calibration in the validation cohort was excellent, with a slope of 0.97 (standard error, 0.04; P value [for difference from 1] =0.53) and an intercept of 0.007 (standard error, 0.12; P value [for difference from 0] =0.95). The creation of a validated risk-standardization model for adverse outcomes after congenital cardiac catheterization can support reporting of risk-adjusted outcomes in the IMPACT Registry as a foundation for quality improvement. © 2017 American Heart Association, Inc.

A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients.

PubMed

Costello, John P; Weiderhold, Allison; Louis, Clauden; Shaughnessy, Conner; Peer, Syed M; Zurakowski, David; Jonas, Richard A; Nath, Dilip S

2015-06-01

The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

Incidence and Risk Factors for Perioperative Cardiovascular and Respiratory Adverse Events in Pediatric Patients With Congenital Heart Disease Undergoing Noncardiac Procedures.

PubMed

Lee, Sandra; Reddington, Elise; Koutsogiannaki, Sophia; Hernandez, Michael R; Odegard, Kirsten C; DiNardo, James A; Yuki, Koichi

2018-04-27

While mortality and adverse perioperative events after noncardiac surgery in children with a broad range of congenital cardiac lesions have been investigated using large multiinstitutional databases, to date single-center studies addressing adverse outcomes in children with congenital heart disease (CHD) undergoing noncardiac surgery have only included small numbers of patients with significant heart disease. The primary objective of this study was to determine the incidences of perioperative cardiovascular and respiratory events in a large cohort of patients from a single institution with a broad range of congenital cardiac lesions undergoing noncardiac procedures and to determine risk factors for these events. We identified 3010 CHD patients presenting for noncardiac procedures in our institution over a 5-year period. We collected demographic information, including procedure performed, cardiac diagnosis, ventricular function as assessed by echocardiogram within 6 months of the procedure, and classification of CHD into 3 groups (minor, major, or severe CHD) based on residual lesion burden and cardiovascular functional status. Characteristics related to conduct of anesthesia care were also collected. The primary outcome variables for our analysis were the incidences of intraoperative cardiovascular and respiratory events. Univariable and multivariable logistic regressions were used to determine risk factors for these 2 outcomes. The incidence of cardiovascular events was 11.5% and of respiratory events was 4.7%. Univariate analysis and multivariable analysis demonstrated that American Society of Anesthesiologists (≥3), emergency cases, major and severe CHD, single-ventricle physiology, ventricular dysfunction, orthopedic surgery, general surgery, neurosurgery, and pulmonary procedures were associated with perioperative cardiovascular events. Respiratory events were associated with American Society of Anesthesiologists (≥4) and otolaryngology, gastrointestinal, general surgery, and maxillofacial procedures. Intraoperative cardiovascular events and respiratory events in patients with CHD were relatively common. While cardiovascular events were highly associated with cardiovascular status, respiratory events were not associated with cardiovascular status.

Increased Atrial β-Adrenergic Receptors and GRK-2 Gene Expression Can Play a Fundamental Role in Heart Failure After Repair of Congenital Heart Disease with Cardiopulmonary Bypass.

PubMed

Oliveira, Marcela Silva; Carmona, Fabio; Vicente, Walter V A; Manso, Paulo H; Mata, Karina M; Celes, Mara Rúbia; Campos, Erica C; Ramos, Simone G

2017-04-01

Surgeries to correct congenital heart diseases are increasing in Brazil and worldwide. However, even with the advances in surgical techniques and perfusion, some cases, especially the more complex ones, can develop heart failure and death. A retrospective study of patients who underwent surgery for correction of congenital heart diseases with cardiopulmonary bypass (CPB) in a university tertiary-care hospital that died, showed infarction in different stages of evolution and scattered microcalcifications in the myocardium, even without coronary obstruction. CPB is a process routinely used during cardiac surgery for congenital heart disease. However, CPB has been related to increased endogenous catecholamines that can lead to major injuries in cardiomyocytes. The mechanisms involved are not completely understood. The aim of this study was to evaluate the alterations induced in the β-adrenergic receptors and GRK-2 present in atrial cardiomyocytes of infants with congenital heart disease undergoing surgical repair with CPB and correlate the alterations with functional and biochemical markers of ischemia/myocardial injury. The study consisted of right atrial biopsies of infants undergoing surgical correction in HC-FMRPUSP. Thirty-three cases were selected. Atrial biopsies were obtained at the beginning of CPB (group G1) and at the end of CPB (group G2). Real-time PCR, Western blotting, and immunofluorescence analysis were conducted to evaluate the expression of β 1 , β 2 -adrenergic receptors, and GRK-2 in atrial myocardium. Cardiac function was evaluated by echocardiography and biochemical analysis (N-terminal pro-brain natriuretic peptide (NT-ProBNP), lactate, and cardiac troponin I). We observed an increase in serum lactate, NT-proBNP, and troponin I at the end of CPB indicating tissue hypoxia/ischemia. Even without major clinical consequences in cardiac function, these alterations were followed by a significant increase in gene expression of β 1 and β 2 receptors and GRK-2, suggesting that this is one of the mechanisms responsible for the exacerbated response of cardiomyocytes to circulating catecholamines. These alterations could explain the irreversible myocardial damage and lipid peroxidation of membranes classically attributed to catecholamine excess, observed in some infants who develop heart failure and postoperative death. Although other factors may be involved, this study confirms that CPB acts as a potent inducer of increased gene expression of β- adrenergic receptors and GRK-2, making the myocardium of these infants more susceptible to the effects of circulating endogenous catecholamines, which may contribute to the development of irreversible myocardial damage and death.

Socioeconomic status and impact of treatment on families of children with congenital heart disease.

PubMed

Mughal, Abdul Razzaq; Sadiq, Masood; Hyder, Syed Najam; Qureshi, Ahmad Usaid; A Shah, S Salman; Khan, Mohammad Asim; Nasir, Jamal Abdul

2011-07-01

To assess the socioeconomic status, treatment being offered and the impact of congenital heart disease treatment on families. Observational study. The Children's Hospital / Institute of Child Health, Lahore, from 1st March to 31st August 2010. All patients undergoing a cardiac surgical or angiographic intervention were enrolled. Socioeconomic status was assessed by Kuppuswamy socioeconomic status scale with income group modification. The impact was measured by the source of financing, effect on family financing source and schooling and health of siblings. Of 211 patients undergoing treatment in the study period, surgery was the definitive treatment in 164 (77.7%) and angiographic intervention in 47 (22.3%) patients. Male to female ratio was 1.5:1. The mean age of the patient was 39.1 + 3.2 months (range 01 day to 15 years). Majority of families belonged to middle (66.4%, n=140) and lower (27%, n=57) socioeconomic class. The mean cost of medicines and disposables was PKR 78378.2 ± 8845.9 (US$ 933.1 ± 105.3) in open heart surgery, PKR 12581 ± 7010.8 (US$ 149.8 ± 83.5) in closed heart surgery and PKR 69091 + 60906 in angiographic interventions. In 63.1% patients, families contributed towards these costs either completely (12.3%) or partly (50.8%) with significant contribution from the hospital. Adverse effect on families ranged from leave without pay to losing jobs or business (46%), and selling their assets (11.3%). It also affected schooling and health of siblings (22.7% and 26.1% respectively). Majority of children with congenital heart disease belonged to middle and lower socioeconomic status in this study. Main definitive treatment was surgery. The cost of health care facilities posed a marked socioeconomic burden on those families.

Accuracy of the all patient refined diagnosis related groups classification system in congenital heart surgery.

PubMed

Parnell, Aimee S; Shults, Justine; Gaynor, J William; Leonard, Mary B; Dai, Dingwei; Feudtner, Chris

2014-02-01

Administrative data are increasingly used to evaluate clinical outcomes and quality of care in pediatric congenital heart surgery (CHS) programs. Several published analyses of large pediatric administrative data sets have relied on the All Patient Refined Diagnosis Related Groups (APR-DRG, version 24) diagnostic classification system. The accuracy of this classification system for patients undergoing CHS is unclear. We performed a retrospective cohort study of all 14,098 patients 0 to 5 years of age undergoing any of six selected congenital heart operations, ranging in complexity from isolated closure of a ventricular septal defect to single-ventricle palliation, at 40 tertiary-care pediatric centers in the Pediatric Health Information Systems database between 2007 and 2010. Assigned APR-DRGs (cardiac versus noncardiac) were compared using χ2 or Fisher's exact tests between those patients admitted during the first day of life versus later and between those receiving extracorporeal membrane oxygenation support versus those not. Recursive partitioning was used to assess the greatest determinants of APR-DRG type in the model. Every patient admitted on day 1 of life was assigned to a noncardiac APR-DRG (p<0.001 for each procedure). Similarly, use of extracorporeal membrane oxygenation was highly associated with misclassification of CHS patients into a noncardiac APR-DRG (p<0.001 for each procedure). Cases misclassified into a noncardiac APR-DRG experienced a significantly increased mortality (p<0.001). In classifying patients undergoing CHS, APR-DRG coding has systematic misclassifications, which may result in inaccurate reporting of CHS case volumes and mortality. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Trends in resource utilization associated with the inpatient treatment of neonatal congenital heart disease.

PubMed

Smith, Andrew H; Gay, James C; Patel, Neal R

2014-01-01

While neonates account for a significant proportion of health care expenditures related to inpatient care for congenital heart disease, key drivers of resource utilization among this population are poorly defined. Data from 2005 through 2011 were extracted from the Pediatric Health Information System for patients assigned a discharge All Patient Refined Diagnosis Related Group of 630 (neonates with birthweight >2499 g undergoing a major cardiovascular procedure). Mortality risk adjustment for patients undergoing operative interventions was performed with the Risk Adjusment in Congenital Heart Surgery (RACHS-1) score. A total of 13 156 cases were included in the analysis. Despite only a 3% increase in case mix index and no significant change in operative acuity over the study period (RACHS classifications of 3 or greater 67% in 2005 vs. 66% in 2011, P = .64), there were inflation-adjusted increases in both total estimated cost per case of (50% to $151 760 in 2011, P < .001), and mean charge per case (33% to $433 875 in 2011, P < .001). Pharmacy charges increased by 16% (P < .001), with agents including chlorothiazide and albumin accounting for the highest patient charges over the study period. Imaging charges increased by 42% (P < .001), with an average of 5.7 echocardiograms and $6517 in associated charges per case by 2011. While the proportion of patients receiving nitric oxide remained consistent, mean duration of administration increased by 25% to 6.6 days by 2011, accounting for average charges of $52 141 per patient exposed. Among neonates with serious congenital heart disease, increases in both institutional costs and charges to the patient are associated with relatively consistent utilization practices in recent years. Multiinstitutional collaboration may prove useful in aligning evidence-based reductions in practice variation with limitations in resource utilization without compromising the quality of care. © 2013 Wiley Periodicals, Inc.

Contact force monitoring during catheter ablation of intraatrial reentrant tachycardia in patients with congenital heart disease.

PubMed

Krause, Ulrich; Backhoff, David; Klehs, Sophia; Schneider, Heike E; Paul, Thomas

2016-08-01

Monitoring of catheter contact force during catheter ablation of atrial fibrillation has been shown to increase efficacy and safety. However, almost no data exists on the use of this technology in catheter ablation of intraatrial reentrant tachycardia in patients with congenital heart disease. The aim of the present study was to evaluate the impact of contact force monitoring during catheter ablation of intraatrial reentrant tachycardia in those patients. Catheter ablation of intraatrial reentrant tachycardia using monitoring of catheter contact force was performed in 28 patients with congenital heart disease (CHD). Thirty-two patients matched according to gender, age, and body weight with congenital heart disease undergoing catheter ablation without contact force monitoring served as control group. Parameters reflecting acute procedural success, long-term efficacy, and safety were compared. Acute procedural success was statistically not different in both groups (contact force 93 % vs. control 84 %, p = 0.3). Likewise the recurrence rate 1 year after ablation as shown by Kaplan-Meier analysis did not differ (contact force 28 % vs. control 37 %, p = 0.63). Major complications were restricted to groin vessel injuries and occurred in 3 out of 60 patients (contact force n = 1; control n = 2). Complications related to excessive catheter contact force were not observed. The present study did not show superiority of catheter contact force monitoring during ablation of intraatrial reentrant tachycardia in patients with CHD in terms of efficacy and safety. Higher contact force compared to pulmonary vein isolation might therefore be required to increase the efficacy of catheter ablation of intraatrial reentrant tachycardia in patients with congenital heart disease.

Prevalence of non-cardiovascular findings on CT angiography in children with congenital heart disease.

PubMed

Malik, Archana; Hellinger, Jeffrey C; Servaes, Sabah; Schwartz, Mathew C; Keller, Marc S; Epelman, Monica

2017-03-01

CT angiography is gaining broader acceptance in the evaluation of children with known or suspected congenital heart disease. These studies include non-cardiovascular structures such as the mediastinum, lung parenchyma and upper abdominal organs. It is important to inspect all these structures for potential abnormalities that might be clinically important and, in some cases, may impact care plans. To determine the prevalence of non-cardiovascular findings in CT angiography of children with congenital heart disease. During 28 months, 300 consecutive children (170 males; mean age: 7.1 years, age range: 6 h-26 years), referred from a tertiary pediatric cardiology center, underwent clinically indicated CT angiography to evaluate known or suspected congenital heart disease. Slightly more than half (n = 169) of the patients were postoperative or post-intervention. Examinations were retrospectively reviewed, and non-cardiovascular findings were recorded and tabulated by organ system, congenital heart disease and operative procedure in conjunction with outcomes from medical charts. Non-cardiovascular findings were identified in 83% (n = 250 / 300) of the studies for a total of 857 findings. In 221 patients (n = 73.7% of 300) a total of 813 non-cardiovascular findings were clinically significant, while in 9.7% (n = 29 / 300) of patients, 5.1% (n = 44 / 857) of the findings were nonsignificant. In 38.3% (n = 115 / 300) of patients with significant non-cardiovascular pathology, the findings were unexpected and directly impacted patient care plans. Commonly involved organs with non-cardiovascular findings were the lungs with 280 non-cardiovascular findings in 176 / 300 (58.7%) of patients, the airway with 139 non-cardiovascular findings in 103 / 300 (34.3%) of patients and the liver with 108 non-cardiovascular findings in 72 / 300 (24.0%) of patients. Syndromic associations were noted in 22% (n = 66 / 300) of the patients. Non-cardiovascular findings are common in children with congenital heart disease who undergo CT angiography. Based upon our study population, if a child with congenital heart disease has a CT angiography, five out of six will have non-cardiovascular findings, while nearly three out of four (73.7%; 221 / 300) will have significant non-cardiovascular findings. Close attention to the non-cardiovascular structures in children with congenital heart disease presenting for a CT angiography is recommended as in nearly 40% of these children, findings were unexpected and directly altered patient care.

Congenital heart diseases in a reference service: clinical evolution and associated illnesses.

PubMed

Huber, Janaína; Peres, Vivian Catarino; Santos, Tiago Jeronimo dos; Beltrão, Lauro da Fontoura; Baumont, Angélica Cerveira de; Cañedo, Andrés Delgado; Schaan, Beatriz D'Agord; Pellanda, Lucia Campos

2010-03-01

Several factors, which include prenatal diagnosis and availability of new therapeutic procedures, have contributed to change the profile of patients with congenital heart disease (CHD). Knowing these changes is important to a better health care. Description of profile of patients with CHD in a reference service in the State of Rio Grande do Sul, Brazil. It is a cross-sectional study including 684 patients with CHD in a service of pediatric cardiology from January 2007 to May 2008. We interviewed the patients (and/or their parents) and examined these patients (congenital malformations, anthropometric measures). Moreover, their charts were reviewed in order to detail heart diseases, procedures and echocardiography. Patients were from 16 days to 66 years old, 51.8% were female, and 93.7% were Caucasian. The mean age at diagnosis was 15.8 +/- 46.8 months. Ventricular septal defect, patent ductus arteriosus and Tetralogy of Fallot were the most prevalent CHD. 59.1% of examined patients, whose average age was 44.3 +/- 71.2 months, have been undergoing therapeutic procedures; 30.4% had congenital extracardiac malformations; and 12 patients had genetic syndrome. Regarding development, 46.6% had low weight and height gain, and 13.7% had neuropsychomotor delay. Furthermore, 18.4% had family history of congenital heart disease. Neuropsychomotor delay and low weight and height gain may be related to CHD. Establishing a profile of patients with CHD, who were treated at an institution of reference, may function as a basis in which health care of this population can be planed appropriately.

Functionally univentricular heart and the fontan operation: lessons learned about patterns of practice and outcomes from the congenital heart surgery databases of the European association for cardio-thoracic surgery and the society of thoracic surgeons.

PubMed

Jacobs, Jeffrey Phillip; Maruszewski, Bohdan

2013-10-01

"The term "functionally univentricular heart" describes a spectrum of congenital cardiovascular malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation and another to the pulmonary circulation." The purpose of this article is to review patterns of practice and outcomes in the Congenital Heart Surgery Databases (CHSDBs) of the European Association for Cardio-Thoracic Surgery (EACTS) and the Society of Thoracic Surgeons (STS) in patients with functionally univentricular hearts undergoing the Fontan operation. We examined all index operations performed on patients with functionally univentricular hearts in the EACTS and STS-CHSDBs over 4 years from 2007 to 2010, inclusive. The most common diagnostic categories are hypoplastic left heart syndrome, tricuspid atresia, and double inlet left ventricle. The Fontan operation makes up 3.2% of all cardiac operations in the EACTS and STS-CHSDBs over 4 years from 2007 to 2010, inclusive. Of all the patients undergoing a Fontan procedure, 65.1% had an extracardiac Fontan, 21.5% had a lateral tunnel, and 5.8% had a Fontan revision or conversion (Re-do Fontan). In operations where fenestration status is known, 68.5% of the Fontan operations were fenestrated. During the four years of this analysis, only 5 patients had ventricular septation. Exclusive of Fontan revision or conversion (Re-do Fontan), all remaining Fontan operations had a discharge mortality of 2.3%. Fontan revision or conversion (Re-do Fontan) had a discharge mortality of 12.8%. The STS database is largest CHSDB in North America. The EACTS database is largest CHSDB in Europe. This review of data from EACTS and STS allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that patients with functionally univentricular hearts present a challenging problem; however, exclusive of Fontan revision or conversion (Re-do Fontan), the Fontan operation has a discharge mortality of 2.3%.

Measuring hospital performance in congenital heart surgery: Administrative vs. clinical registry data

PubMed Central

Pasquali, Sara K.; He, Xia; Jacobs, Jeffrey P.; Jacobs, Marshall L.; Gaies, Michael G.; Shah, Samir S.; Hall, Matthew; Gaynor, J. William; Peterson, Eric D.; Mayer, John E.; Hirsch-Romano, Jennifer C.

2015-01-01

Background In congenital heart surgery, hospital performance has historically been assessed using widely available administrative datasets. Recent studies have demonstrated inaccuracies in case ascertainment (coding and inclusion of eligible cases) in administrative vs. clinical registry data, however it is unclear whether this impacts assessment of performance on a hospital-level. Methods Merged data from the Society of Thoracic Surgeons (STS) Database (clinical registry), and Pediatric Health Information Systems Database (administrative dataset) on 46,056 children undergoing heart surgery (2006–2010) were utilized to evaluate in-hospital mortality for 33 hospitals based on their administrative vs. registry data. Standard methods to identify/classify cases were used: Risk Adjustment in Congenital Heart Surgery (RACHS-1) in the administrative data, and STS–European Association for Cardiothoracic Surgery (STAT) methodology in the registry. Results Median hospital surgical volume based on the registry data was 269 cases/yr; mortality was 2.9%. Hospital volumes and mortality rates based on the administrative data were on average 10.7% and 4.7% lower, respectively, although this varied widely across hospitals. Hospital rankings for mortality based on the administrative vs. registry data differed by ≥ 5 rank-positions for 24% of hospitals, with a change in mortality tertile classification (high, middle, or low mortality) for 18%, and change in statistical outlier classification for 12%. Higher volume/complexity hospitals were most impacted. Agency for Healthcare Quality and Research methods in the administrative data yielded similar results. Conclusions Inaccuracies in case ascertainment in administrative vs. clinical registry data can lead to important differences in assessment of hospital mortality rates for congenital heart surgery. PMID:25624057

The impact of obesity on early postoperative outcomes in adults with congenital heart disease.

PubMed

Zaidi, Ali N; Bauer, John A; Michalsky, Marc P; Olshove, Vincent; Boettner, Bethany; Phillips, Alistair; Cook, Stephen C

2011-01-01

As the prevalence of obesity continues to increase, it now includes the growing number of patients with congenital heart disease (CHD). This particular obese patient population may pose additional intraoperative as well as postoperative challenges that may contribute to poor outcomes. Our aims were to determine the influence of obesity on morbidity and mortality in adults with CHD undergoing surgical repair at a free standing children's hospital. A retrospective analysis of adult (≥18 years) CHD surgery cases from 2002 to 2008 was performed. Congenital heart lesions were defined as mild, moderate, or complex. Patients were categorized by body mass index (BMI): underweight (BMI < 20 kg/m(2)), normal (BMI 20-24.9 kg/m(2)), overweight (BMI 25-29.9 kg/m(2)), and obese (BMI ≥ 30 kg/m(2)). Demographics, incidence of mortality, or specific morbidities were statistically compared using Fisher's exact test and analyses of variance (anovas). In this population (n = 165), overweight (29%) and obese (22%) patients were prevalent. Hypertension (HTN) and pre-HTN were more prevalent in obese and overweight patients. Postoperative renal dysfunction was observed in obese patients with complex CHD (P = .04). Mortality was not different among groups. Obesity is becoming increasingly common among adults with CHD. Despite marginal evidence of postoperative renal complications in obese patients with CHD of severe complexity, the overall presence of obesity did not influence mortality or short term postoperative morbidities. © 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.

Subtle hemorrhagic brain injury is associated with neurodevelopmental impairment in infants with repaired congenital heart disease.

PubMed

Soul, Janet S; Robertson, Richard L; Wypij, David; Bellinger, David C; Visconti, Karen J; du Plessis, Adré J; Kussman, Barry D; Scoppettuolo, Lisa A; Pigula, Frank; Jonas, Richard A; Newburger, Jane W

2009-08-01

Perioperative stroke and periventricular leukomalacia have been reported to occur commonly in infants with congenital heart disease. We aimed to determine the incidence and type of brain injury in infants undergoing 2-ventricle repair in infancy and to determine risk factors associated with such injury. Forty-eight infants enrolled in a trial comparing 2 different hematocrits during surgical repair of congenital heart disease underwent brain magnetic resonance imaging scans and neurodevelopmental testing at 1 year of age. Eighteen (38%) of our subjects had tiny foci of hemosiderin by susceptibility imaging, without evidence of abnormalities in corresponding regions on conventional magnetic resonance imaging sequences. Subjects with foci of hemosiderin had a significantly lower Psychomotor Developmental Index at 1 year of age (79.6 +/- 16.5, mean +/- standard deviation) compared with subjects without these foci (89.5 +/- 15.3; P = .04). Older age at surgery and diagnostic group were significantly associated with the presence of hemosiderin foci. Only 1 subject had a small stroke (2%), and 2 subjects had periventricular leukomalacia (4%). Foci of hemosiderin without radiologic evidence of ischemic brain injury are an abnormality associated with adverse neurodevelopmental outcome not previously described in magnetic resonance imaging studies of children with surgically repaired congenital heart disease. The association of hemosiderin foci with older age at surgery and cardiac diagnosis, and not with risk factors associated with brain injury, in previous studies suggests that the cause and pathogenesis of this abnormality are different from ischemic brain lesions reported previously.

General Concepts in Adult Congenital Heart Disease.

PubMed

Mutluer, Ferit Onur; Çeliker, Alpay

2018-01-20

Congenital heart disease in adults (adult congenital heart disease) is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face.

Regional Microstructural and Volumetric Magnetic Resonance Imaging (MRI) Abnormalities in the Corpus Callosum of Neonates With Congenital Heart Defect Undergoing Cardiac Surgery.

PubMed

Hagmann, Cornelia; Singer, Jitka; Latal, Beatrice; Knirsch, Walter; Makki, Malek

2016-03-01

The purpose of the study is to investigate the structural development of the corpus callosum in term neonates with congenital heart defect before and after surgery using diffusion tensor imaging and 3-dimensional T1-weighted magnetic resonance imaging (MRI). We compared parallel and radial diffusions, apparent diffusion coefficient (ADC), fractional anisotropy, and volume of 5 substructures of the corpus callosum: genu, rostral body, body, isthmus, and splenium. Compared to healthy controls, we found a significantly lower volume of the splenium and total corpus callosum and a higher radial diffusion and lower fractional anisotropy in the splenium of patients presurgery; a lower volume in all substructures in the postsurgery group; higher radial diffusion in the rostral body, body, and splenium; and a higher apparent diffusion coefficient in the splenium of postsurgery patients. Similar fractional anisotropy changes in congenital heart defect patients were reported in preterm infants. Our findings in apparent diffusion coefficient in the splenium of these patients (pre and postsurgery) are comparable to findings in preterm neonates with psychomotor delay. Delayed maturation of the isthmus was also reported in preterm infants. © The Author(s) 2015.

Heart morphogenesis gene regulatory networks revealed by temporal expression analysis.

PubMed

Hill, Jonathon T; Demarest, Bradley; Gorsi, Bushra; Smith, Megan; Yost, H Joseph

2017-10-01

During embryogenesis the heart forms as a linear tube that then undergoes multiple simultaneous morphogenetic events to obtain its mature shape. To understand the gene regulatory networks (GRNs) driving this phase of heart development, during which many congenital heart disease malformations likely arise, we conducted an RNA-seq timecourse in zebrafish from 30 hpf to 72 hpf and identified 5861 genes with altered expression. We clustered the genes by temporal expression pattern, identified transcription factor binding motifs enriched in each cluster, and generated a model GRN for the major gene batteries in heart morphogenesis. This approach predicted hundreds of regulatory interactions and found batteries enriched in specific cell and tissue types, indicating that the approach can be used to narrow the search for novel genetic markers and regulatory interactions. Subsequent analyses confirmed the GRN using two mutants, Tbx5 and nkx2-5 , and identified sets of duplicated zebrafish genes that do not show temporal subfunctionalization. This dataset provides an essential resource for future studies on the genetic/epigenetic pathways implicated in congenital heart defects and the mechanisms of cardiac transcriptional regulation. © 2017. Published by The Company of Biologists Ltd.

Prenatal chromosomal microarray analysis in fetuses with congenital heart disease: a prospective cohort study.

PubMed

Wang, Yan; Cao, Li; Liang, Dong; Meng, Lulu; Wu, Yun; Qiao, Fengchang; Ji, Xiuqing; Luo, Chunyu; Zhang, Jingjing; Xu, Tianhui; Yu, Bin; Wang, Leilei; Wang, Ting; Pan, Qiong; Ma, Dingyuan; Hu, Ping; Xu, Zhengfeng

2018-02-01

Currently, chromosomal microarray analysis is considered the first-tier test in pediatric care and prenatal diagnosis. However, the diagnostic yield of chromosomal microarray analysis for prenatal diagnosis of congenital heart disease has not been evaluated based on a large cohort. Our aim was to evaluate the clinical utility of chromosomal microarray as the first-tier test for chromosomal abnormalities in fetuses with congenital heart disease. In this prospective study, 602 prenatal cases of congenital heart disease were investigated using single nucleotide polymorphism array over a 5-year period. Overall, pathogenic chromosomal abnormalities were identified in 125 (20.8%) of 602 prenatal cases of congenital heart disease, with 52.0% of them being numerical chromosomal abnormalities. The detection rates of likely pathogenic copy number variations and variants of uncertain significance were 1.3% and 6.0%, respectively. The detection rate of pathogenic chromosomal abnormalities in congenital heart disease plus additional structural anomalies (48.9% vs 14.3%, P < .0001) or intrauterine growth retardation group (50.0% vs 14.3%, P = .044) was significantly higher than that in isolated congenital heart disease group. Additionally, the detection rate in congenital heart disease with additional structural anomalies group was significantly higher than that in congenital heart disease with soft markers group (48.9% vs 19.8%, P < .0001). No significant difference was observed in the detection rates between congenital heart disease with additional structural anomalies and congenital heart disease with intrauterine growth retardation groups (48.9% vs 50.0%), congenital heart disease with soft markers and congenital heart disease with intrauterine growth retardation groups (19.8% vs 50.0%), or congenital heart disease with soft markers and isolated congenital heart disease groups (19.8% vs 14.3%). The detection rate in fetuses with congenital heart disease plus mild ventriculomegaly was significantly higher than in those with other types of soft markers (50.0% vs 15.6%, P < .05). Our study suggests chromosomal microarray analysis is a reliable and high-resolution technology and should be used as the first-tier test for prenatal diagnosis of congenital heart disease in clinical practice. Copyright © 2017 Elsevier Inc. All rights reserved.

Congenital Heart Disease: Causes, Diagnosis, Symptoms, and Treatments.

PubMed

Sun, RongRong; Liu, Min; Lu, Lei; Zheng, Yi; Zhang, Peiying

2015-07-01

The congenital heart disease includes abnormalities in heart structure that occur before birth. Such defects occur in the fetus while it is developing in the uterus during pregnancy. About 500,000 adults have congenital heart disease in USA (WebMD, Congenital heart defects medications, www.WebMD.com/heart-disease/tc/congenital-heart-defects-medications , 2014). 1 in every 100 children has defects in their heart due to genetic or chromosomal abnormalities, such as Down syndrome. The excessive alcohol consumption during pregnancy and use of medications, maternal viral infection, such as Rubella virus, measles (German), in the first trimester of pregnancy, all these are risk factors for congenital heart disease in children, and the risk increases if parent or sibling has a congenital heart defect. These are heart valves defects, atrial and ventricular septa defects, stenosis, the heart muscle abnormalities, and a hole inside wall of the heart which causes defect in blood circulation, heart failure, and eventual death. There are no particular symptoms of congenital heart disease, but shortness of breath and limited ability to do exercise, fatigue, abnormal sound of heart as heart murmur, which is diagnosed by a physician while listening to the heart beats. The echocardiogram or transesophageal echocardiogram, electrocardiogram, chest X-ray, cardiac catheterization, and MRI methods are used to detect congenital heart disease. Several medications are given depending on the severity of this disease, and catheter method and surgery are required for serious cases to repair heart valves or heart transplantation as in endocarditis. For genetic study, first DNA is extracted from blood followed by DNA sequence analysis and any defect in nucleotide sequence of DNA is determined. For congenital heart disease, genes in chromosome 1 show some defects in nucleotide sequence. In this review the causes, diagnosis, symptoms, and treatments of congenital heart disease are described.

Prevalence of Non-cardiac and Genetic abnormalities in Neonates Undergoing Cardiac Surgery: Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database

PubMed Central

Patel, Angira; Costello, John M.; Backer, Carl L.; Pasquali, Sara K.; Hill, Kevin D.; Wallace, Amelia S.; Jacobs, Jeffrey P.; Jacobs, Marshall L.

2016-01-01

Background Among congenital heart disease (CHD) patients, the coexistence of non-cardiac congenital anatomic abnormalities (NC), genetic abnormalities (GA), and syndromes (S) may influence therapeutic strategies and outcomes. The appreciated prevalence of these abnormalities has risen, as increased screening and improved diagnostic precision enable identification of these comorbidities in a larger fraction of neonates with CHD. We examined the contemporary prevalence and distribution of NC/GA/S across diagnostic groups among neonates undergoing cardiac surgery using a large, nationally representative clinical registry. Methods The Society of Thoracic Surgeons-Congenital Heart Surgery Database (STS-CHSD) was queried to identify neonates (≤ 30 days) who underwent index cardiac operations from 2010–2013. The fundamental cardiac diagnosis was used to identify 10 diagnostic groups. The prevalence of NC/GA/S was reported across each group. Results The cohort included 15,376 index neonatal operations from 112 centers. Overall 18.8% (2,894/15,376) of operations were performed on neonates with NC/GA/S. Patients with atrioventricular septal defect (212/357, 59.4%), interrupted aortic arch (248/567, 43.7%), truncus arteriosus (204/554, 36.8%), tetralogy of Fallot (417/1383, 30.2%) had the highest prevalence of NC/GA/S abnormalities, whereas those with transposition (111/2778, 4.0%) had the lowest prevalence. The most commonly identified NC/GA/S included: heterotaxy (597/15,376, 3.9%), DiGeorge/22q11 deletion (550/15,376, 3.6%), Down syndrome/trisomy 21 (318/15, 376, 2.1%), intestinal malrotation (220/15,376, 1.4%), and Turner syndrome/45XO (189/15,376, 1.2%). Conclusions The prevalence of NC/GA/S varies widely across CHD diagnostic groups. This information may be useful for patient counseling, recommendations for screening for anomalies and genetic disorders, and perioperative management. PMID:27319986

Risk Factors for Mortality in Reoperations for Pediatric and Congenital Heart Surgery in a Developing Country.

PubMed

Villa-Hincapie, Carlos A; Carreno-Jaimes, Marisol; Obando-Lopez, Carlos E; Camacho-Mackenzie, Jaime; Umaña-Mallarino, Juan P; Sandoval-Reyes, Nestor F

2017-07-01

The survival of patients with congenital heart disease has increased in the recent years, because of enhanced diagnostic capabilities, better surgical techniques, and improved perioperative care. Many patients will require reoperation as part of staged procedures or to treat grafts deterioration and residual or recurrent lesions. Reoperations favor the formation of cardiac adhesions and consequently increase surgery time; however, the impact on morbidity and operative mortality is certain. The objective of the study was to describe the risk factors for mortality in pediatric patients undergoing a reoperation for congenital heart disease. Historic cohort of patients who underwent reoperation after pediatric cardiac surgery from January 2009 to December 2015. Operations with previous surgical approach different to sternotomy were excluded from the analysis. In seven years, 3,086 surgeries were performed, 481 were reoperations, and 238 patients fulfilled the inclusion criteria. Mean number of prior surgeries was 1.4 ± 0.6. Median age at the time of reoperation was 6.4 years. The most common surgical procedures were staged palliation for functionally univentricular heart (17.6%). Median cross-clamp time was 66 minutes. Younger age at the moment of resternotomy, longer cross-clamp time, and Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) Mortality Categories risk category greater than three were risk factors for mortality. The number of resternotomies was not associated with mortality. Mortality prior to hospital discharge was 4.6%, and mortality after discharge but prior to 30 days after surgery was 0.54%. Operative mortality was 5.1%. Resternotomy in pediatric cardiac surgery is a safe procedure in our center.

Congenital heart defects and extracardiac malformations.

PubMed

Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

2013-06-01

To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

History of the Congenital Heart Surgeons' Society.

PubMed

Mavroudis, Constantine; Williams, William G

2015-10-01

The Congenital Heart Surgeons' Society is a group of over 100 pediatric heart surgeons representing 72 institutions that specialize in the treatment of patients with congenital heart defects. The Society began in 1972 and incorporated as a not-for-profit charitable organization in 2004. It has become the face and voice of congenital heart surgery in North America. In 1985, the Society established a data center for multicenter clinical research studies to encourage congenital heart professionals to participate in improving outcomes for our patients. The goals of the Congenital Heart Surgeons' Society are to stimulate the study of congenital cardiac physiology, pathology, and management options which are instantiated in data collection, multi-institutional studies, and scientific meetings. Honest and open discussion of problems with possible solutions to the challenges facing congenital heart professionals have been the strength of the Congenital Heart Surgeons' Society. It is imperative for the growth of an organization to know from where it came in order to know to where it is going. The purpose of this article is to review the history of the Congenital Heart Surgeons' Society. © The Author(s) 2015.

In-Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium: Completeness of Ascertainment Using the National Death Index and United Network for Organ Sharing Datasets.

PubMed

Spector, Logan G; Menk, Jeremiah S; Vinocur, Jeffrey M; Oster, Matthew E; Harvey, Brian A; St Louis, James D; Moller, James; Kochilas, Lazaros K

2016-08-09

The long-term outcomes of patients undergoing interventions for congenital heart disease (CHD) remain largely unknown. We linked the Pediatric Cardiac Care Consortium (PCCC) with the National Death Index (NDI) and the United Network for Organ Sharing Dataset (UNOS) registries to study mortality and transplant occurring up to 32 years postintervention. The objective of the current analysis was to determine the sensitivity of this linkage in identifying patients who are known to have died or undergone heart transplant. We used direct identifiers from 59 324 subjects registered in the PCCC between 1982 and 2003 to test for completeness of case ascertainment of subjects with known vital and heart transplant status by linkage with the NDI and UNOS registries. Of the 4612 in-hospital deaths, 3873 were identified by the NDI as "true" matches for a sensitivity of 84.0% (95% CI, 82.9-85.0). There was no difference in sensitivity across 25 congenital cardiovascular conditions after adjustment for age, sex, race, presence of first name, death year, and residence at death. Of 455 known heart transplants in the PCCC, there were 408 matches in the UNOS registry, for a sensitivity of 89.7% (95% CI, 86.9-92.3). An additional 4851 deaths and 363 transplants that occurred outside the PCCC were identified through 2014. The linkage of the PCCC with the NDI and UNOS national registries is feasible with a satisfactory sensitivity. This linkage provides a conservative estimate of the long-term death and heart transplant events in this cohort. © 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

Subtle Hemorrhagic Brain Injury is Associated with Neurodevelopmental Impairment in Infants with Repaired Congenital Heart Disease

PubMed Central

Soul, Janet S.; Robertson, Richard L.; Wypij, David; Bellinger, David C.; Visconti, Karen J.; du Plessis, Adré J.; Kussman, Barry D.; Scoppettuolo, Lisa A.; Pigula, Frank; Jonas, Richard A.; Newburger, Jane W.

2009-01-01

Objective Perioperative stroke and periventricular leukomalacia have been reported to occur commonly in infants with congenital heart disease. We aimed to determine the incidence and type of brain injury in infants undergoing two-ventricle repair in infancy and to determine risk factors associated with such injury. Methods Forty-eight infants enrolled in a trial comparing two different hematocrits during surgical repair of congenital heart disease underwent brain MRI scans and neurodevelopmental testing at one year of age. Results Eighteen (38%) of our subjects had tiny foci of hemosiderin by susceptibility imaging, without evidence of abnormalities in corresponding regions on conventional MRI sequences. Subjects who had foci of hemosiderin had a significantly lower Psychomotor Developmental Index at one year of age (79.6 ± 16.5, mean ± SD) compared with subjects who did not have these foci (89.5 ± 15.3; p=0.04). Older age at surgery and diagnostic group were significantly associated with presence of hemosiderin foci. Only one subject had a small stroke (2%) and two had periventricular leukomalacia (4%). Conclusions Foci of hemosiderin without radiologic evidence of ischemic brain injury are an abnormality associated with adverse neurodevelopmental outcome not previously described in MRI studies of children with surgically repaired congenital heart disease. The association of hemosiderin foci with older age at surgery and cardiac diagnosis and not risk factors associated with brain injury in previous studies suggests that the etiology and pathogenesis of this abnormality is different from ischemic brain lesions reported previously. PMID:19619781

Pre-operative renal volume predicts peak creatinine after congenital heart surgery in neonates.

PubMed

Carmody, J Bryan; Seckeler, Michael D; Ballengee, Cortney R; Conaway, Mark; Jayakumar, K Anitha; Charlton, Jennifer R

2014-10-01

Acute kidney injury is common in neonates following surgery for congenital heart disease. We conducted a retrospective analysis to determine whether neonates with smaller pre-operative renal volume were more likely to develop post-operative acute kidney injury. We conducted a retrospective review of 72 neonates who underwent congenital heart surgery for any lesion other than patent ductus arteriosus at our institution from January 2007 to December 2011. Renal volume was calculated by ultrasound using the prolate ellipsoid formula. The presence and severity of post-operative acute kidney injury was determined both by measuring the peak serum creatinine in the first 7 days post-operatively and by using the Acute Kidney Injury Network scoring system. Using a linear change point model, a threshold renal volume of 17 cm³ was identified. Below this threshold, there was an inverse linear relationship between renal volume and peak post-operative creatinine for all patients (p = 0.036) and the subgroup with a single morphologic right ventricle (p = 0.046). There was a non-significant trend towards more acute kidney injury using Acute Kidney Injury Network criteria in all neonates with renal volume ≤17 cm³ (p = 0.11) and in the subgroup with a single morphologic right ventricle (p = 0.17). Pre-operative renal volume ≤17 cm³ is associated with a higher peak post-operative creatinine and potentially greater risk for post-operative acute kidney injury for neonates undergoing congenital heart surgery. Neonates with a single right ventricle may be at higher risk.

Congenital heart disease infant death rates decrease as gestational age advances from 34 to 40 weeks.

PubMed

Cnota, James F; Gupta, Resmi; Michelfelder, Erik C; Ittenbach, Richard F

2011-11-01

To describe congenital heart disease death rates in infants born between 34 and 40 weeks, estimate the relationship between gestational age and congenital heart disease infant death rates, and compare congenital heart disease death rates across 1- and 2-week intervals in gestational age. The 2000 to 2003 national linked birth/infant death cohort datasets were obtained. Congenital heart disease deaths were identified by using International Statistical Classification of Diseases, 10th Revision codes. Proportional death rates were calculated by using congenital heart disease deaths and all live births. The relationship between congenital heart disease death rates and gestational age was determined. Death rates were compared across intervals. A total of 14.9 million records were analyzed. Congenital heart disease deaths occurred in 4736 infants (0.04%) born between 34 and 40 weeks. There was a significant, negative linear relationship between congenital heart disease death rate and gestational age (R(2) = 0.97). Comparisons across 1-week intervals varied (P = .02-.23). All 2-week intervals were statistically significant (P < .01). Congenital heart disease death rates decrease as gestational age approaches 40 weeks. These results should be considered before elective delivery for the sole indication of prenatally diagnosed congenital heart disease. Copyright © 2011 Mosby, Inc. All rights reserved.

The Perioperative Use of Dexmedetomidine in Pediatric Patients with Congenital Heart Disease: An Analysis from the Congenital Cardiac Anesthesia Society-Society of Thoracic Surgeons Congenital Heart Disease Database.

PubMed

Schwartz, Lawrence I; Twite, Mark; Gulack, Brian; Hill, Kevin; Kim, Sunghee; Vener, David F

2016-09-01

Dexmedetomidine is a selective α-2 receptor agonist with a sedative and cardiopulmonary profile that makes it an attractive anesthetic for pediatric patients with congenital heart disease (CHD). Although several smaller, single-center studies suggest that dexmedetomidine use is gaining traction in the perioperative setting in children with CHD, there are limited multicenter data, with little understanding of the variation in use across age ranges, procedural complexity, and centers. The aim of this study was to use the Congenital Cardiac Anesthesia Society-Society of Thoracic Surgeons (CCAS-STS) registry to describe patient- and center-level variability in the use of dexmedetomidine in the perioperative setting in children with heart disease. To describe the use of dexmedetomidine in patients for CHD surgery, we analyzed all index cardiopulmonary bypass operations entered in the CCAS-STS database from 2010 to 2013. Patient and operative characteristics were compared between those who received intraoperative dexmedetomidine and those who did not. Selective outcomes associated with dexmedetomidine use were also described. Of the 12,142 operations studied, 3600 (29.6%) received perioperative dexmedetomidine (DEX) and 8542 did not receive the drug (NoDEX). Patient characteristics were different between the 2 groups with the DEX group generally exhibiting both lower patient and procedural risk factors. Patients who received dexmedetomidine were more likely to have a lower level of Society of Thoracic Surgeons mortality complexity than patient who did not receive it. Consistent with their overall lower risk profile, children in the DEX group also demonstrated improved outcomes compared with patients who did not receive dexmedetomidine. We described the growing use of dexmedetomidine in children anesthetized for surgical repair of CHD. Dexmedetomidine appears to be preferentially given to older and larger children who are undergoing less complex CHD surgery. We believe that the data provided in this study are the largest investigating the use of an anesthetic drug in CHD patients. It is also the first analysis of the anesthesia data in the CCAS-STS Congenital Heart Disease database.

Risk factors for systemic inflammatory response after congenital cardiac surgery.

PubMed

Güvener, Murat; Korun, Oktay; Demirtürk, Orhan Saim

2015-01-01

This study aims to assess the frequency of systemic inflammatory response syndrome (SIRS) following congenital heart surgery and risk factors associated with this clinical syndrome. Charts of all patients undergoing surgery for congenital heart disease in a single institution over a five-year period were analyzed retrospectively. The presence of SIRS was evaluated based on the criteria of the International Pediatric Sepsis Consensus Conference. Of the 246 patients included in the study 22 (8.9%) had clinical parameters indicating SIRS. The patients in the SIRS group had significantly longer cardiopulmonary bypass time (105.14 ± 27.27 vs. 66.86 ± 26.64 min; p < 0.01), aortic cross clamp time (69.36 ± 21.52 vs. 44.30 ± 24.27 min; p < 0.01), higher postoperative alanine aminotransferase (1419.00 ± 3260.99 vs. 81.95 ± 808.61 U/L; p < 0.01) and aspartate aminotransferase (2137.14 ± 4905.40 vs. 171.33 ± 1303.21 U/L; p < 0.01), white blood cell counts (20,827 ± 3603 vs. 12,242 ± 3782/µL; p < 0.01) and lower body surface area (0.52 ± 0.32 vs. 0.71 ± 0.36 m(2) ; p < 0.05) compared to patients in the no-SIRS group. Binary logistic regression revealed cardiopulmonary bypass time (OR: 1.05, p < 0.05), low body weight (<10 kg) (OR: 2.44; p < 0.05), and preoperative diagnosis of right to left shunt congenital heart disease (OR: 8.06; p < 0.01) as independent predictors of SIRS. SIRS was also found to be a strong independent predictor of mortality (OR: 10.13, p < 0.01). SIRS after congenital heart surgery is associated with increased mortality. Independent risk factors for SIRS in the patient population of the study were cardiopulmonary bypass time, body weight below 10 kg and preoperative diagnosis of right to left shunt congenital heart disease. © 2014 Wiley Periodicals, Inc.

The frequency of anesthesia-related cardiac arrests in patients with congenital heart disease undergoing cardiac surgery.

PubMed

Odegard, Kirsten C; DiNardo, James A; Kussman, Barry D; Shukla, Avinash; Harrington, James; Casta, Al; McGowan, Francis X; Hickey, Paul R; Bacha, Emile A; Thiagarajan, Ravi R; Laussen, Peter C

2007-08-01

The frequency of anesthesia-related cardiac arrests during pediatric anesthesia has been reported between 1.4 and 4.6 per 10,000 anesthetics. ASA physical status >III and younger age are risk factors. Patients with congenital cardiac disease may also be at increased risk. Therefore, in this study, we evaluated the frequency of cardiac arrest in patients with congenital heart disease undergoing cardiac surgery at a large pediatric tertiary referral center. Using an established data registry, all cardiac arrests from January 2000 through December 2005 occurring in the cardiac operating rooms were reviewed. A cardiac arrest was defined as any event requiring external or internal chest compressions, with or without direct cardioversion. Events determined to be anesthesia-related were classified as likely related or possibly related. There were 41 cardiac arrests in 40 patients (median age, 2.9 mo; range, 2 days to 23 yr) during 5213 anesthetics over the time period, for an overall frequency of 0.79%; 78% were open procedures requiring cardiopulmonary bypass and 22% closed procedures not requiring cardiopulmonary bypass. Eleven cardiac arrests (26.8%) were classified as either likely (n = 6) or possibly related (n = 5) to anesthesia, (21.1 per 10,000 anesthetics) but with no mortality; 30 were categorized as procedure-related. The incidence of anesthesia-related and procedure-related cardiac arrests was highest in neonates (P < 0.001). There was no association with year of event or experience of the anesthesiologist. The frequency of anesthesia-related cardiac arrest in patients undergoing cardiac surgery is increased, but is not associated with an increase in mortality. Neonates and infants are at higher risk. Careful preparation and anticipation is important to ensure timely and effective resuscitation.

Correlation of a Novel Noninvasive Tissue Oxygen Saturation Monitor to Serum Central Venous Oxygen Saturation in Pediatric Patients with Postoperative Congenital Cyanotic Heart Disease

PubMed Central

Yadlapati, Ajay; Grogan, Tristan; Elashoff, David; Kelly, Robert B.

2013-01-01

Abstract: Using a novel noninvasive, visible-light optical diffusion oximeter (T-Stat VLS Tissue Oximeter; Spectros Corporation, Portola Valley, CA) to measure the tissue oxygen saturation (StO2) of the buccal mucosa, the correlation between StO2 and central venous oxygen saturation (ScvO2) was examined in children with congenital cyanotic heart disease undergoing a cardiac surgical procedure. Paired StO2 and serum ScvO2 measurements were obtained postoperatively and statistically analyzed for agreement and association. Thirteen children (nine male) participated in the study (age range, 4 days to 18 months). Surgeries included Glenn shunt procedures, Norwood procedures, unifocalization procedures with Blalock-Taussig shunt placement, a Kawashima/Glenn shunt procedure, a Blalock-Taussig shunt placement, and a modified Norwood procedure. A total of 45 paired StO2-ScvO2 measurements was obtained. Linear regression demonstrated a Pearson’s correlation of .58 (95% confidence interval [CI], .35–.75; p < .0001). The regression slope coefficient estimate was .95 (95% CI, .54–1.36) with an interclass correlation coefficient of .48 (95% CI, .22–.68). Below a clinically relevant average ScvO2 value, a receiver operator characteristic analysis yielded an area under the curve of .78. Statistical methods to control for repeatedly measuring the same subjects produced similar results. This study shows a moderate relationship and agreement between StO2 and ScvO2 measurements in pediatric patients with a history of congenital cyanotic heart disease undergoing a cardiac surgical procedure. This real-time monitoring device can act as a valuable adjunct to standard noninvasive monitoring in which serum ScvO2 sampling currently assists in the diagnosis of low cardiac output after pediatric cardiac surgery. PMID:23691783

Immunologic Aging in Adults with Congenital Heart Disease: Does Infant Sternotomy Matter?

PubMed

Elder, Robert W; George, Roshan P; McCabe, Nancy M; Rodriguez, Fred H; Book, Wendy M; Mahle, William T; Kirk, Allan D

2015-10-01

Thymectomy is performed routinely in infants undergoing cardiothoracic surgery. Children post-sternotomy have decreased numbers of T lymphocytes, although the mechanisms involved and long-term consequences of this have not been defined. We hypothesized that lymphopenia in patients with adult congenital heart disease (ACHD) would be reflective of premature T cell maturation and exhaustion. Adults with ACHD who had sternotomy to repair congenital heart disease as infants (<1 year) and age-matched ACHD patients without prior sternotomy were studied using polychromatic flow cytometry interrogating markers of lymphocyte maturation, exhaustion and senescence. Group differences were analyzed using Mann-Whitney U and Fisher's exact tests. Eighteen ACHD patients aged 21-40 years participated: 10 cases and 8 controls. Median age at sternotomy for cases was 52 days. Cases and controls were matched for age (28.9 vs. 29.1 years; p = 0.83), gender (p = 0.15) and race (p = 0.62) and had similar case complexity. Cases had a lower mean percentage of cytotoxic CD8 lymphocytes compared to controls (26.8 vs. 33.9 %; p = 0.016), with fewer naive, undifferentiated CD8 T cells (31.0 vs. 53.6 %; p = 0.027). CD8 cells expressing PD1, a marker of immune exhaustion, trended higher in cases versus controls (25.6 vs. 19.0 %; p = 0.083). Mean percentage of CD4 cells was higher in cases versus controls (65.6 vs. 59.6 %; p = 0.027), without differences in CD4 T cell maturation subtype. In summary, ACHD patients who undergo sternotomy as infants exhibit differences in T lymphocyte composition compared to ACHD controls, suggesting accelerated immunologic exhaustion. Investigation is warranted to assess the progressive nature and clinical impact of this immune phenotypic change.

Heart transplantation in adults with congenital heart disease.

PubMed

Houyel, Lucile; To-Dumortier, Ngoc-Tram; Lepers, Yannick; Petit, Jérôme; Roussin, Régine; Ly, Mohamed; Lebret, Emmanuel; Fadel, Elie; Hörer, Jürgen; Hascoët, Sébastien

2017-05-01

With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients. This population is deemed to be at high risk of mortality after heart transplantation, although their long-term survival is similar to that of patients transplanted for other reasons. Indeed, heart transplantation in adults with congenital heart disease is often challenging, because of several potential problems: complex cardiac and vascular anatomy, multiple previous palliative and corrective surgeries, and effects on other organs (kidney, liver, lungs) of long-standing cardiac dysfunction or cyanosis, with frequent elevation of pulmonary vascular resistance. In this review, we focus on the specific problems relating to heart and heart-lung transplantation in this population, revisit the indications/contraindications, and update the long-term outcomes. Copyright © 2017. Published by Elsevier Masson SAS.

Genetics of Congenital Heart Disease: Past and Present.

PubMed

Muntean, Iolanda; Togănel, Rodica; Benedek, Theodora

2017-04-01

Congenital heart disease is the most common congenital anomaly, representing an important cause of infant morbidity and mortality. Congenital heart disease represents a group of heart anomalies that include septal defects, valve defects, and outflow tract anomalies. The exact genetic, epigenetic, or environmental basis of congenital heart disease remains poorly understood, although the exact mechanism is likely multifactorial. However, the development of new technologies including copy number variants, single-nucleotide polymorphism, next-generation sequencing are accelerating the detection of genetic causes of heart anomalies. Recent studies suggest a role of small non-coding RNAs, micro RNA, in congenital heart disease. The recently described epigenetic factors have also been found to contribute to cardiac morphogenesis. In this review, we present past and recent genetic discoveries in congenital heart disease.

An empowerment health education program for children undergoing surgery for congenital heart diseases.

PubMed

Ni, Zhihong; Chao, Yannfen; Xue, Xiaoling

2016-09-01

Since the surgery for congenital heart disease (CHD) is considered highly risky, appropriate postoperative care is crucial. After the surgery, children are often discharged with unhealed wounds, incomplete recovery, and continuing pain. Health education programs based on empowerment education model can assist clients to develop skills in self-management. This study aimed to evaluate the effectiveness of an empowerment health education program for improving caregiving knowledge, caring behaviors, and self-efficacy of parents caring for children after corrective surgery for CHD. This prospective clinical trial enrolled pediatric patients undergoing surgical correction for CHD. Patients were divided into two groups: the control group (n = 42), which received the standard education program, and the intervention group (n = 44), which participated in the empowerment theory-based education program. We collected data on left ventricular ejection fraction (LVEF); peripheral oxygen saturation (SpO2); New York Heart Association classification of the patients; and the parents' caregiving knowledge, caring behaviors, and self-efficacy before surgery and one month and three months after surgery. At one month and three months after surgery, the intervention group scored higher than the control group in caregiving knowledge, caring behavior, and self-efficacy. By the third month after surgery, the intervention group had significantly higher values of LVEF and SpO2 than the control group. © The Author(s) 2015.

Initial validation of a healthcare needs scale for young people with congenital heart disease.

PubMed

Chen, Chi-Wen; Ho, Ciao-Lin; Su, Wen-Jen; Wang, Jou-Kou; Chung, Hung-Tao; Lee, Pi-Chang; Lu, Chun-Wei; Hwang, Be-Tau

2018-01-01

To validate the initial psychometric properties of a Healthcare Needs Scale for Youth with Congenital Heart Disease. As the number of patients with congenital heart disease surviving to adulthood increases, the transitional healthcare needs for adolescents and young adults with congenital heart disease require investigation. However, few tools comprehensively identify the healthcare needs of youth with congenital heart disease. A cross-sectional study was employed to examine the psychometric properties of the Healthcare Needs Scale for Youth with Congenital Heart Disease. The sample consisted of 500 patients with congenital heart disease, aged 15-24 years, from paediatric cardiology departments and covered the period from March-August 2015. The patients completed the 25-item Healthcare Needs Scale for Youth with Congenital Heart Disease, the questionnaire on health needs for adolescents and the WHO Quality of Life-BREF. Reliability and construct, concurrent, predictive and known-group validity were examined. The Healthcare Needs Scale for Youth with Congenital Heart Disease includes three dimensions, namely health management, health policy and individual and interpersonal relationships, which consist of 25 items. It demonstrated excellent internal consistency and sound construct, concurrent, predictive and known-group validity. The Healthcare Needs Scale for Youth with Congenital Heart Disease is a psychometrically robust measure of the healthcare needs of youth with congenital heart disease. It has the potential to provide nurses with a means to assess and identify the concerns of youth with congenital heart disease and to help them achieve a successful transition to adult care. © 2017 John Wiley & Sons Ltd.

Long-term follow-up of implantable cardioverter-defibrillators in adult congenital heart disease patients: indications and outcomes.

PubMed

Santharam, Sandhya; Hudsmith, Lucy; Thorne, Sara; Clift, Paul; Marshall, Howard; De Bono, Joseph

2017-03-01

Ventricular arrhythmias are a major cause of mortality in adult congenital heart disease (ACHD) patients. The European Society of Cardiology guidelines state that implantable cardioverter-defibrillators (ICD) should be considered in patients with congenital heart disease following spontaneous sustained ventricular tachycardia (VT) or cardiac arrest and in patients at presumed high risk. This study sought to analyse the circumstances in which ACHD patients received ICD and to assess outcomes of ICD implantation, including therapies delivered and the rate of complications. A retrospective review was performed of all adult patients with congenital heart disease undergoing ICD implant between 2000 and 2014, in a large quaternary referral centre with over 4000 adults with congenital heart disease under active follow-up. Demographics: 42 patients with congenital heart disease had ICD implants: 55% male; age range 21-71 years and mean age 45 years. Mean age at implantation of ICD was 41 years. Mean follow-up was 5 years. Diagnosis: 50% of patients had repaired tetralogy of Fallot (TOF). Twelve per cent of patients had repaired transposition of the great arteries. Reason for ICD: 15 patients (35.7%) received ICD after sustained VT. Eleven patients (26.2%) received ICD after cardiac arrest. Sixteen (38%) had ICD implanted as primary prophylaxis. Outcome: Since implantation, six patients received an appropriate full-output shock for VT from ICD. Nineteen (45%) patients suffered significant complications (inappropriate shocks 11, inappropriate anti-tachycardia pacing resulting in VF 1, infection requiring extraction 3, lead abnormalities 3, and pneumothorax 1). Equal proportions of primary and secondary prevention patients received appropriate shocks. Most patients had ICD for secondary prevention (62%), and the majority had repaired TOF. There was a 2.9% annual appropriate shock rate. However, there was a high incidence of complications with more than a third suffering a major complication (9% per annum). The risks and benefits of ICD implantation are patient and disease specific, and must be clearly discussed prior to implantation. Further research is warranted into the use of primary prevention ICD in ACHD and in alternatives to ICD such as ablation in specific patient groups. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.

Clinical feasibility of pre-operative neurodevelopmental assessment of infants undergoing open heart surgery.

PubMed

Campbell, Miranda; Rabbidge, Bridgette; Ziviani, Jenny; Sakzewski, Leanne

2017-08-01

Assessing the neurodevelopmental status of infants with congenital heart disease before surgery provides a means of identifying those at heightened risk of developmental delay. This study aimed to investigate factors impacting clinical feasibility of pre-operative neurodevelopmental assessment of infants undergoing early open heart surgery. Infants who underwent open heart surgery prior to 4 months of age participated in this cross-sectional study. The Test of Infant Motor Performance and Prechtl's Assessment of General Movements were undertaken on infants pre-operatively. When assessments could not be undertaken, reasons were ascribed to either infant or environmental circumstances. Demographic data and Aristotle scores were compared between groups of infants who did or did not undergo assessment. Binary logistic regression was used to explore associations. A total of 60 infants participated in the study. Median gestational age was 38.78 weeks (interquartile range: 36.93-39.72). Of these infants, 37 (62%) were unable to undergo pre-operative assessment. Twenty-four (40%) could not complete assessment due to infant-related factors and 13 (22%) due to environmental-related factors. For every point increase in the Aristotle Patient-Adjusted Complexity score, the infants likelihood of being unable to undergo assessment increased by 35% (odds ratio: 0.35; 95% confidence interval: 1.03-1.77, P = 0.03). Over half of the infants undergoing open heart surgery were unable to complete pre-operative neurodevelopmental assessment. The primary reason for this was infant-related medical instability. Findings suggest further research is warranted to investigate whether the Aristotle Patient-Adjusted Complexity score might serve as an indicator to inform developmental surveillance with this medically fragile cohort. © 2017 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).

Comorbid Conditions in Neonates With Congenital Heart Disease.

PubMed

Krishnamurthy, Ganga; Ratner, Veniamin; Bacha, Emile; Aspelund, Gudrun

2016-08-01

The objectives of this review are to discuss the pathophysiology, clinical impact and treatment of major noncardiac anomalies, and prematurity in infants with congenital heart disease. MEDLINE and PubMed. Mortality risk is significantly higher in patients with congenital heart disease and associated anomalies compared with those in whom the heart defect occurs in isolation. Although most noncardiac structural anomalies do not require surgery in the neonatal period, several require surgery for survival. Management of such infants poses multiple challenges. Premature infants with congenital heart disease face challenges imposed by their immature organ systems, which are susceptible to injury or altered function by congenital heart disease and abnormal circulatory physiology independent of congenital heart disease. For optimal outcomes in premature infants or in infants with multiple congenital anomalies, a collaborative interdisciplinary approach is necessary.

Prevalence and spectrum of in utero structural brain abnormalities in fetuses with complex congenital heart disease.

PubMed

Brossard-Racine, M; du Plessis, A J; Vezina, G; Robertson, R; Bulas, D; Evangelou, I E; Donofrio, M; Freeman, D; Limperopoulos, C

2014-08-01

Brain injury is a major complication in neonates with complex congenital heart disease. Preliminary evidence suggests that fetuses with congenital heart disease are at greater risk for brain abnormalities. However, the nature and frequency of these brain abnormalities detected by conventional fetal MR imaging has not been examined prospectively. Our primary objective was to determine the prevalence and spectrum of brain abnormalities detected on conventional clinical MR imaging in fetuses with complex congenital heart disease and, second, to compare the congenital heart disease cohort with a control group of fetuses from healthy pregnancies. We prospectively recruited pregnant women with a confirmed fetal congenital heart disease diagnosis and healthy volunteers with normal fetal echocardiogram findings who underwent a fetal MR imaging between 18 and 39 weeks gestational age. A total of 338 fetuses (194 controls; 144 with congenital heart disease) were studied at a mean gestational age of 30.61 ± 4.67 weeks. Brain abnormalities were present in 23% of the congenital heart disease group compared with 1.5% in the control group (P < .001). The most common abnormalities in the congenital heart disease group were mild unilateral ventriculomegaly in 12/33 (36.4%) and increased extra-axial spaces in 10/33 (30.3%). Subgroup analyses comparing the type and frequency of brain abnormalities based on cardiac physiology did not reveal significant associations, suggesting that the brain abnormalities were not limited to those with the most severe congenital heart disease. This is the first large prospective study reporting conventional MR imaging findings in fetuses with congenital heart disease. Our results suggest that brain abnormalities are prevalent but relatively mild antenatally in fetuses with congenital heart disease. The long-term predictive value of these findings awaits further study. © 2014 by American Journal of Neuroradiology.

Compliance with Adult Congenital Heart Disease Guidelines: Are We Following the Recommendations?

PubMed

Gerardin, Jennifer F; Menk, Jeremiah S; Pyles, Lee A; Martin, Cindy M; Lohr, Jamie L

2016-05-01

As the adult congenital heart disease population increases, poor transition from pediatric to adult care can lead to suboptimal quality of care and an increase in individual and institutional costs. In 2008, the American College of Cardiology and American Heart Association updated the adult congenital heart disease practice guidelines and in 2011, the American Heart Association recommended transition guidelines to standardize and encourage appropriate timing of transition to adult cardiac services. The objective of this study was to evaluate if patient age or complexity of congenital heart disease influences pediatric cardiologists' decision to transfer care to adult providers and to evaluate the compliance of different types of cardiology providers with current adult congenital heart disease treatment guidelines. A single-center retrospective review of 991 adult congenital heart disease patients identified by ICD-9 code from 2010 to 2012. Academic and community outpatient cardiology clinics. Nine hundred ninety-one patients who are 18 years and older with congenital heart disease. None. The compliance with health maintenance and transfer of care recommendations in the outpatient setting. For patients seen by pediatric cardiologists, only 20% had transfer of care discussions documented, most often in younger simple patients. Significant differences in compliance with preventative health guidelines were found between cardiology provider types. Even though a significant number of adults with congenital heart disease are lost to appropriate follow-up in their third and fourth decades of life, pediatric cardiologists discussed transfer of care with moderate and complex congenital heart disease patients less frequently. Appropriate transfer of adults with congenital heart disease to an adult congenital cardiologist provides an opportunity to reinforce the importance of regular follow-up in adulthood and may improve outcomes as adult congenital cardiologists followed the adult congenital heart disease guidelines more consistently than pediatric or adult cardiologists. © 2015 Wiley Periodicals, Inc.

Effect of steroids on inflammatory markers and clinical parameters in congenital open heart surgery: a randomised controlled trial.

PubMed

Amanullah, Muhammad M; Hamid, Mohammad; Hanif, Hashim M; Muzaffar, Marium; Siddiqui, Maria T; Adhi, Fatima; Ahmad, Khabir; Khan, Shahjahan; Hasan, Zahra

2016-03-01

Cardiopulmonary bypass is associated with systemic inflammatory response. Steroids suppress this response, although the therapeutic evidence remains controversial. We hypothesised that intravenous steroids in children undergoing open-heart surgery would decrease inflammation leading to better early post-operative outcomes. We conducted a randomised controlled trial to evaluate the trends in the levels of immunomodulators and their effects on clinical parameters. To assess the effects of intravenous steroids on early post-operative inflammatory markers and clinical parameters in children undergoing open-heart surgery. A randomised controlled trial involving 152 patients, from one month up to 18 years of age, who underwent open-heart surgery for congenital heart disease from April 2010-2012 was carried out. Patients were randomised and administered either three scheduled intravenous pulse doses of dexamethasone (1 mg/kg) or placebo. Blood samples were drawn at four time intervals and serum levels of inflammatory cytokines - Interleukin-6, 8, 10, 18, and tumour necrosis factor-alpha - were measured. Clinical parameters were also assessed. Blood cytokine levels were compared between the dexamethasone (n=65) and placebo (n=64) groups. Interleukin-6 levels were lower at 6 and 24 hours post-operatively (p<0.001), and Interleukin-10 levels were higher 6 hours post-operatively (p<0.001) in the steroid group. Interleukin-8, 18, and tumour necrosis factor-alpha levels did not differ between the groups at any time intervals. The clinical parameters were similar in both the groups. Dexamethasone caused quantitative suppression of Interleukin-6 and increased Interleukin-10 activation, contributing to reduced immunopathology, but it did not translate into clinical benefit in the short term.

Comparative study of the Aristotle Comprehensive Complexity and the Risk Adjustment in Congenital Heart Surgery scores.

PubMed

Bojan, Mirela; Gerelli, Sébastien; Gioanni, Simone; Pouard, Philippe; Vouhé, Pascal

2011-09-01

The Aristotle Comprehensive Complexity (ACC) and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) scores have been proposed for complexity adjustment in the analysis of outcome after congenital heart surgery. Previous studies found RACHS-1 to be a better predictor of outcome than the Aristotle Basic Complexity score. We compared the ability to predict operative mortality and morbidity between ACC, the latest update of the Aristotle method and RACHS-1. Morbidity was assessed by length of intensive care unit stay. We retrospectively enrolled patients undergoing congenital heart surgery. We modeled each score as a continuous variable, mortality as a binary variable, and length of stay as a censored variable. We compared performance between mortality and morbidity models using likelihood ratio tests for nested models and paired concordance statistics. Among all 1,384 patients enrolled, 30-day mortality rate was 3.5% and median length of intensive care unit stay was 3 days. Both scores strongly related to mortality, but ACC made better prediction than RACHS-1; c-indexes 0.87 (0.84, 0.91) vs 0.75 (0.65, 0.82). Both scores related to overall length of stay only during the first postoperative week, but ACC made better predictions than RACHS-1; U statistic=0.22, p<0.001. No significant difference was noted after adjusting RACHS-1 models on age, prematurity, and major extracardiac abnormalities. The ACC was a better predictor of operative mortality and length of intensive care unit stay than RACHS-1. In order to achieve similar performance, regression models including RACHS-1 need to be further adjusted on age, prematurity, and major extracardiac abnormalities. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

The Aristotle Comprehensive Complexity score predicts mortality and morbidity after congenital heart surgery.

PubMed

Bojan, Mirela; Gerelli, Sébastien; Gioanni, Simone; Pouard, Philippe; Vouhé, Pascal

2011-04-01

The Aristotle Comprehensive Complexity (ACC) score has been proposed for complexity adjustment in the analysis of outcome after congenital heart surgery. The score is the sum of the Aristotle Basic Complexity score, largely used but poorly related to mortality and morbidity, and of the Comprehensive Complexity items accounting for comorbidities and procedure-specific and anatomic variability. This study aims to demonstrate the ability of the ACC score to predict 30-day mortality and morbidity assessed by the length of the intensive care unit (ICU) stay. We retrospectively enrolled patients undergoing congenital heart surgery in our institution. We modeled the ACC score as a continuous variable, mortality as a binary variable, and length of ICU stay as a censored variable. For each mortality and morbidity model we performed internal validation by bootstrapping and assessed overall performance by R(2), calibration by the calibration slope, and discrimination by the c index. Among all 1,454 patients enrolled, 30-day mortality rate was 3.4% and median length of ICU stay was 3 days. The ACC score strongly related to mortality, but related to length of ICU stay only during the first postoperative week. For the mortality model, R(2) = 0.24, calibration slope = 0.98, c index = 0.86, and 95% confidence interval was 0.82 to 0.91. For the morbidity model, R(2) = 0.094, calibration slope = 0.94, c index = 0.64, and 95% confidence interval was 0.62 to 0.66. The ACC score predicts 30-day mortality and length of ICU stay during the first postoperative week. The score is an adequate tool for complexity adjustment in the analysis of outcome after congenital heart surgery. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Measuring hospital performance in congenital heart surgery: administrative versus clinical registry data.

PubMed

Pasquali, Sara K; He, Xia; Jacobs, Jeffrey P; Jacobs, Marshall L; Gaies, Michael G; Shah, Samir S; Hall, Matthew; Gaynor, J William; Peterson, Eric D; Mayer, John E; Hirsch-Romano, Jennifer C

2015-03-01

In congenital heart surgery, hospital performance has historically been assessed using widely available administrative data sets. Recent studies have demonstrated inaccuracies in case ascertainment (coding and inclusion of eligible cases) in administrative versus clinical registry data; however, it is unclear whether this impacts assessment of performance on a hospital level. Merged data from The Society of Thoracic Surgeons (STS) database (clinical registry) and the Pediatric Health Information Systems (PHIS) database (administrative data set) for 46,056 children undergoing cardiac operations (2006-2010) were used to evaluate in-hospital mortality for 33 hospitals based on their administrative versus registry data. Standard methods to identify/classify cases were used: Risk Adjustment in Congenital Heart Surgery, version 1 (RACHS-1) in the administrative data and STS-European Association for Cardiothoracic Surgery (STAT) methodology in the registry. Median hospital surgical volume based on the registry data was 269 cases per year; mortality was 2.9%. Hospital volumes and mortality rates based on the administrative data were on average 10.7% and 4.7% lower, respectively, although this varied widely across hospitals. Hospital rankings for mortality based on the administrative versus registry data differed by 5 or more rank positions for 24% of hospitals, with a change in mortality tertile classification (high, middle, or low mortality) for 18% and a change in statistical outlier classification for 12%. Higher volume/complexity hospitals were most impacted. Agency for Healthcare Quality and Research (AHRQ) methods in the administrative data yielded similar results. Inaccuracies in case ascertainment in administrative versus clinical registry data can lead to important differences in assessment of hospital mortality rates for congenital heart surgery. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Variability in Non-Cardiac Surgical Procedures in Children with Congenital Heart Disease

PubMed Central

Sulkowski, Jason P.; Cooper, Jennifer N.; McConnell, Patrick I.; Pasquali, Sara K.; Shah, Samir S.; Minneci, Peter C.; Deans, Katherine J.

2014-01-01

Background The purpose of this study was to examine the volume and variability of non-cardiac surgeries performed in children with congenital heart disease (CHD) requiring cardiac surgery in the first year of life. Methods Patients who underwent cardiac surgery by 1 year of age and had a minimum 5-year follow-up at 22 of the hospitals contributing to the Pediatric Health Information System database between 2004–2012 were included. Frequencies of non-cardiac surgical procedures by age 5 years were determined and categorized by subspecialty. Patients were stratified according to their maximum RACHS-1 (Risk Adjustment in Congenital Heart Surgery) category. The proportions of patients across hospitals who had a non-cardiac surgical procedure for each subspecialty were compared using logistic mixed effects models. Results 8,857 patients underwent congenital heart surgery during the first year of life, 3,621 (41%) of whom had 13,894 non-cardiac surgical procedures by 5 years. Over half of all procedures were in general surgery (4,432; 31.9%) or otolaryngology (4,002; 28.8%). There was significant variation among hospitals in the proportion of CHD patients having non-cardiac surgical procedures. Compared to children in the low risk group (RACHS-1 categories 1–3), children in the high-risk group (categories 4–6) were more likely to have general, dental, orthopedic, and thoracic procedures. Conclusions Children with CHD requiring cardiac surgery frequently also undergo non-cardiac surgical procedures; however, considerable variability in the frequency of these procedures exists across hospitals. This suggests a lack of uniformity in indications used for surgical intervention. Further research should aim to better standardize care for this complex patient population. PMID:25475794

[Prenatal screening of congenital heart defects in population at low risk of congenital defects. A reality today].

PubMed

Sainz, J A; Zurita, M J; Guillen, I; Borrero, C; García-Mejido, J; Almeida, C; Turmo, E; Garrido, R

2015-01-01

We propose to demonstrate that it is possible to implement a valid (diagnostic sensitivity for major cardiac malformations 90%), and universal (applied to over 90% of pregnant women), prenatal screening method for congenital heart defects. Prospective study. A total of 12478 pregnant women were evaluated between January 2008 and December 2010. Congenital heart diseases were screened using fetal extended basic echocardiography (cardiac ultrasound). The prevalence of birth defects in general and congenital heart disease was 2.5% (2.2-2.7%) and 0.9% (0.7-1%) respectively. Congenital heart disease had a higher rate of association with other structural abnormalities with 11.5% (5.6-17.4%), 21% for major congenital heart disease (9.9-32%), and chromosomal abnormalities of 15.9% (9.1-22.7%), with 32.6% for major congenital heart disease (19.8-45.3%). A fetal cardiac ultrasound assessment was performed on 99.2% of pregnant women. The fetal echocardiography is useful for the diagnosis of congenital heart disease in general, and major congenital heart disease, with a sensitivity of 42.8% (33.5-52.5%) and 90.4% (78.9-96.8%), respectively, and a specificity for both of 99.9% (99.8-99.9%). It is possible to perform a valid prenatal and universal screening of major congenital heart disease. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

Congenital heart disease

MedlinePlus

... page: //medlineplus.gov/ency/article/001114.htm Congenital heart disease To use the sharing features on this page, please enable JavaScript. Congenital heart disease (CHD) is a problem with the heart's structure ...

The genetics of congenital heart disease… understanding and improving long-term outcomes in congenital heart disease: a review for the general cardiologist and primary care physician.

PubMed

Simmons, M Abigail; Brueckner, Martina

2017-10-01

This review has two purposes: to provide an updated review of the genetic causes of congenital heart disease (CHD) and the clinical implications of these genetic mutations, and to provide a clinical algorithm for clinicians considering a genetics evaluation of a CHD patient. A large portion of congenital heart disease is thought to have a significant genetic contribution, and at this time a genetic cause can be identified in approximately 35% of patients. Through the advances made possible by next generation sequencing, many of the comorbidities that are frequently seen in patients with genetic congenital heart disease patients can be attributed to the genetic mutation that caused the congenital heart disease. These comorbidities are both cardiac and noncardiac and include: neurodevelopmental disability, pulmonary disease, heart failure, renal dysfunction, arrhythmia and an increased risk of malignancy. Identification of the genetic cause of congenital heart disease helps reduce patient morbidity and mortality by improving preventive and early intervention therapies to address these comorbidities. Through an understanding of the clinical implications of the genetic underpinning of congenital heart disease, clinicians can provide care tailored to an individual patient and continue to improve the outcomes of congenital heart disease patients.

[Congenital heart disease mortality in Spain during a 10 year period (2003-2012)].

PubMed

Pérez-Lescure Picarzo, Javier; Mosquera González, Margarita; Latasa Zamalloa, Pello; Crespo Marcos, David

2018-05-01

Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012. Mortality rate and relative risk of mortality were estimated by Poisson regression. There were 2,970 (4.58%) infant deaths in a population of 64,831 patients with congenital heart disease, with 73.8% of deaths occurring during first week of life. Infant mortality rate in patients with congenital heart disease was 6.23 per 10,000 live births, and remained constant during the ten-year period of the study, representing 18% of total infant mortality rate in Spain. The congenital heart diseases with highest mortality rates were hypoplastic left heart syndrome (41.4%), interruption of aortic arch (20%), and total anomalous pulmonary drainage (16.8%). Atrial septal defect (1%) and pulmonary stenosis (1.1%) showed the lowest mortality rate. Congenital heart disease was a major cause of infant mortality with no variations during the study period. The proportion of infants who died in our study was similar to other similar countries. In spite of current medical advances, some forms of congenital heart disease show very high mortality rates. Copyright © 2017 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome.

PubMed

Krieger, Eric V; Leary, Peter J; Opotowsky, Alexander R

2015-11-01

Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient's hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology, diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations. Copyright © 2015 Elsevier Inc. All rights reserved.

Congenital Heart Information Network

MedlinePlus

... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

Health in adults with congenital heart disease.

PubMed

Cuypers, Judith A A E; Utens, Elisabeth M W J; Roos-Hesselink, Jolien W

2016-09-01

Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many adults with congenital heart disease encounter late complications. Late morbidity can be related to the congenital heart defect itself, but may also be the consequence of the surgical or medical treatment or longstanding alterations in hemodynamics, neurodevelopment and psychosocial development. This narrative review describes the cardiac and non-cardiac long-term morbidity in the adult population with congenital heart disease. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Adult Congenital Heart Disease: Scope of the Problem.

PubMed

Mazor Dray, Efrat; Marelli, Ariane J

2015-11-01

This article reviews the changing epidemiology of congenital heart disease summarizing its impact on the demographics of the congenital heart disease population and the progress made in order to improve outcomes in this patient population. Birth prevalence of congenital heart disease can be modified by many factors. As a result of decreasing mortality and increasing survival in all forms of congenital heart disease, the median age of patients has increased and adults now compose two-thirds of patients with congenital heart disease. Disease burden and resulting health services utilization increase significantly across the lifespan. Bridging the gap between policy and quality of care can be improved by referral to specialized adult congenital heart disease centers and planning delivery of specialized services that are commensurate with population needs, program accreditation criteria and certified training of designated workforce. Copyright © 2015 Elsevier Inc. All rights reserved.

The World Society for Pediatric and Congenital Heart Surgery: its mission and history.

PubMed

Tchervenkov, Christo I; Stellin, Giovanni; Kurosawa, Hiromi; Jacobs, Jeffrey P; Mavroudis, Constantine; Bernier, Pierre-Luc; Maruszewski, Bohdan; Kreutzer, Christian; Cicek, Sertac; Kinsley, Robin H; Nunn, Graham R; Jonas, Richard A

2009-01-01

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

Case complexity scores in congenital heart surgery: a comparative study of the Aristotle Basic Complexity score and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) system.

PubMed

Al-Radi, Osman O; Harrell, Frank E; Caldarone, Christopher A; McCrindle, Brian W; Jacobs, Jeffrey P; Williams, M Gail; Van Arsdell, Glen S; Williams, William G

2007-04-01

The Aristotle Basic Complexity score and the Risk Adjustment in Congenital Heart Surgery system were developed by consensus to compare outcomes of congenital cardiac surgery. We compared the predictive value of the 2 systems. Of all index congenital cardiac operations at our institution from 1982 to 2004 (n = 13,675), we were able to assign an Aristotle Basic Complexity score, a Risk Adjustment in Congenital Heart Surgery score, and both scores to 13,138 (96%), 11,533 (84%), and 11,438 (84%) operations, respectively. Models of in-hospital mortality and length of stay were generated for Aristotle Basic Complexity and Risk Adjustment in Congenital Heart Surgery using an identical data set in which both Aristotle Basic Complexity and Risk Adjustment in Congenital Heart Surgery scores were assigned. The likelihood ratio test for nested models and paired concordance statistics were used. After adjustment for year of operation, the odds ratios for Aristotle Basic Complexity score 3 versus 6, 9 versus 6, 12 versus 6, and 15 versus 6 were 0.29, 2.22, 7.62, and 26.54 (P < .0001). Similarly, odds ratios for Risk Adjustment in Congenital Heart Surgery categories 1 versus 2, 3 versus 2, 4 versus 2, and 5/6 versus 2 were 0.23, 1.98, 5.80, and 20.71 (P < .0001). Risk Adjustment in Congenital Heart Surgery added significant predictive value over Aristotle Basic Complexity (likelihood ratio chi2 = 162, P < .0001), whereas Aristotle Basic Complexity contributed much less predictive value over Risk Adjustment in Congenital Heart Surgery (likelihood ratio chi2 = 13.4, P = .009). Neither system fully adjusted for the child's age. The Risk Adjustment in Congenital Heart Surgery scores were more concordant with length of stay compared with Aristotle Basic Complexity scores (P < .0001). The predictive value of Risk Adjustment in Congenital Heart Surgery is higher than that of Aristotle Basic Complexity. The use of Aristotle Basic Complexity or Risk Adjustment in Congenital Heart Surgery as risk stratification and trending tools to monitor outcomes over time and to guide risk-adjusted comparisons may be valuable.

Arrhythmias in Adults with Congenital Heart Disease: What Are Risk Factors for Specific Arrhythmias?

PubMed

Loomba, Rohit S; Buelow, Matthew W; Aggarwal, Saurabh; Arora, Rohit R; Kovach, Joshua; Ginde, Salil

2017-04-01

An increasing number of patients with congenital heart disease are now surviving into adulthood. This has also led to the emergence of complications from the underlying congenital heart disease, related surgical interventions, and associated combordities. While the prevalence of particular arrhythmias with specific congenital heart disease has been previously described, a detailed analysis of all lesions and a large number of comorbidities has not been previously published. Admissions with congenital heart disease were identified in the National Inpatient Sample. Associated comorbidities were also identified for these patients. Univariate analysis was done to compare those risk factors associated with specific arrhythmias in the setting of congenital heart disease. Next, regression analysis was done to identify what patient characteristics and comorbidities were associated with increased risk of specific arrhythmias. A total of 52,725,227 admissions were included in the analysis. Of these, 109,168 (0.21%) had congenital heart disease. Of those with congenital heart disease, 27,088 (25%) had an arrhythmia at some point. The most common arrhythmia in those with congenital heart disease was atrial fibrillation, which was noted in 86% of those with arrhythmia followed by atrial flutter which was noted in 20% of those with congenital heart disease. The largest burden of arrhythmia was found to be in those with tricuspid atresia with a 51% prevalence of arrhythmia in this group followed by Ebstein anomaly which had an arrhythmia prevalence of 39%. Increasing age, male gender, double outlet right ventricle, atrioventricular septal defect, heart failure, obstructive sleep apnea, transposition of the great arteries, congenitally corrected transposition, and tetralogy of Fallot were frequently noted to be independent risk factors of specific arrhythmias. Approximately, 25% of adult admissions with congenital heart disease are associated with arrhythmia. The burden of arrhythmia varies by the specific lesion and other risk factors as well. Understanding of these can help in risk stratification and can help devise strategies to lower this risk. © 2016 Wiley Periodicals, Inc.

Care and Treatment for Congenital Heart Defects

MedlinePlus

... Physical Activity Recommendations for Heart Health • Tools & Resources Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

Association of brain abscess with a mycotic aneurysm of the contralateral internal carotid artery in a patient of cyanotic heart disease

PubMed Central

Bansal, Sumit; Borkar, Sachin A.; Mahapatra, Ashok K.

2017-01-01

Congenital cyanotic heart disease can lead to intra-cranial involvement. Authors report a very rare case of right intra-cerebral abscess diagnosed on computerized tomography (CT) scan and simultaneous presence of an aneurysm of the left internal carotid artery diagnosed on CT angiogram in a 15-year-old child with congenital cyanotic heart disease with recent onset left hemiparesis. Right cerebral abscess was tapped and left internal carotid aneurysm was planned to be followed up by giving antibiotics and serial angiograms, but he could not survive and died due to non-cranial cause. We conclude that cerebral angiography is necessary to diagnose cerebro-vascular complications, including infectious aneurysms, in cases presenting with unusual findings on neuroimaging study. Patient must undergo serial angiography while being on intravenous antibiotics. Intervention (either surgical or endovascular) should be considered if there are no signs of regression of size of aneurysm or in the presence of aneurysm rupture. We have not been able to find a similar case in the English literature. PMID:28484536

Prophylactic levosimendan for the prevention of low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease.

PubMed

Hummel, Johanna; Rücker, Gerta; Stiller, Brigitte

2017-08-02

Low cardiac output syndrome remains a serious complication, and accounts for substantial morbidity and mortality in the postoperative course of paediatric patients undergoing surgery for congenital heart disease. Standard prophylactic and therapeutic strategies for low cardiac output syndrome are based mainly on catecholamines, which are effective drugs, but have considerable side effects. Levosimendan, a calcium sensitiser, enhances the myocardial function by generating more energy-efficient myocardial contractility than achieved via adrenergic stimulation with catecholamines. Thus potentially, levosimendan is a beneficial alternative to standard medication for the prevention of low cardiac output syndrome in paediatric patients after open heart surgery. To review the efficacy and safety of the postoperative prophylactic use of levosimendan for the prevention of low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease. We identified trials via systematic searches of CENTRAL, MEDLINE, Embase, and Web of Science, as well as clinical trial registries, in June 2016. Reference lists from primary studies and review articles were checked for additional references. We only included randomised controlled trials (RCT) in our analysis that compared prophylactic levosimendan with standard medication or placebo, in infants and children up to 18 years of age, who were undergoing surgery for congenital heart disease. Two review authors independently extracted data and assessed risk of bias according to a pre-defined protocol. We obtained additional information from all but one of the study authors of the included studies. We used the five GRADE considerations (study limitations, consistency of effect, imprecision, indirectness, and publication bias) to assess the quality of evidence from the studies that contributed data to the meta-analyses for the prespecified outcomes. We created a 'Summary of findings' table to summarise the results and the quality of evidence for each outcome. We included five randomised controlled trials with a total of 212 participants in the analyses. All included participants were under five years of age. Using GRADE, we assessed there was low-quality evidence for all analysed outcomes. We assessed high risk of performance and detection bias for two studies due to their unblinded setting. Levosimendan showed no clear effect on risk of mortality (risk ratio (RR) 0.47, 95% confidence interval (CI) 0.12 to 1.82; participants = 123; studies = 3) and no clear effect on low cardiac output syndrome (RR 0.64, 95% CI 0.39 to 1.04; participants = 83; studies = 2) compared to standard treatments. Data on time-to-death were not available from any of the included studies.There was no conclusive evidence on the effect of levosimendan on the secondary outcomes. The length of intensive care unit stays (mean difference (MD) 0.33 days, 95% CI -1.16 to 1.82; participants = 188; studies = 4), length of hospital stays (MD 0.26 days, 95% CI -3.50 to 4.03; participants = 75; studies = 2), duration of mechanical ventilation (MD -0.04 days, 95% CI -0.08 to 0.00; participants = 208; studies = 5), and the risk of mechanical circulatory support or cardiac transplantation (RR 1.49, 95% CI 0.19 to 11.37; participants = 60; studies = 2) did not clearly differ between the groups. Published data about adverse effects of levosimendan were limited. A meta-analysis of hypotension, one of the most feared side effects of levosimendan, was not feasible because of the heterogeneous expression of blood pressure values. The current level of evidence is insufficient to judge whether prophylactic levosimendan prevents low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease. So far, no significant differences have been detected between levosimendan and standard inotrope treatments in this setting.The authors evaluated the quality of evidence as low, using the GRADE approach. Reasons for downgrading were serious risk of bias (performance and detection bias due to unblinded setting of two RCTs), serious risk of inconsistency, and serious to very serious risk of imprecision (small number of included patients, low event rates).

Brain abnormalities and neurodevelopmental delay in congenital heart disease: systematic review and meta-analysis.

PubMed

Khalil, A; Suff, N; Thilaganathan, B; Hurrell, A; Cooper, D; Carvalho, J S

2014-01-01

Studies have demonstrated an association between congenital heart disease (CHD) and neurodevelopmental delay. Neuroimaging studies have also demonstrated a high incidence of preoperative brain abnormalities. The aim of this study was to perform a systematic review to quantify the non-surgical risk of brain abnormalities and of neurodevelopmental delay in infants with CHD. MEDLINE, EMBASE and The Cochrane Library were searched electronically without language restrictions, utilizing combinations of the terms congenital heart, cardiac, neurologic, neurodevelopment, magnetic resonance imaging, ultrasound, neuroimaging, autopsy, preoperative and outcome. Reference lists of relevant articles and reviews were hand-searched for additional reports. Cohort and case-control studies were included. Studies reporting neurodevelopmental outcomes and/or brain lesions on neuroimaging in infants with CHD before heart surgery were included. Cases of chromosomal or genetic abnormalities, case reports and editorials were excluded. Between-study heterogeneity was assessed using the I(2) test. The search yielded 9129 citations. Full text was retrieved for 119 and the following were included in the review: 13 studies (n = 425 cases) reporting on brain abnormalities either preoperatively or in those who did not undergo congenital cardiac surgery and nine (n = 512 cases) reporting preoperative data on neurodevelopmental assessment. The prevalence of brain lesions on neuroimaging was 34% (95% CI, 24-46; I(2) = 0%) in transposition of the great arteries, 49% (95% CI, 25-72; I(2) = 65%) in left-sided heart lesions and 46% (95% CI, 40-52; I(2) =18.1%) in mixed/unspecified cardiac lesions, while the prevalence of neurodevelopmental delay was 42% (95% CI, 34-51; I(2) = 68.9). In the absence of chromosomal or genetic abnormalities, infants with CHD are at increased risk of brain lesions as revealed by neuroimaging and of neurodevelopmental delay. These findings are independent of the surgical risk, but it is unclear whether the time of onset is fetal or postnatal. Copyright © 2013 ISUOG. Published by John Wiley & Sons Ltd.

Oral triiodothyronine normalizes triiodothyronine levels after surgery for pediatric congenital heart disease*.

PubMed

Marwali, Eva M; Boom, Cindy E; Sakidjan, Indriwanto; Santoso, Anwar; Fakhri, Dicky; Kartini, Ay; Kekalih, Aria; Schwartz, Steven M; Haas, Nikolaus A

2013-09-01

This study was conducted to determine if oral triiodothyronine supplementation could prevent the decrease of serum triiodothyronine levels that commonly occurs after cardiopulmonary bypass for pediatric congenital heart surgery. Secondary objectives included identifying any significant adverse effects of oral triiodothyronine supplementation, including any effects on the thyroid/pituitary axis. Randomized, placebo-controlled, doubleblind clinical trial Operating room and ICU. Infants and children younger than 2 years of age undergoing congenital heart surgery using cardiopulmonary bypass (n = 43). Subjects were assigned to placebo (n = 15, group A) or one of two treatment groups: a low-dose group (group B, n = 14, 0.5 mcg/kg triiodothyronine orally every 24 hr for 3 d) or a high-dose group (group C, n = 14, 0.5 mcg/kg triiodothyronine orally every 12 hr for 3 d). Thyroid hormone, including total and free triiodothyronine levels at predetermined time points, potential side effects indicating hyperthyroidism, indicators of the thyroid-pituitary axis, and clinical endpoints. Oral triiodothyronine supplementation twice-daily maintained serum triiodothyronine levels within normal limits in group C, whereas serum levels progressively declined in groups A and B. A statistically significant difference in triiodothyronine levels between the treatment groups occurred between 18 and 36 hours post cross-clamp release, with the largest difference in serum levels between group C and group A noted at 36 hours post cross-clamp release (total triiodothyronine, 0.71 ± 0.15 [0.34-1.08] ng/mL [p < 0.01]; free triiodothyronine, 2.56 ± 0.49 [1.33-3.79] pg/mL [p < 0.01]). There was no evidence of hyperthyroidism or suppression of the pituitary-thyroid axis in either treatment group Oral triiodothyronine supplementation at a dose of 0.5 mcg/kg every 12 hours for 3 days can maintain total and free triiodothyronine levels within normal limits after open-heart surgery using cardiopulmonary bypass for congenital heart disease.

Prevalence of Noncardiac and Genetic Abnormalities in Neonates Undergoing Cardiac Operations: Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

PubMed

Patel, Angira; Costello, John M; Backer, Carl L; Pasquali, Sara K; Hill, Kevin D; Wallace, Amelia S; Jacobs, Jeffrey P; Jacobs, Marshall L

2016-11-01

Among patients with congenital heart disease (CHD), the coexistence of noncardiac congenital anatomic abnormalities (NC), genetic abnormalities (GA), and syndromes (S) may influence therapeutic strategies and outcomes. The appreciated prevalence of these abnormalities has risen because increased screening and improved diagnostic precision enable identification of these comorbidities in a larger fraction of neonates with CHD. We examined the contemporary prevalence and distribution of NC/GA/S across diagnostic groups among neonates undergoing cardiac operations using a large nationally representative clinical registry. The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) was queried to identify neonates (≤30 days) who underwent index cardiac operations from 2010 to 2013. The fundamental cardiac diagnosis was used to identify 10 diagnostic groups. The prevalence of NC/GA/S was reported across each group. The cohort included 15,376 index neonatal operations from 112 centers. Overall, 18.8% (2,894 of 15,376) of operations were performed in neonates with NC/GA/S. Patients with atrioventricular septal defect (212 of 357 [59.4%]), interrupted aortic arch (248 of 567 [43.7%]), truncus arteriosus (204 of 554 [36.8%]), and tetralogy of Fallot (417 of 1,383 [30.2%]) had the highest prevalence of NC/GA/S abnormalities, whereas those with transposition of the great arteries (111 of 2,778 [4.0%]) had the lowest prevalence. The most commonly identified NC/GA/S included heterotaxy (597 of 15,376 [3.9%]), DiGeorge syndrome or 22q11 deletion (550 of 15,376 [3.6%]), Down syndrome or trisomy 21 (318 of 15, 376 [2.1%]), intestinal malrotation (220 of 15,376 [1.4%]), and Turner syndrome or 45XO (189 of 15,376 [1.2%]). The prevalence of NC/GA/S varies widely across CHD diagnostic groups. This information may be useful for patient counseling, recommendations for screening for anomalies and genetic disorders, and perioperative management. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Cardiac Embryology and Molecular Mechanisms of Congenital Heart Disease: A Primer for Anesthesiologists.

PubMed

Kloesel, Benjamin; DiNardo, James A; Body, Simon C

2016-09-01

Congenital heart disease is diagnosed in 0.4% to 5% of live births and presents unique challenges to the pediatric anesthesiologist. Furthermore, advances in surgical management have led to improved survival of those patients, and many adult anesthesiologists now frequently take care of adolescents and adults who have previously undergone surgery to correct or palliate congenital heart lesions. Knowledge of abnormal heart development on the molecular and genetic level extends and improves the anesthesiologist's understanding of congenital heart disease. In this article, we aim to review current knowledge pertaining to genetic alterations and their cellular effects that are involved in the formation of congenital heart defects. Given that congenital heart disease can currently only occasionally be traced to a single genetic mutation, we highlight some of the difficulties that researchers face when trying to identify specific steps in the pathogenetic development of heart lesions.

Report of the 2005 STS Congenital Heart Surgery Practice and Manpower Survey.

PubMed

Jacobs, Marshall L; Mavroudis, Constantine; Jacobs, Jeffrey P; Tchervenkov, Christo I; Pelletier, Glenn J

2006-09-01

Limited information is available concerning the congenital heart surgery workforce in North America. To obtain reliable data, The Society of Thoracic Surgeons (STS) Workforce on Congenital Heart Surgery undertook a subspecialty focused survey. Preliminary research of websites and databases revealed a potential target group of 263 congenital heart surgeons, including 248 surgeons at 121 US centers and 15 at eight Canadian sites. Surveys were sent to these, plus any additional individuals who listed an interest in congenital or pediatric heart surgery on CTSNet or STS websites. Responders include active congenital heart surgeons, inactive, or retired surgeons, adult cardiac surgeons, and surgeons in training. Analysis is based exclusively on the responses of 217 active congenital heart surgeons (>80% of target). Average age was 48.3 +/- 8.3 years (range, 33 to 78). Ten were female (5%), 207 male (95%). American Medical School graduates were 170 (79%), with 9.2 +/- 1.6 years of postgraduate training. Eighty-five percent are Thoracic Board certified. Congenital heart surgery training outside the United States or Canada was obtained by 29 (14%). One hundred twenty-eight (59%) do exclusively congenital heart surgery. One third perform fewer than 100 congenital cases per year, one third perform 100 to 199, and one third perform 200 or more. Congenital heart surgeons have been in their current positions for 9.5 +/- 8.2 years. Eleven are in their first year of practice. Mean anticipated years to retirement is 15.7 +/- 7.5 (range, 1 to 34). Three anticipate retirement within 1 year. At the same time, 39 fellows will complete postgraduate training at 28 centers this year, and 19 will seek positions in North America. These data should help to facilitate rational plans to meet manpower needs, including evolving policies concerning training and certification.

Selected environmental risk factors and congenital heart defects.

PubMed

Kuciene, Renata; Dulskiene, Virginija

2008-01-01

The aim of the article is to review the published scientific literature and epidemiological studies about the effect of selected environmental risk factors on congenital heart defects in infants. According to recent reports, the prevalence of congenital heart defects is around 1% of live births. Congenital heart malformations are the leading cause of infant mortality. Unfortunately, the majority of the causes of heart defects remain unknown. These malformations are caused by interaction of genetic and environmental factors. The article reviews selected environmental risk factors: maternal illnesses and conditions associated with metabolic disorder (maternal diabetes, obesity, phenylketonuria), maternal lifestyle factors (alcohol use, smoking), which may increase the risk of congenital heart defects.

Potential implications of the helical heart in congenital heart defects.

PubMed

Corno, Antonio F; Kocica, Mladen J

2007-01-01

The anatomic and functional observations made by Francisco Torrent-Guasp, in particular his discovery of the helical ventricular myocardial band (HVMB), have challenged what has been taught to cardiologists and cardiac surgeons over centuries. A literature debate is ongoing, with interdependent articles and comments from supporters and critics. Adequate understanding of heart structure and function is obviously indispensable for the decision-making process in congenital heart defects. The HVMB described by Torrent-Guasp and the potential impact on the understanding and treatment of congenital heart defects has been analyzed in the following settings: embryology, ventriculo-arterial discordance (transposition of great arteries), Ebstein's anomaly, pulmonary valve regurgitation after repair of tetralogy of Fallot, Ross operation, and other congenital heart defects. The common structural spiral feature is only one of the elements responsible for the functional interaction of right and left ventricles, and understanding the form/function relationship in congenital heart defects is more difficult than for acquired heart disease because of the variety and complexity of congenital heart defects. Individuals involved in the care of patients with congenital heart defects have to be stimulated to consider further investigations and alternative surgical strategies.

Gastrointestinal system malformations in children are associated with congenital heart defects.

PubMed

Orün, Utku Arman; Bilici, Meki; Demirçeken, Fulya G; Tosun, Mahya; Ocal, Burhan; Cavuşoğlu, Yusuf Hakan; Erdoğan, Derya; Senocak, Filiz; Karademir, Selmin

2011-03-01

To determine the frequency of congenital heart defects (CHD) in children with gastrointestinal malformations (GISM) and mortality rates in patients with GISM. Two hundred and forty two consecutive children patients with GISM followed up in Pediatric Surgery Clinics of our hospital were examined for cardiovascular anomaly by the Department of Pediatric Cardiology, and the CHD incidence was investigated by examining the records of the patients retrospectively. Chi-square test was used for the statistical analysis of data. Two hundred and forty two patients with gastrointestinal system malformations were included in the study. Of 242 patients, 135 (55.8%) were male and 107 (44.2%) were female, and their age range was 0-15 years. The most frequent GISM were anorectal malformations (43.2%), atresia involving stomach, ileum or colon (21%) and esophageal atresia/tracheoesophageal fistula (18.3%). Congenital heart defects were observed in 28.5% of the participants. The most frequent defects were as follows; atrial septal defect (31 patients, 44.9%) a, ventricular septal defect (17 patients, 24.6%) and patent ductus arteriosus (5 patients, 7.2%). There was no significant difference (p>0.05) in mortality rate in patients with CHD (16.7%) and without CHD (13.3%) undergoing operations for GISM. We would like to emphasize the importance of the earliest possible cardiological evaluation of all patients with gastrointestinal system malformations.

Intrapartum electrocardiogram alteration in fetuses with congenital heart disease: a case-control study.

PubMed

Gay, Estelle; Bornallet, Géraldine; Gaucherand, Pascal; Doret, Muriel

2015-11-01

To assess if the fetal electrocardiogram especially ST segment is modified by congenital heart diseases: modifications in frequencies of the different ST events and modifications in signal quality. A retrospective case-control study, comparing frequencies of the different ST events and the quality of the signal between fetuses with congenital heart diseases and fetuses without congenital heart disease. From 2000 to 2011, fifty-eight fetuses with congenital heart disease had their heart rate recording using a STAN device during labor. Control group was fetuses who were born just before a case and had a STAN as a second line for intrapartum surveillance. Cases and controls were matched on parity, gestational age at birth, presence of growth restriction and umbilical artery pH. Frequencies of the different ST event and quality of the signal were first analyzed for the global labor recording, and then separately for the first and the second phase of labor. No statistically significant difference in ST event frequencies between fetuses with congenital heart disease and the control group was found. Regarding the quality of the signal, 11.49% (±18.82) of recording time is a signal loss for fetus with congenital heart disease whereas only 5.18% (±10.67) for the control group (p=0.028). This is the first study investigating for intrapartum electrocardiogram modification in fetus with congenital heart disease. Congenital heart diseases do not modify frequencies of ST events. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Congenital cardiac disease in the newborn infant: past, present, and future.

PubMed

Sadowski, Sharyl L

2009-03-01

Congenital heart defects are the most common of all congenital malformations, with a review of the literature reporting the incidence at 6 to 8 per 1000 live births. The Centers for Disease Control reports cyanotic heart defects occurred in 56.9 per 100,000 live births in the United States in 2005, with higher rates noted when maternal age exceeded 40 years. The incidence of congenital heart disease in premature infants is 12.5 per 1000 live births, excluding isolated patent ductus arteriosus and atrial septal defect. Despite advances in detection and treatment, congenital heart disease accounts for 3% of all infant deaths and 46% of death from congenital malformations. This article discusses the embryology, pathogenesis, clinical presentation, incidence, classifications, and management of congenital heart diseases.

Xenopus: An Emerging Model for Studying Congenital Heart Disease

PubMed Central

Kaltenbrun, Erin; Tandon, Panna; Amin, Nirav M.; Waldron, Lauren; Showell, Chris; Conlon, Frank L.

2011-01-01

Congenital heart defects affect nearly 1% of all newborns and are a significant cause of infant death. Clinical studies have identified a number of congenital heart syndromes associated with mutations in genes that are involved in the complex process of cardiogenesis. The African clawed frog, Xenopus, has been instrumental in studies of vertebrate heart development and provides a valuable tool to investigate the molecular mechanisms underlying human congenital heart diseases. In this review, we discuss the methodologies that make Xenopus an ideal model system to investigate heart development and disease. We also outline congenital heart conditions linked to cardiac genes that have been well-studied in Xenopus and describe some emerging technologies that will further aid in the study of these complex syndromes. PMID:21538812

Instrument to assess educational programs for parents of children with congenital heart disease undergoing cardiac surgery.

PubMed

Pino Armijo, Paola; Ramírez León, Muriel; Clavería Rodríguez, Cristian

2017-10-01

To design and validate an instrument to assess the relevance of educational programs for parents of children with congenital heart disease (CHD) undergoing cardiac surgery. In October 2011, an instrument was designed based on Kaufman's model and on the bibliography, and a survey was developed in the form of a checklist with 32 close-ended questions about received education and desired education, categorized into 5 items: educator, time, place, means, and content. The survey was reviewed by 4 academic professionals and 9 experts in the care of children with CHD, and the checklist was extended to include 42 close-ended questions and 5 open questions. The instrument was administered on the day before discharge to the parents of children with CHD undergoing cardiac surgery at the Department of Pediatrics between February and August 2013. The survey was self-administered by the first participants and administered by the investigator among the remaining participants. Fifty-five children met inclusion criteria; a total of 60 parents took part in the study. Agreement was observed between received education and desired education, which was statistically significant only in terms of education provided by a cardiologist (p= 0.000, K= 0.659) and in the hall (p= 0.000, K= 0.655). Statistically significant differences were observed between the 19 self-administered surveys and the 41 surveys administered by the investigator. Among the latter, a greater level of completion was observed for all items. A validated instrument was developed to assess the relevance of educational programs for parents of children with CHD undergoing cardiac surgery. This survey should be administered by a health care provider for a better understanding of information. Sociedad Argentina de Pediatría

Oral Triiodothyronine for Infants and Children Undergoing Cardiopulmonary Bypass.

PubMed

Marwali, Eva M; Boom, Cindy E; Budiwardhana, Novik; Fakhri, Dicky; Roebiono, Poppy S; Santoso, Anwar; Sastroasmoro, Sudigdo; Slee, April; Portman, Michael A

2017-08-01

This study evaluated the efficacy and safety of oral triiodothyronine (T3; Tetronine, Dalim BioTech, Korea) for infants and children undergoing cardiopulmonary bypass in an Indonesian population. We performed a single-center, randomized, double-blind, and placebo-controlled trial in children aged 3 years and younger undergoing congenital heart disease operations with cardiopulmonary bypass. We administered oral T3 (1 μg/kg per body weight/dose) or placebo (saccharum lactis) by nasogastric tube every 6 hours for 60 hours after induction of anesthesia. The primary end point, time to extubation, was compared with Cox regression. The modified intention-to-treat group included 101 placebo and 104 treated patients. The stratified log-rank test did not show a significant treatment difference (p = 0.061) for time to extubation, but after adjustment for age, the nutritional Z score, and Aristotle surgical complexity, the hazard ratio was 1.33 (95% confidence interval, 1.00 to 1.76; p = 0.049). The effect of T3 was stronger in the strata aged 5 months and younger (hazard ratio, 1.86; 95% confidence interval 1.02 to 3.39; p = 0.043). Median intubation time was 47.3 hours for the placebo and 32.1 hours for the T3 group in aged 5 months and younger. Adverse events rates, including arrhythmia, were similar between groups, although sepsis was more frequent with placebo. Oral T3 supplementation may shorten time to extubation in children undergoing congenital heart disease operations, particularly infants aged 5 months or younger. Administration is relatively safe, simple and inexpensive. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Hypoplastic left heart syndrome

MedlinePlus

HLHS; Congenital heart - hypoplastic left heart; Cyanotic heart disease - hypoplastic left heart ... Hypoplastic left heart is a rare type of congenital heart disease. It is more common in males than in females. As ...

Congenital Anomaly of Single Dominant Right Coronary Artery with Hypoplastic Left Coronary Artery.

PubMed

Chuang, Cheng-Yen; Chen, Yen-Chou; Cheng, Ho-Shun; Hsieh, Ming-Hsiung

2015-11-01

With the popularization of new imaging technology, more people are deciding to undergo non-invasive studies such as multidetector computerized tomography (MDCT) before receiving coronary angiography. For this reason, coronary anomalies of coronary artery are being encountered more frequently. We here report a 68-year-old male presenting with typical angina. The MDCT images suggested chronic total occlusion of the left anterior descending (LAD) artery with collateral circulation from the right coronary artery (RCA). The patient's coronary angiography showed a congenital coronary anomaly with a single dominant RCA supplying the entire coronary circulation of the heart with both LAD and left circumflex artery hypoplasia. Angiography; Anomaly; Computerized tomography; Coronary artery.

Chronic kidney disease in congenital heart disease patients: a narrative review of evidence.

PubMed

Morgan, Catherine; Al-Aklabi, Mohammed; Garcia Guerra, Gonzalo

2015-01-01

Patients with congenital heart disease have a number of risk factors for the development of chronic kidney disease (CKD). It is well known that CKD has a large negative impact on health outcomes. It is important therefore to consider that patients with congenital heart disease represent a population in whom long-term primary and secondary prevention strategies to reduce CKD occurrence and progression could be instituted and significantly change outcomes. There are currently no clear guidelines for clinicians in terms of renal assessment in the long-term follow up of patients with congenital heart disease. Consolidation of knowledge is critical for generating such guidelines, and hence is the purpose of this view. This review will summarize current knowledge related to CKD in patients with congenital heart disease, to highlight important work that has been done to date and set the stage for further investigation, development of prevention strategies, and re-evaluation of appropriate renal follow-up in patients with congenital heart disease. The literature search was conducted using PubMed and Google Scholar. Current epidemiological evidence suggests that CKD occurs in patients with congenital heart disease at a higher frequency than the general population and is detectable early in follow-up (i.e. during childhood). Best evidence suggests that approximately 30 to 50 % of adult patients with congenital heart disease have significantly impaired renal function. The risk of CKD is higher with cyanotic congenital heart disease but it is also present with non-cyanotic congenital heart disease. Although significant knowledge gaps exist, the sum of the data suggests that patients with congenital heart disease should be followed from an early age for the development of CKD. There is an opportunity to mitigate CKD progression and negative renal outcomes by instituting interventions such as stringent blood pressure control and reduction of proteinuria. There is a need to invest time, thought and money to fill existing knowledge gaps to improve health outcomes in this population. This review should serve as an impetus for generation of follow-up guidelines of kidney health evaluation in patients with congenital heart disease.

Acquired heart conditions in adults with congenital heart disease: a growing problem.

PubMed

Tutarel, Oktay

2014-09-01

The number of adults with congenital heart disease is increasing due to the great achievements in the field of paediatric cardiology, congenital heart surgery and intensive care medicine over the last decades. Mortality has shifted away from the infant and childhood period towards adulthood. As congenital heart disease patients get older, a high prevalence of cardiovascular risk factors is encountered similar to the general population. Consequently, the contribution of acquired morbidities, especially acquired heart conditions to patient outcome, is becoming increasingly important. Therefore, to continue the success story of the last decades in the treatment of congenital heart disease and to further improve the outcome of these patients, more attention has to be given to the prevention, detection and adequate therapy of acquired heart conditions. The aim of this review is to give an overview about acquired heart conditions that may be encountered in adults with congenital heart disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Increased arterial stiffness in children with congenital heart disease.

PubMed

Häcker, Anna-Luisa; Reiner, Barbara; Oberhoffer, Renate; Hager, Alfred; Ewert, Peter; Müller, Jan

2018-01-01

Objective Central systolic blood pressure (SBP) is a measure of arterial stiffness and strongly associated with atherosclerosis and end-organ damage. It is a stronger predictor of cardiovascular events and all-cause mortality than peripheral SBP. In particular, for children with congenital heart disease, a higher central SBP might impose a greater threat of cardiac damage. The aim of the study was to analyse and compare central SBP in children with congenital heart disease and in healthy counterparts. Patients and methods Central SBP was measured using an oscillometric method in 417 children (38.9% girls, 13.0 ± 3.2 years) with various congenital heart diseases between July 2014 and February 2017. The test results were compared with a recent healthy reference cohort of 1466 children (49.5% girls, 12.9 ± 2.5 years). Results After correction for several covariates in a general linear model, central SBP of children with congenital heart disease was significantly increased (congenital heart disease: 102.1 ± 10.2 vs. healthy reference cohort: 100.4 ± 8.6, p < .001). The analysis of congenital heart disease subgroups revealed higher central SBP in children with left heart obstructions (mean difference: 3.6 mmHg, p < .001), transpositions of the great arteries after arterial switch (mean difference: 2.2 mmHg, p = .017) and univentricular hearts after total cavopulmonary connection (mean difference: 2.1 mmHg, p = .015) compared with the reference. Conclusion Children with congenital heart disease have significantly higher central SBP compared with healthy peers, predisposing them to premature heart failure. Screening and long-term observations of central SBP in children with congenital heart disease seems warranted in order to evaluate the need for treatment.

Association of maternal chronic disease with risk of congenital heart disease in offspring

PubMed Central

Chou, Hsin-Hsu; Chiou, Meng-Jiun; Liang, Fu-Wen; Chen, Lea-Hua; Lu, Tsung-Hsueh; Li, Chung-Yi

2016-01-01

Background: Information about known risk factors for congenital heart disease is scarce. In this population-based study, we aimed to investigate the relation between maternal chronic disease and congenital heart disease in offspring. Methods: The study cohort consisted of 1 387 650 live births from 2004 to 2010. We identified chronic disease in mothers and mild and severe forms of congenital heart disease in their offspring from Taiwan’s National Health Insurance medical claims. We used multivariable logistic regression analysis to assess the associations of all cases and specific types of congenital heart disease with various maternal chronic diseases. Results: For mothers with the following chronic diseases, the overall prevalence of congenital heart disease in their children was significantly higher than for mothers without these diseases: diabetes mellitus type 1 (adjusted odds ratio [OR] 2.32, 95% confidence interval [CI] 1.66–3.25), diabetes mellitus type 2 (adjusted OR 2.85, 95% CI 2.60–3.12), hypertension (adjusted OR 1.87, 95% CI 1.69–2.07), congenital heart defects (adjusted OR 3.05, 95% CI 2.45–3.80), anemia (adjusted OR 1.31, 95% CI 1.25–1.38), connective tissue disorders (adjusted OR 1.39, 95% CI 1.19–1.62), epilepsy (adjusted OR 1.37, 95% CI 1.08–1.74) and mood disorders (adjusted OR 1.25, 95% CI 1.11–1.41). The same pattern held for mild forms of congenital heart disease. A higher prevalence of severe congenital heart disease was seen only among offspring of mothers with congenital heart defects or type 2 diabetes. Interpretation: The children of women with several kinds of chronic disease appear to be at risk for congenital heart disease. Preconception counselling and optimum treatment of pregnant women with chronic disease would seem prudent. PMID:27729382

Association of maternal chronic disease with risk of congenital heart disease in offspring.

PubMed

Chou, Hsin-Hsu; Chiou, Meng-Jiun; Liang, Fu-Wen; Chen, Lea-Hua; Lu, Tsung-Hsueh; Li, Chung-Yi

2016-12-06

Information about known risk factors for congenital heart disease is scarce. In this population-based study, we aimed to investigate the relation between maternal chronic disease and congenital heart disease in offspring. The study cohort consisted of 1 387 650 live births from 2004 to 2010. We identified chronic disease in mothers and mild and severe forms of congenital heart disease in their offspring from Taiwan's National Health Insurance medical claims. We used multivariable logistic regression analysis to assess the associations of all cases and specific types of congenital heart disease with various maternal chronic diseases. For mothers with the following chronic diseases, the overall prevalence of congenital heart disease in their children was significantly higher than for mothers without these diseases: diabetes mellitus type 1 (adjusted odds ratio [OR] 2.32, 95% confidence interval [CI] 1.66-3.25), diabetes mellitus type 2 (adjusted OR 2.85, 95% CI 2.60-3.12), hypertension (adjusted OR 1.87, 95% CI 1.69-2.07), congenital heart defects (adjusted OR 3.05, 95% CI 2.45-3.80), anemia (adjusted OR 1.31, 95% CI 1.25-1.38), connective tissue disorders (adjusted OR 1.39, 95% CI 1.19-1.62), epilepsy (adjusted OR 1.37, 95% CI 1.08-1.74) and mood disorders (adjusted OR 1.25, 95% CI 1.11-1.41). The same pattern held for mild forms of congenital heart disease. A higher prevalence of severe congenital heart disease was seen only among offspring of mothers with congenital heart defects or type 2 diabetes. The children of women with several kinds of chronic disease appear to be at risk for congenital heart disease. Preconception counselling and optimum treatment of pregnant women with chronic disease would seem prudent. © 2016 Canadian Medical Association or its licensors.

Adults with Congenital Heart Defects

MedlinePlus

... Peripheral Artery Disease Venous Thromboembolism Aortic Aneurysm More Web Booklet: Adults With Congenital Heart Defects Updated:Aug ... topic from the list below to learn more. Web Booklet: Adults With Congenital Heart Defects Introduction Introduction: ...

Genetic Counseling for Congenital Heart Defects

MedlinePlus

... Artery Disease Venous Thromboembolism Aortic Aneurysm More Genetic Counseling for Congenital Heart Defects Updated:Jan 19,2018 ... with congenital heart disease considers having children. Genetic counseling can help answer these questions and address your ...

Between invisible defects and visible impact: the life experiences of adolescents and young adults with congenital heart disease.

PubMed

Chiang, Yueh-Tao; Chen, Chi-Wen; Su, Wen-Jen; Wang, Jou-Kou; Lu, Chun-Wei; Li, Yuh-Fen; Moons, Philip

2015-03-01

To describe the life experiences of adolescents and young adults with congenital heart disease. Owing to medical advances, most children with congenital heart disease are expected to survive into adulthood. The transitional development from adolescence to adult is the critical period for fostering self-care. Descriptive phenomenological study. Thirty-five patients of 15-24 years old with congenital heart disease were recruited from paediatric cardiology clinics by purposive sampling. They were individually interviewed between October 2012-February 2013 using a semi-structured interview guideline and joined adult congenital heart disease clinics at two medical centres in northern Taiwan. The data were analysed using descriptive phenomenological method developed by Giorgi. The essence of the life experience of adolescents and young adults with congenital heart disease involves a dynamic process of moving between invisible defects and coexistence with the disease. Six themes emerged: (1) invisible defects: the existence of imperfect understanding; (2) conflict: interpersonal frustrations; (3) imbalance: the loss of self-balance; (4) suffering: increasing anxiety; (5) encounters: meeting needs; and (6) coexistence: positive coping strategies. As patients with congenital heart disease transition from adolescence into adulthood, they must learn about their disease, overcome frustration and anxiety and develop self-care strategies for coexisting with congenital heart disease. Results of this study may serve as clinical care guidelines for adolescents and young adults with congenital heart disease and give a reference for developing transitional intervention strategies. © 2014 John Wiley & Sons Ltd.

Antenatal diagnosis of congenital heart disease and Down's syndrome: the potential effect on the practice of paediatric cardiology.

PubMed Central

Abu-Harb, M.; Wyllie, J.; Hey, E.; Richmond, S.; Wren, C.

1995-01-01

OBJECTIVE--To predict the effect of antenatal ultrasound screening for congenital heart disease and maternal serum screening of Down's syndrome on the practice of paediatric cardiology and paediatric cardiac surgery. DESIGN--A retrospective and prospective ascertainment of all congenital heart disease diagnosed in infancy in 1985-1991. SETTING--One English health region. PATIENTS--All congenital heart disease diagnosed in infancy by echocardiography, cardiac catheterisation, surgery, or necropsy was classified as "complex", "significant", or "minor" and as "detectable" or "not detectable" on a routine antenatal ultrasound scan. RESULTS--1347 infants had congenital heart disease which was "complex" in 13%, "significant" in 55%, and "minor" in 32%. 15% of cases were "detectable" on routine antenatal ultrasound. Assuming 20% detection and termination of 67% of affected pregnancies, liveborn congenital heart disease would be reduced by 2%, infant mortality from congenital heart disease by 5%, and paediatric cardiac surgical activity by 3%. Maternal screening for Down's syndrome, assuming 75% uptake, 60% detection, and termination of all affected pregnancies, would reduce liveborn cases of Down's syndrome by 45%, liveborn cases of congenital heart disease by 3.5%, and cardiac surgery by 2.6%. CONCLUSIONS--Screening for congenital heart disease using the four chamber view in routine obstetric examinations and maternal serum screening for Down's syndrome is likely to have only a small effect on the requirements for paediatric cardiology services and paediatric cardiac surgery. PMID:7547001

Two case reports of anophthalmia and congenital heart disease: Adding a new dimension to this association.

PubMed

Wang, Jenny; Steelman, Charlotte K; Vincent, Robert; Richburg, Delene; Chang, Tiffany S; Shehata, Bahig M

2010-01-01

Anophthalmia is the congenital absence of ocular tissue from the orbit. Many syndromes and malformations (e.g., anophthalmia-esophageal-genital syndrome, Matthew-Wood syndrome, CHARGE syndrome, oculo-facial-cardio-dental-syndome, heterotaxy, and Fraser syndrome) have been associated with anophthalmia. However, its relation with congenital heart disease has not been fully elucidated. In this article, we discuss two cases of patients with anophthalmia and congenital heart defects, and we compare these findings with other syndromes with which anophthalmia has been associated. One of our two patients showed complex congenital heart disease with heterotaxia, polysplenia, and normal lung lobation. These findings may reflect a new dimension of anophthalmia, heterotaxia, and congenital heart disease associations.

Congenital heart defects in cats: A retrospective study of 162 cats (1996-2013).

PubMed

Tidholm, Anna; Ljungvall, Ingrid; Michal, Jenny; Häggström, Jens; Höglund, Katja

2015-12-01

To study the prevalence and distribution of congenital heart defects in cats presented at two referral centers in Sweden between 1996 and 2013. 162 client-owned cats with congenital heart defects. Case records of cats diagnosed with congenital heart disease were reviewed retrospectively. The overall prevalence of congenital heart disease was 0.2% of the total number of patient cats, and 8% of cats diagnosed with heart disease. A total of 182 heart defects were identified as 16 cats were diagnosed with more than one defect. Ventricular septal defect (VSD) was most prevalent, found in 50% of cats, followed by tricuspid valve dysplasia (11%), pulmonic stenosis (10%), atrial septal defect (10%), aortic stenosis (9%), mitral valve dysplasia (9%), tetralogy of Fallot (5%), patent ductus arteriosus (3%), common atrioventricular canal (2%), and the following defects that each accounted for 0.6% of cats: double chamber right ventricle, double outlet right ventricle, endocardial fibroelastosis, dextroposition of the aorta, persistent right aortic arch, and pulmonary atresia. The prevalence of congenital heart disease was 0.2% of the total number of patient cats, and 8% of cats diagnosed with heart disease. Ventricular septal defect was the most common congenital heart defect in this study. Copyright © 2014 Elsevier B.V. All rights reserved.

Correlation between NFATC1 gene polymorphisms and congenital heart disease in children.

PubMed

Li, C-L; Niu, L; Fu, M-Y; Tian, J; Wang, Q-W; An, X-J

2017-08-01

To analyze the links between NFATC1 gene polymorphism and congenital heart disease in children. In the present study, we selected 85 children patients with congenital heart disease who were hospitalized from February 2013 to February 2015 as research subjects (observation group), and 92 healthy subjects as control group. Restriction fragment length polymorphism (RFLP) was used for analysis of NFATC1 gene in samples from each group. The distribution of NFATC1 genotype and allele between the observation group (children with congenital heart disease) and the control group showed no significant difference (p >0.05), but AA, GG genotypes, and allele frequency between pathological samples of children with congenital heart disease and the control group displayed significant difference (p <0.05) (X2 = 16.04, p <0.05; X2 = 16.29, p <0.05). Further analyses showed that AA, GG, AG genotype and allele frequency among children with congenital heart disease in observation group also showed a difference, i.e., homozygote (AA, GG) ratio in children with severe congenital heart disease is relatively high. There is a correlation between NFATC1 genes and the incidence of congenital heart disease in children, and a correlation between different genotypes and allele frequency and the incidence of the disease.

Objectively assessed physical activity and sedentary behaviour does not differ between children and adolescents with and without a congenital heart defect: a pilot examination.

PubMed

Ewalt, Lauren A; Danduran, Michael J; Strath, Scott J; Moerchen, Victoria; Swartz, Ann M

2012-02-01

To objectively evaluate and describe physical activity levels in children with a stable congenital heart defect and compare those levels with children who do not have a congenital heart defect. We matched 21 pairs of children for gender and grade in school and gave them an accelerometer-based motion sensor to wear for 7 consecutive days. Physical activity levels did not differ between children with and without a congenital heart defect. During the 7 days of monitoring, children in this study spent most of their time in sedentary behaviours, that is, 6.7 hours of the 13 monitored hours, 54 minutes in moderate-intensity physical activity, and 12 minutes in vigorous-intensity physical activity. Less than one-fifth of all participants, with or without a congenital heart defect, accumulated sufficient physical activity to meet current physical activity recommendations for children and adolescents. Children with a stable congenital heart defect have activity behaviours that are similar to children without a congenital heart defect. Habitual physical activity in children with a congenital heart defect should be encouraged early on in life to develop strong physical activity habits that will hopefully follow them across their lifespan.

A systematic review of trends and patterns of congenital heart disease in children in Nigeria from 1964-2015.

PubMed

Abdulkadir, Mohammed; Abdulkadir, Zainab

2016-06-01

Congenital heart diseases cause significant childhood morbidity and mortality. Several restricted studies have been conducted on the epidemiology in Nigeria. No truly nationwide data on patterns of congenital heart disease exists. To determine the patterns of congenital heart disease in children in Nigeria and examine trends in the occurrence of individual defects across 5 decades. We searched PubMed database, Google scholar, TRIP database, World Health Organisation libraries and reference lists of selected articles for studies on patterns of congenital heart disease among children in Nigeria between 1964 and 2015. Two researchers reviewed the papers independently and extracted the data. Seventeen studies were selected that included 2,953 children with congenital heart disease. The commonest congenital heart diseases in Nigeria are ventricular septal defect (40.6%), patent ductus arteriosus (18.4%), atrial septal defect (11.3%) and tetralogy of Fallot (11.8%). There has been a 6% increase in the burden of VSD in every decade for the 5 decades studied and a decline in the occurrence of pulmonary stenosis. Studies conducted in Northern Nigeria demonstrated higher proportions of atrial septal defects than patent ductus arteriosus. Ventricular septal defects are the commonest congenital heart diseases in Nigeria with a rising burden.

Sequential segmental classification of feline congenital heart disease.

PubMed

Scansen, Brian A; Schneider, Matthias; Bonagura, John D

2015-12-01

Feline congenital heart disease is less commonly encountered in veterinary medicine than acquired feline heart diseases such as cardiomyopathy. Understanding the wide spectrum of congenital cardiovascular disease demands a familiarity with a variety of lesions, occurring both in isolation and in combination, along with an appreciation of complex nomenclature and variable classification schemes. This review begins with an overview of congenital heart disease in the cat, including proposed etiologies and prevalence, examination approaches, and principles of therapy. Specific congenital defects are presented and organized by a sequential segmental classification with respect to their morphologic lesions. Highlights of diagnosis, treatment options, and prognosis are offered. It is hoped that this review will provide a framework for approaching congenital heart disease in the cat, and more broadly in other animal species based on the sequential segmental approach, which represents an adaptation of the common methodology used in children and adults with congenital heart disease. Copyright © 2015 Elsevier B.V. All rights reserved.

Early life environment and social determinants of cardiac health in children with congenital heart disease.

PubMed

Wong, Peter; Denburg, Avram; Dave, Malini; Levin, Leo; Morinis, Julia Orkin; Suleman, Shazeen; Wong, Jonathan; Ford-Jones, Elizabeth; Moore, Aideen M

2018-04-01

Congenital heart disease is a significant cause of infant mortality. Epidemiology and social context play a crucial role in conditioning disease burden and modulating outcomes, while diagnosis and treatment remain resource intensive. This review will address the role of social demographics, environmental exposure, epigenetics and nutrition in the aetiology of congenital heart disease. We then discuss the determinant effect of social factors on the provision and outcomes of care for congenital heart disease and implications for practice. It is our hope that enhanced knowledge of the intersection of social determinants of health and congenital heart disease will facilitate effective preventative strategies at the individual and population levels to optimize heart health outcomes across the life course.

Severe portopulmonary hypertension in congenital hepatic fibrosis.

PubMed

Hsu, Chen-Ming; Chiu, Cheng-Tang; Lien, Jau-Min; Ng, Kwai-Fong

2003-03-01

Portopulmonary hypertension is a rare complication of portal hypertension. Although epoprostenol infusion, nitric oxide inhalation, isosorbide-5-mononitrate, nitroglycerin, and calcium channel blockers may reduce pulmonary artery pressure in patients with portopulmonary hypertension, the prognosis remains poor. We present a case of congenital hepatic fibrosis associated with pulmonary hypertension. A 42-year-old man with congenital hepatic fibrosis visited our hospital with syncope. The man had suffered from breathlessness on exertion for 2 weeks before the episode of syncope. He also had a history of portal hypertension with documented gastric cardiac varices at the age of 28 years. Despite undergoing intensive care, the patient died 1 week after admission owing to severe right-sided heart failure. Autopsy revealed dilatation of the right atrium and right ventricle grossly and plexogenic pulmonary arteriopathy microscopically. Accurate diagnosis of portopulmonary hypertension requires awareness of the disease and a high index of suspicion when examining patients with portal hypertension and dyspnea.

Report of the 2010 society of thoracic surgeons congenital heart surgery practice and manpower survey.

PubMed

Jacobs, Marshall L; Daniel, Megan; Mavroudis, Constantine; Morales, David L S; Jacobs, Jeffrey P; Fraser, Charles D; Turek, Joseph W; Mayer, John E; Tchervenkov, Christo; Conte, John

2011-08-01

The Society of Thoracic Surgeons (STS) Workforce on Congenital Heart Surgery undertook a second subspecialty Practice and Manpower Survey (2005, 2010) to obtain contemporary data. Preliminary research suggested a potential target group of 273 congenital heart surgeons, including 258 at 125 US centers and 15 at 8 Canadian centers. The web-based survey was sent to these surgeons, plus all individuals listing pediatric cardiac surgery on CTSNet. Two hundred forty-six responders included 213 active congenital heart surgeons, 16 retired congenital heart surgeons, and 17 surgeons in training. Retirement age was 63.5±7.5 years. Two hundred thirteen active congenital heart surgeon responders represent 78% of the original estimate. Their responses generated the following data: The mean age was 49.2±8.5 (range, 35 to 75 years). American medical school graduates included 159 of 201 respondents (79%). Years of postgraduate training was 9.7±1.7. One hundred ninety-seven (92%) respondents were certified in thoracic surgery by the American Board of Thoracic Surgery (ABTS). Twenty-eight of 200 (14%) received their congenital heart surgery training outside the United States or Canada. One hundred forty-three of 190 respondents (75%) perform exclusively congenital heart operations; 31 (16%) perform exclusively pediatric heart operations. Of 186 respondents, 54 (29%) perform fewer than 100 major congenital heart operations per year, 78 (42%) perform 100 to 199 procedures, and 54 (29%) perform 200 procedures or more. Active congenital heart surgeons have been in their current positions for 9.3±8.6 years. Eight respondents are in their first year of practice. For 203 respondents, mean anticipated years to retirement is 16.1±7.6. Twenty-eight anticipate retirement within 5 years; 31 in 6 to 10 years. These data should help facilitate rational plans to meet workforce needs for an expanding patient population. Copyright © 2011. Published by Elsevier Inc. All rights reserved.

Risks and Benefits of Exercise Training in Adults With Congenital Heart Disease.

PubMed

Chaix, Marie-A; Marcotte, François; Dore, Annie; Mongeon, François-Pierre; Mondésert, Blandine; Mercier, Lise-Andrée; Khairy, Paul

2016-04-01

Exercise capacity in adults with various forms of congenital heart disease is substantially lower than that of the general population. Although the underlying congenital heart defect, and its sequelae, certainly contribute to observed exercise limitations, there is evidence suggesting that deconditioning and a sedentary lifestyle are important implicated factors. The prevalence of acquired cardiovascular comorbidities is on the increase in the aging population with congenital heart disease, such that obesity and a sedentary lifestyle confer increased risk. Health fears and misconceptions are common barriers to regular physical activity in adults with congenital heart disease, despite evidence linking lower functional capacity to poor outcomes, and data supporting the safety and efficacy of exercise in bestowing numerous physical and psychosocial rewards. With few exceptions, adults with congenital heart disease should be counselled to exercise regularly. In this contemporary review, we provide a practical approach to assessing adults with congenital heart disease before exercise training. We examine available evidence supporting the safety and benefits of exercise training. Risks associated with exercise training in adults with congenital heart disease are discussed, particularly with regard to sudden cardiac death. Finally, recommendations for exercise training are provided, with consideration for the type of congenital heart disease, the nature (ie, static vs dynamic) and intensity (ie, low, medium, high) of the physical activity, and associated factors such as systemic ventricular dysfunction and residual defects. Further research is required to determine optimal exercise regimens and to identify effective strategies to implement exercise training as a key determinant of healthy living. Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

Mothers and Fathers Experience Stress of Congenital Heart Disease Differently: Recommendations for Pediatric Critical Care.

PubMed

Sood, Erica; Karpyn, Allison; Demianczyk, Abigail C; Ryan, Jennie; Delaplane, Emily A; Neely, Trent; Frazier, Aisha H; Kazak, Anne E

2018-03-10

To inform pediatric critical care practice by examining how mothers and fathers experience the stress of caring for a young child with congenital heart disease and use hospital and community supports. Qualitative study of mothers and fathers of young children with congenital heart disease. Tertiary care pediatric hospital in the Mid-Atlantic region of the United States. Thirty-four parents (20 mothers, 14 fathers) from diverse backgrounds whose child previously underwent cardiac surgery during infancy. Subjects participated in semi-structured, individual interviews about their experiences and psychosocial needs at the time of congenital heart disease diagnosis, surgical admission, and discharge to home after surgery. Qualitative interview data were coded, and consistent themes related to emotional states, stressors, and supports were identified. Fathers experience and respond to the stressors and demands of congenital heart disease in unique ways. Fathers often described stress from not being able to protect their child from congenital heart disease and the associated surgeries/pain and from difficulties balancing employment with support for their partner and care of their congenital heart disease child in the hospital. Fathers were more likely than mothers to discuss support from the work environment (coworkers/managers, flexible scheduling, helpful distraction) and were less likely to describe the use of hospital-based resources or congenital heart disease peer-to-peer supports. This study highlights the importance of understanding the paternal experience and tailoring interventions to the unique needs of both mothers and fathers. Opportunities for critical care practice change to promote the mental health of mothers and fathers following a diagnosis of congenital heart disease are discussed.

Genetic testing in congenital heart disease: ethical considerations.

PubMed

Lin, Kimberly Y; D'Alessandro, Lisa C A; Goldmuntz, Elizabeth

2013-01-01

On March 16, 2012, the Ethics of the Heart 2012: Ethical and Policy Challenges in Pediatric and Adult Congenital Heart Disease Conference took place in Philadelphia, Pennsylvania. The first session focused on the ethics surrounding genetic testing in patients with congenital heart disease. Summarized here is the introductory presentation given by Dr Elizabeth Goldmuntz entitled "The Role of Genetic Testing in Congenital Heart Disease," followed by a case presentation given by Dr Lisa D'Alessandro. The case and the panel discussion that ensued highlight several ethical principles and challenges in this unique patient population.

Incremenal Value of Cardiac Magnetic Resonance for Assessing Pulmonic Valve Regurgitation.

PubMed

Zdradzinski, Michael; Elkin, Rachel; Flamm, Scott; Krasuski, Richard

2015-07-01

Cardiac magnetic resonance (CMR) is the 'gold standard' for quantifying pulmonic regurgitation (PR) in adults with congenital heart disease, but remains costly and is less readily available than echocardiography. Qualitative echocardiographic assessment of PR is challenging, and guiding criteria are limited. It is unknown if echocardiography is sufficient to screen for significant PR. The study aim was to determine whether cardiac MRI provides additional benefit in the assessment of PR in adults with congenital heart disease. Patients with repaired tetralogy of Fallot or congenital pulmonic stenosis after valvotomy undergoing transthoracic echocardiography and CMR with no interval intervention were identified from a prospective registry. Patients with greater than mild pulmonic stenosis, residual ventricular septal defect or poor echocardiographic windows were excluded. Whole-cohort and subgroup (tetralogy of Fallot versus pulmonic stenosis) analyses for inter-modality agreement were performed. A total of 48 patients (24 men, 24 women; mean age 43 +/- 12 years) was included in the analysis. The unweighted kappa value for the two modalities was 0.30, suggesting 'fair' agreement, though only 52% had matching PR assessments. The indexed right ventricular end-systolic volume (RVESVi) correlated closely with cardiac MRI-monitored PR (p = 0.011 by analysis of variance), but not with that monitored with echocardiography (p = 0.081). Subgroup analysis demonstrated less inter-modality agreement in the tetralogy of Fallot population (kappa 0.25) than in the pulmonic stenosis population (kappa 0.35). CMR measurement of PR correlates closely with the RVESVi, and appears superior to echocardiography when assessing patients at risk for PR. The study results suggest a vital role for CMR whenever significant PR is suspected in the adult congenital heart disease population.

[Surgery of grown up congenital heart disease. About 540 cases].

PubMed

Haddad, A; Bourezak, R; Aouiche, M; Ait Mohand, R; Hamzaoui, A; Bourezak, S E

2015-09-01

With advances in recent decades in the field of congenital heart disease both for imaging in medical therapy, a large number of heart disease is diagnosed before birth. Many of them benefit from surgery and reach adulthood, they do not require further action. Some of them develop later in their lives other problems requiring reoperation in adulthood. This sparked the birth of a subspecialty within the department of congenital heart disease: GUCH Unit "grown up congenital heart disease". In developing countries, little heart are detected in childhood, a minority of them are operated and very few reach adulthood or with minor heart disease or become advanced enough then inoperable. Only part may still take advantage of surgery at this age. The aim of our study is to describe the spectrum and characteristics of congenital heart disease in adulthood in Algiers a center of cardiovascular surgery. A retrospective descriptive study of patients aged 15 and above operated for congenital heart defects between 1995 and 2011. Five hundred and forty patients aged 15 to 76years (29±10 years), including 314 women and 226 men are operated congenital heart defects between 1995 and 2011. The left-right shunts represent two thirds of heart disease, represented mainly (50%) by the atrial septal defect. Barriers to the ejection of the left heart represent one forth of cases with a predominance of subvalvular aortic stenosis. We find the native heart whose survival is considered exceptional in adulthood in the absence of surgery, such as tetralogy of Fallot, aortopulmonary windows wide, double outlet right ventricle and atrioventricular canal that take advantage of always surgery. The results are encouraging with low perioperative mortality (2%). The approach of congenital heart disease in developing countries is different from that of developed countries. Efforts need to be made in early detection and monitoring of congenital heart disease and improve access to surgery centers in close collaboration with pediatricians, cardiologists and obstetricians. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

The Brain in Congenital Heart Disease across the Lifespan: The Cumulative Burden of Injury

PubMed Central

Marelli, Ariane; Miller, Steven P.; Marino, Bradley Scott; Jefferson, Angela L.; Newburger, Jane W.

2017-01-01

The number of patients surviving with congenital heart disease (CHD) has soared over the last three decades. Adults constitute the fastest growing segment of the CHD population, now outnumbering children. Research to date on the heart-brain intersection in this population has largely been focused on neurodevelopmental outcomes in childhood and adolescence. Mutations in genes that are highly expressed in heart and brain may cause cerebral dysgenesis. Together with altered cerebral perfusion in utero, these factors are associated with abnormalities of brain structure and brain immaturity in a significant portion of neonates with critical CHD even before they undergo cardiac surgery. In infancy and childhood, the brain may be affected by risk factors related to heart disease itself or to its interventional treatments. As children with CHD become adults, they increasingly develop heart failure, atrial fibrillation, hypertension, diabetes and coronary disease. These acquired cardiovascular comorbidities can be expected to have effects similar to those in the general population on cerebral blood flow, brain volumes, and dementia. In both children and adults, cardiovascular disease may have adverse effects on achievement, executive function, memory, language, social interactions, and quality of life. In summary, against the backdrop of shifting demographics, risk factors for brain injury in the CHD population are cumulative and synergistic. As neurodevelopmental sequelae in children with CHD evolve to cognitive decline or dementia during adulthood, a growing population of CHD can be expected to require support services. We highlight evidence gaps and future research directions. PMID:27185022

Maternal obesity and congenital heart defects: a population-based study123

PubMed Central

Mills, James L; Troendle, James; Conley, Mary R; Carter, Tonia; Druschel, Charlotte M

2010-01-01

Background: Obesity affects almost one-third of pregnant women and causes many complications, including neural tube defects. It is not clear whether the risk of congenital heart defects, the most common malformations, is also increased. Objective: This study was conducted to determine whether obesity is associated with an increased risk of congenital heart defects. Design: A population-based, nested, case-control study was conducted in infants born with congenital heart defects and unaffected controls from the cohort of all births (n = 1,536,828) between 1993 and 2003 in New York State, excluding New York City. The type of congenital heart defect, maternal body mass index (BMI; in kg/m2), and other risk factors were obtained from the Congenital Malformations Registry and vital records. Mothers of 7392 congenital heart defect cases and 56,304 unaffected controls were studied. Results: All obese women (BMI ≥ 30) were significantly more likely than normal-weight women (BMI: 19–24.9) to have children with a congenital heart defect [odds ratio (OR): 1.15; 95% CI: 1.07, 1.23; P < 0.0001]. Overweight women were not at increased risk (OR: 1.00; 95% CI: 0.94, 1.06). The risk in morbidly obese women (BMI ≥ 40) was higher (OR: 1.33; 95% CI: 1.15, 1.54; P = 0.0001) than that in obese women with a BMI of 30–39.9 (OR: 1.11; 95% CI: 1.04, 1.20; P = 0.004). There was a highly significant trend of increasing OR for congenital heart defects with increasing maternal obesity (P < 0.0001). The offspring of obese women had significantly higher ORs for atrial septal defects, hypoplastic left heart syndrome, aortic stenosis, pulmonic stenosis, and tetralogy of Fallot. Conclusions: Obese, but not overweight, women are at significantly increased risk of bearing children with a range of congenital heart defects, and the risk increases with increasing BMI. Weight reduction as a way to reduce risk should be investigated. PMID:20375192

Understand Your Risk for Heart Failure

MedlinePlus

... Heart.org Arrhythmia About Arrhythmia Why Arrhythmia Matters Understand Your Risk for Arrhythmia Symptoms, Diagnosis & Monitoring of ... Heart Defects The Impact of Congenital Heart Defects Understand Your Risk for Congenital Heart Defects Symptoms & Diagnosis ...

Certification in echocardiography of congenital heart disease: experience of the first 6 years of a European process.

PubMed

Mertens, Luc; Miller, Owen; Fox, Kevin; Simpson, John

2013-02-01

Certification in congenital heart disease echocardiography presents unique challenges compared with certification of adult transthoracic and transoesophageal echocardiography. We report our experience in collaboratively developing an exam process that covers the size and age range of congenital heart patients, the varying professional backgrounds of echocardiography practitioners across the field and our approach to the challenge of introducing a pan-European certification endorsed by the major stakeholder groups; the European Association of Echocardiography (EAE), the Association for European Paediatric and Congenital Cardiology (AEPC) and the Grown Up Congenital Heart Working Group of the European Society of Cardiology (ESC). Since its inception in 2006 the exam has been held seven times; 137 candidates from 27 countries have sat the exam, 107 candidates (78%) have passed the exam components and 60 candidates have successfully completed the logbook submission and have been certified in echocardiography of congenital heart disease echocardiography by the EAE. In addition to the certification process, a comprehensive curriculum, teaching programme, and teaching courses have been developed. The instititution of a European certification process for echocardiography of congenital heart disease has proved feasible.

Employment after heart transplantation among adults with congenital heart disease.

PubMed

Tumin, Dmitry; Chou, Helen; Hayes, Don; Tobias, Joseph D; Galantowicz, Mark; McConnell, Patrick I

2017-12-01

Adults with congenital heart disease may require heart transplantation for end-stage heart failure. Whereas heart transplantation potentially allows adults with congenital heart disease to resume their usual activities, employment outcomes in this population are unknown. Therefore, we investigated the prevalence and predictors of work participation after heart transplantation for congenital heart disease. Retrospective review of a prospective registry. United Network for Organ Sharing registry of transplant recipients in the United States. Adult recipients of first-time heart transplantation with a primary diagnosis of congenital heart disease, performed between 2004 and 2015. None. Employment status reported by transplant centers at required follow-up intervals up to 5 y posttransplant. Among 470 patients included in the analysis (mean follow-up: 5 ± 3 y), 127 (27%) worked after transplant, 69 (15%) died before beginning or returning to work, and 274 (58%) survived until censoring, but did not participate in paid work. Multivariable competing-risks regression analysis examined characteristics associated with posttransplant employment, accounting for mortality as a competing outcome. In descriptive and multivariable analysis, pretransplant work participation was associated with a greater likelihood of posttransplant employment, while the use of Medicaid insurance at the time of transplant was associated with a significantly lower likelihood of working after transplant (subhazard ratio compared to private insurance: 0.55; 95% confidence interval: 0.32, 0.95; P = .032). Employment was rare after heart transplantation for congenital heart disease, and was significantly less common than in the broader population of adults with congenital heart disease. Differences in return to work were primarily related to pretransplant employment and the use of public insurance, rather than clinical characteristics. © 2017 Wiley Periodicals, Inc.

Theory of mind deficit in adult patients with congenital heart disease.

PubMed

Chiavarino, Claudia; Bianchino, Claudia; Brach-Prever, Silvia; Riggi, Chiara; Palumbo, Luigi; Bara, Bruno G; Bosco, Francesca M

2015-10-01

This article provides the first assessment of theory of mind, that is, the ability to reason about mental states, in adult patients with congenital heart disease. Patients with congenital heart disease and matched healthy controls were administered classical theory of mind tasks and a semi-structured interview which provides a multidimensional evaluation of theory of mind (Theory of Mind Assessment Scale). The patients with congenital heart disease performed worse than the controls on the Theory of Mind Assessment Scale, whereas they did as well as the control group on the classical theory-of-mind tasks. These findings provide the first evidence that adults with congenital heart disease may display specific impairments in theory of mind. © The Author(s) 2013.

Origins and consequences of congenital heart defects affecting the right ventricle.

PubMed

Woudstra, Odilia I; Ahuja, Suchit; Bokma, Jouke P; Bouma, Berto J; Mulder, Barbara J M; Christoffels, Vincent M

2017-10-01

Congenital heart disease is a major health issue, accounting for a third of all congenital defects. Improved early surgical management has led to a growing population of adults with congenital heart disease, including patients with defects affecting the right ventricle, which are often classified as severe. Defects affecting the right ventricle often cause right ventricular volume or pressure overload and affected patients are at high risk for complications such as heart failure and sudden death. Recent insights into the developmental mechanisms and distinct developmental origins of the left ventricle, right ventricle, and the outflow tract have shed light on the common features and distinct problems arising in specific defects. Here, we provide a comprehensive overview of the current knowledge on the development into the normal and congenitally malformed right heart and the clinical consequences of several congenital heart defects affecting the right ventricle. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

Imaging techniques for visualizing and phenotyping congenital heart defects in murine models.

PubMed

Liu, Xiaoqin; Tobita, Kimimasa; Francis, Richard J B; Lo, Cecilia W

2013-06-01

Mouse model is ideal for investigating the genetic and developmental etiology of congenital heart disease. However, cardiovascular phenotyping for the precise diagnosis of structural heart defects in mice remain challenging. With rapid advances in imaging techniques, there are now high throughput phenotyping tools available for the diagnosis of structural heart defects. In this review, we discuss the efficacy of four different imaging modalities for congenital heart disease diagnosis in fetal/neonatal mice, including noninvasive fetal echocardiography, micro-computed tomography (micro-CT), micro-magnetic resonance imaging (micro-MRI), and episcopic fluorescence image capture (EFIC) histopathology. The experience we have gained in the use of these imaging modalities in a large-scale mouse mutagenesis screen have validated their efficacy for congenital heart defect diagnosis in the tiny hearts of fetal and newborn mice. These cutting edge phenotyping tools will be invaluable for furthering our understanding of the developmental etiology of congenital heart disease. Copyright © 2013 Wiley Periodicals, Inc.

The Role of Beta-Blocker in Heart Failure in Adults with Congenital Heart Disease.

PubMed

Norozi, Kambiz

2014-01-01

Thanks to the enormous progress in the field of cardiac surgery and paediatric cardiology since the mid of 20th century, more and more children with congenital heart defects reach the adulthood. This on the other hand encounter physician and patients various problems due to late complications after the heart surgery like congestive heart failure, arrhythmia and sudden death. One of the challenging area is the medical management of heart failure in these patients with complex anatomy and hemodynamics. The lack of evidence of the effectiveness of the anti congestive medications in this population in from of large randomized controlled trials, makes it difficult to establish universally accepted therapy guidelines. In this article we will review the evidence of the beta-blockers in heart failure in patients with congenital heart disease. Also we will discuss the mechanisms of heart failure in this patient's cohort and will review the literature with respect to the use of neurohormonal antagonists in congenital heart disease. There is an urgent need to initiate well-designed clinical trials to prove if the positive results of neurohormonal blockade in acquired heart failure in adults can be translated in patients with congenital heart disease.

Extra-cardiac manifestations of adult congenital heart disease.

PubMed

Gaeta, Stephen A; Ward, Cary; Krasuski, Richard A

2016-10-01

Advancement in correction or palliation of congenital cardiac lesions has greatly improved the lifespan of congenital heart disease patients, resulting in a rapidly growing adult congenital heart disease (ACHD) population. As this group has increased in number and age, emerging science has highlighted the systemic nature of ACHD. Providers caring for these patients are tasked with long-term management of multiple neurologic, pulmonary, hepatic, renal, and endocrine manifestations that arise as syndromic associations with congenital heart defects or as sequelae of primary structural or hemodynamic abnormalities. In this review, we outline the current understanding and recent research into these extra-cardiac manifestations. Copyright © 2016 Elsevier Inc. All rights reserved.

Postnatal Cardiac Autonomic Nervous Control in Pediatric Congenital Heart Disease

PubMed Central

Nederend, Ineke; Jongbloed, Monique R. M.; de Geus, Eco J. C.; Blom, Nico A.; ten Harkel, Arend D. J.

2016-01-01

Congenital heart disease is the most common congenital defect. During childhood, survival is generally good but, in adulthood, late complications are not uncommon. Abnormal autonomic control in children with congenital heart disease may contribute considerably to the pathophysiology of these long term sequelae. This narrative review of 34 studies aims to summarize current knowledge on function of the autonomic nervous system in children with a congenital heart defect. Large scale studies that measure both branches of the nervous system for prolonged periods of time in well-defined patient cohorts in various phases of childhood and adolescence are currently lacking. Pending such studies, there is not yet a good grasp on the extent and direction of sympathetic and parasympathetic autonomic function in pediatric congenital heart disease. Longitudinal studies in homogenous patient groups linking autonomic nervous system function and clinical outcome are warranted. PMID:29367565

Medical therapy in adults with congenital heart disease.

PubMed

Book, Wendy M; Shaddy, Robert E

2014-01-01

Heart failure is a common late complication in adults with congenital heart defects, both repaired and unrepaired. The onset of clinical heart failure is associated with increased morbidity and mortality. Some patients with congenital heart disease may benefit from medications shown to improve survival in the population with acquired heart failure, but these same therapies may be of no benefit to other patients. Further studies are needed to better guide the choice of medical therapies. Copyright © 2014 Elsevier Inc. All rights reserved.

[Sex differences in congenital heart disease].

PubMed

Aubry, P; Demian, H

2016-12-01

Gender influences the clinical presentation and the management of some acquired cardiovascular diseases, such as coronary artery disease, resulting in different outcomes. Differences between women and men are also noticed in congenital heart disease. They are mainly related to the prevalence and severity of some congenital heart defects at birth, and in adulthood to the prognosis, incidence of Eisenmenger syndrome and risks of pregnancy. The role of gender on the risk of operative mortality of congenital heart surgery remains debated. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Parental age and birth order in Chinese children with congenital heart disease.

PubMed Central

Tay, J S; Yip, W C; Joseph, R

1982-01-01

Parental age and birth order were studied in 100 Chinese children with congenital heart disease (proven by cardiac catheterisation) and in 100 controls. A higher incidence of congenital heart disease was present in the children with higher birth orders. No relationship was found between the incidence and the paternal or maternal ages. Using the method of multiple regression analysis this birth order effect was significant (p less than 0.01) and independent of parental age. This finding provides indirect evidence of environmental influence in the causation of congenital heart disease, which is known to be inherited in a multifactorial manner. Family planning to limit the size of the family may possibly contribute to the reduction of the incidence of congenital heart disease. PMID:7154041

Dermatoglyphs in congenital heart disease.

PubMed Central

David, T J

1981-01-01

The palmar dermatoglyphs of 800 patients with anatomically proven congenital heart disease were compared with prints from 1000 controls. A review of the previous studies revealed major technical deficiencies, and the present study failed to confirm most of the previously reported positive findings. An overall increase in the incidence of hypothenar patterns was found, probably explaining the previous suggestion of increased atd angle in congenital heart disease. A large number of statistical comparisons inevitably produced a few 'significant' results, most of which were inconsistent in various ways. Two percent of cases were found to have rare epidermal ridge malformation, ridge dissociation. The nature of the relationship between this and congenital heart disease is obscure. Claims that there are diagnostically useful dermatoglyphic changes in congenital heart disease can be disregarded. PMID:7328614

Improving the quality of transition and transfer of care in young adults with congenital heart disease.

PubMed

Everitt, Ian K; Gerardin, Jennifer F; Rodriguez, Fred H; Book, Wendy M

2017-05-01

The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been shown to decrease mortality in the adult population. This paper reviews the transition and transfer of care processes and outlines current congenital heart disease specific guidelines in the United States and compares these recommendations to Canadian and European guidelines. It then reviews perceived and real barriers to successful transition and identifies predictors of success during transfer to adult congenital heart disease care. Lastly, it explores how disease-specific markers of outcomes and quality indicators are being utilized to guide transition and transfer of care in other chronic childhood illnesses, and identifies existing knowledge gaps and structural impediments to improving the management of transition and transfer among congenital heart disease patients. © 2017 Wiley Periodicals, Inc.

Congenital heart disease and chromossomopathies detected by the karyotype

PubMed Central

Trevisan, Patrícia; Rosa, Rafael Fabiano M.; Koshiyama, Dayane Bohn; Zen, Tatiana Diehl; Paskulin, Giorgio Adriano; Zen, Paulo Ricardo G.

2014-01-01

OBJECTIVE: To review the relationship between congenital heart defects and chromosomal abnormalities detected by the karyotype. DATA SOURCES: Scientific articles were searched in MEDLINE database, using the descriptors "karyotype" OR "chromosomal" OR "chromosome" AND "heart defects, congenital". The research was limited to articles published in English from 1980 on. DATA SYNTHESIS: Congenital heart disease is characterized by an etiologically heterogeneous and not well understood group of lesions. Several researchers have evaluated the presence of chromosomal abnormalities detected by the karyotype in patients with congenital heart disease. However, most of the articles were retrospective studies developed in Europe and only some of the studied patients had a karyotype exam. In this review, only one study was conducted in Latin America, in Brazil. It is known that chromosomal abnormalities are frequent, being present in about one in every ten patients with congenital heart disease. Among the karyotype alterations in these patients, the most important is the trisomy 21 (Down syndrome). These patients often have associated extra-cardiac malformations, with a higher risk of morbidity and mortality, which makes heart surgery even more risky. CONCLUSIONS: Despite all the progress made in recent decades in the field of cytogenetic, the karyotype remains an essential tool in order to evaluate patients with congenital heart disease. The detailed dysmorphological physical examination is of great importance to indicate the need of a karyotype. PMID:25119760

Near-infrared spectroscopy as a predictor of clinical deterioration: a case report of two infants with duct-dependent congenital heart disease.

PubMed

Mebius, Mirthe J; du Marchie Sarvaas, Gideon J; Wolthuis, Diana W; Bartelds, Beatrijs; Kneyber, Martin C J; Bos, Arend F; Kooi, Elisabeth M W

2017-03-16

Some infants with congenital heart disease are at risk of in-hospital cardiac arrest. To better foresee cardiac arrest in infants with congenital heart disease, it might be useful to continuously assess end-organ perfusion. Near-infrared spectroscopy is a non-invasive method to continuously assess multisite regional tissue oxygen saturation. We report on two infants with duct-dependent congenital heart disease who demonstrated a gradual change in cerebral and/or renal tissue oxygen saturation before cardiopulmonary resuscitation was required. In both cases, other clinical parameters such as heart rate, arterial oxygen saturation and blood pressure did not indicate that deterioration was imminent. These two cases demonstrate that near-infrared spectroscopy might contribute to detecting a deteriorating clinical condition and might therefore be helpful in averting cardiopulmonary collapse and need for resuscitation in infants with congenital heart disease.

Preparing Children for Heart Surgery

MedlinePlus

... Physical Activity Recommendations for Heart Health • Tools & Resources Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

Correlates of posttraumatic stress disorder in adults with congenital heart disease.

PubMed

Eslami, Bahareh

2017-05-01

The aims of this study were to compare the level of posttraumatic stress disorder between adults with and without congenital heart disease, and to examine the correlates of posttraumatic stress disorder (e.g., sociodemographics). Cross-sectional. Two university-affiliated heart hospitals in Tehran, Iran. A sample of 347 adults with congenital heart disease aged 18-64 years (52% women), and 353 adults without congenital heart disease matched by sex and age (±2 years) was recruited. The PTSD Scale: Self-report version was used to assess the diagnosis and severity of posttraumatic stress disorder. Hierarchical multivariate logistic regression analyses were performed to explore correlates of likely posttraumatic stress disorder diagnosis among each group of participants. The posttraumatic stress disorder in the patients was comparable to those of the control group, except for increased arousal (P = .027) which was scored higher among the patients. Over 52% of adults with congenital heart disease met the criteria for a likely posttraumatic stress disorder diagnosis compared with 48% of adults without congenital heart disease. The regression analyses among patients revealed that elevated depressive symptoms (OR = 1.27) and a positive history of cardiac surgery (OR = 2.02) were significantly associated with posttraumatic stress disorder. The model could explain 29% of the variance in posttraumatic stress disorder. The high and comparable prevalence of posttraumatic stress disorder among patients and nonpatients highlight the significance of the context in which adults with congenital heart disease may face other/additional stressors than disease-related ones, an issue that clinicians need also take into account. Furthermore, the association of posttraumatic stress disorder with elevated depressive symptoms warrant a comprehensive psychological assessment and management of adults with congenital heart disease, in particular among those with a history of invasive procedures. © 2017 Wiley Periodicals, Inc.

A positive perspective of knowledge, attitude, and practices for health-promoting behaviors of adolescents with congenital heart disease.

PubMed

Huang, Hui-Ru; Chen, Chi-Wen; Chen, Chin-Mi; Yang, Hsiao-Ling; Su, Wen-Jen; Wang, Jou-Kou; Tsai, Pei-Kwei

2018-03-01

Health-promoting behaviors could serve as a major strategy to optimize long-term outcomes for adolescents with congenital heart disease. The associations assessed from a positive perspective of knowledge, attitudes, and practice model would potentially cultivate health-promoting behaviors during adolescence. The purpose of this study was to examine the relationships between disease knowledge, resilience, family functioning, and health-promoting behaviors in adolescents with congenital heart disease. A total of 320 adolescents with congenital heart disease who were aged 12-18 years were recruited from pediatric cardiology outpatient departments, and participated in a cross-sectional survey. The participants completed the Leuven Knowledge Questionnaire for Congenital Heart Disease; Haase Adolescent Resilience in Illness Scale; Family Adaptability, Partnership, Growth, Affection, and Resolve; and Adolescent Health Promotion scales. The collected data were analyzed using descriptive statistics and three multiple regression models. Greater knowledge of prevention of complications and higher resilience had a more powerful effect in enhancing health-promoting behaviors. Having symptoms and moderate or severe family dysfunction were significantly more negatively predictive of health-promoting behaviors than not having symptoms and positive family function. The third model explained 40% of the variance in engaging in health-promoting behaviors among adolescents with congenital heart disease. The findings of this study provide new insights into the role of disease knowledge, resilience, and family functioning in the health-promoting behavior of adolescents with congenital heart disease. Continued efforts are required to plan family care programs that promote the acquisition of sufficient disease knowledge and the development of resilience for adolescents with congenital heart disease.

[Is psychoprophylaxis a valid approach for heart surgery in children?].

PubMed

Pereira Ruschel, P; Pierini Cidade, D; Daudt, N S; Rossi Filho, R I

1995-10-01

To validate the hypothesys that a psycological preparation for children who will undergo cardiac surgery may improve the outcome. Sixty patients, with ages ranging between 3 and 10 years, submitted to heart surgery for treatment of congenital heart defects, were evaluated. They were divided in 2 groups: experimental and control. A questionnaire was designed for collecting data about psychological and clinical aspects of each patient. The following data was found to be of statistical significance: acceptance of peripheral vein puncture in the surgical group (chi 2 = 11.59, p < 0.05), calm awakening following general anesthesia (chi 2 = 9.64 p < 0.05), cooperation with the physiotherapy staff (chi 2 = 13.30, p < 0.05), coping with parents absence (chi 2 = 9.64, p < 0.05), acceptance of fluid restriction (chi 2 = 17.78, p < 0.05) and cooperation with removal of stitches and pacemaker electrodes (chi 2 = 19.20, p < 0.05). There was not statistical significance on demand of sedation, cooperation at removal of the orotracheal tube and during examination, necessity of reintubation and occurrence of clinical complications. However, the prepared group showed a slight tendency to have less postoperative complications (20%) than the control (27%). It was found that children who had adequated psychologic preparation prior to the correction of congenital heart defects had better psychological results with the imposed trauma.

Tricuspid atresia

MedlinePlus

Tri atresia; Valve disorder - tricuspid atresia; Congenital heart - tricuspid atresia; Cyanotic heart disease - tricuspid atresia ... Tricuspid atresia is an uncommon form of congenital heart disease. It affects about 5 in every 100, ...

THE EFFECT OF PRE-OPERATIVE NUTRITIONAL STATUS ON POST-OPERATIVE OUTCOMES IN CHILDREN UNDERGOING SURGERY FOR CONGENITAL HEART DEFECTS IN SAN FRANCISCO (UCSF) AND GUATEMALA CITY (UNICAR)

PubMed Central

Radman, Monique; Mack, Ricardo; Barnoya, Joaquin; Castañeda, Aldo; Rosales, Monica; Azakie, Anthony; Mehta, Nilesh; Keller, Roberta; Datar, Sanjeev; Oishi, Peter; Fineman, Jeffrey

2013-01-01

Objective To determine the association between preoperative nutritional status and postoperative outcomes in children undergoing surgery for congenital heart defects (CHD). Methods Seventy-one patients with CHD were enrolled in a prospective, two-center cohort study. We adjusted for baseline risk differences using a standardized risk adjustment score for surgery for CHD. We assigned a World Health Organization Z-score for each subjects’ preoperative triceps skinfold measurement, an assessment of total body fat mass. We obtained preoperative plasma concentrations of markers of nutritional status (prealbumin, albumin) and myocardial stress (B-type natriuretic peptide, BNP). Associations between indices of preoperative nutritional status and clinical outcomes were sought. Results Subjects had a median (IQR) age of 10.2 (33) months. In the UCSF cohort, duration of mechanical ventilation (median 19 hours, IQR 29), length of ICU stay (median 5 days, IQR 5), duration of any continuous inotropic infusion (median 66 hours, IQR 72) and preoperative BNP levels (median 30 pg/mL, IQR 75) were associated with a lower preoperative triceps skinfold Z-score (p<0.05). Longer duration of any continuous inotropic infusion and higher preoperative BNP levels were also associated with lower preoperative prealbumin (12.1 ± 0.5 mg/dL) and albumin (3.2 ± 0.1) (p<0.05). Conclusions Lower total body fat mass and acute and chronic malnourishment are associated with worse clinical outcomes in children undergoing surgery for CHD at UCSF, a resource-abundant institution. There is an inverse correlation between total body fat mass and BNP levels. Duration of inotropic support and BNP increase concomitantly as measures of nutritional status decrease, supporting the hypothesis that malnourishment is associated with decreased myocardial function. PMID:23583172

"Just-In-Time" Simulation Training Using 3-D Printed Cardiac Models After Congenital Cardiac Surgery.

PubMed

Olivieri, Laura J; Su, Lillian; Hynes, Conor F; Krieger, Axel; Alfares, Fahad A; Ramakrishnan, Karthik; Zurakowski, David; Marshall, M Blair; Kim, Peter C W; Jonas, Richard A; Nath, Dilip S

2016-03-01

High-fidelity simulation using patient-specific three-dimensional (3D) models may be effective in facilitating pediatric cardiac intensive care unit (PCICU) provider training for clinical management of congenital cardiac surgery patients. The 3D-printed heart models were rendered from preoperative cross-sectional cardiac imaging for 10 patients undergoing congenital cardiac surgery. Immediately following surgical repair, a congenital cardiac surgeon and an intensive care physician conducted a simulation training session regarding postoperative care utilizing the patient-specific 3D model for the PCICU team. After the simulation, Likert-type 0 to 10 scale questionnaire assessed participant perception of impact of the training session. Seventy clinicians participated in training sessions, including 22 physicians, 38 nurses, and 10 ancillary care providers. Average response to whether 3D models were more helpful than standard hand off was 8.4 of 10. Questions regarding enhancement of understanding and clinical ability received average responses of 9.0 or greater, and 90% of participants scored 8 of 10 or higher. Nurses scored significantly higher than other clinicians on self-reported familiarity with the surgery (7.1 vs. 5.8; P = .04), clinical management ability (8.6 vs. 7.7; P = .02), and ability enhancement (9.5 vs. 8.7; P = .02). Compared to physicians, nurses and ancillary providers were more likely to consider 3D models more helpful than standard hand off (8.7 vs. 7.7; P = .05). Higher case complexity predicted greater enhancement of understanding of surgery (P = .04). The 3D heart models can be used to enhance congenital cardiac critical care via simulation training of multidisciplinary intensive care teams. Benefit may be dependent on provider type and case complexity. © The Author(s) 2016.

Choosing Between MRI and CT Imaging in the Adult with Congenital Heart Disease.

PubMed

Bonnichsen, Crystal; Ammash, Naser

2016-05-01

Improvements in the outcomes of surgical and catheter-based interventions and medical therapy have led to a growing population of adult patients with congenital heart disease. Adult patients with previously undiagnosed congenital heart disease or those previously palliated or repaired may have challenging echocardiographic examinations. Understanding the distinct anatomic and hemodynamic features of the congenital anomaly and quantifying ventricular function and valvular dysfunction plays an important role in the management of these patients. Rapid advances in imaging technology with magnetic resonance imaging (MRI) and computed tomography angiography (CTA) allow for improved visualization of complex cardiac anatomy in the evaluation of this unique patient population. Although echocardiography remains the most widely used imaging tool to evaluate congenital heart disease, alternative and, at times, complimentary imaging modalities should be considered. When caring for adults with congenital heart disease, it is important to choose the proper imaging study that can answer the clinical question with the highest quality images, lowest risk to the patient, and in a cost-efficient manner.

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Congenital left-sided heart obstruction

PubMed Central

Carr, Michelle; Curtis, Stephanie; Marek, Jan

2018-01-01

Congenital obstruction of the left ventricular outflow tract remains a significant problem and multilevel obstruction can often coexist. Obstruction can take several morphological forms and may involve the subvalvar, valvar or supravalvar portion of the aortic valve complex. Congenital valvar stenosis presenting in the neonatal period represents a spectrum of disorders ranging from the hypoplastic left heart syndrome to almost normal hearts. Treatment options vary dependent on the severity of the left ventricular outflow tract obstruction (LVOTO) and the variable degree of left ventricular hypoplasia as well as the associated lesions such as arch hypoplasia and coarctation. PMID:29681546

Health condition and familial factors associated with health-related quality of life in adolescents with congenital heart disease: a cross sectional study.

PubMed

Im, Yu-Mi; Yun, Tae-Jin; Lee, Sunhee

2018-01-10

The focus of clinical care after the repair of congenital heart disease has shifted from saving life of the patient to the patient's quality of life. The purpose of this study was to examine the health condition and familial factors associated with the health related quality of life of adolescents with congenital heart disease. Ninety-eight adolescents aged 13-19 years were collected from a congenital heart clinic from July 22 to August 23, 2013. Perceptions of parental rearing behaviors, health related quality of life of adolescent with congenital heart disease, and general characteristics were measured. We used multiple linear regression analysis to explore factors that are associated with the health related quality of life of adolescents with congenital heart disease. New York heart association class (Adj R 2  = .186, p = .000), presence of siblings (Adj R 2  = .240, p = .010), and mother's emotional warmth (Adj R 2  = .265, p = .043) were significantly associated with the health related quality of life of adolescents with congenital heart disease. Emotionally warm parental rearing behaviors and the presence of siblings were important familial factors that were positively associated with HRQOL in adolescents with CHD. Therefore, it is important for healthcare providers to develop a greater sensitivity to, and awareness of, the familial influences that may be impacting a subject's HRQOL, as well as the exigencies of the CHD, itself.

The World Database for Pediatric and Congenital Heart Surgery: The Dawn of a New Era of Global Communication and Quality Improvement in Congenital Heart Disease.

PubMed

St Louis, James D; Kurosawa, Hiromi; Jonas, Richard A; Sandoval, Nestor; Cervantes, Jorge; Tchervenkov, Christo I; Jacobs, Jeffery P; Sakamoto, Kisaburo; Stellin, Giovanni; Kirklin, James K

2017-09-01

The World Society for Pediatric and Congenital Heart Surgery was founded with the mission to "promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service." Early on, the Society's members realized that a crucial step in meeting this goal was to establish a global database that would collect vital information, allowing cardiac surgical centers worldwide to benchmark their outcomes and improve the quality of congenital heart disease care. With tireless efforts from all corners of the globe and utilizing the vast experience and invaluable input of multiple international experts, such a platform of global information exchange was created: The World Database for Pediatric and Congenital Heart Disease went live on January 1, 2017. This database has been thoughtfully designed to produce meaningful performance and quality analyses of surgical outcomes extending beyond immediate hospital survival, allowing capture of important morbidities and mortalities for up to 1 year postoperatively. In order to advance the societal mission, this quality improvement program is available free of charge to WSPCHS members. In establishing the World Database, the Society has taken an essential step to further the process of global improvement in care for children with congenital heart disease.

Brain Perfusion Is Increased at Term in the White Matter of Very Preterm Newborns and Newborns with Congenital Heart Disease: Does this Reflect Activated Angiogenesis?

PubMed

Wintermark, Pia; Lechpammer, Mirna; Kosaras, Bela; Jensen, Frances E; Warfield, Simon K

2015-10-01

This study aims to evaluate brain perfusion at term in very preterm newborns and newborns with congenital heart disease before their corrective surgery, and to search for histopathological indicators of whether the brain perfusion abnormalities of these newborns may be related to an activated angiogenesis. Using magnetic resonance imaging and arterial spin labeling, regional cerebral blood flow was measured at a term-equivalent age for three very preterm newborns (born at < 32 weeks), one newborn with congenital heart disease before his corrective surgery and three healthy newborns. In addition, a histopathological analysis was performed on a newborn with congenital heart disease. The very preterm newborns and the newborn with congenital heart disease included in this study all displayed an increased signal in their white matter on T2-weighted imaging. The cerebral blood flow of these newborns was increased in their white matter, compared with the healthy term newborns. The vascular endothelial growth factor was overexpressed in the injured white matter of the newborn with congenital heart disease. Brain perfusion may be increased at term in the white matter, in very preterm newborns, and newborns with congenital heart disease, and it correlates with white matter abnormalities on conventional imaging. Georg Thieme Verlag KG Stuttgart · New York.

Description and initial evaluation of an educational and psychosocial support model for adults with congenitally malformed hearts.

PubMed

Rönning, Helén; Nielsen, Niels Erik; Swahn, Eva; Strömberg, Anna

2011-05-01

Various programmes for adults with congenitally malformed hearts have been developed, but detailed descriptions of content, rationale and goals are often missing. The aim of this study was to describe and make an initial evaluation of a follow-up model for adults with congenitally malformed hearts, focusing on education and psychosocial support by a multidisciplinary team (EPS). The model is described in steps and evaluated with regards to perceptions of knowledge, anxiety and satisfaction. The EPS model included a policlinic visit to the physician/nurse (medical consultation, computer-based and individual education face-to-face as well as psychosocial support) and a 1-month telephone follow-up. Fifty-five adults (mean age 34, 29 women) with the nine most common forms of congenitally malformed hearts participated in the EPS model as well as the 3-months follow-up. Knowledge about congenital heart malformation had increased in 40% of the participants at the 3-months follow-up. This study describes and evaluates a model that combines a multidisciplinary approach and computer-based education for follow-up of adults with congenitally malformed hearts. The EPS model was found to increase self-estimated knowledge, but further evaluations need to be conducted to prove patient-centred outcomes over time. The model is now ready to be implemented in adults with congenitally malformed hearts. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

Vascular ring

MedlinePlus

... with aberrant subclavian and left ligamentum ateriosus; Congenital heart defect - vascular ring; Birth defect heart - vascular ring ... accounts for less than 1% of all congenital heart problems. The condition occurs as often in males ...

Facts about Congenital Heart Defects

MedlinePlus

... Living With Heart Defects Data & Statistics Tracking & Research Articles & Key Findings Free Materials Multimedia and Tools Links to Other Websites Information For… Media Policy Makers Basics about Congenital Heart Defects Language: ...

Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association.

PubMed

Canobbio, Mary M; Warnes, Carole A; Aboulhosn, Jamil; Connolly, Heidi M; Khanna, Amber; Koos, Brian J; Mital, Seema; Rose, Carl; Silversides, Candice; Stout, Karen

2017-02-21

Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management. © 2017 American Heart Association, Inc.

A computer-based matrix for rapid calculation of pulmonary hemodynamic parameters in congenital heart disease

PubMed Central

Lopes, Antonio Augusto; dos Anjos Miranda, Rogério; Gonçalves, Rilvani Cavalcante; Thomaz, Ana Maria

2009-01-01

BACKGROUND: In patients with congenital heart disease undergoing cardiac catheterization for hemodynamic purposes, parameter estimation by the indirect Fick method using a single predicted value of oxygen consumption has been a matter of criticism. OBJECTIVE: We developed a computer-based routine for rapid estimation of replicate hemodynamic parameters using multiple predicted values of oxygen consumption. MATERIALS AND METHODS: Using Microsoft® Excel facilities, we constructed a matrix containing 5 models (equations) for prediction of oxygen consumption, and all additional formulas needed to obtain replicate estimates of hemodynamic parameters. RESULTS: By entering data from 65 patients with ventricular septal defects, aged 1 month to 8 years, it was possible to obtain multiple predictions for oxygen consumption, with clear between-age groups (P <.001) and between-methods (P <.001) differences. Using these predictions in the individual patient, it was possible to obtain the upper and lower limits of a likely range for any given parameter, which made estimation more realistic. CONCLUSION: The organized matrix allows for rapid obtainment of replicate parameter estimates, without error due to exhaustive calculations. PMID:19641642

Predictors of postnatal complications and congenital cardiac diseases in infants of mothers with pregestational and gestational diabetes.

PubMed

Demirpençe, Savaş; Demirpençe, Banu İnce; Meşe, Timur; Arslanoğlu, Sertaç; Tavlı, Vedide; Çalkavur, Şebnem; Olukman, Özgür; Firuzan, Ali Rıza

2014-12-01

In this study, we aimed to evaluate the postnatal problems of infants of mothers with pregestational and gestational diabetes and the clinical properties of infants who were found to have congenital cardiac disease. We retrospectively examined the records of 337 newborns who were followed up with a diagnosis of infant of diabetic mother between January 2010 and January 2012 in our Neonatology Unit. The demographic data of the diabetic mothers and their babies, the postnatal problems of the babies of diabetic mothers and congenital heart diseases found on transthoracic echocardiography were examined. The patients were classified as group A, B and C in accordance with the recommendations of The American Congress of Obstetricians and Gynecologists (ACOG) according to the type of diabetes. The most common postnatal problems included hyperbilirubinemia, respiratory distress, hypoglycemia and hypocalcemia. The rate of congenital heart disease was found be 17.3% in group A, 50% in group B and 9% in group C. No correlation was found between congenital heart disease and gender, multiple pregnancy, diabetes type, diet treatment, use of oral antidiabetic drugs and drug usage. A positive significant correlation was found between congenital heart disease and genetic disease, murmur, cyanosis and presence of gestational hypertension. It was shown that use of insulin, genetic disease and presence of gestational diabetes increased the risk of congenital heart disease. In our study, the overall incidence of congenital heart disease was found to be 24% in infants of diabetic mothers. It should be kept in mind that it is important to investigate the infants of mothers with pregestational and gestational diabetes in terms of the risk of congenital heart disease.

Design and Implementation of a Prospective Adult Congenital Heart Disease Biobank.

PubMed

Opotowsky, Alexander R; Loukas, Brittani; Ellervik, Christina; Moko, Lilamarie E; Singh, Michael N; Landzberg, Elizabeth I; Rimm, Eric B; Landzberg, Michael J

2016-11-01

Adults with congenital heart disease (ACHD) comprise a growing, increasingly complex population. The Boston Adult Congenital Heart Disease Biobank is a program for the collection and storage of biospecimens to provide a sustainable resource for scientific biomarker investigation in ACHD. We describe a protocol to collect, process, and store biospecimens for ACHD or associated diagnoses developed based on existing literature and consultation with cardiovascular biomarker epidemiologists. The protocol involves collecting urine and ∼48.5 mL of blood. A subset of the blood and urine undergoes immediate clinically relevant testing. The remaining biospecimens are processed soon after collection and stored at -80°C as aliquots of ethylenediaminetetraacetic acid (EDTA) and lithium heparin plasma, serum, red cell and buffy coat pellet, and urine supernatant. Including tubes with diverse anticoagulant and clot accelerator contents will enable flexible downstream use. Demographic and clinical data are entered into a database; data on biospecimen collection, processing, and storage are managed by an enterprise laboratory information management system. Since implementation in 2012, we have enrolled more than 650 unique participants (aged 18-80 years, 53.3% women); the Biobank contains over 11,000 biospecimen aliquots. The most common primary CHD diagnoses are single ventricle status-post Fontan procedure (18.8%), repaired tetralogy of Fallot with pulmonary stenosis or atresia (17.6%), and left-sided obstructive lesions (17.5%). We describe the design and implementation of biospecimen collection, handling, and storage protocols with multiple levels of quality assurance. These protocols are feasible and reflect the size and goals of the Boston ACHD Biobank. © The Author(s) 2016.

[Genetics of congenital heart diseases].

PubMed

Bonnet, Damien

2017-06-01

Developmental genetics of congenital heart diseases has evolved from analysis of serial slices in embryos towards molecular genetics of cardiac morphogenesis with a dynamic view of cardiac development. Genetics of congenital heart diseases has also changed from formal genetic analysis of familial recurrences or population-based analysis to screening for mutations in candidates genes identified in animal models. Close cooperation between molecular embryologists, pathologists involved in heart development and pediatric cardiologists is crucial for further increase of knowledge in the field of cardiac morphogenesis and genetics of cardiac defects. The genetic model for congenital heart disease has to be revised to favor a polygenic origin rather than a monogenic one. The main mechanism is altered genic dosage that can account for heart diseases in chromosomal anomalies as well as in point mutations in syndromic and isolated congenital heart diseases. The use of big data grouping information from cardiac development, interactions between genes and proteins, epigenetic factors such as chromatin remodeling or DNA methylation is the current source for improving our knowledge in the field and to give clues for future therapies. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Increasing mortality burden among adults with complex congenital heart disease.

PubMed

Greutmann, Matthias; Tobler, Daniel; Kovacs, Adrienne H; Greutmann-Yantiri, Mehtap; Haile, Sarah R; Held, Leonhard; Ivanov, Joan; Williams, William G; Oechslin, Erwin N; Silversides, Candice K; Colman, Jack M

2015-01-01

Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease. © 2014 The Authors. Congenital Heart Disease Published by Wiley Periodicals, Inc.

Histone methylations in heart development, congenital and adult heart diseases.

PubMed

Zhang, Qing-Jun; Liu, Zhi-Ping

2015-01-01

Heart development comprises myocyte specification, differentiation and cardiac morphogenesis. These processes are regulated by a group of core cardiac transcription factors in a coordinated temporal and spatial manner. Histone methylation is an emerging epigenetic mechanism for regulating gene transcription. Interplay among cardiac transcription factors and histone lysine modifiers plays important role in heart development. Aberrant expression and mutation of the histone lysine modifiers during development and in adult life can cause either embryonic lethality or congenital heart diseases, and influences the response of adult hearts to pathological stresses. In this review, we describe current body of literature on the role of several common histone methylations and their modifying enzymes in heart development, congenital and adult heart diseases.

Congenital Heart Disease: Guidelines of Care for Children with Special Health Care Needs.

ERIC Educational Resources Information Center

Minnesota State Dept. of Health, Minneapolis. Services for Children with Handicaps.

These guidelines were written to help families coordinate the health care that may be needed by a child with congenital heart disease. The booklet begins with general information about congenital heart disease. It then discusses the goals of health care, the health care team, the importance of periodic health care, and record keeping procedures.…

School Children with Congenital Heart Disease: Quality of Life and Policy Implications

ERIC Educational Resources Information Center

Roberts, Jillian; Massie, Kendra; Mortimer, Tamara; Maxwell, Lani

2005-01-01

Congenital heart disease is one of the most common pediatric chronic illnesses that can have an impact on the lives of affected children and their families. Despite the growing number of school-age children who live with congenital heart disease, few researchers have investigated the lives and well-being of these children and their families. The…

Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease

PubMed Central

2013-01-01

Cardiovascular magnetic resonance (CMR) has taken on an increasingly important role in the diagnostic evaluation and pre-procedural planning for patients with congenital heart disease. This article provides guidelines for the performance of CMR in children and adults with congenital heart disease. The first portion addresses preparation for the examination and safety issues, the second describes the primary techniques used in an examination, and the third provides disease-specific protocols. Variations in practice are highlighted and expert consensus recommendations are provided. Indications and appropriate use criteria for CMR examination are not specifically addressed. PMID:23763839

Quality of Life and Congenital Heart Disease in Childhood and Adolescence

PubMed Central

Bertoletti, Juliana; Marx, Giovana Caroline; Hattge, Sérgio Pedro; Pellanda, Lucia Campos

2014-01-01

Advances in cardiac surgery techniques and early diagnosis have enabled the increased survival of individuals with congenital heart disease. The investigation of the quality of life in children and adolescents with congenital heart disease provides complementary information to clinical data that can assist in decision making on the part of health professionals. Although many studies have been conducted to investigate the quality of life of children and adolescents with congenital heart disease, the results prove to be contradictory; while some studies show that congenital heart disease can impact the quality of life, others describe a better perception of quality of life among children and adolescents who suffer from the disease when compared with healthy control subjects. The purpose of this study is to review the literature on the assessment of health related quality of life in children and adolescents with congenital heart disease, in order to systematize the existing knowledge on this topic today. It is observed that research seeks to investigate aspects of personality in cardiac patients, their coping strategies used and perceived social support, aiming at better understanding the association of these variables with the level of quality of life in this population. PMID:24676375

Systemic venous anomalies in the Middle East

PubMed Central

Corno, Antonio F.; Alahdal, Sami A.; Das, Karuna Moy

2013-01-01

Introduction: Systemic venous anomalies are quite rare and can be associated with congenital heart disease requiring surgery. Materials and Methods: All consecutive patients (pts) undergoing surgery for congenital heart defects were retrospectively analyzed for presence of systemic venous anomalies: (a) Persistent left superior vena cava (PLSVC)(b) Inferior vena cava (IVC) interruption(c) Retro-aortic innominate vein Results: From 9/2010 to 5/2012 155 pts, median age 7 months, mean age 1.3 years (3 days–50 years), median weight 4 kg, mean weight 7.2 kg (0.6–110 kg) underwent congenital heart surgery. Twenty-nine systemic venous anomalies were identified in 28/155 patients (=18.1%). PLSVC was present in 21 pts (=13.5%), median age 4 months, mean age 2.7 years (3 days–22 years), median weight 6 kg, mean weight 10.1 kg (2.4–43.0 kg). IVC interruption was identified in 5 pts (=3.2%), median age 2 months, mean age 5.4 years (30 days–26 years), median weight 3.7 kg, median weight 17 kg (2.3–68.0 kg). Retro-aortic innominate vein was diagnosed in 3 pts (=1.9%), median age 5 years, mean age 3.7 years (10 months–5 years), median weight 12 kg, mean weight 10.1 kg (4.5–14 kg). Complete pre-operative diagnosis was obtained in 14/28 (=50%) pts with echocardiography and in other 8/28 (=28.6%) only after computed tomography (CT) scan, for a total of 22/28 (=78.6%) correct pre-operative diagnosis. In 6/28 (=21.4%) patients the diagnosis was intra-operative. Total incidence of systemic venous anomalies was 18.1% (vs. 4% in the literature, P = 0.0009), with presence of PLSVC = 13.5% (vs. 0.3–4.0%, respectively P = 0.0004 and P = 0.0012), IVC interruption = 3.2% (vs. 0.1–1.3%, N.S.), and retro-aortic innominate vein = 1.9% (vs. 0.2–1%, N.S.). Conclusions: Our study showed an incidence of systemic venous anomalies in Middle Eastern pts with congenital heart defects higher than previously reported. In 78.6% of pts the diagnosis was correctly made before surgery (echocardiography or CT scan), with 21.4% of complete diagnosis made at surgery. A careful pre-operative screening should be performed in all pts with congenital heart defects from this region to better identify all systemic venous anomalies for a more accurate surgical planning. PMID:24400249

Congenital Heart Defects (For Parents)

MedlinePlus

... or loses consciousness, call 911 . More treatments than ever are available for congenital heart defects, and most defects are treated successfully. Children with heart problems are best cared for by a team of specialists, which ...

Living with a Congenital Heart Defect

MedlinePlus

... to live as healthy a life as possible. Stories: Living with a Congenital Heart Defect William's Story ... Story Shandler's story Nicholas' Story Ken's Story William's Story William’s Story I was born with a heart ...

Assessment of Diastolic Function in Congenital Heart Disease

PubMed Central

Panesar, Dilveer Kaur; Burch, Michael

2017-01-01

Diastolic function is an important component of left ventricular (LV) function which is often overlooked. It can cause symptoms of heart failure in patients even in the presence of normal systolic function. The parameters used to assess diastolic function often measure flow and are affected by the loading conditions of the heart. The interpretation of diastolic function in the context of congenital heart disease requires some understanding of the effects of the lesions themselves on these parameters. Individual congenital lesions will be discussed in this paper. Recently, load-independent techniques have led to more accurate measurements of ventricular compliance and remodeling in heart disease. The combination of inflow velocities and tissue Doppler measurements can be used to estimate diastolic function and LV filling pressures. This review focuses on diastolic function and assessment in congenital heart disease. PMID:28261582

Loan applications in adult patients with congenital heart disease: a French study.

PubMed

Ladouceur, Magalie; Dugardin, Bertrand; Gourdin, Stéphanie; Sidi, Daniel; Bonnet, Damien; Iserin, Laurence

2011-01-01

Improvements in the treatment of children with congenital heart disease have led to most of these patients reaching adulthood. Despite the increase in lifespan, very little is known about their quality of life - in particular, their ability to obtain a mortgage or consumer loan. To investigate the outcome of mortgage and loan applications made by adults with differential severities of congenital heart disease. Four hundred and seventy-six patients were invited to participate in a questionnaire-based interview by phone. Of these patients, one hundred and forty-two responded. Respondents were classified into three categories ('significant', 'complex' and 'mild') based on congenital heart disease severity according to the Bethesda conference. Ninety patients (64%) had applied for loans; 17 (16.5%) did not report their heart disease to the insurance company, 13 were refused insurance and 39 were asked to pay surplus fees. The imposed fees concerned patients classified in the 'significant' and 'complex' groups (P<0.0001 and P<0.003, respectively, compared with those classified in the 'mild' group). Age, sex, other diseases, cardiovascular risk factors and duration of the loan had no influence on loan application outcomes. Adults with congenital heart disease are considerably more likely to have difficulty obtaining a mortgage or loan, independent of their congenital heart disease severity. Moreover, despite an increased obtainment of a loan in patients classified as 'mild', the refusal rates were identical for patients classified as having 'significant' or 'complex' congenital heart disease, although their prognosis is different. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

[Change in our approach in the surgical management of congenital heart defects in patients with Down syndrome, 1974-2016].

PubMed

Hartyánszky, István; Bogáts, Gábor

2016-10-01

Congenital heart defects are frequently present in patients with Down syndrome. The authors analyzed the impact of changing approach in surgical management of congenital heart defect on the life expectancy of patients with Down syndrome. Between 1974 and 1997 the data of 359 children with Down syndrome were collected. Among them 255 patients had no surgery and the mortality in this group was 25.9%, whereas the mortality in the group of 104 patients who underwent palliative surgery was 8.6%. Surgical management of congenital heart defects provides the same life expectancy for these patients as compared to Down patients without cardiac defects. Primary reconstruction is the preferable surgical procedure in infancy that provides good results. Nowadays the number of the operated grown-up congenital heart disease patients with Down syndrome is increasing. During the last three years 82 grown-up congenital heart disease patients, including 4 patients with Down syndrome (aged between 24 and 60 years) were reconstructed successfully. Due to the successful surgery in infancy the population of grown-up congenital heart disease patients with Down syndrome is increasing. The cardiac surgeons are ready to do everything for the optimal life expectancy of these patients. However, management of special problems (indication and necessity of reoperation, optimal age) in patients with Down syndrome poses a great challenge for cardiologists and cardiac surgeons. Orv. Hetil., 2016, 157(40), 1601-1603.

Development and Validation of an Agency for Healthcare Research and Quality Indicator for Mortality After Congenital Heart Surgery Harmonized With Risk Adjustment for Congenital Heart Surgery (RACHS-1) Methodology.

PubMed

Jenkins, Kathy J; Koch Kupiec, Jennifer; Owens, Pamela L; Romano, Patrick S; Geppert, Jeffrey J; Gauvreau, Kimberlee

2016-05-20

The National Quality Forum previously approved a quality indicator for mortality after congenital heart surgery developed by the Agency for Healthcare Research and Quality (AHRQ). Several parameters of the validated Risk Adjustment for Congenital Heart Surgery (RACHS-1) method were included, but others differed. As part of the National Quality Forum endorsement maintenance process, developers were asked to harmonize the 2 methodologies. Parameters that were identical between the 2 methods were retained. AHRQ's Healthcare Cost and Utilization Project State Inpatient Databases (SID) 2008 were used to select optimal parameters where differences existed, with a goal to maximize model performance and face validity. Inclusion criteria were not changed and included all discharges for patients <18 years with International Classification of Diseases, Ninth Revision, Clinical Modification procedure codes for congenital heart surgery or nonspecific heart surgery combined with congenital heart disease diagnosis codes. The final model includes procedure risk group, age (0-28 days, 29-90 days, 91-364 days, 1-17 years), low birth weight (500-2499 g), other congenital anomalies (Clinical Classifications Software 217, except for 758.xx), multiple procedures, and transfer-in status. Among 17 945 eligible cases in the SID 2008, the c statistic for model performance was 0.82. In the SID 2013 validation data set, the c statistic was 0.82. Risk-adjusted mortality rates by center ranged from 0.9% to 4.1% (5th-95th percentile). Congenital heart surgery programs can now obtain national benchmarking reports by applying AHRQ Quality Indicator software to hospital administrative data, based on the harmonized RACHS-1 method, with high discrimination and face validity. © 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

[Congenital cardiopathy and cerebral abscess].

PubMed

Paixão, A; de Andrade, F F; Sampayo, F

1989-01-01

During 1986 the authors came across two cases of brain abscess among children with congenital heart disease followed at the Pediatric Cardiology Service and decided to evaluate their global experience on the subject. In a retrospective study of 860 infants and children with cyanotic congenital heart disease and final diagnosis, there were four cases complicated with brain abscess. The following items were evaluated: prevalence of the complication, type of congenital heart disease, date and age at the diagnosis of brain abscess, diagnostic methods, neurosurgical treatment and results. The main findings were: all patients were above two years of age and had noncorrected cyanotic congenital heart disease belonging to the classic high risk group; the first two cases had been treated in other institutions and only scanty information was available; two recent cases had early diagnosis on CAT scan followed by neurosurgical treatment. All children survived. brain abscess is a rare but severe complication occurring in patients with noncorrected cyanotic congenital heart disease above two years of age; whenever prevention turns impossible, early diagnosis and treatment provide good short term and long term results. A multidisciplinar approach with full cooperation is advocated.

Emerging Research Directions in Adult Congenital Heart Disease: A Report from a National Heart, Lung, and Blood Institute/Adult Congenital Heart Association Working Group

PubMed Central

Gurvitz, Michelle; Burns, Kristin M.; Brindis, Ralph; Broberg, Craig S.; Daniels, Curt J.; Fuller, Stephanie M.P.N.; Honein, Margaret A.; Khairy, Paul; Kuehl, Karen S.; Landzberg, Michael J.; Mahle, William T.; Mann, Douglas L.; Marelli, Ariane; Newburger, Jane W.; Pearson, Gail D.; Starling, Randall C.; Tringali, Glenn R.; Valente, Anne Marie; Wu, Joseph C.; Califf, Robert M.

2016-01-01

Congenital heart disease (CHD) is the most common birth defect, affecting about 0.8% of live births. Advances in recent decades have allowed >85% of children with CHD to survive to adulthood, creating a growing population of adults with CHD. Little information exists regarding survival, demographics, late outcomes, and comorbidities in this emerging group, and multiple barriers impede research in adult CHD (ACHD). The National Heart, Lung, and Blood Institute and the Adult Congenital Heart Association convened a multidisciplinary Working Group to identify high-impact research questions in ACHD. This report summarizes the meeting discussions in the broad areas of CHD-related heart failure, vascular disease and multisystem complications. High-priority subtopics identified included heart failure in tetralogy of Fallot, mechanical circulatory support/transplantation, sudden cardiac death, vascular outcomes in coarctation of the aorta, late outcomes in single ventricle disease, cognitive and psychiatric issues, and pregnancy. PMID:27102511

Heparin-Induced Thrombocytopenia with Associated Thrombosis in Children after the Fontan Operation

PubMed Central

Porcelli, Rosalia; Moskowitz, Bonnie C.; Cetta, Frank; Graham, Lynn C.; Godwin, John E.; Eidem, Benjamin W.; Prechel, M. Margaret; Walenga, Jeanine M.

2003-01-01

Heparin-induced thrombocytopenia is a widely recognized clinical disorder. The spectrum of disease ranges from clinically insignificant to severe thrombosis (heparin-induced thrombocytopenia with associated thrombosis). Overall, thrombosis occurs in approximately 33% of adults diagnosed with heparin-induced thrombocytopenia and has been associated with high morbidity and mortality rates. Diagnostic testing for this disorder is not standard in children with thrombocytopenia who are receiving heparin, despite the fact that children with congenital heart disease may be exposed to heparin frequently. There are few reported cases of heparin-induced thrombocytopenia with associated thrombosis in children; herein, we describe the cases of 2 children who developed this disorder after undergoing a Fontan operation. (Tex Heart Inst J 2003;30:58–61) PMID:12638673

The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model: Part 1—Statistical Methodology

PubMed Central

O’Brien, Sean M.; Jacobs, Jeffrey P.; Pasquali, Sara K.; Gaynor, J. William; Karamlou, Tara; Welke, Karl F.; Filardo, Giovanni; Han, Jane M.; Kim, Sunghee; Shahian, David M.; Jacobs, Marshall L.

2016-01-01

Background This study’s objective was to develop a risk model incorporating procedure type and patient factors to be used for case-mix adjustment in the analysis of hospital-specific operative mortality rates after congenital cardiac operations. Methods Included were patients of all ages undergoing cardiac operations, with or without cardiopulmonary bypass, at centers participating in The Society of Thoracic Surgeons Congenital Heart Surgery Database during January 1, 2010, to December 31, 2013. Excluded were isolated patent ductus arteriosus closures in patients weighing less than or equal to 2.5 kg, centers with more than 10% missing data, and patients with missing data for key variables. Data from the first 3.5 years were used for model development, and data from the last 0.5 year were used for assessing model discrimination and calibration. Potential risk factors were proposed based on expert consensus and selected after empirically comparing a variety of modeling options. Results The study cohort included 52,224 patients from 86 centers with 1,931 deaths (3.7%). Covariates included in the model were primary procedure, age, weight, and 11 additional patient factors reflecting acuity status and comorbidities. The C statistic in the validation sample was 0.858. Plots of observed-vs-expected mortality rates revealed good calibration overall and within subgroups, except for a slight overestimation of risk in the highest decile of predicted risk. Removing patient preoperative factors from the model reduced the C statistic to 0.831 and affected the performance classification for 12 of 86 hospitals. Conclusions The risk model is well suited to adjust for case mix in the analysis and reporting of hospital-specific mortality for congenital heart operations. Inclusion of patient factors added useful discriminatory power and reduced bias in the calculation of hospital-specific mortality metrics. PMID:26245502

A pilot study of expenditures on, and utilization of resources in, health care in adults with congenital heart disease.

PubMed

Moons, P; Siebens, K; De Geest, S; Abraham, I; Budts, W; Gewillig, M

2001-05-01

Congenital cardiac disease may be a chronic condition, necessitating life-long follow-up for a substantial proportion of the patients. Such patients, therefore, are often presumed to be high users of resources for health care. Information on utilization of resources in adults with congenital heart disease, however, is scarce. This retrospective pilot study, performed in Belgium, investigated 192 adults with congenital heart disease to measure the annual expenditures and utilization of health care and compared the findings with data from the general population. We also sought to explore demographic and clinical parameters as predictors for the expenditures. Hospitalization was documented in 20.3% of the patients, with a median length of stay of 5 days. The overall payment by health insurance associations in 1997 was 1794.5 ECU per patient, while patients paid on average 189.5 ECU out-of-pocket. For medication, the average reimbursement and out-of-pocket expenses were estimated at 78 ECU and 20 ECU, respectively. Expenditures for patients with congenital heart disease were considerably higher than the age and gender-corrected expenditures for the general population (411.7 ECU), though this difference was accounted for by only one-eighth of the cohort of those with congenital heart disease. In general, higher expenditures were associated with abnormal left ventricular end-diastolic diameter, female gender, functional impairment and higher age, although the explained variance was limited. Our study has provided pilot data on the economic outcomes for patients with congenital heart diseases. We have identified parameters that could predict expenditure, but which will have to be examined in future research. This is needed to develop guidelines for health insurance for those with congenital heart diseases.

DNA methylation abnormalities in congenital heart disease.

PubMed

Serra-Juhé, Clara; Cuscó, Ivon; Homs, Aïda; Flores, Raquel; Torán, Núria; Pérez-Jurado, Luis A

2015-01-01

Congenital heart defects represent the most common malformation at birth, occurring also in ∼50% of individuals with Down syndrome. Congenital heart defects are thought to have multifactorial etiology, but the main causes are largely unknown. We have explored the global methylation profile of fetal heart DNA in comparison to blood DNA from control subjects: an absolute correlation with the type of tissue was detected. Pathway analysis revealed a significant enrichment of differential methylation at genes related to muscle contraction and cardiomyopathies in the developing heart DNA. We have also searched for abnormal methylation profiles on developing heart-tissue DNA of syndromic and non-syndromic congenital heart defects. On average, 3 regions with aberrant methylation were detected per sample and 18 regions were found differentially methylated between groups. Several epimutations were detected in candidate genes involved in growth regulation, apoptosis and folate pathway. A likely pathogenic hypermethylation of several intragenic sites at the MSX1 gene, involved in outflow tract morphogenesis, was found in a fetus with isolated heart malformation. In addition, hypermethylation of the GATA4 gene was present in fetuses with Down syndrome with or without congenital heart defects, as well as in fetuses with isolated heart malformations. Expression deregulation of the abnormally methylated genes was detected. Our data indicate that epigenetic alterations of relevant genes are present in developing heart DNA in fetuses with both isolated and syndromic heart malformations. These epimutations likely contribute to the pathogenesis of the malformation by cis-acting effects on gene expression.

Burden and impact of congenital syndromes and comorbidities among adults with congenital heart disease.

PubMed

Bracher, Isabelle; Padrutt, Maria; Bonassin, Francesca; Santos Lopes, Bruno; Gruner, Christiane; Stämpfli, Simon F; Oxenius, Angela; De Pasquale, Gabriella; Seeliger, Theresa; Lüscher, Thomas F; Attenhofer Jost, Christine; Greutmann, Matthias

2017-08-01

Our aim was to assess the overall burden of congenital syndromes and non-cardiac comorbidities among adults with congenital heart disease and to assess their impact on circumstances of living and outcomes. Within a cohort of 1725 adults with congenital heart defects (65% defects of moderate or great complexity) followed at a single tertiary care center, congenital syndromes and comorbidities were identified by chart review. Their association with arrhythmias, circumstances of living and survival was analyzed. Within the study cohort, 232 patients (13%) had a genetic syndrome, 51% at least one comorbidity and 23% ≥2 comorbidities. Most prevalent comorbidities were systemic arterial hypertension (11%), thyroid dysfunction (9%), psychiatric disorders (9%), neurologic disorders (7%), chronic lung disease (7%), and previous stroke (6%). In contrast to higher congenital heart defect complexity, the presence of comorbidities had no impact on living circumstances but patients with comorbidities were less likely to work full-time. Atrial arrhythmias were more common among patients with moderate/great disease complexity and those with comorbidities but were less common among patients with congenital syndromes (p<0.01 for all comparisons). Patients with ≥2 comorbidities had lower survival estimates compared to those with ≤1 comorbidity (p=0.013). Congenital syndromes and comorbidities are highly prevalent in adults with congenital heart disease followed at specialist centers and add to the overall complexity of care. The presence of these additional factors has an impact on living circumstances, is associated with arrhythmias and needs to be further explored as prognostic markers. Copyright © 2017 Elsevier B.V. All rights reserved.

A Matter of the Heart: The African Clawed Frog Xenopus as a Model for Studying Vertebrate Cardiogenesis and Congenital Heart Defects

PubMed Central

Hempel, Annemarie; Kühl, Michael

2016-01-01

The African clawed frog, Xenopus, is a valuable non-mammalian model organism to investigate vertebrate heart development and to explore the underlying molecular mechanisms of human congenital heart defects (CHDs). In this review, we outline the similarities between Xenopus and mammalian cardiogenesis, and provide an overview of well-studied cardiac genes in Xenopus, which have been associated with congenital heart conditions. Additionally, we highlight advantages of modeling candidate genes derived from genome wide association studies (GWAS) in Xenopus and discuss commonly used techniques. PMID:29367567

Nursing diagnoses in children with congenital heart disease: a survival analysis.

PubMed

Martins da Silva, Viviane; Lopes, Marcos Venícios de Oliveira; Leite de Araujo, Thelma

2007-01-01

To analyze the relationship between nursing diagnoses and survival rates in children with congenital heart disease. A total of 270 observations were carried out in 45 children with congenital heart disease who were followed for 15 days. Differences in mean survival times were identified in children not more than 4 months of age with respect to the following diagnoses: impaired gas exchange, ineffective breathing pattern, activity intolerance, delayed growth and development, and decreased cardiac output. The main diagnoses are identified early in the hospitalization period and are conditions resulting from hemodynamic alterations and prescribed medical treatment. Congenital heart disease provokes serious hemodynamic alterations that generate human responses, which should be treated proactively.

The World Database for Pediatric and Congenital Heart Surgery: Update.

PubMed

Louis, James St; Kirklin, James

2018-05-01

Following several years of planning and design, the World Database for Pediatric and Congenital Heart Surgery (WDPCHS) went live on January 1, 2017. The first operational year of this valuable database has proven very successful. As of February 28, 2018, more than 4,500 patients have been submitted from 39 centers; participation currently spans 19 countries over 6 continents, with 120 more centers in the enrollment pipeline. This update, the first in a series, is intended to provide World Society for Pediatric and Congenital Heart Surgery members and others who care for children with congenital heart disease with a broad overview of current progress and ongoing activities surrounding the WDPCHS.

Congenital heart disease in relation to maternal use of Bendectin and other drugs in early pregnancy.

PubMed

Zierler, S; Rothman, K J

1985-08-08

To clarify the association of Bendectin and other drugs used in early pregnancy with the occurrence of congenital heart disease, we interviewed 298 mothers of children with congenital heart disease and 738 mothers of healthy controls. Differential recall of drug use by mothers of affected children and mothers of controls was evaluated by comparison of information collected by interview with that recorded in the prenatal record. Data derived from maternal interviews were generally consistent with the record data. Reported Bendectin use was minimally associated with congenital heart disease (prevalence odds ratio, 1.1; 90 per cent confidence interval, 0.8 to 1.5). The data from this study were consistent with previously reported associations of other drugs with congenital heart disease. In particular, aspirin use in early pregnancy was associated with about a twofold increase in the frequency of defects in septation of the truncus arteriosus (prevalence odds ratio, 2.1; 90 per cent confidence interval, 1.1 to 3.9).

Improving Neurodevelopmental Outcomes in Children with Congenital Heart Disease: An Intervention Study

DTIC Science & Technology

2017-10-01

AWARD NUMBER: W81XWH-16-1-0741 TITLE: Improving Neurodevelopmental Outcomes in Children with Congenital Heart Disease: An Intervention Study ...2017 4. TITLE AND SUBTITLE Improving Neurodevelopmental Outcomes in Children with Congenital Heart Disease: An Intervention Study 5a. CONTRACT NUMBER...executive function in several pediatric populations, but has not been studied in the CHD population. 15. SUBJECT TERMS- 16. SECURITY CLASSIFICATION OF

Annual Research Progress Report, Fiscal Year 1981,

DTIC Science & Technology

1981-10-01

Joseph P. Murgo, M.D., COL, MC Key Words: Patent ductus arteriosus Cyanotic congenital heart disease Accumulative MEDCASE Est Accumulative Periodic Cost... Ductus Arteriosus in Neonates 47 with Cyanotic Congenital Heart Disease. (T) C-37-79 Ankle Trauma Study. (0) 48 C-5-80 Lopressor Intervention Trial. (0...Maintenance of Patency of the Ductus Arteriosus in Neonates with Cyanotic Congenital Heart Disease Start Date: Aug 79 Est Comp Date: Principal

The helical ventricular myocardial band of Torrent-Guasp: potential implications in congenital heart defects.

PubMed

Corno, Antonio F; Kocica, Mladen J; Torrent-Guasp, Francisco

2006-04-01

The new concepts of cardiac anatomy and physiology, based on the observations made by Francisco Torrent-Guasp's discovery of the helical ventricular myocardial band, can be useful in the context of the surgical strategies currently used to manage patients with congenital heart defects. The potential impact of the Torrent-Guasp's Heart on congenital heart defects have been analyzed in the following settings: ventriculo-arterial discordance (transposition of the great arteries), double (atrio-ventricular and ventriculo-arterial) discordance (congenitally corrected transposition of the great arteries), Ebstein's anomaly, pulmonary valve regurgitation after repair of tetralogy of Fallot, Ross operation, and complex intra-ventricular malformations. The functional interaction of right and left ventricles occurs not only through their arrangements in series but also thanks to the structural spiral features. Changes in size and function of either ventricle may influence the performance of the other ventricle. The variety and complexity of congenital heart defects make the recognition of the relationship between form and function a vital component, especially when compared to acquired disease. The new concepts of cardiac anatomy and function proposed by Francisco Torrent-Guasp, based on his observations, should stimulate further investigations of alternative surgical strategies by individuals involved with the management of patients with congenital heart defects.

Cardiovascular Conditions of Childhood

MedlinePlus

... Learn the symptoms, diagnosis and treatment for Kawasaki disease. Congenital Heart Defects If your child is born with ... and read personal stories of young stroke survivors. Congenital Heart Defects ... about children and heart disease. Login or Sign Up to save and share ...

Percutaneous Pulmonary Valve Placement

PubMed Central

Prieto, Lourdes R.

2015-01-01

Patients with congenital heart disease and pulmonary valve disease need multiple procedures over their lifetimes to replace their pulmonary valves. Chronic pulmonary stenosis, regurgitation, or both have untoward effects on ventricular function and on the clinical status of these patients. To date, all right ventricle–pulmonary artery conduits have had relatively short lifespans. Percutaneous pulmonary valve implantation, although relatively new, will probably reduce the number of operative procedures that these patients will have to undergo over a lifetime. Refinement and further development of this procedure holds promise for the extension of this technology to other patient populations. PMID:26175629

Implant and clinical characteristics for pediatric and congenital heart patients in the national cardiovascular data registry implantable cardioverter defibrillator registry.

PubMed

Jordan, Christopher P; Freedenberg, Vicki; Wang, Yongfei; Curtis, Jeptha P; Gleva, Marye J; Berul, Charles I

2014-12-01

In 2010, the National Cardiovascular Data Registry enhanced pediatric, nonatherosclerotic structural heart disease and congenital heart disease (CHD) data collection. This report characterizes CHD and pediatric patients undergoing implantable cardioverter defibrillator implantation. In this article, we report implantable cardioverter defibrillator procedures (April 2010 to December 2012) in the registry for 2 cohorts: (1) all patients with CHD (atrial septal defect, ventricular septal defect, tetralogy of Fallot, Ebstein anomaly, transposition of the great vessels, and common ventricle) and (2) patients <21 years. We evaluated indications and characteristics to include transvenous and nontransvenous lead implants, CHD type, and New York Heart Association class. There were 3139 CHD procedures, 1601 for patients <21 years and 126 for CHD <21 years. Implantable cardioverter defibrillator indications for patients with CHD were primary prevention in 1943 (61.9%) and secondary prevention in 1107 (35.2%). Pediatric patients had 935 (58.4%) primary prevention and 588 (36.7%) secondary prevention devices. Primary prevention had higher New York Heart Association class. Nontransvenous age (35.9 ± 23.2 versus 40.1 ± 24.6 years; P=0.05) and nontransvenous height (167.1 ± 18.9 cm; range, 53-193 cm versus 170.4 ± 13.1 cm; range, 61-203 cm; P<0.01) were lower than for transvenous patients. CHD and pediatrics had similar rates of transvenous (97%) and nontransvenous (3%) leads and did not differ from the overall registry. Transposition of the great vessels and common ventricle had higher rates of nontransvenous leads. Primary prevention exceeds secondary prevention for CHD and pediatrics. Nontransvenous lead patients were younger, with higher rates of transposition of the great vessels and common ventricle patients compared with transvenous lead patients. © 2014 American Heart Association, Inc.

Mechanisms Linking Red Blood Cell Disorders and Cardiovascular Diseases

PubMed Central

2015-01-01

The present paper aims to review the main pathophysiological links between red blood cell disorders and cardiovascular diseases, provides a brief description of the latest studies in this area, and considers implications for clinical practice and therapy. Anemia is associated with a special risk in proatherosclerotic conditions and heart disease and became a new therapeutic target. Guidelines must be updated for the management of patients with red blood cell disorders and cardiovascular diseases, and targets for hemoglobin level should be established. Risk scores in several cardiovascular diseases should include red blood cell count and RDW. Complete blood count and hemorheological parameters represent useful, inexpensive, widely available tools for the management and prognosis of patients with coronary heart disease, heart failure, hypertension, arrhythmias, and stroke. Hypoxia and iron accumulation cause the most important cardiovascular effects of sickle cell disease and thalassemia. Patients with congenital chronic hemolytic anemia undergoing splenectomy should be monitored, considering thromboembolic and cardiovascular risk. PMID:25710019

RSV prophylaxis guideline changes and outcomes in children with congenital heart disease.

PubMed

Walpert, Adam S; Thomas, Ian D; Lowe, Merlin C; Seckeler, Michael D

2018-05-01

The aim of this study was to compare inpatient outcomes and costs for children with respiratory syncytial virus and congenital heart disease before and after the change in management guidelines for respiratory syncytial virus prophylaxis. Hospital discharge data from the Vizient (formerly University HealthSystem Consortium) were queried from October 2012 to June 2014 (Era 1) and July 2014 to April 2016 (Era 2) for patients aged <24 months with an any International Classification of Disease (ICD)-9 or ICD-10 code for congenital heart disease (745-747.49, Q20.0-Q26.4) and a primary or secondary admitting diagnosis of respiratory syncytial virus infection (079.6, J20.5), acute bronchiolitis due to respiratory syncytial virus (466.11, J21.0) or respiratory syncytial virus pneumonia (480.1, J12.1). This study is a review of a national administrative discharge database. Respiratory syncytial virus admissions were identified in 1269 patients aged <24 months with congenital heart disease, with 644 patients in Era 1 and 625 in Era 2. Patients 0-12 months old represented 83% of admissions. Prior to 2014, children aged 0-24 months with congenital heart disease were eligible to receive respiratory syncytial virus prophylaxis. Updated guidelines, published in 2014, restricted the recommendation to administer palivizumab respiratory syncytial virus prophylaxis to children with congenital heart disease only if they are ≤12 months old. The outcome measures are hospital length of stay, ICU admission rate, mortality, and direct costs. There was no change in length of stay, ICU admission rate, in-hospital mortality, or direct costs for children 13-24 months old with congenital heart disease after the change in guidelines. There were no deaths in 13-24 month olds, regardless of era. Our findings provide additional support for the new guideline recommendations to provide respiratory syncytial virus prophylaxis only for children ≤12 months old with congenital heart disease. © 2018 Wiley Periodicals, Inc.

[Immunological status of the pediatric patient who has undergone heart surgery].

PubMed

Valenzuela Flores, A; Wakida, G; Limón Rojas, A; Obregón, C; Orihuela, O; Romero, C

1995-01-01

Communication of results a study the effect of open and closed-heart surgery in the immune system of infants and children. Data collected 24 hrs before anesthesia and surgery and five days after surgery. Operating room and pediatric intensive care of Hospital Central de Petróleos Mexicanos (PEMEX) in the South, Mexico City. Children undergoing surgery for correction of congenital heart disease (age 16 months to 14 years). A total of 16 patients. increased neutrophil counts with luymphopenia in both groups (p < 0.05), serum levels of the complement components C3 and C4 were higher after surgery, serum immunoglobulin IgG, IgA and IgM were higher after surgery, serum immunoglobulin IgG, IgA and IgM were decreased form preoperative levels (p < 0.01). Two patients had infection in the surgical wound. The effect of open and closed-heart surgery produced transitory immunodeficiency with recuperation of his immune systems and 5th day after surgery.

2013 update on congenital heart disease, clinical cardiology, heart failure, and heart transplant.

PubMed

Subirana, M Teresa; Barón-Esquivias, Gonzalo; Manito, Nicolás; Oliver, José M; Ripoll, Tomás; Lambert, Jose Luis; Zunzunegui, José L; Bover, Ramon; García-Pinilla, José Manuel

2014-03-01

This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters in assessing the systemic right ventricle, problems in patients with repaired tetralogy of Fallot and indication for pulmonary valve replacement, and confirmation of the role of specific factors in the selection of candidates for Fontan surgery. The most recent publications in clinical cardiology include a study by a European working group on correct diagnostic work-up in cardiomyopathies, studies on the cost-effectiveness of percutaneous aortic valve implantation, a consensus document on the management of type B aortic dissection, and guidelines on aortic valve and ascending aortic disease. The most noteworthy developments in heart failure and transplantation include new American guidelines on heart failure, therapeutic advances in acute heart failure (serelaxin), the management of comorbidities such as iron deficiency, risk assessment using new biomarkers, and advances in ventricular assist devices. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

[Percutaneous intervention in the correction of congenital heart deffects (DCC): experience in as UMAE].

PubMed

Campos-García, Vicente; Ordóñez-Toquero, Guillermo; Monjaraz-Rodríguez, Sarain; Gómez-Conde, Eduardo

Congenital heart defects are common in infants and adults, affecting quality of life if not corrected. Unlike open surgery, percutaneous intervention allows correction with a high success rate and speedy recovery. In Mexico, there are not enough studies to describe their efficacy and safety. A cohort study was conducted in the Hospital "Manuel Avila Camacho", in Puebla, Mexico, including 149 patients with congenital heart defects repaired by percutaneous intervention, recording data from clinical records. The following were documented: post-guided fluoroscopy, hemodynamic changes, cardiac catheterization drilling anatomical changes, and complications six months later such as infection or bleeding at the puncture site, device migration, endocarditis, or death. SPSS was used, using descriptive and inferential statistics. The patients' congenital heart defects treated were ductus arteriosus, atrial septal defect, and aortic coarctation, with ductus arteriosus being recorded as the most frequent congenital heart defect. Primary angioplasties were performed in 75% and stenting in the rest. Anatomical corrections of congenital defects were successful in 96.4% of patients (p < 0.01), with minimal adverse effects (p < 0.01). We conclude that our hospital has good efficacy and safety in percutaneous intervention, comparable to published reports.

Intraventricular Hemorrhage in Moderate to Severe Congenital Heart Disease.

PubMed

Ortinau, Cynthia M; Anadkat, Jagruti S; Smyser, Christopher D; Eghtesady, Pirooz

2018-01-01

Determine the prevalence of intraventricular hemorrhage in infants with moderate to severe congenital heart disease, investigate the impact of gestational age, cardiac diagnosis, and cardiac intervention on intraventricular hemorrhage, and compare intraventricular hemorrhage rates in preterm infants with and without congenital heart disease. A single-center retrospective review. A tertiary care children's hospital. All infants admitted to St. Louis Children's Hospital from 2007 to 2012 with moderate to severe congenital heart disease requiring cardiac intervention in the first 90 days of life and all preterm infants without congenital heart disease or congenital anomalies/known genetic diagnoses admitted during the same time period. None. Cranial ultrasound data were reviewed for presence/severity of intraventricular hemorrhage. Head CT and brain MRI data were also reviewed in the congenital heart disease infants. Univariate analyses were undertaken to determine associations with intraventricular hemorrhage, and a final multivariate logistic regression model was performed. There were 339 infants with congenital heart disease who met inclusion criteria and 25.4% were born preterm. Intraventricular hemorrhage was identified on cranial ultrasound in 13.3% of infants, with the majority of intraventricular hemorrhage being low-grade (grade I/II). The incidence increased as gestational age decreased such that intraventricular hemorrhage was present in 8.7% of term infants, 19.2% of late preterm infants, 26.3% of moderately preterm infants, and 53.3% of very preterm infants. There was no difference in intraventricular hemorrhage rates between cardiac diagnoses. Additionally, the rate of intraventricular hemorrhage did not increase after cardiac intervention, with only three infants demonstrating new/worsening high-grade (grade III/IV) intraventricular hemorrhage after surgery. In a multivariate model, only gestational age at birth and African-American race were predictors of intraventricular hemorrhage. In the subset of infants with CT/MRI data, there was good sensitivity and specificity of cranial ultrasound for presence of intraventricular hemorrhage. Infants with congenital heart disease commonly develop intraventricular hemorrhage, particularly when born preterm. However, the vast majority of intraventricular hemorrhage is low-grade and is associated with gestational age and African-American race.

Sex differences in mortality in children undergoing congenital heart disease surgery: a United States population-based study.

PubMed

Marelli, Ariane; Gauvreau, Kimberlee; Landzberg, Mike; Jenkins, Kathy

2010-09-14

The changing demographics of the adult congenital heart disease (CHD) population requires an understanding of the factors that impact patient survival to adulthood. We sought to investigate sex differences in CHD surgical mortality in children. Children <18 years old hospitalized for CHD surgery were identified using the Kids' Inpatient Database in 2000, 2003, and 2006. Demographic, diagnostic, and procedural variables were grouped according to RACHS-1 (Risk Adjustment for Congenital Heart Surgery) method. Logistic regression was used to determine the odds ratio of death in females versus males adjusting for RACHS-1 risk category, age, prematurity, major noncardiac anomalies, and multiple procedures. Analyses were stratified by RACHS-1 risk categories and age. Of 33 848 hospitalizations for CHD surgery, 54.7% were in males. Males were more likely than females to have CHD surgery in infancy, high-risk CHD surgery, and multiple CHD procedures. Females had more major noncardiac structural anomalies and more low-risk procedures. However, the adjusted risk of in-hospital death was higher in females (odds ratio, 1.21; 95% confidence interval, 1.08 to 1.36) on account of the subgroup with high-risk surgeries who were <1 year of age (odds ratio, 1.39; 95% confidence interval, 1.16 to 1.67). In this large US population study, more male children underwent CHD surgery and had high-risk procedures. Female infants who had high-risk procedures were at higher risk for death, but this accounted for a small proportion of females and is therefore unlikely to have a major impact on the changing demographics in adults in CHD.

Prophylactic milrinone for the prevention of low cardiac output syndrome and mortality in children undergoing surgery for congenital heart disease.

PubMed

Burkhardt, Barbara E U; Rücker, Gerta; Stiller, Brigitte

2015-03-25

Children with congenital heart disease often undergo heart surgery at a young age. They are at risk for postoperative low cardiac output syndrome (LCOS) or death. Milrinone may be used to provide inotropic and vasodilatory support during the immediate postoperative period. This review examines the effectiveness of prophylactic postoperative use of milrinone to prevent LCOS or death in children having undergone surgery for congenital heart disease. Electronic and manual literature searches were performed to identify randomised controlled trials. We searched CENTRAL, MEDLINE, EMBASE and Web of Science in February 2014 and conducted a top-up search in September 2014 as well as clinical trial registries and reference lists of published studies. We did not apply any language restrictions. Only randomised controlled trials were selected for analysis. We considered studies with newborn infants, infants, toddlers, and children up to 12 years of age. Two review authors independently extracted data according to a pre-defined protocol. We obtained additional information from all study authors. Three of the five included studies compared milrinone versus levosimendan, one study compared milrinone with placebo, and one compared milrinone verus dobutamine, with 101, 242, and 50 participants, respectively. Three trials were at low risk of bias while two were at higher risk of bias. The number and definitions of outcomes were non-uniform as well. In one study comparing two doses of milrinone and placebo, there was some evidence in an overall comparison of milrinone versus placebo that milrinone lowered risk for LCOS (risk ratio (RR) 0.52, 95% confidence interval (CI) 0.28 to 0.96; 227 participants). The results from two small studies do not provide enough information to determine whether milrinone increases the risk of LCOS when compared to levosimendan (RR 1.22, 95% CI 0.32 to 4.65; 59 participants). Mortality rates in the studies were low, and there was insufficient evidence to draw conclusions on the effect of milrinone compared to placebo or levosimendan or dobutamine regarding mortality, the duration of intensive care stay, hospital stay, mechanical ventilation, or maximum inotrope score (where available). Numbers of patients requiring mechanical cardiac support were also low and did not allow a comparison between studies, and none of the participants of any study received a heart transplantation up to the end of the respective follow-up period. Time to death within three months was not reported in any of the included studies. A number of adverse events was examined, but differences between the treatment groups could not be proven for hypotension, intraventricular haemorrhage, hypokalaemia, bronchospasm, elevated serum levels of liver enzymes, or a reduced left ventricular ejection fraction < 50% or reduced left ventricular fraction of shortening < 28%. Our analysis did not prove an increased risk of arrhythmias in patients treated prophylactically with milrinone compared with placebo (RR 3.59, 95% CI 0.83 to 15.42; 238 participants), a decreased risk of pleural effusions (RR 1.78, 95% CI 0.92 to 3.42; 231 participants), or a difference in risk of thrombocytopenia on milrinone compared with placebo (RR 0.86, 95% CI 0.39 to 1.88; 238 participants). Comparisons of milrinone with levosimendan or with dobutamine, respectively, did not clarify the risk of arrhythmia and were not possible for pleural effusions or thrombocytopenia. There is insufficient evidence of the effectiveness of prophylactic milrinone in preventing death or low cardiac output syndrome in children undergoing surgery for congenital heart disease, compared to placebo. So far, no differences have been shown between milrinone and other inodilators, such as levosimendan or dobutamine, in the immediate postoperative period, in reducing the risk of LCOS or death. The existing data on the prophylactic use of milrinone has to be viewed cautiously due to the small number of small trials and their risk of bias.

Right upper limb bud triplication and polythelia, left sided hemihypertrophy and congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis: disorganisation-like spectrum or patterning gene defect?

PubMed

Sabry, M A; al-Saleh, Q; al-Saw'an, R; al-Awadi, S A; Farag, T I

1995-07-01

A Somali female baby with right upper limb triplication, polythelia, left sided hemihypertrophy, congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis is described. It seems that the above described pattern of anomalies has not been reported before. The possible developmental genetic mechanism responsible for this phenotype is briefly discussed.

Deletion of ETS-1, a gene in the Jacobsen syndrome critical region, causes ventricular septal defects and abnormal ventricular morphology in mice

PubMed Central

Ye, Maoqing; Coldren, Chris; Liang, Xingqun; Mattina, Teresa; Goldmuntz, Elizabeth; Benson, D. Woodrow; Ivy, Dunbar; Perryman, M.B.; Garrett-Sinha, Lee Ann; Grossfeld, Paul

2010-01-01

Congenital heart defects comprise the most common form of major birth defects, affecting 0.7% of all newborn infants. Jacobsen syndrome (11q-) is a rare chromosomal disorder caused by deletions in distal 11q. We have previously determined that a wide spectrum of the most common congenital heart defects occur in 11q-, including an unprecedented high frequency of hypoplastic left heart syndrome (HLHS). We identified an ∼7 Mb ‘cardiac critical region’ in distal 11q that contains a putative causative gene(s) for congenital heart disease. In this study, we utilized chromosomal microarray mapping to characterize three patients with 11q- and congenital heart defects that carry interstitial deletions overlapping the 7 Mb cardiac critical region. We propose that this 1.2 Mb region of overlap harbors a gene(s) that causes at least a subset of the congenital heart defects that occur in 11q-. We demonstrate that one gene in this region, ETS-1 (a member of the ETS family of transcription factors), is expressed in the endocardium and neural crest during early mouse heart development. Gene-targeted deletion of ETS-1 in mice in a C57/B6 background causes, with high penetrance, large membranous ventricular septal defects and a bifid cardiac apex, and less frequently a non-apex-forming left ventricle (one of the hallmarks of HLHS). Our results implicate an important role for the ETS-1 transcription factor in mammalian heart development and should provide important insights into some of the most common forms of congenital heart disease. PMID:19942620

Maternal and infant genetic variants, maternal periconceptional use of selective serotonin reuptake inhibitors, and risk of congenital heart defects in offspring: population based study.

PubMed

Nembhard, Wendy N; Tang, Xinyu; Hu, Zhuopei; MacLeod, Stewart; Stowe, Zachary; Webber, Daniel

2017-03-06

Objective  To evaluate whether the association between maternal periconceptional use of selective serotonin reuptake inhibitors (SSRIs) and increased risk of congenital heart defects in offspring is modified by maternal or infant genetic variants in folate, homocysteine, or transsulfuration pathways. Design  Population based study. DNA from mothers, fathers, and infants was genotyped with an Illumina GoldenGate custom single nucleotide polymorphism panel. A hybrid design based on a log linear model was used to calculate relative risks and Bayesian false discovery probabilities (BFDP) to identify polymorphisms associated with congenital heart defects modified by SSRI use. Data sources  Data from the US National Birth Defects Prevention Study on 1180 liveborn infants with congenital heart defects and 1644 controls, born 1997-2008. Main outcome measures  Cases included infants with selected congenital heart defects and control infants had no major defects. SSRI use was obtained from telephone interviews with mothers. Results  For women who reported taking SSRIs periconceptionally, maternal SHMT1 (rs9909104) GG and AGgenotypes were associated with a 5.9 and 2.4 increased risk of select congenital heart defects in offspring, respectively, versus the AA genotype (BFDP=0.69). Compared with the AA genotype, BHMT (rs492842 and rs542852) GG and AG genotypes were associated with twice the riskof congenital heart defects (BFDP=0.74 and 0.79, respectively). MGST1 (rs2075237) CC and ACgenotypes were associated with an increased risk compared with the GG genotype (8.0 and 2.8, respectively; BFDP=0.79). Single nucleotide polymorphism in infant genes in the folate (MTHFS rs12438477), homocysteine (TRDMT1 rs6602178 and GNMT rs11752813) and transsulfuration (GSTP1 rs7941395 and MGST1 rs7294985) pathways were also associated with an increased risk of congenital heart defects. Conclusions  Common maternal or infant genetic variants in folate, homocysteine, or transsulfuration pathways are associated with an increased risk of certain congenital heart defects among children of women taking SSRIs during cardiogenesis. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

A systematic review of prenatal screening for congenital heart disease by fetal electrocardiography.

PubMed

Verdurmen, Kim M J; Eijsvoogel, Noortje B; Lempersz, Carlijn; Vullings, Rik; Schroer, Christian; van Laar, Judith O E H; Oei, S Guid

2016-11-01

Congenital heart disease (CHD) is the most common severe congenital anomaly worldwide. Diagnosis early in pregnancy is important, but the detection rate by two-dimensional ultrasonography is only 65%-81%. To evaluate existing data on CHD and noninvasive abdominal fetal electrocardiography (ECG). A systematic review was performed through a search of the Cochrane Library, PubMed, and Embase for studies published up to April 2016 using the terms "congenital heart disease," "fetal electrocardiogram," and other similar keywords. Primary articles that described changes in fetal ECG among fetuses with CHD published in English were included. Outcomes of interest were changes in fetal ECG parameters observed for fetuses with congenital heart disease. Findings were reported descriptively. Only five studies described changes observed in the fetal electrocardiogram for fetuses with CHD, including heart rate, heart rate variability, and PR, QRS, and QT intervals. Fetal ECG reflects the intimate relationship between the cardiac nerve conduction system and the structural morphology of the heart. It seems particularly helpful in detecting the electrophysiological effects of cardiac anatomic defects (e.g. hypotrophy, hypertrophy, and conduction interruption). Fetal ECG might be a promising clinical tool to complement ultrasonography in the screening program for CHD. Copyright © 2016 International Federation of Gynecology and Obstetrics. Published by Elsevier Ireland Ltd. All rights reserved.

Epidemiology of congenital heart disease in Brazil

PubMed Central

Pinto Júnior, Valdester Cavalcante; Branco, Klébia Magalhães P. Castello; Cavalcante, Rodrigo Cardoso; Carvalho Junior, Waldemiro; Lima, José Rubens Costa; de Freitas, Sílvia Maria; Fraga, Maria Nazaré de Oliveira; de Souza, Nayana Maria Gomes

2015-01-01

Introduction Congenital heart disease is an abnormality in the structure or cardiocirculatory function, occurring from birth, even if diagnosed later. It can result in intrauterine death in childhood or in adulthood. Accounted for 6% of infant deaths in Brazil in 2007. Objective To estimate underreporting in the prevalence of congenital heart disease in Brazil and its subtypes. Methods The calculations of prevalence were performed by applying coefficients, giving them function rates for calculations of health problems. The study makes an approach between the literature and the governmental registries. It was adopted an estimate of 9: 1000 births and prevalence rates for subtypes applied to births of 2010. Estimates of births with congenital heart disease were compared with the reports to the Ministry of Health and were studied by descriptive methods with the use of rates and coefficients represented in tables. Results The incidence in Brazil is 25,757 new cases/year, distributed in: North 2,758; Northeast 7,570; Southeast 10,112; South 3,329; and Midwest 1,987. In 2010, were reported to System of Live Birth Information of Ministry of Health 1,377 cases of babies with congenital heart disease, representing 5.3% of the estimated for Brazil. In the same period, the most common subtypes were: ventricular septal defect (7,498); atrial septal defect (4,693); persistent ductus arteriosus (2,490); pulmonary stenosis (1,431); tetralogy of Fallot (973); coarctation of the aorta (973); transposition of the great arteries (887); and aortic stenosis 630. The prevalence of congenital heart disease, for the year of 2009, was 675,495 children and adolescents and 552,092 adults. Conclusion In Brazil, there is underreporting in the prevalence of congenital heart disease, signaling the need for adjustments in the methodology of registration. PMID:26107454

Noninvasive Screening for Pulmonary Hypertension by Exercise Testing in Congenital Heart Disease.

PubMed

Müller, Jan; Heck, Pinar Bambul; Ewert, Peter; Hager, Alfred

2017-05-01

Patients with congenital heart disease and native or palliated conditions are at risk to develop pulmonary hypertension (PH) in later life. Screening for PH is currently performed by regular echocardiographic follow-up, which appears to be difficult in several congenital conditions. This study evaluated the screening for PH in congenital heart disease by cardiopulmonary exercise testing (CPET). We analyzed our database including all patients with congenital heart disease referred for CPET in our institution from June 2001 to September 2013 and identified 683 patients who had an accompanied heart catheterization less than 6 month after CPET. Those 130 patients with proven PH were compared with the other 563 patients with congenital heart disease but without PH. Peak oxygen uptake was the most discriminative variable, showing two thresholds at 16.3 mL/min per kg and 25.2 mL/min per kg. The highest specificity of 95% for PH was found in patients with a peak oxygen uptake of 16.3 mL/min per kg or less and a breathing reserve of 37.4% or less. In patients with a peak oxygen uptake exceeding 16.3 mL/min per kg, there was a high specificity of 86.3% but a low sensitivity of 53.1%. With 25.2 mL/min per kg as the threshold, the sensitivity for PH was only 10.0%. Detection of PH in patients with congenital heart disease by CPET is difficult because of many falsely positive tests. However, a peak oxygen uptake higher than 25.2 mL/min per kg makes the diagnosis of PH unlikely. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

[Respiratory infections, Down's syndrome and congenital heart disease: the CIVIC 21 study].

PubMed

Medrano López, C; García-Guereta Silva, L; Lirio Casero, J; García Pérez, J

2009-07-01

We compare hospitalisation rates for acute respiratory tract infection in children younger than 24 months with significant congenital heart disease without Down's syndrome with those with Down's syndrome with or without congenital heart disease. This was an epidemiological, multicentre (53 Spanish hospitals), observational and prospective study, from October 2006 to April 2007. A total of 1085 patients were followed-up, of which 806 did not have Down's syndrome and 279 with Down's syndrome: 135 with significant, 38 with non significant and 105 without congenital heart disease. A total of 147 patients (13.1%; 95% CI, 11.2-15.2%) required hospitalisation due to respiratory infection. Rates in patients without and with Down's syndrome were 11% vs 19.1%. In the Down's group, 26.3% had no significant, a 23% had significant and 11.4% had no congenital heart disease. The main diagnosis was bronchiolitis (59.4%). An infectious agent was found in 36.2% cases. The specific admission rate due to respiratory syncytial virus was 4.4%, with differences in children without vs with Down's syndrome (3.2% vs 7.8%). In the Down's patients we found rates of 15.8%, 9.3% and 3% in the non-significant, significant and no congenital heart disease. Immunoprophylaxis against respiratory syncytial virus rates were 83.4% vs 39.9% in no Down's versus Down's syndrome patients. No differences were found in hospital stay or the severity. Hospital admission rates due to respiratory infection, and specifically respiratory syncytial virus, were higher in the Down's patients, particularly in the group with no significant congenital heart disease and low immunoprophylaxis against respiratory syncytial virus.

Assessment of the role of copy-number variants in 150 patients with congenital heart defects.

PubMed

Derwińska, Katarzyna; Bartnik, Magdalena; Wiśniowiecka-Kowalnik, Barbara; Jagła, Mateusz; Rudziński, Andrzej; Pietrzyk, Jacek J; Kawalec, Wanda; Ziółkowska, Lidia; Kutkowska-Kaźmierczak, Anna; Gambin, Tomasz; Sykulski, Maciej; Shaw, Chad A; Gambin, Anna; Mazurczak, Tadeusz; Obersztyn, Ewa; Bocian, Ewa; Stankiewicz, Paweł

2012-01-01

Congenital heart defects are the most common group of major birth anomalies and one of the leading causes of infant deaths. Mendelian and chromosomal syndromes account for about 20% of congenital heart defects and in some cases are associated with other malformations, intellectual disability, and/or dysmorphic features. The remarkable conservation of genetic pathways regulating heart development in animals suggests that genetic factors can be responsible for a significantly higher percentage of cases. Assessment of the role of CNVs in the etiology of congenital heart defects using microarray studies. Genome-wide array comparative genomic hybridization, targeting genes known to play an important role in heart development or responsible for abnormal cardiac phenotype was used in the study on 150 patients. In addition, we have used multiplex ligation-dependent probe amplification specific for chromosome 22q11.2 region. We have identified 21 copy-number variants, including 13 known causative recurrent rearrangements (12 deletions 22q11.2 and one deletion 7q11.23), three potentially pathogenic duplications (5q14.2, 15q13.3, and 22q11.2), and five variants likely benign for cardiac anomalies. We suggest that abnormal copy-number of the ARRDC3 and KLF13 genes can be responsible for heart defects. Our study demonstrates that array comparative genomic hybridization enables detection of clinically significant chromosomal imbalances in patients with congenital heart defects.

Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children.

PubMed

Iannucci, Glen J; Adisa, Olufolake A; Oster, Matthew E; McConnell, Michael; Mahle, William T

2016-12-01

Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction. The surviving patient had developmental delays. On the basis of this series, we suggest mitigating hypoxemia, and thus the risk of stroke, in patients who have sickle cell disease and cyanotic congenital heart disease. Potential therapies include chronic blood transfusions, hydroxyurea, earlier surgical correction to reduce the duration of hypoxemia, and heart or bone marrow transplantation.

Impact of Telemedicine in the Screening for Congenital Heart Disease in a Center from Northeast Brazil.

PubMed

de Araújo, Juliana Sousa Soares; Regis, Cláudio Teixeira; Gomes, Renata Grigório Silva; Mourato, Felipe Alves; Mattos, Sandra da Silva

2016-12-01

To describe the incidence of congenital heart disease before and after the establishment of a telemedicine screening program, in a reference center from Northeast Brazil. This is a descriptive, retrospective and comparative study based on the institutional data from a reference center in perinatology for a period of 16 years. Institutional data were collected from a 16-year period (2001-15). Data were divided into two periods: prior to (2001-11) and after (2012-15) the establishment of a telemedicine screening program. After the implementation of the screening process, almost all kinds of heart disease showed a significant increase in their incidence (p  <  0.05). With this, the incidence of major heart diseases approached those specified in developed regions. The implementation of a screening process model for congenital heart diseases can change the context of patients with congenital heart diseases in poor regions. © The Author [2016]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Outpatient nursing clinic for congenital heart disease patients: Copenhagen Transition Program.

PubMed

Berg, Selina Kikkenborg; Hertz, Pernille Grarup

2007-01-01

Adolescents with congenital heart disease need support transitioning from childhood to adulthood. To become independent, adolescents need to possess knowledge about the disease and how to handle life with congenital heart disease. Outpatient nursing clinics can address issues relevant for adolescents. Creating an environment in which worries and questions can be discussed better prepares adolescents to meet the struggles of living with congenital heart disease. The Copenhagen Transition Program was established in 2004. This outpatient nursing clinic was established by (1) defining the target group, (2) conducting a literature review, (3) collaborating with interdisciplinary colleagues, (4) scheduling visits to the transition-nursing clinic, (5) arranging clinic visits, (6) charting, and (7) testing and evaluating the clinic. Adolescents with congenital heart disease are invited to a consultation with a specially trained nurse, and it is the patients themselves who decide the content of the consultation. Topics such as knowledge about the disease, endocarditis, acute situations, nutrition, contraception, alcohol/drugs, smoking, physical activity, sleep/rest, and education may be discussed. Parents are asked not to participate.

Recreational scuba diving in patients with congenital heart disease: Time for new guidelines.

PubMed

Schleich, Jean-Marc; Schnell, Frédéric; Brouant, Benoît; Phan, Gerald; Lafay, Vincent; Bonnemains, Laurent; Bédossa, Marc

2016-01-01

The number of recreational scuba divers is steadily increasing. In its latest recommendations, the French Federation of Undersea Studies and Sports listed congenital heart disease as a formal and final contraindication to scuba diving. On the other hand, with the progress made in their management, the prognosis and quality of life of patients with congenital heart diseases have improved considerably, enabling them to engage in physical and sports endeavours, which are known to confer general health and psychological benefits. As a consequence, the ability of these patients to dive has become a regular and recurrent issue. We review the various types of scuba diving, the physical performance required for its practice, its effects on cardiovascular function and the elements that need to be considered before recommending whether it can be practiced safely at various levels of difficulty. Because of the diversity and broad heterogeneity of congenital heart diseases, a detailed evaluation of each patient's performance based on clinical criteria common to all congenital heart diseases is recommended. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Case Report: First Reported Combined Heart-Liver Transplant in a Patient With a Congenital Solitary Kidney.

PubMed

Hanna, R M; Kamgar, M; Hasnain, H; Khorsan, R; Nsair, A; Kaldas, F; Baas, A; Bunnapradist, S; Wilson, J M

2018-04-01

We report a case of successful combined heart liver transplant in a patient with a congenital solitary kidney. The patient had normal renal function before combined heart-liver transplantation and developed acute kidney injury requiring slow continuous dialysis and subsequent intermittent dialysis for almost 8 weeks post transplantation. Her renal function recovered and she remains off dialysis now 7 months post transplantation. She only currently has mild chronic renal insufficiency. We believe this is the first reported case of successful heart liver transplant in a patient with a congenital solitary kidney. Published by Elsevier Inc.

Right upper limb bud triplication and polythelia, left sided hemihypertrophy and congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis: disorganisation-like spectrum or patterning gene defect?

PubMed Central

Sabry, M A; al-Saleh, Q; al-Saw'an, R; al-Awadi, S A; Farag, T I

1995-01-01

A Somali female baby with right upper limb triplication, polythelia, left sided hemihypertrophy, congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis is described. It seems that the above described pattern of anomalies has not been reported before. The possible developmental genetic mechanism responsible for this phenotype is briefly discussed. Images PMID:7562971

Neurodevelopmental Outcomes in Congenital Heart Disease

MedlinePlus

... school and independent living, such as attention, organization, social interaction, coordination, and self-care. The American Heart Association has described categories of children at high risk for neurodevelopmental impairment (see the Table ) and recommends these children be formally evaluated with ... Media Neurodevelopmental Outcomes in Congenital Heart Disease Caitlin ...

Bioengineering and Stem Cell Technology in the Treatment of Congenital Heart Disease

PubMed Central

Bosman, Alexis; Edel, Michael J.; Blue, Gillian; Dilley, Rodney J.; Harvey, Richard P.; Winlaw, David S.

2015-01-01

Congenital heart disease places a significant burden on the individual, family and community despite significant advances in our understanding of aetiology and treatment. Early research in ischaemic heart disease has paved the way for stem cell technology and bioengineering, which promises to improve both structural and functional aspects of disease. Stem cell therapy has demonstrated significant improvements in cardiac function in adults with ischaemic heart disease. This finding, together with promising case studies in the paediatric setting, demonstrates the potential for this treatment in congenital heart disease. Furthermore, induced pluripotent stems cell technology, provides a unique opportunity to address aetiological, as well as therapeutic, aspects of disease. PMID:26239354

Rare association of anophthalmia, complex congenital heart disease and pulmonary hypertension: case report.

PubMed

Ríos-Méndez, Raúl Enrique; Lozano Chinga, Michell Marola

2016-10-07

Clinical congenital anophthalmia is described as the uni- or bilateral absence of the eyeball that might occur in isolation or as part of a syndrome. It has a very low prevalence and its etiology is heterogeneous. Complex congenital cardiac malformations are also rare. The association of congenital anophthalmia and congenital heart disease is rarer still, and the etiology of those associations is not well understood yet. We report the case of a patient who had the very rare association of bilateral anophthalmia, multiple cardiac malformations and severe pulmonary hypertension.

Infective Endocarditis

MedlinePlus

... material occurs within six months after the procedure. Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

Prenatal screening for major congenital heart disease: assessing performance by combining national cardiac audit with maternity data.

PubMed

Gardiner, Helena M; Kovacevic, Alexander; van der Heijden, Laila B; Pfeiffer, Patricia W; Franklin, Rodney Cg; Gibbs, John L; Averiss, Ian E; Larovere, Joan M

2014-03-01

Determine maternity hospital and lesion-specific prenatal detection rates of major congenital heart disease (mCHD) for hospitals referring prenatally and postnatally to one Congenital Cardiac Centre, and assess interhospital relative performance (relative risk, RR). We manually linked maternity data (3 hospitals prospectively and another 16 retrospectively) with admissions, fetal diagnostic and surgical cardiac data from one Congenital Cardiac Centre. This Centre submits verified information to National Institute for Cardiovascular Outcomes Research (NICOR-Congenital), which publishes aggregate antenatal diagnosis data from infant surgical procedures. We included 120 198 unselected women screened prospectively over 11 years in 3 maternity hospitals (A, B, C). Hospital A: colocated with fetal medicine, proactive superintendent, on-site training, case-review and audit, hospital B: on-site training, proactive superintendent, monthly telemedicine clinics, and hospital C: sonographers supported by local obstetrician. We then studied 321 infants undergoing surgery for complete transposition (transposition of the great arteries (TGA), n=157) and isolated aortic coarctation (CoA, n=164) screened in hospitals A, B, C prospectively, and 16 hospitals retrospectively. 385 mCHD recorded prospectively from 120 198 (3.2/1000) screened women in 3 hospitals. Interhospital relative performance (RR) in Hospital A:1.68 (1.4 to 2.0), B:0.70 (0.54 to 0.91), C:0.65 (0.5 to 0.8). Standardised prenatal detection rates (funnel plots) demonstrating inter-hospital variation across 19 hospitals for TGA (37%, 0.00 to 0.81) and CoA (34%, 0.00 to 1.06). Manually linking data sources produced hospital-specific and lesion-specific prenatal mCHD detection rates. More granular, rather than aggregate, data provides meaningful feedback to improve screening performance. Automatic maternal and infant record linkage on a national scale, requires verified, prospective maternity audit and integration of health information systems.

Subclinical hypothyroidism: A common finding in adult patients with cyanotic congenital heart disease.

PubMed

Bak, Peter; Hjortshøj, Cristel S; Gaede, Peter; Idorn, Lars; Søndergaard, Lars; Jensen, Annette S

2018-03-01

Cyanotic congenital heart disease is a systemic disease, with effects on multiple organ systems. A high prevalence of subclinical hypothyroidism (SCH) has been reported in a small cohort of cyanotic congenital heart disease patients. Subclinical hypothyroidism has been associated with various adverse cardiovascular effects, as well as an increased risk of progression to overt hypothyroidism. The aim of this study was to examine the prevalence of SCH in cyanotic congenital heart disease patients, consider possible etiologies, and evaluate thyroid function over time. First, 90 clinically stable cyanotic congenital heart disease patients were examined with blood samples (thyroid-stimulating hormone, C-reactive protein, hemoglobin, hematocrit, and N-terminal pro-brain-natriuretic peptide) in a cross-sectional descriptive study. Second, a longitudinal follow-up study of 43 patients originating from the first study part, was carried out. These patients had thyroid function parameters (thyroid-stimulating hormone, thyroid hormones, and thyroid peroxidase antibodies) evaluated biannually. Elevated thyroid-stimulating hormone was present in 24% of the 90 screened patients. During follow-up (6.5 ± 1.0 years), SCH (defined as ≥2 consecutive elevated thyroid-stimulating hormone values) was present in 26%. Three patients progressed to overt hypothyroidism. Patients with SCH were younger (34 ± 12 vs 42 ± 16 years; P = .01) and had a lower oxygen saturation (80 ± 5 vs 84 ± 6%; P = .03). Subclinical hypothyroidism is a very common finding in cyanotic congenital heart disease. This is not associated with increased levels of C-reactive protein, heart failure, or autoimmunity but appears to be associated with cyanosis and age. Since the clinical impact of SCH is uncertain, further studies are needed to determine this. Regular thyroid evaluation is recommended in cyanotic congenital heart disease patients since SCH can develop to overt hypothyroidism. © 2017 Wiley Periodicals, Inc.

CONGENITAL HEART MALFORMATIONS IN NEWBORN BABIES WITH LOW BIRTH WEIGHT.

PubMed

Luca, Alina-Costina; Holoc, Andreea-Simona; Iordache, C

2015-01-01

Congenital heart malformations represent a public health problem, holding a significant percentage of the total of heart diseases. Beside the elevated frequency of the malformations, we also notice their occurrence in newborn babies with low birth weight, increasing, thus, the risk of complications and late therapeutic approach. The goal of the study was to highlight the general and particular aspects of cardiovascular malformations epidemiology in newborn babies with low weight at birth, the correlation of the malformations with implied genetic and environmental factors, assessing the complications and their procedures on the therapeutic management. Our study was performed on a group of 271 patients, hospitalized in the Department of Pediatric Cardiology of "Sf. Maria" Emergency Clinical Hospital for Children of Iasi, during January 2011-December 2013. The patients were assessed based on anamnesis, clinical, biological and imagistic exam. The study lot was divided according to the type of the structural defect: 95% of the patients were diagnosed with non-cyanogenic congenital heart malformations and 5% with cyanogenic congenital heart malformations. Regarding the patient's origin background, we notice an elevated frequency of the rural environment (71%). The incidence of the malformations was high in premature low birth weight (48%), followed by premature very low birth weight (22%). In evolution, congenital heart malformations often get more complicated heart failure, arterial hypertension and respiratory infections being most often met. Mortality was maximum in the first year of life, a third of the cases being associated with chromosomal malformations. Congenital heart malformations in newborn patients with low weight at birth represented an elevated percentage of 44.13% of the total of the cases hospitalized for cardiovascular diseases from the Department of Pediatric Cardiology of Iasi. Many cases were associated with other congenital malformations or different complications, being necessary an interdisciplinary collaboration to adequately monitor the anatom5ical and functional parameters and to ensure a somatic and mental development as normal as possible.

Genetic and flow anomalies in congenital heart disease.

PubMed

Rugonyi, Sandra

2016-01-01

Congenital heart defects are the most common malformations in humans, affecting approximately 1% of newborn babies. While genetic causes of congenital heart disease have been studied, only less than 20% of human cases are clearly linked to genetic anomalies. The cause for the majority of the cases remains unknown. Heart formation is a finely orchestrated developmental process and slight disruptions of it can lead to severe malformations. Dysregulation of developmental processes leading to heart malformations are caused by genetic anomalies but also environmental factors including blood flow. Intra-cardiac blood flow dynamics plays a significant role regulating heart development and perturbations of blood flow lead to congenital heart defects in animal models. Defects that result from hemodynamic alterations, however, recapitulate those observed in human babies, even those due to genetic anomalies and toxic teratogen exposure. Because important cardiac developmental events, such as valve formation and septation, occur under blood flow conditions while the heart is pumping, blood flow regulation of cardiac formation might be a critical factor determining cardiac phenotype. The contribution of flow to cardiac phenotype, however, is frequently ignored. More research is needed to determine how blood flow influences cardiac development and the extent to which flow may determine cardiac phenotype.

The Adult Congenital and Pediatric Cardiology Section: increasing the opportunities for the congenital heart disease community within the American College of Cardiology.

PubMed

Martin, Gerard R; Mitchell, Stephanie; Beekman, Robert H; Feinstein, Jeffrey A; Jenkins, Kathy J; Landzberg, Michael; Webb, Gary

2012-01-03

The Adult Congenital and Pediatric Cardiology (AC/PC) Section was established to develop a clear voice within the American College of Cardiology and address the myriad issues facing the congenital heart disease profession. The Section is governed by the AC/PC Council, which includes pediatric cardiologists, adult congenital cardiologists, a cardiac care associate, and a fellow-in-training member. The Council is responsible for bidirectional communication between the College's Board of Trustees and the AC/PC Section members. Since its founding in 2004, Section objectives have been defined by the College's mission: to advocate for quality cardiovascular care through education, research promotion, and the development and application of standards and guidelines and to influence health care policy. The pillars of the College-advocacy, quality, education, and member engagement-serve as the defining template for the Section's strategy. The Section has developed work groups in advocacy, clinical practice, education and training, quality, and publications. A separate leadership group has been developed for adult congenital heart disease. Work groups are open to all Section members. Recognition of the importance of lifelong care in congenital heart disease led Section leaders to incorporate pediatric cardiology and adult congenital heart disease content into each of the work groups. There are more than 1,200 Section members, with nearly 400 members actively contributing to Section activities. This article outlines Section efforts to date and highlights significant successes to date. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Dynamics in prevalence of Down syndrome in children with congenital heart disease.

PubMed

Pfitzer, Constanze; Helm, Paul C; Rosenthal, Lisa-Maria; Berger, Felix; Bauer, Ulrike M M; Schmitt, Katharina Rl

2018-01-01

We assessed the dynamics in the prevalence of children with congenital heart disease (CHD) and Down syndrome in Germany with regard to phenotype, severity, and gender. Data from patients with CHD and Down syndrome born between 1980 and 2014 were analyzed, who are registered with the German National Register for Congenital Heart Defects. One thousand six hundred eighteen CHD patients with Down syndrome were identified. The prevalence of children born with both Down syndrome and CHD was constant from 2005 to 2009 but increased from 2010 to 2014. Regarding CHD groups, complex and simple lesions have become more equal since 2005. The number of simple lesions with shunt has a peak prevalence in the period of 2010-2014. Atrioventricular septal defect was the most common CHD phenotype, but temporal changes were found within the group of CHD phenotypes over the observation period. Our findings suggest a growing number of CHD and Down syndrome, which may be the result of improved medical management and progress in educational, social, and financial support. This development is noteworthy as it adds new aspects to present discussions in the media and political settings. What is known: • Congenital heart disease is regarded to be the most important clinical phenomenon in children with Down syndrome, due to its significant impact on morbidity and mortality. • New developments in prenatal diagnostic and therapy management of congenital heart disease continue to influence the number of patients diagnosed with congenital heart disease and Down syndrome. What is New: • This study provides essential data giving the first overview of the dynamics in the prevalence of congenital heart disease and Down syndrome over an extended length of time up to 2015 in a large patient cohort, taking recent developments into account. • Our data suggest a growing prevalence of congenital heart disease and Down syndrome, which may be the result of improved medical management for Down syndrome patients and progress in educational, social, and financial support for their families; this development is noteworthy as it adds new aspects to the present discussion in the media and political settings.

Changing Landscape of Congenital Heart Disease.

PubMed

Bouma, Berto J; Mulder, Barbara J M

2017-03-17

Congenital heart disease is the most frequently occurring congenital disorder affecting ≈0.8% of live births. Thanks to great efforts and technical improvements, including the development of cardiopulmonary bypass in the 1950s, large-scale repair in these patients became possible, with subsequent dramatic reduction in morbidity and mortality. The ongoing search for progress and the growing understanding of the cardiovascular system and its pathophysiology refined all aspects of care for these patients. As a consequence, survival further increased over the past decades, and a new group of patients, those who survived congenital heart disease into adulthood, emerged. However, a large range of complications raised at the horizon as arrhythmias, endocarditis, pulmonary hypertension, and heart failure, and the need for additional treatment became clear. Technical solutions were sought in perfection and creation of new surgical techniques by developing catheter-based interventions, with elimination of open heart surgery and new electronic devices enabling, for example, multisite pacing and implantation of internal cardiac defibrillators to prevent sudden death. Over time, many pharmaceutical studies were conducted, changing clinical treatment slowly toward evidence-based care, although results were often limited by low numbers and clinical heterogeneity. More attention has been given to secondary issues like sports participation, pregnancy, work, and social-related difficulties. The relevance of these issues was already recognized in the 1970s when the need for specialized centers with multidisciplinary teams was proclaimed. Finally, research has become incorporated in care. Results of intervention studies and registries increased the knowledge on epidemiology of adults with congenital heart disease and their complications during life, and at the end, several guidelines became easily accessible, guiding physicians to deliver care appropriately. Over the past decades, the landscape of adult congenital heart disease has changed dramatically, which has to be continued in the future. © 2017 American Heart Association, Inc.

Congenital Heart Defects in Adults : A Field Guide for Cardiologists

PubMed Central

Romfh, Anitra; Pluchinotta, Francesca Romana; Porayette, Prashob; Valente, Anne Marie; Sanders, Stephen P.

2013-01-01

Advances in cardiology and cardiac surgery allow a large proportion of patients with congenital heart defects to survive into adulthood. These patients frequently develop complications characteristic of the defect or its treatment. Consequently, adult cardiologists participating in the care of these patients need a working knowledge of the more common defects. Occasionally, patients with congenital heart defects such as atrial septal defect, Ebstein anomaly or physiologically corrected transposition of the great arteries present for the first time in adulthood. More often patients previously treated in pediatric cardiology centers have transitioned to adult congenital heart disease centers for ongoing care. Some of the more important defects in this category are tetralogy of Fallot, transposition of the great arteries, functionally single ventricle defects, and coarctation. Through this field guide, we provide an overview of the anatomy of selected defects commonly seen in an adult congenital practice using pathology specimens and clinical imaging studies. In addition, we describe the physiology, clinical presentation to the adult cardiologist, possible complications, treatment options, and outcomes. PMID:24294540

The effect of preoperative nutritional status on postoperative outcomes in children undergoing surgery for congenital heart defects in San Francisco (UCSF) and Guatemala City (UNICAR).

PubMed

Radman, Monique; Mack, Ricardo; Barnoya, Joaquin; Castañeda, Aldo; Rosales, Monica; Azakie, Anthony; Mehta, Nilesh; Keller, Roberta; Datar, Sanjeev; Oishi, Peter; Fineman, Jeffrey

2014-01-01

The objective of this study was to determine the association between preoperative nutritional status and postoperative outcomes in children undergoing surgery for congenital heart defects (CHD). Seventy-one patients with CHD were enrolled in a prospective, 2-center cohort study. We adjusted for baseline risk differences using a standardized risk adjustment score for surgery for CHD. We assigned a World Health Organization z score for each subject's preoperative triceps skin-fold measurement, an assessment of total body fat mass. We obtained preoperative plasma concentrations of markers of nutritional status (prealbumin, albumin) and myocardial stress (B-type natriuretic peptide [BNP]). Associations between indices of preoperative nutritional status and clinical outcomes were sought. Subjects had a median (interquartile range [IQR]) age of 10.2 (33) months. In the University of California at San Francisco (UCSF) cohort, duration of mechanical ventilation (median, 19 hours; IQR, 29 hours), length of intensive care unit stay (median, 5 days; IQR 5 days), duration of any continuous inotropic infusion (median, 66 hours; IQR 72 hours), and preoperative BNP levels (median, 30 pg/mL; IQR, 75 pg/mL) were associated with a lower preoperative triceps skin-fold z score (P < .05). Longer duration of any continuous inotropic infusion and higher preoperative BNP levels were also associated with lower preoperative prealbumin (12.1 ± 0.5 mg/dL) and albumin (3.2 ± 0.1; P < .05) levels. Lower total body fat mass and acute and chronic malnourishment are associated with worse clinical outcomes in children undergoing surgery for CHD at UCSF, a resource-abundant institution. There is an inverse correlation between total body fat mass and BNP levels. Duration of inotropic support and BNP increase concomitantly as measures of nutritional status decrease, supporting the hypothesis that malnourishment is associated with decreased myocardial function. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

PubMed

Hornik, Christoph P; Collins, Ronnie Thomas; Jaquiss, Robert D B; Jacobs, Jeffrey P; Jacobs, Marshall L; Pasquali, Sara K; Wallace, Amelia S; Hill, Kevin D

2015-06-01

Patients with Williams syndrome (WS) undergoing cardiac surgery are at risk for major adverse cardiac events (MACE). Prevalence and risk factors for such events have not been well described. We sought to define frequency and risk of MACE in patients with WS using a multicenter clinical registry. We identified cardiac operations performed in patients with WS using the Society of Thoracic Surgeons Congenital Heart Surgery Database (2000-2012). Operations were divided into 4 groups: isolated supravalvular aortic stenosis, complex left ventricular outflow tract (LVOT), isolated right ventricular outflow tract (RVOT), and combined LVOT/RVOT procedures. The proportion of patients with MACE (in-hospital mortality, cardiac arrest, or postoperative mechanical circulatory support) was described and the association with preoperative factors was examined. Of 447 index operations (87 centers), median (interquartile range) age and weight at surgery were 2.4 years (0.6-7.4 years) and 10.6 kg (6.5-21.5 kg), respectively. Mortality occurred in 20 patients (5%). MACE occurred in 41 patients (9%), most commonly after combined LVOT/RVOT (18 out of 87; 21%) and complex LVOT (12 out of 131; 9%) procedures, but not after isolated RVOT procedures. Odds of MACE decreased with age (odds ratio [OR], 0.99; 95% confidence interval [CI], 0.98-0.99), weight (OR, 0.97; 95% CI, 0.93-0.99), but increased in the presence of any preoperative risk factor (OR, 2.08; 95% CI, 1.06-4.00), and in procedures involving coronary artery repair (OR, 5.37; 95% CI, 2.05-14.06). In this multicenter analysis, MACE occurred in 9% of patients with WS undergoing cardiac surgery. Demographic and operative characteristics were associated with risk. Further study is needed to elucidate mechanisms of MACE in this high-risk population. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Sleep-Disordered Breathing in Patients with Pulmonary Valve Incompetence Complicating Congenital Heart Disease.

PubMed

Miles, Susan; Ahmad, Waheed; Bailey, Amy; Hatton, Rachael; Boyle, Andrew; Collins, Nicholas

2016-12-01

Long standing pulmonary regurgitation results in deleterious effects on right heart size and function with late consequences of right heart volume overload including ventricular dilatation, propensity to arrhythmia and right heart failure. As sleep disordered breathing may predispose to elevations in pulmonary vascular resistance and associated negative effects on right ventricular function, we sought to assess this in patients with underlying congenital heart disease. We performed a pilot study to evaluate the incidence of sleep-disordered breathing in a patient population with a history of long standing pulmonary valve incompetence in patients with congenital heart disease using overnight oximetry. Patients with a background of tetralogy of Fallot repair or residual pulmonary incompetence following previous pulmonary valve intervention for congenital pulmonary stenosis were included. Twenty-two patients underwent overnight oximetry. The mean age of the cohort was 34.3 ± 15.2 years with no patients observed to have severe underlying pulmonary hypertension. Abnormal overnight oximetry was seen in 13/22 patients (59.1%) with 2/22 (9.1%) patients considered to have severe abnormalities. An important proportion of patients with a background of pulmonary incompetence complicating congenital heart disease are prone to the development of sleep-disordered breathing as assessed by overnight oximetry. Further study into the prevalence and mechanisms of sleep-disordered breathing in a larger cohort are warranted. © 2016 Wiley Periodicals, Inc.

Risk of thromboembolic complications in adult congenital heart disease: A literature review.

PubMed

Karsenty, Clement; Zhao, Alexandre; Marijon, Eloi; Ladouceur, Magalie

2018-05-30

Adult congenital heart disease (ACHD) is a constantly expanding population with challenging issues. Initial medical and surgical treatments are seldom curative, and the majority of patients still experience late sequelae and complications, especially thromboembolic events. These common and potentially life-threating adverse events are probably dramatically underdiagnosed. Better identification and understanding of thromboembolic risk factors are essential to prevent long-term related morbidities. In addition to specific situations associated with a high risk of thromboembolic events (Fontan circulation, cyanotic congenital heart disease), atrial arrhythmia has been recognized as an important risk factor for thromboembolic events in ACHD. Unlike in patients without ACHD, thromboembolic risk stratification scores, such as the CHA 2 DS 2 -VASc score, may not be applicable in ACHD. Overall, after a review of the scientific data published so far, it is clear that the complexity of the underlying congenital heart disease represents a major risk factor for thromboembolic events. As a consequence, prophylactic anticoagulation is indicated in patients with complex congenital heart disease and atrial arrhythmia, regardless of the other risk factors, as opposed to simple heart defects. The landscape of ACHD is an ongoing evolving process, and specific thromboembolic risk scores are needed, especially in the setting of simple heart defects; these should be coupled with specific trials or long-term follow-up of multicentre cohorts. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Isolated Major Aortopulmonary Collateral as the Sole Pulmonary Blood Supply to an Entire Lung Segment.

PubMed

Kim, Hannah S; Grady, R Mark; Shahanavaz, Shabana

2017-01-01

Congenital systemic-to-pulmonary collateral arteries or major aortopulmonary collaterals are associated with cyanotic congenital heart disease with decreased pulmonary blood flow. Though it is usually associated with congenital heart diseases, there is an increased incidence of isolated acquired aortopulmonary collaterals in premature infants with chronic lung disease. Interestingly, isolated congenital aortopulmonary collaterals can occur without any lung disease, which may cause congestive heart failure and require closure. We present a neonate with an echocardiogram that showed only left-sided heart dilation. Further workup with a CT angiogram demonstrated an anomalous systemic artery from the descending thoracic aorta supplying the left lower lobe. He eventually developed heart failure symptoms and was taken to the catheterization laboratory for closure of the collateral. However, with the collateral being the only source of blood flow to the entire left lower lobe, he required surgical unifocalization. Isolated aortopulmonary collaterals without any other congenital heart disease or lung disease are rare. Our patient is the first reported case to have an isolated aortopulmonary collateral being the sole pulmonary blood supply to an entire lung segment. Due to its rarity, there is still much to learn about the origin and development of these collaterals that possibly developed prenatally.

CHD's Impact on Females

MedlinePlus

... the test around the 18th week of pregnancy. Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

Single Ventricle Defects

MedlinePlus

... Your Risk • Symptoms & Diagnosis • Care & Treatment • Tools & Resources Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

[Medical and surgical health care for congenital heart disease: a panoramic vision of the reality in Mexico. Inquiry 2009].

PubMed

Calderón-Colmenero, Juan; De-la-Llata, Manuel; Vizcaíno, Alfredo; Ramírez, Samuel; Bolio, Alejandro

2011-01-01

The only way to characterize the Mexican problem related to congenital heart disease care is promoting the creation of a national database for registering the organization, resources, and related activities. The Health Secretary of Mexico adopted a Spanish registration model to design a survey for obtaining a national Mexican reference in congenital heart disease. This survey was distributed to all directors of medical and/or surgical health care centers for congenital heart disease in Mexico. This communication presents the results obtained in relation to organization, resources and activities performed during the last year 2009. From the 22 health care centers which answered the survey 10 were reference centers (45%) and 12 were assistant centers (55%). All of them are provided with cardiologic auxiliary diagnostic methods. Except one, all centers have at least one bidimentional echocardiography apparatus. There is a general deficit between material and human resources detected in our study. Therapeutic actions for congenital heart disease (70% surgical and 30% therapeutical interventionism) show a clear centralization tendency for this kind of health care in Mexico City, Monterrey and finally Guadalajara. Due to the participation of almost all cardiac health centers in Mexico, our study provides an important information related to organization, resources, and medical and/or surgical activities for congenital heart disease. The data presented not only show Mexican reality, but allows us to identify better the national problematic for establishing priorities and propose solution alternatives.

The effect of a resilience improvement program for adolescents with complex congenital heart disease.

PubMed

Lee, Sunhee; Lee, Junga; Choi, Jae Young

2017-04-01

Adolescents with congenital heart disease need to increase their resilience in the face of challenges in order to preserve their health and quality of life. This study aimed to develop a resilience improvement program for adolescents with congenital heart disease and also to evaluate any change in resilience and quality of life as a measure of the effectiveness of the resilience improvement programs. A nonequivalent control group pretest-posttest study was designed. Twenty-five adolescents who attended the first resilience improvement program were included in the experimental group, and 31 adolescents who took part in the second program were placed in the control group. Adolescents with congenital heart disease completed a self-report questionnaire on three separate occasions: the pretest, the first posttest and the second posttest. The self-report questionnaire included general characteristics and instruments to measure resilience and quality of life. For the longitudinal analysis, generalized estimating equations were used to evaluate the difference in the estimated average trajectories of resilience and quality of life changes. Independent predictors of resilience improvement in adolescents with congenital heart disease were the experimental group ( p=0.02) and middle and high school students ( p=0.02). Quality of life was not associated with membership in the experimental group. However, males scored higher than females on quality of life measures ( p=0.02). It is essential for healthcare providers to apply various programs, including those targeted at accepting illness, improving autonomy and independently managing disease, to adolescents with congenital heart disease.

Fitness to Fly Testing in Patients with Congenital Heart and Lung Disease.

PubMed

Spoorenberg, Mandy E; van den Oord, Marieke H A H; Meeuwsen, Ted; Takken, Tim

2016-01-01

During commercial air travel passengers are exposed to a low ambient cabin pressure, comparable to altitudes of 5000 to 8000 ft (1524 to 2438 m). In healthy passengers this causes a fall in partial pressure of oxygen, which results in relative hypoxemia, usually without symptoms. Patients with congenital heart or lung disease may experience more severe hypoxemia during air travel. This systematic review provides an overview of the current literature focusing on whether it is safe for patients with congenital heart or lung disease to fly. The Pubmed database was searched and all studies carried out at an (simulated) altitude of 5000-8000 ft (1524-2438 m) for a short time period (several hours) and related to patients with congenital heart or lung disease were reviewed. Included were 11 studies. These studies examined patients with cystic fibrosis, neonatal (chronic) lung disease and congenital (a)cyanotic heart disease during a hypoxic challenge test, in a hypobaric chamber, during commercial air travel, or in the mountains. Peripheral/arterial saturation, blood gases, lung function, and/or the occurrence of symptoms were listed. Based on the current literature, it can be concluded that air travel is safe for most patients. However, those at risk of hypoxia can benefit from supplemental in-flight oxygen. Therefore, patients with congenital heart and lung disease should be evaluated carefully prior to air travel to select the patients at risk for hypoxia using the current studies and guidelines.

Ventricular assist device use in single ventricle congenital heart disease.

PubMed

Carlo, Waldemar F; Villa, Chet R; Lal, Ashwin K; Morales, David L

2017-11-01

As VAD have become an effective therapy for end-stage heart failure, their application in congenital heart disease has increased. Single ventricle congenital heart disease introduces unique physiologic challenges for VAD use. However, with regard to the mixed clinical results presented within this review, we suggest that patient selection, timing of implant, and center experience are all important contributors to outcome. This review focuses on the published experience of VAD use in single ventricle patients and details physiologic challenges and novel approaches in this growing pediatric and adult population. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Health-related quality of life of mothers of children with congenital heart disease in a sub-Saharan setting: cross-sectional comparative study.

PubMed

Sileshi, Lidia; Tefera, Endale

2017-10-26

While the Health Related Quality of Life of the children with congenital heart defects is primarily affected, caring for a child with birth defect has an impact on the family's quality of life as well. Understanding the level of quality of life of the parents, which is likely to vary in different cultural settings, beliefs and parental educational status may help to implement educational programs and other interventional measures that may improve the HRQOL of parents of such children. This cross-sectional comparative study reports the health-related quality of life of mothers of children with congenital heart diseases in a sub-Saharan setting. Mean age of the mothers in the study group was 32.2 ± 7.1 years where as that of the control group was 30.5 ± 6.5 years (p = .054). One hundred-four children had congenital cardiac lesions classified as mild to moderate while 31 patients had severe lesions. On average, mothers in the study group showed poor performance on the Short Form-36 (SF-36) with statistically significant differences on all sub-scales including general health perception, physical functioning, role physical, role emotional, social functioning, bodily pain, vitality and mental health. Severity of the congenital heart defect was not associated with statistically significant difference in the health-related quality of life of the mothers. Mothers of children with congenital heart disease in our study have significantly lower quality of life in all domains of SF-36 compared to the control group. Planning and devising a strategy to support these mothers may need to be part of management and clinical care of children with congenital heart diseases.

No evidence for mosaic pathogenic copy number variations in cardiac tissue from patients with congenital heart malformations.

PubMed

Winberg, Johanna; Berggren, Håkan; Malm, Torsten; Johansson, Sune; Johansson Ramgren, Jens; Nilsson, Boris; Liedén, Agne; Nordenskjöld, Agneta; Gustavsson, Peter; Nordgren, Ann

2015-03-01

The aim of this study was to investigate if pathogenic copy number variations (CNVs) are present in mosaic form in patients with congenital heart malformations. We have collected cardiac tissue and blood samples from 23 patients with congenital heart malformations that underwent cardiac surgery and screened for mosaic gene dose alterations restricted to cardiac tissue using array comparative genomic hybridization (array CGH). We did not find evidence of CNVs in mosaic form after array CGH analysis. Pathogenic CNVs that were present in both cardiac tissue and blood were detected in 2/23 patients (9%), and in addition we found several constitutional CNVs of unclear clinical significance. This is the first study investigating mosaicism for CNVs in heart tissue compared to peripheral blood and the results do not indicate that pathogenic mosaic copy number changes are common in patients with heart malformations. Importantly, in line with previous studies, our results show that constitutional pathogenic CNVs are important factors contributing to congenital heart malformations. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Use of extracorporeal life support in patients with congenital heart disease.

PubMed

Delius, R E; Bove, E L; Meliones, J N; Custer, J R; Moler, F W; Crowley, D; Amirikia, A; Behrendt, D M; Bartlett, R H

1992-09-01

To review a large experience with extracorporeal life support in patients with congenital heart disease. To determine the major causes of mortality and morbidity in order to improve the results of using this technology in this patient population. Retrospective chart review. Twenty-five patients between the ages of 1 day and 8 yrs. These patients had congenital heart disease and were clinically felt to be at high risk for death caused by cardiac failure or by respiratory failure complicated by congenital heart disease. All patients in this report were placed on extracorporeal life support to allow recovery of myocardial or pulmonary function. Of these 25 patients, 52% were weaned from bypass support and 40% survived to discharge. Patients who were not weaned from extracorporeal life support characteristically suffered from irreversible neurologic injury, multiple organ failure, or bleeding complications. Only one patient died of irreversible cardiac failure. Extracorporeal life support can be useful in supporting patients with congenital heart disease with life-threatening cardiac or pulmonary failure. Improvements in limiting neurologic and bleeding complications may lead to improvements in the use of extracorporeal life support for this indication. However, prospective, randomized studies are needed to appreciate the role of extracorporeal life support in these patients.

Velo-Cardio-Facial syndrome and DiGeorge sequence with meningomyelocele and deletions of the 22q11 region

DOE Office of Scientific and Technical Information (OSTI.GOV)

Nickel, R.E.; Pillers, D.M.; Merkens, M.

Approximately 5% of children with neural tube defects (NTDs) have a congenital heart defect and/or cleft lip and palate. The cause of isolated meningomyelocele, congenital heart defects, or cleft lip and palate has been largely thought to be multifactorial. However, chromosomal, teratogenic, and single gene causes of combinations of NTDs with congenital heart defects and/or cleft lip and palate have been reported. We report on 3 patients with meningomyelocele, congenital heart defects, and 22q11 deletions. Two of the children had the clinical diagnosis of velo-cardio-facial syndrome (VCFS); both have bifid uvula. The third child had DiGeorge sequence (DGS). The associationmore » of NTDs with 22q11 deletion has not been reported previously. An accurate diagnosis of the 22q11 deletion is critical as this micro-deletion and its associated clinical problems is transmitted as an autosomal dominant trait due to the inheritance of the deletion-bearing chromosome. We recommend that all children with NTDs and congenital heart defects, with or without cleft palate, have cytogenetic and molecular studies performed to detect 22q11 deletions. 31 refs., 3 figs.« less

[Pregnancy and congenital heart disease].

PubMed

Manso, Begoña; Gran, Ferrán; Pijuán, Antonia; Giralt, Gemma; Ferrer, Queralt; Betrián, Pedro; Albert, Dimpna; Rosés, Ferrán; Rivas, Nuria; Parra, Montserrat; Girona, Josep; Farrán, Inmaculada; Casaldáliga, Jaume

2008-03-01

Since the creation of the Adult Congenital Heart Disease Units and of the High Obstetric Risk Units, there has been increasing interest in hemodynamic and obstetric outcomes in pregnant woman with congenital heart disease. Retrospective descriptive study of 56 women with congenital heart disease aged (mean [range]) 25 (18-40) years, who experienced a total of 84 pregnancies between January 1992 and August 2006. The women were divided into three pregnancy risk groups: A, low-risk; B, moderate-risk, and C, high-risk. The incidence of complications during pregnancy was 1.6%, 15%, and 20% in groups A, B, and C, respectively; the incidence during the puerperium was 2%, 23%, and 50%, respectively; and maternal mortality was 0%, 7.6%, and 25%, respectively. Overall, 69 children were born, and the prematurity rates in the three groups were 11%, 15%, and 100%, respectively. The following risk factors were studied: pulmonary hypertension, cyanosis, arrhythmia, left ventricular outflow tract obstruction, right ventricular dilatation, systemic right ventricle, and anticoagulation therapy. The risk factor most significantly associated with maternal or fetal morbidity or mortality was found to be pulmonary hypertension. Risk stratification in pregnant women with congenital heart disease provides prognostic information that can help multidisciplinary teams to target care to achieve the best results.

Novel de novo pathogenic variant in the NR2F2 gene in a boy with congenital heart defect and dysmorphic features.

PubMed

Upadia, Jariya; Gonzales, Patrick R; Robin, Nathaniel H

2018-04-16

The NR2F2 gene plays an important role in angiogenesis and heart development. Moreover, this gene is involved in organogenesis in many other organs in mouse models. Variants in this gene have been reported in a number of patients with nonsyndromic atrioventricular septal defect, and in one patient with congenital heart defect and dysmorphic features. Here we report an 11-month-old Caucasian male with global developmental delay, dysmorphic features, coarctation of the aorta, and ventricular septal defect. He was later found to have a pathogenic mutation in the NR2F2 gene by whole exome sequencing. This is the second instance in which an NR2F2 mutation has been identified in a child with a congenital heart defect and other anomalies. This case suggests that some variants in NR2F2 may cause syndromic forms of congenital heart defect. © 2018 Wiley Periodicals, Inc.

Drug Therapy in Adult Congenital Heart Disease.

PubMed

Contractor, Tahmeed; Levin, Vadim; Mandapati, Ravi

2017-06-01

Adults with congenital heart disease are at risk for atrial and ventricular arrhythmias that can lead to an increased morbidity as well as mortality. When catheter ablation is not an option or unsuccessful, antiarrhythmic drugs are the mainstay of treatment. There is limited data on the use of antiarrhythmics in this population. The purpose of this article is to discuss the practical aspects of the use of antiarrhythmics in adults with congenital heart disease. Several tables have been provided to provide clinicians a reference for daily use. Copyright © 2017 Elsevier Inc. All rights reserved.

Chronic Hypoxemia in Children With Congenital Heart Defect Impairs Airway Epithelial Sodium Transport.

PubMed

Kaskinen, Anu K; Helve, Otto; Andersson, Sture; Kirjavainen, Turkka; Martelius, Laura; Mattila, Ilkka P; Rautiainen, Paula; Pitkänen, Olli M

2016-01-01

Ambient hypoxia impairs the airway epithelial Na transport, which is crucial in lung edema reabsorption. Whether chronic systemic hypoxemia affects airway Na transport has remained largely unknown. We have therefore investigated whether chronic systemic hypoxemia in children with congenital heart defect affects airway epithelial Na transport, Na transporter-gene expression, and short-term lung edema accumulation. Prospective, observational study. Tertiary care medical center responsible for nationwide pediatric cardiac surgery. Ninety-nine children with congenital heart defect or acquired heart disease (age range, 6 d to 14.8 yr) were divided into three groups based on their level of preoperative systemic hypoxemia: 1) normoxemic patients (SpO2% ≥ 95%; n = 44), 2) patients with cyanotic congenital heart defect and moderate hypoxemia (SpO2 86-94%; n = 16), and 3) patients with cyanotic congenital heart defect and profound systemic hypoxemia (SpO2 ≤ 85%; n = 39). Nasal transepithelial potential difference served as a surrogate measure for epithelial Na transport of the respiratory tract. Profoundly hypoxemic patients had 29% lower basal nasal transepithelial potential difference (p = 0.02) and 55% lower amiloride-sensitive nasal transepithelial potential difference (p = 0.0003) than normoxemic patients. In profoundly hypoxemic patients, nasal epithelial messenger RNA expressions of two airway Na transporters (amiloride-sensitive epithelial Na channel and β1- Na-K-ATPase) were not attenuated, but instead α1-Na-K-ATPase messenger RNA levels were higher (p = 0.03) than in the normoxemic patients, indicating that posttranscriptional factors may impair airway Na transport. The chest radiograph lung edema score increased after congenital cardiac surgery in profoundly hypoxemic patients (p = 0.0004) but not in patients with normoxemia or moderate hypoxemia. The impaired airway epithelial amiloride-sensitive Na transport activity in profoundly hypoxemic children with cyanotic congenital heart defect may hinder defense against lung edema after cardiac surgery.

Brain Injury in Neonates with Complex Congenital Heart Disease: What Is the Predictive Value of MRI in the Fetal Period?

PubMed

Brossard-Racine, M; du Plessis, A; Vezina, G; Robertson, R; Donofrio, M; Tworetzky, W; Limperopoulos, C

2016-07-01

Brain injury in neonates with congenital heart disease is an important predictor of adverse neurodevelopmental outcome. Impaired brain development in congenital heart disease may have a prenatal origin, but the sensitivity and specificity of fetal brain MR imaging for predicting neonatal brain lesions are currently unknown. We sought to determine the value of conventional fetal MR imaging for predicting abnormal findings on neonatal preoperative MR imaging in neonates with complex congenital heart disease. MR imaging studies were performed in 103 fetuses with confirmed congenital heart disease (mean gestational age, 31.57 ± 3.86 weeks) and were repeated postnatally before cardiac surgery (mean age, 6.8 ± 12.2 days). Each MR imaging study was read by a pediatric neuroradiologist. Brain abnormalities were detected in 17/103 (16%) fetuses by fetal MR imaging and in 33/103 (32%) neonates by neonatal MR imaging. Only 9/33 studies with abnormal neonatal findings were preceded by abnormal findings on fetal MR imaging. The sensitivity and specificity of conventional fetal brain MR imaging for predicting neonatal brain abnormalities were 27% and 89%, respectively. Brain abnormalities detected by in utero MR imaging in fetuses with congenital heart disease are associated with higher risk of postnatal preoperative brain injury. However, a substantial proportion of anomalies on postnatal MR imaging were not present on fetal MR imaging; this result is likely due to the limitations of conventional fetal MR imaging and the emergence of new lesions that occurred after the fetal studies. Postnatal brain MR imaging studies are needed to confirm the presence of injury before open heart surgery. © 2016 by American Journal of Neuroradiology.

Feasibility of a Team Approach to Complex Congenital Heart Defect Neurodevelopmental Follow-Up: Early Experience of a Combined Cardiology/Neonatal Intensive Care Unit Follow-Up Program.

PubMed

Chorna, Olena; Baldwin, H Scott; Neumaier, Jamie; Gogliotti, Shirley; Powers, Deborah; Mouvery, Amanda; Bichell, David; Maitre, Nathalie L

2016-07-01

Infants with complex congenital heart disease are at high risk for poor neurodevelopmental outcomes. However, implementation of dedicated congenital heart disease follow-up programs presents important infrastructure, personnel, and resource challenges. We present the development, implementation, and retrospective review of 1- and 2-year outcomes of a Complex Congenital Heart Defect Neurodevelopmental Follow-Up program. This program was a synergistic approach between the Pediatric Cardiology, Cardiothoracic Surgery, Pediatric Intensive Care, and Neonatal Intensive Care Unit Follow-Up teams to provide a feasible and responsible utilization of existing infrastructure and personnel, to develop and implement a program dedicated to children with congenital heart disease. Trained developmental testers administered the Ages and Stages Questionnaire-3 over the phone to the parents of all referred children at least once between 6 and 12 months' corrected age. At 18 months' corrected age, all children were scheduled in the Neonatal Intensive-Care Unit Follow-Up Clinic for a visit with standardized neurological exams, Bayley III, multidisciplinary therapy evaluations and continued follow-up. Of the 132 patients identified in the Cardiothoracic Surgery database and at discharge from the hospital, a total number of 106 infants were reviewed. A genetic syndrome was identified in 23.4% of the population. Neuroimaging abnormalities were identified in 21.7% of the cohort with 12.8% having visibly severe insults. As a result, 23 (26.7%) received first-time referrals for early intervention services, 16 (13.8%) received referrals for new services in addition to their existing ones. We concluded that utilization of existing resources in collaboration with established programs can ensure targeted neurodevelopmental follow-up for all children with complex congenital heart disease. © 2016 American Heart Association, Inc.

Total Anomalous Pulmonary Venous Connection (TAPVC)

MedlinePlus

... Your Risk • Symptoms & Diagnosis • Care & Treatment • Tools & Resources Web Booklets on Congenital Heart Defects These online publications ... to you or your child’s defect and concerns. Web Booklet: Adults With Congenital Heart Defects Web Booklet: ...

Supporting Optimal Neurodevelopmental Outcomes in Infants and Children With Congenital Heart Disease.

PubMed

Peterson, Jennifer K

2018-06-01

Improved survival has led to increased recognition of developmental delays in infants and children with congenital heart disease. Risk factors for developmental delays in congenital heart disease survivors may not be modifiable; therefore, it is important that lifesaving, high-technology critical care interventions be combined with nursing interventions that are also developmentally supportive. Implementing developmental care in a pediatric cardiac intensive care unit requires change implementation strategies and widespread support from all levels of health care professionals. This manuscript reviews developmentally supportive interventions such as massage, developmentally supportive positioning, kangaroo care, cue-based feeding, effective pain/anxiety management, and procedural preparation and identifies strategies to implement developmentally supportive interventions in the care of infants and children with congenital heart disease. Improving developmental support for these infants and children at high risk for developmental delay may improve their outcomes and help promote family-centered care. ©2018 American Association of Critical-Care Nurses.

Society of Thoracic Surgeons 2008 cardiac risk models predict in-hospital mortality of heart valve surgery in a Chinese population: a multicenter study.

PubMed

Wang, Lv; Lu, Fang-Lin; Wang, Chong; Tan, Meng-Wei; Xu, Zhi-yun

2014-12-01

The Society of Thoracic Surgeons 2008 cardiac surgery risk models have been developed for heart valve surgery with and without coronary artery bypass grafting. The aim of our study was to evaluate the performance of Society of Thoracic Surgeons 2008 cardiac risk models in Chinese patients undergoing single valve surgery and the predicted mortality rates of those undergoing multiple valve surgery derived from the Society of Thoracic Surgeons 2008 risk models. A total of 12,170 patients underwent heart valve surgery from January 2008 to December 2011. Combined congenital heart surgery and aortal surgery cases were excluded. A relatively small number of valve surgery combinations were excluded. The final research population included the following isolated heart valve surgery types: aortic valve replacement, mitral valve replacement, and mitral valve repair. The following combined valve surgery types were included: mitral valve replacement plus tricuspid valve repair, mitral valve replacement plus aortic valve replacement, and mitral valve replacement plus aortic valve replacement and tricuspid valve repair. Evaluation was performed by using the Hosmer-Lemeshow test and C-statistics. Data from 9846 patients were analyzed. The Society of Thoracic Surgeons 2008 cardiac risk models showed reasonable discrimination and poor calibration (C-statistic, 0.712; P = .00006 in Hosmer-Lemeshow test). Society of Thoracic Surgeons 2008 models had better discrimination (C-statistic, 0.734) and calibration (P = .5805) in patients undergoing isolated valve surgery than in patients undergoing multiple valve surgery (C-statistic, 0.694; P = .00002 in Hosmer-Lemeshow test). Estimates derived from the Society of Thoracic Surgeons 2008 models exceeded the mortality rates of multiple valve surgery (observed/expected ratios of 1.44 for multiple valve surgery and 1.17 for single valve surgery). The Society of Thoracic Surgeons 2008 cardiac surgery risk models performed well when predicting the mortality for Chinese patients undergoing valve surgery. The Society of Thoracic Surgeons 2008 models were suitable for single valve surgery in a Chinese population; estimates of mortality for multiple valve surgery derived from the Society of Thoracic Surgeons 2008 models were less accurate. Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Rapamycin attenuates pathological hypertrophy caused by an absence of trabecular formation.

PubMed

Fleming, Nicole D; Samsa, Leigh A; Hassel, David; Qian, Li; Liu, Jiandong

2018-06-05

Cardiac trabeculae are mesh-like muscular structures within ventricular walls. Subtle perturbations in trabeculation are associated with many congenital heart diseases (CHDs), and complete failure to form trabeculae leads to embryonic lethality. Despite the severe consequence of an absence of trabecular formation, the exact function of trabeculae remains unclear. Since ErbB2 signaling plays a direct and essential role in trabecular initiation, in this study, we utilized the erbb2 zebrafish mutant as a model to address the function of trabeculae in the heart. Intriguingly, we found that the trabeculae-deficient erbb2 mutant develops a hypertrophic-like (HL) phenotype that can be suppressed by inhibition of Target of Rapamycin (TOR) signaling in a similar fashion to adult mammalian hearts subjected to mechanical overload. Further, cell transplantation experiments demonstrated that erbb2 mutant cells in an otherwise wildtype heart did not undergo hypertrophy, indicating that erbb2 mutant HL phenotypes are due to a loss of trabeculae. Together, we propose that trabeculae serve to enhance contractility and that defects in this process lead to wall-stress induced hypertrophic remodeling.

Remote Magnetic Navigation for Catheter Ablation in Patients With Congenital Heart Disease: A Review.

PubMed

Roy, Karine; Gomez-Pulido, Federico; Ernst, Sabine

2016-03-01

In patients with congenital heart disease, challenges to catheter-based arrhythmia interventions are unique and numerous given the complexity of the underlying defects, anatomic and surgical intervention variants including baffles, conduits, patches, and/or shunts. Remote magnetic navigation offers significant advantages in these cases that may present with limited vascular access or difficult access to the target cardiac chambers implicated by the previous surgical interventions. We reviewed the data available on the safety, feasibility, and effectiveness of magnetic navigation for the treatment of arrhythmia in congenital heart disease and discussed the specific challenges related to various congenital defects and repair with the potential advantages offered by magnetic navigation in these circumstances. © 2016 Wiley Periodicals, Inc.

Radiation dose management for pediatric cardiac computed tomography: a report from the Image Gently 'Have-A-Heart' campaign.

PubMed

Rigsby, Cynthia K; McKenney, Sarah E; Hill, Kevin D; Chelliah, Anjali; Einstein, Andrew J; Han, B Kelly; Robinson, Joshua D; Sammet, Christina L; Slesnick, Timothy C; Frush, Donald P

2018-01-01

Children with congenital or acquired heart disease can be exposed to relatively high lifetime cumulative doses of ionizing radiation from necessary medical imaging procedures including radiography, fluoroscopic procedures including diagnostic and interventional cardiac catheterizations, electrophysiology examinations, cardiac computed tomography (CT) studies, and nuclear cardiology examinations. Despite the clinical necessity of these imaging studies, the related ionizing radiation exposure could pose an increased lifetime attributable cancer risk. The Image Gently "Have-A-Heart" campaign is promoting the appropriate use of medical imaging studies in children with congenital or acquired heart disease while minimizing radiation exposure. The focus of this manuscript is to provide a comprehensive review of radiation dose management and CT performance in children with congenital or acquired heart disease.

Software development, nomenclature schemes, and mapping strategies for an international pediatric cardiac surgery database system.

PubMed

Jacobs, Jeffrey P

2002-01-01

The field of congenital heart surgery has the opportunity to create the first comprehensive international database for a medical subspecialty. An understanding of the demographics of congenital heart disease and the rapid growth of computer technology leads to the realization that creating a comprehensive international database for pediatric cardiac surgery represents an important and achievable goal. The evolution of computer-based data analysis creates an opportunity to develop software to manage an international congenital heart surgery database and eventually become an electronic medical record. The same database data set for congenital heart surgery is now being used in Europe and North America. Additional work is under way to involve Africa, Asia, Australia, and South America. The almost simultaneous publication of the European Association for Cardio-thoracic Surgery/Society of Thoracic Surgeons coding system and the Association for European Paediatric Cardiology coding system resulted in the potential for multiple coding. Representatives of the Association for European Paediatric Cardiology, Society of Thoracic Surgeons, European Association for Cardio-thoracic Surgery, and European Congenital Heart Surgeons Foundation agree that these hierarchical systems are complementary and not competitive. An international committee will map the two systems. The ideal coding system will permit a diagnosis or procedure to be coded only one time with mapping allowing this code to be used for patient care, billing, practice management, teaching, research, and reporting to governmental agencies. The benefits of international data gathering and sharing are global, with the long-term goal of the continued upgrade in the quality of congenital heart surgery worldwide. Copyright 2002 by W.B. Saunders Company

Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease.

PubMed

Bhat, Aarti Hejmadi; Sahn, David J

2004-10-01

As the specialties of pediatrics and pediatric cardiology continue to forge ahead with better diagnoses, medical care, and surgical results, an expanding population of patients with congenital heart disease (CHD) outgrows the pediatric age group, yet does not quite graduate to routine adult cardiology or general medicine. The adult with congenital heart disease (ACHD) faces medical, surgical, and psychosocial issues that are unique to this population and must be addressed as such. This review attempts to discuss and highlight some of the important advances and controversies brought up in the past year, in the care and management of these patients. The past five to 10 years have seen dynamic interest in understanding sequelae of corrected, uncorrected, or palliated congenital heart disease. The search for the ideal surgery, optimal prosthesis, and a smooth transition to adult care continues and is reflected in the vast amount of academic work and publications in this field. Of particular interest, conduit reoperations and single ventricle pathway modifications are still an art and a science in evolution. While all are agreed that there is a pressing need to focus on the delivery of care to the adult with congenital heart disease, this essentially requires a clearer understanding of late sequelae of CHD. The sheer heterogeneity of anatomy, age, surgery, and institutional management protocols can make it difficult to develop clear guidelines. This review attempts to give an up-to-date perspective on some of the new findings related to the more common lesions and problems faced in this group.

Perioperative Management of a Child with Hypoplastic Left Heart Syndrome of the Jehovah's Witness Faith Presenting for Hybrid Comprehensive Stage II Procedure.

PubMed

Karuppiah, Sathappan; Mckee, Christopher; Hodge, Ashley; Galantowicz, Mark; Tobias, Joseph; Naguib, Aymen

2016-09-01

Over the years, there has been a growing recognition of the potential negative sequelae of allogeneic blood products on postoperative outcomes following cardiac surgery. In addition, followers of the Jehovah's Witness (JW) faith have a religious restriction against receiving blood or blood components. Advances in perioperative care, cardiopulmonary bypass (CPB), and surgical technique have minimized the need for allogeneic blood products. Specific blood conservation strategies include maximizing the preoperative hematocrit and coagulation function as well as intraoperative strategies, such as acute normovolemic hemodilution and adjustments of the technique of CPB. We report a 7-month-old patient whose parents were of the JW faith who underwent a comprehensive stage II procedure for hypoplastic left heart syndrome without exposure to blood or blood products during his hospital stay. Perioperative techniques for blood avoidance are discussed with emphasis on their application to infants undergoing surgery for congenital heart disease.

Perioperative Management of a Child with Hypoplastic Left Heart Syndrome of the Jehovah's Witness Faith Presenting for Hybrid Comprehensive Stage II Procedure

PubMed Central

Karuppiah, Sathappan; Mckee, Christopher; Hodge, Ashley; Galantowicz, Mark; Tobias, Joseph; Naguib, Aymen

2016-01-01

Abstract: Over the years, there has been a growing recognition of the potential negative sequelae of allogeneic blood products on postoperative outcomes following cardiac surgery. In addition, followers of the Jehovah's Witness (JW) faith have a religious restriction against receiving blood or blood components. Advances in perioperative care, cardiopulmonary bypass (CPB), and surgical technique have minimized the need for allogeneic blood products. Specific blood conservation strategies include maximizing the preoperative hematocrit and coagulation function as well as intraoperative strategies, such as acute normovolemic hemodilution and adjustments of the technique of CPB. We report a 7-month-old patient whose parents were of the JW faith who underwent a comprehensive stage II procedure for hypoplastic left heart syndrome without exposure to blood or blood products during his hospital stay. Perioperative techniques for blood avoidance are discussed with emphasis on their application to infants undergoing surgery for congenital heart disease. PMID:27729708

Pattern of congenital heart disease in Southern Yemeni children referred for echocardiography.

PubMed

Saleh, Hussein K

2009-06-01

To study the distribution of age, gender, and the relative frequency of congenital heart defects at the time of the diagnosis in Southern Yemeni children. This retrospective study focused on echocardiographic findings of 393 symptomatic children affected by congenital heart disease. It was conducted in the Echocardiography Department of a referral hospital for Aden city and surrounding governorates, Yemen, from January 2001 to December 2005. Out of 987 referred children, congenital heart defects were detected in 393 (39.8%); mean age was 3.45+/-4 years; of them, 48% males and 52% females. They were 85% non-cyanotic and 15% cyanotic. Patients comprised neonates, 5 (1.3%); infants under one year, 156 (39.7%), and children more than one year, 232 (59%). Most cyanotic patients (66%) presented during their first year of life, but only 8.5% were neonates. Most non-cyanotic (64%) presented after their first year mean age 3.9 years, none of them were neonates. The most frequent defects were: ventricular septal defect (26.5%), pulmonary stenosis (17.6%), patent ductus arteriosus (17.3%), and atrial septal defect (15.8%). Tetralogy of Fallot (8.9%) and transposition of great vessels (3.1%) were the most frequent cyanotic defects. The pattern of congenital heart diseases in Southern Yemen, is characterized by simple, potentially correctable heart defects, under-representation of cyanotic, and absence of critical defects that provokes high mortality during infancy.

Interventional Cardiology for Congenital Heart Disease

PubMed Central

2018-01-01

Congenital heart interventions are now replacing surgical palliation and correction in an evolving number of congenital heart defects. Right ventricular outflow tract and ductus arteriosus stenting have demonstrated favorable outcomes compared to surgical systemic to pulmonary artery shunting, and it is likely surgical pulmonary valve replacement will become an uncommon procedure within the next decade, mirroring current practices in the treatment of atrial septal defects. Challenges remain, including the lack of device design focused on smaller infants and the inevitable consequences of somatic growth. Increasing parental and physician expectancy has inevitably lead to higher risk interventions on smaller infants and appreciation of the consequences of these interventions on departmental outcome data needs to be considered. Registry data evaluating congenital heart interventions remain less robust than surgical registries, leading to a lack of insight into the longer-term consequences of our interventions. Increasing collaboration with surgical colleagues has not been met with necessary development of dedicated equipment for hybrid interventions aimed at minimizing the longer-term consequences of scar to the heart. Therefore, great challenges remain to ensure children and adults with congenital heart disease continue to benefit from an exponential growth in minimally invasive interventions and technology. This can only be achieved through a concerted collaborative approach from physicians, industry, academia and regulatory bodies supporting great innovators to continue the philosophy of thinking beyond the limits that has been the foundation of our specialty for the past 50 years. PMID:29671282

VEGFA polymorphisms and cardiovascular anomalies in 22q11 microdeletion syndrome: a case-control and family-based study.

PubMed

Calderón, Juan Francisco; Puga, Alonso R; Guzmán, M Luisa; Astete, Carmen Paz; Arriaza, Marta; Aracena, Mariana; Aravena, Teresa; Sanz, Patricia; Repetto, Gabriela M

2009-01-01

Microdeletion 22q11 in humans causes velocardiofacial and DiGeorge syndromes. Most patients share a common 3Mb deletion, but the clinical manifestations are very heterogeneous. Congenital heart disease is present in 50-80% of patients and is a significant cause of morbidity and mortality. The phenotypic variability suggests the presence of modifiers. Polymorphisms in the VEGFA gene, coding for the vascular endothelial growth factor A, have been associated with non-syndromic congenital heart disease, as well as with the presence of cardiovascular anomalies in patients with microdeletion 22q11. We evaluated the association of VEGFA polymorphisms c.-2578C>A (rs699947), c.-1154G>A (rs1570360) and c.-634C>G (rs2010963) with congenital heart disease in Chilean patients with microdeletion 22q11. The study was performed using case-control and family-based association designs. We evaluated 122 patients with microdeletion 22q11 and known anatomy of the heart and great vessels, and their parents. Half the patients had congenital heart disease. We obtained no evidence of association by either method of analysis. Our results provide further evidence of the incomplete penetrance of the cardiovascular phenotype of microdeletion 22ql 1, but do not support association between VEGFA promoter polymorphisms and the presence of congenital heart disease in Chilean patients with this syndrome.

The evolving role of the total artificial heart in the management of end-stage congenital heart disease and adolescents.

PubMed

Ryan, Thomas D; Jefferies, John L; Zafar, Farhan; Lorts, Angela; Morales, David L S

2015-01-01

Advances in medical therapies have yielded improvement in morbidity and a decrease in mortality for patients with congenital heart disease, both surgically palliated and uncorrected. An unintended consequence is a cohort of adolescent and adult patients with heart failure who require alternative therapies. One intriguing option is placement of a total artificial heart (TAH) either as a bridge to transplant or as a destination therapy. Of the 1091 Jarvik-7 type TAH (Symbion, CardioWest and SynCardia) placed between 1985 and 2012, only 24 have been performed in patients with congenital heart disease, and a total of 51 were placed in patients younger than 21. At our institution, the SynCardia TAH was implanted in a 19-year-old patient with cardiac allograft failure because of chronic rejection and related multisystem organ failure including need for hemodialysis. Over the next year, she was nutritionally and physically rehabilitated, as were her end organs, allowing her to come off dialysis, achieve normal renal function and eventually be successfully transplanted. Given the continued growth of adolescent and adult congenital heart disease populations with end-stage heart failure, the TAH may offer therapeutic options where previously there were few. In addition, smaller devices such as the SynCardia 50/50 will open the door for applications in smaller children. The Freedom Driver offers the chance for patients to leave the hospital with a TAH, as does the AbioCor, which is a fully implantable TAH option. In this report, we review the history of the TAH and potential applications in adolescent patients and congenital heart disease.

Cardiac troponin T is necessary for normal development in the embryonic chick heart.

PubMed

England, Jennifer; Pang, Kar Lai; Parnall, Matthew; Haig, Maria Isabel; Loughna, Siobhan

2016-09-01

The heart is the first functioning organ to develop during embryogenesis. The formation of the heart is a tightly regulated and complex process, and alterations to its development can result in congenital heart defects. Mutations in sarcomeric proteins, such as alpha myosin heavy chain and cardiac alpha actin, have now been associated with congenital heart defects in humans, often with atrial septal defects. However, cardiac troponin T (cTNT encoded by gene TNNT2) has not. Using gene-specific antisense oligonucleotides, we have investigated the role of cTNT in chick cardiogenesis. TNNT2 is expressed throughout heart development and in the postnatal heart. TNNT2-morpholino treatment resulted in abnormal atrial septal growth and a reduction in the number of trabeculae in the developing primitive ventricular chamber. External analysis revealed the development of diverticula from the ventricular myocardial wall which showed no evidence of fibrosis and still retained a myocardial phenotype. Sarcomeric assembly appeared normal in these treated hearts. In humans, congenital ventricular diverticulum is a rare condition, which has not yet been genetically associated. However, abnormal haemodynamics is known to cause structural defects in the heart. Further, structural defects, including atrial septal defects and congenital diverticula, have previously been associated with conduction anomalies. Therefore, to provide mechanistic insights into the effect that cTNT knockdown has on the developing heart, quantitative PCR was performed to determine the expression of the shear stress responsive gene NOS3 and the conduction gene TBX3. Both genes were differentially expressed compared to controls. Therefore, a reduction in cTNT in the developing heart results in abnormal atrial septal formation and aberrant ventricular morphogenesis. We hypothesize that alterations to the haemodynamics, indicated by differential NOS3 expression, causes these abnormalities in growth in cTNT knockdown hearts. In addition, the muscular diverticula reported here suggest a novel role for mutations of structural sarcomeric proteins in the pathogenesis of congenital cardiac diverticula. From these studies, we suggest TNNT2 is a gene worthy of screening for those with a congenital heart defect, particularly atrial septal defects and ventricular diverticula. © 2016 The Authors. Journal of Anatomy published by John Wiley & Sons Ltd on behalf of Anatomical Society.

Type 2 diabetes mellitus induces congenital heart defects in murine embryos by increasing oxidative stress, endoplasmic reticulum stress, and apoptosis.

PubMed

Wu, Yanqing; Reece, E Albert; Zhong, Jianxiang; Dong, Daoyin; Shen, Wei-Bin; Harman, Christopher R; Yang, Peixin

2016-09-01

Maternal type 1 and 2 diabetes mellitus are strongly associated with high rates of severe structural birth defects, including congenital heart defects. Studies in type 1 diabetic embryopathy animal models have demonstrated that cellular stress-induced apoptosis mediates the teratogenicity of maternal diabetes leading to congenital heart defect formation. However, the mechanisms underlying maternal type 2 diabetes mellitus-induced congenital heart defects remain largely unknown. We aim to determine whether oxidative stress, endoplasmic reticulum stress, and excessive apoptosis are the intracellular molecular mechanisms underlying maternal type 2 diabetes mellitus-induced congenital heart defects. A mouse model of maternal type 2 diabetes mellitus was established by feeding female mice a high-fat diet (60% fat). After 15 weeks on the high-fat diet, the mice showed characteristics of maternal type 2 diabetes mellitus. Control dams were either fed a normal diet (10% fat) or the high-fat diet during pregnancy only. Female mice from the high-fat diet group and the 2 control groups were mated with male mice that were fed a normal diet. At E12.5, embryonic hearts were harvested to determine the levels of lipid peroxides and superoxide, endoplasmic reticulum stress markers, cleaved caspase 3 and 8, and apoptosis. E17.5 embryonic hearts were harvested for the detection of congenital heart defect formation using India ink vessel patterning and histological examination. Maternal type 2 diabetes mellitus significantly induced ventricular septal defects and persistent truncus arteriosus in the developing heart, along with increasing oxidative stress markers, including superoxide and lipid peroxidation; endoplasmic reticulum stress markers, including protein levels of phosphorylated-protein kinase RNA-like endoplasmic reticulum kinase, phosphorylated-IRE1α, phosphorylated-eIF2α, C/EBP homologous protein, and binding immunoglobulin protein; endoplasmic reticulum chaperone gene expression; and XBP1 messenger RNA splicing, as well as increased cleaved caspase 3 and 8 in embryonic hearts. Furthermore, maternal type 2 diabetes mellitus triggered excessive apoptosis in ventricular myocardium, endocardial cushion, and outflow tract of the embryonic heart. Similar to those observations in type 1 diabetic embryopathy, maternal type 2 diabetes mellitus causes heart defects in the developing embryo manifested with oxidative stress, endoplasmic reticulum stress, and excessive apoptosis in heart cells. Copyright © 2016 Elsevier Inc. All rights reserved.

Two congenital coronary abnormalities affecting heart function: anomalous origin of the left coronary artery from the pulmonary artery and congenital left main coronary artery atresia.

PubMed

Xiao, Yanyan; Jin, Mei; Han, Ling; Ding, Wenhong; Zheng, Jianyong; Sun, Chufan; Lyu, Zhenyu

2014-01-01

The anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) and congenital left main coronary artery atresia (CLMCA-A) are two kinds of very rare coronary heart diseases which affect heart function profoundly. This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients. From April 1984 to July 2012, in Beijing Anzhen Hospital, 23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A. We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation, diagnosis, and treatments of these two kinds of congenital coronary abnormalities. The 23 patients (13 males and 10 females, aged ranging from 2.5 months to 65 years) identified with ALCAPA were classified into infantile type (age of onset younger than 12 months, 16 cases) and adult type (age of onset older than 12 months, 7 cases). Four patients were diagnosed with CLMCA-A (three males and one female, aged ranging from 3 months to 2 years). The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection, heart failure, dyspnea, feeding intolerance, diaphoresis, and failure to thrive. And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis, dilated cardiomyopathy, and acute myocardial infarction. As for the adult-type ALCAPA, cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease, myocarditis, or patent ductus arteriosus. In ECG examination: Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6, especially in lead avL. However, ECG of adult-type ALCAPA lacked distinct features. In chest radiography: pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A, while pulmonary artery segment dilation was more common in adult type. In echocardiography, the common features of infantile-type ALCAPA and CLMCA-A included left ventricular enlargement, left ventricular systolic function normal or mildly reduced in CLMCA-A or significantly reduced in ALCAPA, and moderate to large mitral valve. It was performed in 9 of 23 cases of ALCAPA and showed the origin of the dilated right coronary artery (RCA) from the right sinus of the aortic root and absence of LCA origin in angiography. After opacification of RCA, reverse flow in the LCA and pulmonary artery was visualized through coronary artery collateral circulation. Angio was performed in three of the four cases of CLMCA-A and showed left main coronary artery was a blind end, with diameter of only 1.1-2.0 mm. Treatment and prognosis: 21 patients with ALCAPA had cardiac surgery and 6 of them died postoperatively. Fifteen postoperative patients survived without overt symptoms within the follow-up period of 6-166 months (median 17 months). As for treatment of CLMCA-A, four patients took digoxin and diuretics without undergoing cardiac surgery. Their clinical symptoms improved during the close follow-ups. ALCAPA and CLMCA-A are two rare coronary artery abnormalities that affect cardiac function in infants and children. In younger patients with cardiomegaly and heart dysfunction these two congenital coronary diseases should be noticed.

Percutaneous pulmonary valve implantation: an update.

PubMed

Lurz, Philipp; Bonhoeffer, Philipp; Taylor, Andrew M

2009-07-01

The field of percutaneous valvular interventions is one of the most exciting and rapidly developing within interventional cardiology. Percutaneous pulmonary valve implantation (PPVI) represents the first in-human application of these techniques and is a nonsurgical option for treating right ventricular outflow tract/pulmonary trunk dysfunction. With the growing numbers of patients with right ventricle to pulmonary artery conduit dysfunction after repair of congenital heart disease, the importance of a technique with lower morbidity and mortality, good patient acceptance and efficacy, that is comparable to surgery, cannot be underestimated. Over the last 9 years, PPVI has become a feasible, safe and effective treatment for both conduit stenosis and regurgitation. Median follow-up data show good freedom from reoperation and recatheterization and demonstrate that PPVI can postpone open-heart surgery, thereby potentially reducing the number of operations that patients have to undergo within their lifetime. Complications seen after PPVI, in particular stent fractures, can require reintervention in some cases (second stent-in-stent PPVI); however, valve competency remains good, with significant regurgitation during follow-up only seen in the context of occasional endocarditis. Attempts are now being made to prolong the lifespan of the device by reducing the incidence of stent fractures. Further, meticulous patient selection must be maintained to ensure that hemodynamic results are optimized and the safety of the procedure remains high. Finally, new devices have to be developed that will allow for PPVI in dilated, distensible outflow tracts, to offer this nonsurgical treatment option to a larger patient population with congenital heart disease.

Gender differences in the utilisation of surgery for congenital heart disease in India.

PubMed

Ramakrishnan, Sivasubramanian; Khera, Rohan; Jain, Snigdha; Saxena, Anita; Kailash, Suparna; Karthikeyan, Ganesan; Kothari, Shyam S; Juneja, Rajnish; Bhargava, Balram; Kalaivani, Mani; Mehta, Manju; Bahl, Vinay K; Airan, Balram

2011-12-01

Corrective surgery for congenital heart disease may be life-saving, but its utilisation depends upon several social and economic factors. Girls with cardiac defects may not receive equitable care in India, but this has not been systematically studied. In this prospective study, parents or guardians of 405 consecutive children aged up to 12 years (mean ± SD age 3.43 ± 3.44 years; 271 boys) who had been advised to undergo elective paediatric cardiac surgery were interviewed using a validated questionnaire. The status of the patients was reviewed after a year and the factors associated with non-compliance with treatment were analysed. In a qualitative sub-study the parents of 20 children who had not undergone surgery were interviewed. Qualitative data were analysed using an inductive analytical approach. Of the 405 patients studied, 44% (59/134) of girls had undergone surgery at 1 year compared with 70% (189/271) of boys (χ²=24.97; p<0.001). Independent predictors for non-compliance with surgery included female gender (OR 3.46, 95% CI -2.06 to 5.80; p<0.0001), lower socioeconomic classes (lower-middle: OR 18.62, 95% CI -2.14 to 161.8, p=0.008; upper-lower: OR 34.27, 95% CI -3.72 to 316.0, p=0.002) and higher cost of surgery (OR 1.92, 95% CI -1.06 to 3.47, p=0.03). In the in-depth interviews, apprehensions about future matrimonial prospects of girls and lack of social support emerged as the major factors responsible for delays in undergoing surgery. Female gender is an important determinant of non-compliance with paediatric cardiac surgery. Deep-seated social factors underlie this gender bias.

Admission to dedicated pediatric cardiac intensive care units is associated with decreased resource use in neonatal cardiac surgery.

PubMed

Johnson, Joyce T; Wilkes, Jacob F; Menon, Shaji C; Tani, Lloyd Y; Weng, Hsin-Yi; Marino, Bradley S; Pinto, Nelangi M

2018-06-01

Neonates undergoing congenital heart surgery require highly specialized, resource-intensive care. Location of care and degree of specialization can vary between and within institutions. Using a multi-institutional cohort, we sought to determine whether location of admission is associated with an increase in health care costs, resource use and mortality. We retrospectively analyzed admission for neonates (<30 days) undergoing congenital heart surgery between 2004 and 2013 by using the Pediatric Health Information Systems database (44 children's hospitals). Multivariate generalized estimating equations adjusted for center- and patient-specific risk factors and stratified by age at admission were performed to examine the association of admission intensive care unit (ICU) with total hospital costs, mortality, and length of stay. Of 19,984 neonates (60% male) identified, 39% were initially admitted to a cardiac ICU (CICU), 48% to a neonatal ICU (NICU), and 13% to a pediatric ICU. In adjusted models, admission to a CICU versus NICU was associated with a $20,440 reduction in total hospital cost for infants aged 2 to 7 days at admission (P = .007) and a $23,700 reduction in total cost for infants aged 8 to 14 days at admission (P = .01). Initial admission to a CICU or pediatric ICU versus NICU at <15 days of age was associated with shorter hospital and ICU length of stay and fewer days of mechanical ventilation. There was no difference in adjusted mortality by admission location. Admission to an ICU specializing in cardiac care is associated with significantly decreased hospital costs and more efficient resource use for neonates requiring cardiac surgery. Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Risk of Congenital Heart Defects after Ambient Heat Exposure Early in Pregnancy.

PubMed

Auger, Nathalie; Fraser, William D; Sauve, Reg; Bilodeau-Bertrand, Marianne; Kosatsky, Tom

2017-01-01

Congenital heart defects may be environmentally related, but the association with elevated ambient temperature has received little attention. We studied the relationship between outdoor heat during the first trimester of pregnancy and risk of congenital heart defects. We carried out a retrospective cohort study of 704,209 fetuses between 2 and 8 weeks postconception from April to September in Quebec, Canada, 1988-2012. We calculated the prevalence of congenital heart defects at birth according to the number of days women were exposed to maximum temperature ≥ 30°C. In log-binomial regression models, we estimated prevalence ratios (PR) and 95% confidence intervals (CI) for the relationship of temperature with seven critical and eight noncritical heart defects, adjusted for pregnancy characteristics. Prevalence of congenital heart defects was 979.5 per 100,000 for 10 days or more of temperature ≥ 30°C compared with 878.9 per 100,000 for 0 days of exposure. Temperature was more precisely associated with noncritical than critical defects, which had lower prevalence. Fetuses exposed to 15 days of temperature ≥ 30°C between 2 and 8 weeks postconception had 1.06 times the risk of critical defects (95% CI: 0.67, 1.67) and 1.12 times the risk of noncritical defects (95% CI: 0.98, 1.29) relative to 0 days. Associations were higher for atrial septal defects (PR 1.37, 95% CI: 1.10, 1.70) than for other noncritical defects. For atrial septal defects, associations with elevated temperatures began the 3rd week postconception. Extreme heat exposure during the first trimester may be associated with noncritical heart defects, especially of the atrial septum. Citation: Auger N, Fraser WD, Sauve R, Bilodeau-Bertrand M, Kosatsky T. 2017. Risk of congenital heart defects after ambient heat exposure early in pregnancy. Environ Health Perspect 125:8-14; http://dx.doi.org/10.1289/EHP171.

Cerebral oxygen delivery is reduced in newborns with congenital heart disease.

PubMed

Lim, Jessie Mei; Kingdom, Theodore; Saini, Brahmdeep; Chau, Vann; Post, Martin; Blaser, Susan; Macgowan, Christopher; Miller, Steven P; Seed, Mike

2016-10-01

To investigate preoperative cerebral hemodynamics in newborns with congenital heart disease. We hypothesized that cerebral blood flow and oxygen delivery would be decreased in newborns with congenital heart disease compared with controls. Using a "feed-and-sleep" approach to performing neonatal magnetic resonance imaging, we measured cerebral blood flow by using a slice prescription perpendicular to the right and left internal carotid arteries and basilar artery at the level of the clivus. We calculated brain volume by segmenting a 3-dimensional steady-state free procession acquisition of the whole brain, allowing quantification of cerebral blood flow indexed to brain volume. Cerebral oxygen delivery was calculated as the product of cerebral blood flow and preductal systemic arterial oxygen content obtained via a combination of conventional pulse oximetry and laboratory analysis of venous blood samples for hemoglobin concentration. A complete set of measurements were obtained in 32 newborns with heart disease and 31 controls. There was no difference in gestational age between the heart disease and control groups. There was no difference in cerebral blood flow compared with controls (103.5 ± 34.0 vs 119.7 ± 40.4 mL/min), whereas cerebral oxygen delivery was significantly lower in the congenital heart disease subjects (1881 ± 625.7 vs 2712 ± 915.7 mLO2/min). Ten newborns with congenital heart disease had diffuse excessive high signal intensity in their white matter and 2 had white matter injury whereas another 5 had both. Newborns with unrepaired cyanotic congenital heart disease have decreased cerebral oxygen delivery due to arterial desaturation. If brain growth and development are adversely affected through oxygen conformance, our findings could have clinical implications in terms of timing of surgical repair. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Heart MRI

MedlinePlus

Magnetic resonance imaging - cardiac; Magnetic resonance imaging - heart; Nuclear magnetic resonance - cardiac; NMR - cardiac; MRI of the heart; Cardiomyopathy - MRI; Heart failure - MRI; Congenital heart disease - MRI

Pre-anesthetic echocardiographic findings in children undergoing non-cardiac surgery at the University of Benin Teaching Hospital, Nigeria

PubMed Central

Wilson, E Sadoh,; Paul, Ikhurionan; Charles, Imarengiaye,

2016-01-01

Summary Background A pre-anaesthestic echocardiogram (echo) is requested for most non-cardiac surgeries to identify possible cardiac structural anomalies Objective To describe the prevalence and spectrum of structural cardiac abnormalities seen in various non-cardiac conditions Methods We carried out a retrospective review of pre-anaesthetic echos performed over five years on children scheduled for non-cardiac surgery. The requests were categorised according to referring specialities, and the biodata and echo findings were noted Results A total of 181 children and 181 echocardiograms were studied, and 100 (55.2%) of the patients were male. Most of the children (87, 48.1%) with oro-facial clefts were referred from dentistry. Of the 181 children, 39 (21.5%) had cardiac abnormalities, most (34, 87.2%) of whom had congenital heart disease (CHD). Ophthalmic requests with suspected congenital rubella syndrome (CRS) had the highest prevalence of 8/12 (66.7%) while the lowest was oro-facial clefts at 15/87 (17.2%). Atrial septal defect was the commonest abnormality, found in 14 patients (35.9%) Conclusion Pre-anaesthetic echo should be performed, especially for children with suspected CRS and other congenital anomalies, requiring non-cardiac surgery. PMID:27701485

Slit–Robo signalling in heart development

PubMed Central

Zhao, Juanjuan; Mommersteeg, Mathilda T M

2018-01-01

Abstract The Slit ligands and their Robo receptors are well-known for their roles during axon guidance in the central nervous system but are still relatively unknown in the cardiac field. However, data from different animal models suggest a broad involvement of the pathway in many aspects of heart development, from cardiac cell migration and alignment, lumen formation, chamber formation, to the formation of the ventricular septum, semilunar and atrioventricular valves, caval veins, and pericardium. Absence of one or more of the genes in the pathway results in defects ranging from bicuspid aortic valves to ventricular septal defects and abnormal venous connections to the heart. Congenital heart defects are the most common congenital malformations found in life new-born babies and progress in methods for large scale human genetic testing has significantly enhanced the identification of new causative genes involved in human congenital heart disease. Recently, loss of function variants in ROBO1 have also been linked to ventricular septal defects and tetralogy of Fallot in patients. Here, we will give an overview of the role of the Slit–Robo signalling pathway in Drosophila, zebrafish, and mouse heart development. The extent of these data warrant further attention on the SLIT–ROBO signalling pathway as a candidate for an array of human congenital heart defects. PMID:29538649

Introduction: December 2015 HeartWeek Issue of Cardiology in the Young - Highlights of HeartWeek 2015: Challenges and Dilemmas of Pediatric Cardiac Care including Heart Failure in Children and Congenital Abnormalities of the Coronary Arteries.

PubMed

Jacobs, Jeffrey P

2015-12-01

This December Issue of Cardiology in the Young represents the 13th annual publication in Cardiology in the Young generated from the two meetings that composed "HeartWeek in Florida". "HeartWeek in Florida", the joint collaborative project sponsored by the Cardiac Centre at the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, together with Johns Hopkins All Children's Heart Institute of Saint Petersburg, Florida, averages over 1000 attendees every year and is now recognised as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of cardiac disease in the foetus, neonate, infant, child, and adult. "HeartWeek in Florida" combines the International Symposium on Congenital Heart Disease, organised by All Children's Hospital and Johns Hopkins Medicine, and entering its 16th year, with the Annual Postgraduate Course in Paediatric Cardiovascular Disease, organised by The Children's Hospital of Philadelphia entering its 19th year. This December 2015 Issue of Cardiology in the Young features highlights of the two meetings that compose HeartWeek. Johns Hopkins All Children's Heart Institute's 15th Annual International Symposium on Congenital Heart Disease was held at the Renaissance Vinoy Resort & Golf Club, Saint Petersburg, Florida, from Friday, 6 February, 2015, to Monday, 9 February, 2015. This Symposium was co-sponsored by The American Association for Thoracic Surgery and its special focus was "Congenital Abnormalities of the Coronary Arteries". The Children's Hospital of Philadelphia's annual meeting - Cardiology 2015, the 18th Annual Update on Paediatric and Congenital Cardiovascular Disease: "Challenges and Dilemmas" - was held at the Hyatt Regency Scottsdale Resort and Spa at Gainey Ranch, Scottsdale, Arizona, from Wednesday, 11 February, 2015, to Sunday, 15 February, 2015. We would like to acknowledge the tremendous contributions made to paediatric and congenital cardiac care by Juan Valentín Comas, MD, PhD (13 May, 1960 to 16 June, 2015) and Donald Nixon Ross, FRCS (4 October, 1922 to 7 July, 2014); and therefore, we dedicate this December 2015 HeartWeek Issue of Cardiology in the Young to them.

Prolonged Tp-e Interval in Down Syndrome Patients with Congenitally Normal Hearts.

PubMed

Kucuk, Mehmet; Karadeniz, Cem; Ozdemir, Rahmi; Meşe, Timur

2018-03-25

Heterogeneity of ventricular repolarization has been assessed by using the QT dispersion in Down syndrome (DS) patients with congenitally normal hearts. However, novel repolarization indexes, the Tp-e interval and Tp-e/QT ratio, have not previously been evaluated in these patients. The aim of this study was to evaluate the Tp-e interval and Tp-e/QT ratio in DS patients without congenital heart defects. Twelve-lead surface electrocardiograms of 160 DS patients and 110 age- and sex-matched healthy controls were used to evaluate and compare the Tp-e interval, Tp-e dispersion, and Tp-e/QT ratio. Heart rate, Tp-e interval, Tp-e dispersion, Tp-e/QT and Tp-e/QTc ratios were significantly higher in DS group than in the controls. Myocardial repolarization indexes in DS patients with congenitally normal hearts were found to be prolonged compared to those in normal controls. Further evaluation is warranted to reveal a relationship between prolonged repolarization indexes and arrhythmic events in these patients. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Arsenic in drinking water and congenital heart anomalies in Hungary.

PubMed

Rudnai, Tamás; Sándor, János; Kádár, Mihály; Borsányi, Mátyás; Béres, Judit; Métneki, Júlia; Maráczi, Gabriella; Rudnai, Péter

2014-11-01

Inorganic arsenic can get easily through the placenta however there are very few human data on congenital anomalies related to arsenic exposure. Objective of our study was to explore the associations between arsenic content of drinking water and prevalence of some congenital anomalies. Four anomalies reported to the Hungarian Congenital Anomalies Registry between 1987 and 2003 were chosen to be analysed in relation to arsenic exposure: congenital anomalies of the circulatory system (n=9734) were considered as cases, while Down syndrome, club foot and multiple congenital malformations were used as controls (n=5880). Arsenic exposure of the mothers during pregnancy was estimated by using archive measurement data for each year and for each settlement where the mothers lived. Analysis of the associations between the prevalence of congenital heart anomalies and arsenic exposure during pregnancy was performed by logistic regression. The child's gender and age of the mother were adjusted for. The associations were evaluated by using the present EU health limit value of 10.0 μg/L arsenic concentration as a cut-off point. Regular consumption of drinking water with arsenic concentration above 10 μg/L during pregnancy was associated with an increased risk of congenital heart anomalies in general (adjusted OR=1.41; 95% C.I.: 1.28-1.56), and especially that of ductus Botalli persistens (adjusted OR=1.81, 95%C.I.: 1.54-2.11) and atrial septal defect (adjusted OR=1.79; 95%C.I.: 1.59-2.01). The presented results showed an increased risk of congenital heart anomalies among infants whose mothers were exposed to drinking water with arsenic content above 10 μg/L during pregnancy. Further studies of possible similar effects of concentrations below 10 μg/L are warranted. Copyright © 2014 Elsevier GmbH. All rights reserved.

Management of pulmonary arterial hypertension associated with congenital heart disease.

PubMed

Togănel, Rodica; Benedek, I; Suteu, Carmen; Blesneac, Cristina

2007-01-01

Congenital heart diseases are the most common congenital malformations and account for about eight cases per 1000 births and are often associated with pulmonary arterial hypertension. Increased shear stress and the excess flow through the pulmonary vascular bed due to a systemic-to-pulmonary shunt lead to the development of pulmonary vascular disease and an increase in pulmonary vascular resistance. Without surgical repair approximately 30% of patients develop pulmonary vascular disease. Eisenmenger syndrome represents the extreme end of pulmonary arterial hypertension with congenital heart disease. We summarized the current therapeutic options for pulmonary arterial hypertension; conventional treatments including calcium channel blockers, anticoagulation, digitalis, diuretics, and new treatment: prostacyclin, bosentan, sildenafil, ambrisentan. Preliminary data of new therapies are encouraging with disease significantly improved natural history, but there is need for more evidence-based data.

Cardiac Hemodynamics in the Pathogenesis of Congenital Heart Disease and Aortic Valve Calcification

NASA Astrophysics Data System (ADS)

Nigam, Vishal

2011-11-01

An improved understanding of the roles of hemodynamic forces play in cardiac development and the pathogenesis of cardiac disease will have significant scientific and clinical impact. I will focus on the role of fluid dynamics in congenital heart disease and aortic valve calcification. Congenital heart defects are the most common form of birth defect. Aortic valve calcification/stenosis is the third leading cause of adult heart disease and the most common form of acquired valvular disease in developed countries. Given the high incidence of these diseases and their associated morbidity and mortality, the potential translational impact of an improved understanding of cardiac hemodynamic forces is very large. Division of Pediatric Cardiology, Rady Children's Hospital, San Diego

Evaluation of athletes with complex congenital heart disease.

PubMed

Bates, Benjamin A; Richards, Camille; Hall, Michael; Kerut, Edmund K; Campbell, William; McMullan, Michael R

2017-06-01

As a result of improvements in congenital heart surgery, there are more adults alive today with congenital heart disease (CHD) than children. Individuals with cardiac birth defects may be able to participate in physical activities but require proper cardiovascular evaluation. The American Heart Association and American College of Cardiology released guidelines in 2015 for athletes with cardiovascular abnormalities. The guidelines express that although restriction from competitive athletics may be indicated for some, the majority of individuals with CHD can and should engage in some form of physical activity. This case study demonstrates the importance of combining all aspects of history, physical examination, ECG, and imaging modalities to evaluate cardiac anatomy and function in young athletes with complex CHD. © 2017, Wiley Periodicals, Inc.

[Isolated left ventricular non-compaction associated with Ebstein's anomaly. Multimodality non-invasive imaging for the assessment of congenital heart disease].

PubMed

Renilla, Alfredo; Santamarta, Elena; Corros, Cecilia; Martín, María; Barreiro, Manuel; de la Hera, Jesús

2013-01-01

To establish the etiology of heart failure in patients with congenital heart disease can be challenging. Multiple concomitant anomalies that can be missed after an initial diagnosis could be seen in these patients. In patients with congenital heart disease, a more accurate evaluation of cardiac morphology and left ventricular systolic function could be evaluated by recent non-invasive cardiac imaging techniques. We present a rare case where multimodal cardiac imaging was useful to establish the final diagnosis of left ventricular non-compaction associated with Ebstein's anomaly. Copyright © 2012 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

Patient-Specific Modeling of Interventricular Hemodynamics in Single Ventricle Physiology

NASA Astrophysics Data System (ADS)

Vedula, Vijay; Feinstein, Jeffrey; Marsden, Alison

2016-11-01

Single ventricle (SV) congenital heart defects, in which babies are born with only functional ventricle, lead to significant morbidity and mortality with over 30% of patients developing heart failure prior to adulthood. Newborns with SV physiology typically undergo three palliative surgeries, in which the SV becomes the systemic pumping chamber. Depending on which ventricle performs the systemic function, patients are classified as having either a single left ventricle (SLV) or a single right ventricle (SRV), with SRV patients at higher risk of failure. As the native right ventricles are not designed to meet systemic demands, they undergo remodeling leading to abnormal hemodynamics. The hemodynamic characteristics of SLVs compared with SRVs is not well established. We present a validated computational framework for performing patient-specific modeling of ventricular flows, and apply it across 6 SV patients (3SLV + 3SRV), comparing hemodynamic conditions between the two subgroups. Simulations are performed with a stabilized finite element method coupled with an immersed boundary method for modeling heart valves. We discuss identification of hemodynamic biomarkers of ventricular remodeling for early risk assessment of failure. This research is supported in part by the Stanford Child Health Research Institute and the Stanford NIH-NCATS-CTSA through Grant UL1 TR001085 and due to U.S. National Institute of Health through NIH NHLBI R01 Grants 5R01HL129727-02 and 5R01HL121754-03.

Micro-RNA-208a, -208b, and -499 as Biomarkers for Myocardial Damage After Cardiac Surgery in Children.

PubMed

Bolkier, Yoav; Nevo-Caspi, Yael; Salem, Yishay; Vardi, Amir; Mishali, David; Paret, Gideon

2016-04-01

To test the hypothesis that cardiac-enriched micro-RNAs can serve as accurate biomarkers that reflect myocardial injury and to predict the postoperative course following pediatric cardiac surgery. Micro-RNAs have emerged as plasma biomarkers for many pathologic states. We aimed to quantify preoperative and postoperative plasma levels of cardiac-enriched micro-RNA-208a, -208b, and -499 in children undergoing cardiac surgery and to evaluate correlations between their levels, the extent of myocardial damage, and the postoperative clinical course. PICU. Thirty pediatric patients that underwent open heart surgery for the correction of congenital heart defects between January 2012 to July 2013. None. At 12 hours post surgery, the plasma levels of the micro-RNAs increased by 300- to 4,000-fold. At 24 hours, their levels decreased but remained significantly higher than before surgery. Micro-RNA levels were associated with troponin levels, longer cardiopulmonary bypass and aortic crossclamp times, maximal postoperative aspartate aminotransferase levels, and delayed hospital discharge. Circulating micro-RNA-208a, -208b, and -499 are detectable in the plasma of children undergoing cardiac surgery and may serve as novel biomarkers for monitoring and forecasting postoperative myocardial injury and recovery.

Short-term outcomes following implementation of a dedicated young adult congenital heart disease transition program.

PubMed

Vaikunth, Sumeet S; Williams, Roberta G; Uzunyan, Merujan Y; Tun, Han; Barton, Cheryl; Chang, Philip M

2018-01-01

Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease. We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January 2012 to December 2015 within a single academic institution that delivered pediatric and adult care at separate children's and adult hospitals, respectively. Demographic characteristics including congenital heart disease severity, gender, age, presence of comorbidities, presence of cardiac implantable electronic devices, and type of insurance were correlated to success of transfer. Rate of successful transfer was evaluated, and multivariate analysis was performed to determine which demographic variables were favorably associated with transfer. Thirty-nine percent of patients successfully transferred from pediatric to adult services during the study period. Severe congenital heart disease (OR 4.44, 95% CI 1.25-15.79, P = .02) and presence of a cardiac implantable electronic device (OR 4.93, 95% CI 1.18-20.58, P = .03) correlated with transfer. Trends favoring successful transfer with presence of comorbidities and private insurance were also noted. Despite a dedicated transition clinic, successful transfer rates remained relatively low though comparable to previously published rates. Severity of disease and presence of implantable devices correlated with successful transfer. Other obstacles to transfer remain and require combined efforts from pediatric and adult care systems, insurance carriers, and policy makers to improve transfer outcomes. © 2017 Wiley Periodicals, Inc.

Extraction of SelectSecure leads compared to conventional pacing leads in patients with congenital heart disease and congenital atrioventricular block.

PubMed

Shepherd, Emma; Stuart, Graham; Martin, Rob; Walsh, Mark A

2015-06-01

SelectSecure™ pacing leads (Medtronic Inc) are increasingly being used in pediatric patients and adults with structural congenital heart disease. The 4Fr lead is ideal for patients who may require lifelong pacing and can be advantageous for patients with complex anatomy. The purpose of this study was to compare the extraction of SelectSecure leads with conventional (stylette-driven) pacing leads in patients with structural congenital heart disease and congenital atrioventricular block. The data on lead extractions from pediatric and adult congenital heart disease (ACHD) patients from August 2004 to July 2014 at Bristol Royal Hospital for Children and the Bristol Heart Institute were reviewed. Multivariable regression analysis was used to determine whether conventional pacing leads were associated with a more difficult extraction process. A total of 57 patients underwent pacemaker lead extractions (22 SelectSecure, 35 conventional). No deaths occurred. Mean age at the time of extraction was 17.6 ± 10.5 years, mean weight was 47 ± 18 kg, and mean lead age was 5.6 ± 2.6 years (range 1-11 years). Complex extraction (partial extraction/femoral extraction) was more common in patients with conventional pacing leads at univariate (P < .01) and multivariate (P = .04) levels. Lead age was also a significant predictor of complex extraction (P < .01). SelectSecure leads can be successfully extracted using techniques that are used for conventional pacing leads. They are less likely to be partially extracted and are less likely to require extraction using a femoral approach compared with conventional pacing leads. Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

Clinicians' perspectives on patient satisfaction in adult congenital heart disease clinics--a dimension of health care quality whose time has come.

PubMed

Rozenblum, Ronen; Gianola, Ann; Ionescu-Ittu, Raluca; Verstappen, Amy; Landzberg, Michael; Gurvitz, Michelle; Jenkins, Kathy; Bates, David W; Marelli, Ariane J

2015-01-01

Patient-centered care and patient satisfaction represent key dimensions of health care quality. This is relevant for the growing number of patients with life-long conditions. In the present study, our goal was to examine clinicians' attitudes and behavior with respect to patient satisfaction in adult congenital heart disease outpatient clinics. A 34-question survey was developed to assess adult congenital heart disease clinicians' awareness, attitudes, and behavior relative to patient satisfaction and administered in-person or online to clinicians from the largely U.S.-based Adult Congenital Heart Association's database of adult congenital heart disease health care providers. Overall, 267 questionnaires were filled out: 108 were collected in person (79% response rate) and 159 online (17.5% response rate). Responses were received from physicians (161); nurses (73); physician assistants (20); and others (13). Although 85% of clinicians believed it was important to inquire about patient satisfaction, only 28% reported routinely inquiring about this dimension of care. Only 34% claimed they had adequate training to cope with varying levels of patient satisfaction, 44% stated that their department utilized patient satisfaction surveys, and 37% received feedback from the hospital management in the preceding 12 months. In multivariate analyses, clinicians that received feedback from the hospital management and had adequate training were more likely to inquire about patient satisfaction. Although patient satisfaction is perceived as an important dimension of quality care by adult congenital heart disease clinicians, most of them reported insufficient institutional support to achieve this. Our findings suggest that clinicians would benefit from health care organizations engaging them in the delivery of this dimension of health care quality. © 2014 Wiley Periodicals, Inc.

Self-efficacy as a predictor of patient-reported outcomes in adults with congenital heart disease.

PubMed

Thomet, Corina; Moons, Philip; Schwerzmann, Markus; Apers, Silke; Luyckx, Koen; Oechslin, Erwin N; Kovacs, Adrienne H

2018-04-01

Self-efficacy is a known predictor of patient-reported outcomes in individuals with acquired diseases. With an overall objective of better understanding patient-reported outcomes in adults with congenital heart disease, this study aimed to: (i) assess self-efficacy in adults with congenital heart disease, (ii) explore potential demographic and medical correlates of self-efficacy and (iii) determine whether self-efficacy explains additional variance in patient-reported outcomes above and beyond known predictors. As part of a large cross-sectional international multi-site study (APPROACH-IS), we enrolled 454 adults (median age 32 years, range: 18-81) with congenital heart disease in two tertiary care centres in Canada and Switzerland. Self-efficacy was measured using the General Self-Efficacy (GSE) scale, which produces a total score ranging from 10 to 40. Variance in the following patient-reported outcomes was assessed: perceived health status, psychological functioning, health behaviours and quality of life. Hierarchical multivariable linear regression analysis was performed. Patients' mean GSE score was 30.1 ± 3.3 (range: 10-40). Lower GSE was associated with female sex ( p = 0.025), not having a job ( p = 0.001) and poorer functional class ( p = 0.048). GSE positively predicted health status and quality of life, and negatively predicted symptoms of anxiety and depression, with an additional explained variance up to 13.6%. No associations between self-efficacy and health behaviours were found. GSE adds considerably to our understanding of patient-reported outcomes in adults with congenital heart disease. Given that self-efficacy is a modifiable psychosocial factor, it may be an important focus for interventions targeting congenital heart disease patients' well-being.

Accuracy of the Masimo SET® LNCS neo peripheral pulse oximeter in cyanotic congenital heart disease.

PubMed

Griksaitis, Michael J; Scrimgeour, Gemma E; Pappachan, John V; Baldock, Andrew J

2016-08-01

Introduction Non-invasive peripheral pulse oximeters are routinely used to measure oxyhaemoglobin saturation (SpO2) in cyanotic congenital heart disease. These probes are calibrated in healthy adult volunteers between arterial saturations of ~75 and 100%, using the gold standard of co-oximetry on arterial blood samples. There are little data to attest their accuracy in cyanotic congenital heart disease. Aims We aimed to assess the accuracy of a commonly used probe in children with cyanotic congenital heart disease. Children with cyanotic congenital heart disease admitted to the Paediatric Intensive Care Unit with an arterial line in situ were included to our study. Prospective simultaneous recordings of SpO2, measured by the Masimo SET® LNCS Neo peripheral probe, and co-oximeter saturations (SaO2) measured by arterial blood gas analysis were recorded. A total of 527 paired measurements of SpO2 and SaO2 (using an ABL800 FLEX analyser) in 25 children were obtained. The mean bias of the pulse oximeter for all SaO2 readings was +4.7±13.8%. The wide standard deviation indicates poor precision. This mean bias increased to +7.0±13.7% at SaO2 recordings <75%. The accuracy root mean square of the recordings was 3.30% across all saturation levels, and this increased to 4.98% at SaO2 <75%. The performance of the Masimo SET® LNCS Neo pulse oximeter is poor when arterial oxyhaemoglobin saturations are below 75%. It tends to overestimate saturations in children with cyanotic congenital heart disease. This may have serious implications for clinical decisions.

Spectrum Of Congenital Heart Disease In Full Term Neonates.

PubMed

Bibi, Saima; Hussain Gilani, Syed Yasir; Bibi, Shawana

2018-01-01

Congenital heart disease is a significant problem world over especially in neonates. Early diagnosis and prompt interventions in neonatal period precludes the mortality associated with this disorder. The objective of this study was to highlight the diversity of congenital cardiac defects in our region so that appropriate interventions are devised to minimize significant morbidity and mortality associated with this disorder. This descriptive cross-sectional study was conducted at the Neonatology Unit of Department of Paediatrics, Ayub Teaching Hospital from January 2015 to December 2016. Approval of ethical committee was taken. All fullterm neonates of either gender who presented in department of neonatology including those delivered in hospital or received from other sources (private settings, home deliveries), diagnosed as having congenital heart disease on echocardiography were included in the study. Preterm neonates of either gender were excluded from the study. Patient characteristics were recorded in a designed proforma. Data was entered in SPSS version 20 and analysed. A total of 89 neonates were included in the study. Mean age of presentation was 6.34±7.058 days and range of 1-28 days. There was a male preponderance with 57 (64%) male patients as compared to 32 (36%) female patients. Ventricular septal defect (VSD) was the commonest cardiac lesion being present in 34 (38.2%) patients. Other defects included complex congenital heart disease in 8 (9%), atrial septal defect (ASD) and transposition of great arteries (TGA) in 7 (7.9%) each, atrioventricular septal defect (AVSD) in 6 (6.7%) and Fallots's tetralogy (TOF) and hypoplastic left heart syndrome in 5 (5.6%) each.. Congenital heart disease is a problem of profound importance. It constitutes approximately one third of the total major congenital malformations. There is a diversity of cardiac lesions in our region that warrant early and prompt interventions so that the disease is recognized and treated at the earliest to reduce morbidity and mortality.

Thromboprophylaxis for atrial arrhythmias in congenital heart disease: A multicenter study.

PubMed

Khairy, Paul; Aboulhosn, Jamil; Broberg, Craig S; Cohen, Scott; Cook, Stephen; Dore, Annie; Fernandes, Susan M; Fournier, Anne; Kay, Joseph; Levesque, Sylvie; Macle, Laurent; Marcotte, François; Mondésert, Blandine; Mongeon, François-Pierre; Opotowsky, Alexander R; Proietti, Anna; Rivard, Lena; Ting, Jennifer; Thibault, Bernard; Zaidi, Ali; Hamilton, Robert

2016-11-15

There is a paucity of data to guide decisions regarding thromboprophylaxis for atrial arrhythmias in congenital heart disease. A retrospective multicenter cohort study enrolled patients with documented sustained atrial arrhythmias and congenital heart disease from 12 North American centers to quantify thromboembolic and bleeding rates associated with antiplatelet and anticoagulation therapy, and explore associated factors. A blinded committee adjudicated all qualifying arrhythmias and outcomes. A total of 482 patients, 45.2% female, age 32.0±18.0years, were followed for 11.3±9.4years since the qualifying arrhythmia. Antiplatelet therapy was administered to 37.8%, anticoagulation to 54.4%, and neither to 7.9%. Congenital heart disease complexity was simple, moderate, and severe in 18.5%, 34.4%, and 47.1%, respectively. Freedom from thromboembolic events was 84.7±2.7% at 15years, with no difference between anticoagulation versus antiplatelet therapy (P=0.97). Congenital heart disease complexity was independently associated with thromboembolic events, with rates of 0.00%, 0.93%, and 1.95%/year in those with simple, moderate, and severe forms (P<0.001). CHADS 2 and CHA 2 DS 2 -VASc scores were not predictive of thromboembolic risk. Annualized bleeding rates with antiplatelet and anticoagulation therapy were 0.66% and 1.82% (P=0.039). In multivariable analyses, anticoagulation [hazard ratio (HR) 4.76, 95% CI (1.05-21.58), P=0.043] and HAS-BLED score [HR 3.15, 95% CI (1.02, 9.78), P=0.047] were independently associated with major bleeds. Current management of atrial arrhythmias in congenital heart disease is associated with a modest rate of thromboembolic events, which is predicted by disease complexity but not CHADS 2 /CHA 2 DS 2 -VASc scores. HAS-BLED score is applicable to the congenital population in predicting major bleeds. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Impaired Mitochondrial Biogenesis Precedes Heart Failure in Right Ventricular Hypertrophy in Congenital Heart Disease

PubMed Central

Karamanlidis, Georgios; Bautista-Hernandez, Victor; Fynn-Thompson, Francis; Nido, Pedro del; Tian, Rong

2011-01-01

Background The outcome of the surgical repair in congenital heart disease (CHD) correlates with the degree of myocardial damage. In this study we determined whether mitochondrial DNA depletion is a sensitive marker of right ventricular (RV) damage and whether impaired mitochondrial DNA (mtDNA) replication contributes to the transition from compensated hypertrophy to failure. Methods and Results RV samples obtained from 31 patients undergoing cardiac surgery were compared to 5 RV samples from non-failing hearts (control). Patients were divided into compensated hypertrophy and failure groups based on preoperative echocardiography, catheterization and/or MRI data. Mitochondrial enzyme activities (citrate synthase and succinate dehydrogenase) were maintained during hypertrophy and decreased by ~40% (p<0.05 vs. control) at the stage of failure. In contrast, mtDNA content was progressively decreased in the hypertrophied RV through failure (by 28±8% and 67±11% respectively, p<0.05 for both), whereas mtDNA encoded gene expression was sustained by increased transcriptional activity during compensated hypertrophy but not in failure. MtDNA depletion was attributed to reduced mtDNA replication in both hypertrophied and failing RV and it was independent of PGC-1 down-regulation but was accompanied by reduced expression of proteins constituting the mtDNA replication fork. Decreased mtDNA content in compensated hypertrophy was also associated with pathological changes of mitochondria ultrastructure. Conclusions Impaired mtDNA replication causes early and progressive depletion of mtDNA in the RV of the CHD patients during the transition from hypertrophy to failure. Decreased mtDNA content is likely a sensitive marker of mitochondrial injury in this patient population. PMID:21840936

Delayed Sternal Closure in Infant Heart Surgery-The Importance of Where and When: An Analysis of the STS Congenital Heart Surgery Database.

PubMed

Nelson-McMillan, Kristen; Hornik, Christoph P; He, Xia; Vricella, Luca A; Jacobs, Jeffrey P; Hill, Kevin D; Pasquali, Sara K; Alejo, Diane E; Cameron, Duke E; Jacobs, Marshall L

2016-11-01

Delayed sternal closure (DSC) is commonly used to optimize hemodynamic stability after neonatal and infant heart surgery. We hypothesized that duration of sternum left open (SLO) was associated with rate of infection complications, and that location of sternal closure may mitigate infection risk. Infants (age ≤365 days) undergoing index operations with cardiopulmonary bypass and DSC at STS Congenital Heart Surgery Database centers (from 2007 to 2013) with adequate data quality were included. Primary outcome was occurrence of infection complication, defined as one or more of the following: endocarditis, pneumonia, wound infection, wound dehiscence, sepsis, or mediastinitis. Multivariable regression models were fit to assess association of infection complication with: duration of SLO (days), location of DSC procedure (operating room versus elsewhere), and patient and procedural factors. Of 6,127 index operations with SLO at 100 centers, median age and weight were 8 days (IQR, 5-24) and 3.3 kg (IQR, 2.9-3.8); 66% of operations were STAT morbidity category 4 or 5. At least one infection complication occurred in 18.7%, compared with 6.6% among potentially eligible neonates and infants without SLO. Duration of SLO (median, 3 days; IQR, 2-5) was associated with an increased rate of infection complications (p < 0.001). Location of DSC procedure was operating room (16%), intensive care unit (67%), or other (17%). Location of DSC was not associated with rate of infection complications (p = 0.45). Rate of occurrence of infectious complications is high among infants with sternum left open following cardiac surgery. Longer duration of SLO is associated with increased infection complications. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Tessier Number 30 Median Mandibular Cleft With Congenital Heart Anomalies in Qena, Egypt.

PubMed

Ali, Ahmed Ali Abdelrahim

2018-01-01

Median cleft deformities of the lower lip and mandible are very rare congenital anomalies. Our patient had median cleft of the lower lip, mandible, and the chin with tongue duplication, ankyloglossia, and cleft strap muscles with 2 neck contracture bands. This anomaly was associated with congenital heart disease transposition of great vessels, large ventricular septal defect, and severe pulmonary stenosis. Early repair was done at 6 months to improve feeding.

Variation in Outcomes for Risk-Stratified Pediatric Cardiac Surgical Operations: An Analysis of the STS Congenital Heart Surgery Database

PubMed Central

Jacobs, Jeffrey Phillip; O'Brien, Sean M.; Pasquali, Sara K.; Jacobs, Marshall Lewis; Lacour-Gayet, François G.; Tchervenkov, Christo I.; Austin III, Erle H.; Pizarro, Christian; Pourmoghadam, Kamal K.; Scholl, Frank G.; Welke, Karl F.; Gaynor, J. William; Clarke, David R.; Mayer, John E.; Mavroudis, Constantine

2013-01-01

Background. We evaluated outcomes for groups of risk-stratified operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database to provide contemporary benchmarks and examine variation between centers. Methods. Patients undergoing surgery from 2005 to 2009 were included. Centers with more than 10% missing data were excluded. Discharge mortality and postoperative length of stay (PLOS) among patients discharged alive were calculated for groups of risk-stratified operations using the five Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery mortality categories (STAT Mortality Categories). Power for analyzing between-center differences in outcome was determined for each STAT Mortality Category. Variation was evaluated using funnel plots and Bayesian hierarchical modeling. Results. In this analysis of risk-stratified operations, 58,506 index operations at 73 centers were included. Overall discharge mortality (interquartile range among programs with more than 10 cases) was as follows: STAT Category 1 = 0.55% (0% to 1.0%), STAT Category 2 = 1.7% (1.0% to 2.2%), STAT Category 3 = 2.6% (1.1% to 4.4%), STAT Category 4 = 8.0% (6.3% to 11.1%), and STAT Category 5 = 18.4% (13.9% to 27.9%). Funnel plots with 95% prediction limits revealed the number of centers characterized as outliers by STAT Mortality Categories was as follows: Category 1 = 3 (4.1%), Category 2 = 1 (1.4%), Category 3 = 7 (9.7%), Category 4 = 13 (17.8%), and Category 5 = 13 (18.6%). Between-center variation in PLOS was analyzed for all STAT Categories and was greatest for STAT Category 5 operations. Conclusions. This analysis documents contemporary benchmarks for risk-stratified pediatric cardiac surgical operations grouped by STAT Mortality Categories and the range of outcomes among centers. Variation was greatest for the more complex operations. These data may aid in the design and planning of quality assessment and quality improvement initiatives. PMID:22704799

Variation in outcomes for risk-stratified pediatric cardiac surgical operations: an analysis of the STS Congenital Heart Surgery Database.

PubMed

Jacobs, Jeffrey Phillip; O'Brien, Sean M; Pasquali, Sara K; Jacobs, Marshall Lewis; Lacour-Gayet, François G; Tchervenkov, Christo I; Austin, Erle H; Pizarro, Christian; Pourmoghadam, Kamal K; Scholl, Frank G; Welke, Karl F; Gaynor, J William; Clarke, David R; Mayer, John E; Mavroudis, Constantine

2012-08-01

We evaluated outcomes for groups of risk-stratified operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database to provide contemporary benchmarks and examine variation between centers. Patients undergoing surgery from 2005 to 2009 were included. Centers with more than 10% missing data were excluded. Discharge mortality and postoperative length of stay (PLOS) among patients discharged alive were calculated for groups of risk-stratified operations using the five Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery mortality categories (STAT Mortality Categories). Power for analyzing between-center differences in outcome was determined for each STAT Mortality Category. Variation was evaluated using funnel plots and Bayesian hierarchical modeling. In this analysis of risk-stratified operations, 58,506 index operations at 73 centers were included. Overall discharge mortality (interquartile range among programs with more than 10 cases) was as follows: STAT Category 1=0.55% (0% to 1.0%), STAT Category 2=1.7% (1.0% to 2.2%), STAT Category 3=2.6% (1.1% to 4.4%), STAT Category 4=8.0% (6.3% to 11.1%), and STAT Category 5=18.4% (13.9% to 27.9%). Funnel plots with 95% prediction limits revealed the number of centers characterized as outliers by STAT Mortality Categories was as follows: Category 1=3 (4.1%), Category 2=1 (1.4%), Category 3=7 (9.7%), Category 4=13 (17.8%), and Category 5=13 (18.6%). Between-center variation in PLOS was analyzed for all STAT Categories and was greatest for STAT Category 5 operations. This analysis documents contemporary benchmarks for risk-stratified pediatric cardiac surgical operations grouped by STAT Mortality Categories and the range of outcomes among centers. Variation was greatest for the more complex operations. These data may aid in the design and planning of quality assessment and quality improvement initiatives. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Rapidly progressive heart failure requiring transplantation in muscular dystrophy: a need for frequent screening.

PubMed

Pick, Justin M; Ellis, Zachary D; Alejos, Juan C; Chang, Anthony C

2017-11-01

Fukuyama congenital muscular dystrophy weakens both skeletal and cardiac muscles, but the rate of cardiomyopathic progression can accelerate faster than that of skeletal muscles. A 14-year-old boy with Fukuyama congenital muscular dystrophy presented with mild skeletal myopathy but severe cardiomyopathy requiring heart transplantation within 1 year of declining heart function. These patients need frequent screening regardless of musculoskeletal symptoms.

Interventional Cardiology for Congenital Heart Disease.

PubMed

Kenny, Damien

2018-05-01

Congenital heart interventions are now replacing surgical palliation and correction in an evolving number of congenital heart defects. Right ventricular outflow tract and ductus arteriosus stenting have demonstrated favorable outcomes compared to surgical systemic to pulmonary artery shunting, and it is likely surgical pulmonary valve replacement will become an uncommon procedure within the next decade, mirroring current practices in the treatment of atrial septal defects. Challenges remain, including the lack of device design focused on smaller infants and the inevitable consequences of somatic growth. Increasing parental and physician expectancy has inevitably lead to higher risk interventions on smaller infants and appreciation of the consequences of these interventions on departmental outcome data needs to be considered. Registry data evaluating congenital heart interventions remain less robust than surgical registries, leading to a lack of insight into the longer-term consequences of our interventions. Increasing collaboration with surgical colleagues has not been met with necessary development of dedicated equipment for hybrid interventions aimed at minimizing the longer-term consequences of scar to the heart. Therefore, great challenges remain to ensure children and adults with congenital heart disease continue to benefit from an exponential growth in minimally invasive interventions and technology. This can only be achieved through a concerted collaborative approach from physicians, industry, academia and regulatory bodies supporting great innovators to continue the philosophy of thinking beyond the limits that has been the foundation of our specialty for the past 50 years. Copyright © 2018. The Korean Society of Cardiology.

Congenital heart defects in Williams syndrome.

PubMed

Yuan, Shi-Min

2017-01-01

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.

Congenital heart surgery in developing countries.

PubMed

Jonas, Richard A

2008-01-01

This article reviews the challenges in caring for individuals with congenital heart disease in developing countries and is drawn in part from the author's Presidential Address to the American Association for Thoracic Surgery in Philadelphia, May 2006. Economic globalization is resulting in demographic and lifestyle changes which are increasing the incidence of ischemic heart disease worldwide. A massive expansion of cardiothoracic surgery in developing countries is beginning. Although the rate of coronary surgery is contracting in Western countries, the population bulge that is approaching 65 years of age will also stretch surgical manpower and resources in developed countries within 10 to 20 years. The incidence of congenital heart disease is stable, but earlier and more accurate diagnosis is rapidly increasing the numbers of children needing the services of a congenital cardiac surgery team. Many opportunities for philanthropy are available, but care should be taken to assist in the most meaningful way by working with a long-term educational program and avoiding surgical tourism.

Current status of cardiovascular surgery in Japan 2013 and 2014: A report based on the Japan Cardiovascular Surgery Database. 2: Congenital heart surgery.

PubMed

Hirata, Yasutaka; Hirahara, Norimichi; Murakami, Arata; Motomura, Noboru; Miyata, Hiroaki; Takamoto, Shinichi

2018-01-01

We analyzed the mortality and morbidity of congenital heart surgery in Japan using the Japan Cardiovascular Surgery Database (JCVSD). Data regarding congenital heart surgery performed between January 2013 and December 2014 were obtained from JCVSD. The 20 most frequent procedures were selected and the mortality rates and major morbidities were analyzed. The mortality rates of atrial septal defect repair and ventricular septal defect repair were less than 1%, and the mortality rates of tetralogy of Fallot repair, complete atrioventricular septal defect repair, bidirectional Glenn, and total cavopulmonary connection were less than 2%. The mortality rates of the Norwood procedure and total anomalous pulmonary venous connection repair were more than 10%. The rates of unplanned reoperation, pacemaker implantation, chylothorax, deep sternal infection, phrenic nerve injury, and neurological deficit were shown for each procedure. Using JCVSD, the national data for congenital heart surgery, including postoperative complications, were analyzed. Further improvements of the database and feedback for clinical practice are required.

The maternal-age-associated risk of congenital heart disease is modifiable.

PubMed

Schulkey, Claire E; Regmi, Suk D; Magnan, Rachel A; Danzo, Megan T; Luther, Herman; Hutchinson, Alayna K; Panzer, Adam A; Grady, Mary M; Wilson, David B; Jay, Patrick Y

2015-04-09

Maternal age is a risk factor for congenital heart disease even in the absence of any chromosomal abnormality in the newborn. Whether the basis of this risk resides with the mother or oocyte is unknown. The impact of maternal age on congenital heart disease can be modelled in mouse pups that harbour a mutation of the cardiac transcription factor gene Nkx2-5 (ref. 8). Here, reciprocal ovarian transplants between young and old mothers establish a maternal basis for the age-associated risk in mice. A high-fat diet does not accelerate the effect of maternal ageing, so hyperglycaemia and obesity do not simply explain the mechanism. The age-associated risk varies with the mother's strain background, making it a quantitative genetic trait. Most remarkably, voluntary exercise, whether begun by mothers at a young age or later in life, can mitigate the risk when they are older. Thus, even when the offspring carry a causal mutation, an intervention aimed at the mother can meaningfully reduce their risk of congenital heart disease.

Real-time Three-dimensional Echocardiography: From Diagnosis to Intervention.

PubMed

Orvalho, João S

2017-09-01

Echocardiography is one of the most important diagnostic tools in veterinary cardiology, and one of the greatest recent developments is real-time three-dimensional imaging. Real-time three-dimensional echocardiography is a new ultrasonography modality that provides comprehensive views of the cardiac valves and congenital heart defects. The main advantages of this technique, particularly real-time three-dimensional transesophageal echocardiography, are the ability to visualize the catheters, and balloons or other devices, and the ability to image the structure that is undergoing intervention with unprecedented quality. This technique may become one of the main choices for the guidance of interventional cardiology procedures. Copyright © 2017 Elsevier Inc. All rights reserved.

[Neonatal tumours and congenital malformations].

PubMed

Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

2008-06-01

The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data exist on the neonatal period and the majority are from medical institutions registers. The prevalence varies from 15 to 31.6%. To explain this association, the hypotheses are based on prenatal exposures (preconceptional and transplacental exposure), to mutagenic and carcinogenic risk factors. Neonatal tumours are more often associated to congenital abnormalities than other pediatric cancers. The inclusion and classification criteria needs to be unified to better understand the association between the neonatal tumours and congenital abnormalities. The environmental history in all neonatal tumours associated to congenital abnormalities, including the constitutional and environmental risk factors, will help to improve our knowledge of the underlying prenatal mechanisms and to an advance in its prevention.

Neurologic sequelae of cardiac surgery in children.

PubMed

Ferry, P C

1987-03-01

Major advances in surgical and cardiopulmonary bypass technology have occurred in the past 30 years. Total correction of previously inoperable congenital cardiac defects is being performed with increasing frequency and in children at progressively younger ages. While the majority of children undergoing cardiac surgery survive without incident, increasing concern is being raised about neurologic sequelae seen in some survivors. Complications such as embolization, hypoxia, inadequate cerebral perfusion, and biochemical disturbances may all lead to brain damage following cardiac surgery. Acute postoperative neurologic problems include seizures, impaired levels of consciousness, focal motor deficits, and movement disorders. Long-term sequelae include language and learning disorders, mental retardation, seizures, and cerebral palsy. Intraoperative cerebral monitoring techniques are as yet imperfect, but their use in combination with meticulous intraoperative and postoperative care currently provides the best means of reducing neurologic morbidity. Future studies should explore other methods of preserving neurologic integrity in children undergoing open heart surgery.

Does the visibility of a congenital anomaly affect maternal-infant attachment levels?

PubMed

Boztepe, Handan; Ay, Ayşe; Kerimoğlu Yıldız, Gizem; Çınar, Sevil

2016-10-01

To determine whether congenital anomaly visibility affects maternal-infant attachment levels. The study population consisted of mothers who had infants with cleft lip/palate or congenital heart anomalies who were receiving treatment in a university hospital. The data were collected using the Structured Questionnaire Form and the Maternal Attachment Inventory. Statistically significant differences in maternal-infant attachment levels were observed between infants with cleft lips/palates and healthy infants and between infants with congenital heart anomalies and healthy infants. It is important to apply appropriate nursing interventions for these mothers during the postpartum period. © 2016, Wiley Periodicals, Inc.

Congenital anomalies of the pulmonary arteries: spectrum of findings on computed tomography.

PubMed

Bueno, J; Flors, L; Mejía, M

Congenital anomalies of the pulmonary arteries are uncommon. They can occur in isolation or in association with congenital heart defects. Isolated congenital anomalies remain undiscovered until they are reported as incidental findings on imaging tests, usually not until adolescence. We review the embryological development and normal anatomy of the pulmonary arteries as well as the spectrum of computed tomography findings for various congenital anomalies: unilateral interruption of the pulmonary artery, anomalous origin of the left pulmonary artery (pulmonary artery sling), idiopathic aneurysm of the pulmonary artery, and other anomalies associated with congenital heart defects. Congenital anomalies of the pulmonary arteries represent a diagnostic challenge for clinicians and radiologists. Computed tomography is useful for their diagnosis, and general radiologists need to be familiar with their imaging appearance because they are often discovered incidentally. Copyright © 2016 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

Hypertension, obesity, and coronary artery disease in the survivors of congenital heart disease.

PubMed

Roche, S Lucy; Silversides, Candice K

2013-07-01

Obesity, hypertension, and coronary artery disease are prevalent in the general population and well recognized as contributors to cardiac morbidity and mortality. With surgical and medical advances, there is a growing and aging population with congenital heart disease who are also at risk of developing these comorbidities. In addition, some congenital cardiac lesions predispose patients to conditions such as hypertension or coronary artery disease. The effect of these comorbidities on the structurally abnormal heart is not well understood, but might be very important, especially in those with residual abnormalities. Thus, in addition to surveillance for and treatment of late complications it is important for the congenital cardiologist to consider and aggressively manage acquired comorbidities. In this review we explore the prevalence of hypertension, obesity, and coronary artery disease, discuss congenital lesions that predispose to these conditions and review management strategies for this unique population. Copyright © 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

Bmp2 and Notch cooperate to pattern the embryonic endocardium.

PubMed

Papoutsi, T; Luna-Zurita, L; Prados, B; Zaffran, S; de la Pompa, J L

2018-05-31

Signaling interactions between myocardium and endocardium pattern embryonic cardiac regions, instructing their development to fulfill specific functions in the mature heart. We show that ectopic Bmp2 expression in the mouse chamber myocardium changes the transcriptional signature of adjacent chamber endocardial cells into valve tissue, and enables them to undergo epithelial-mesenchyme transition. This induction is independent of valve myocardium specification and requires high levels of Notch1 activity. Biochemical experiments suggest that Bmp2-mediated Notch1 induction is achieved through transcriptional activation of the Notch ligand Jag1, and physical interaction of Smad1/5 with the intracellular domain of the Notch1 receptor. Thus, widespread myocardial Bmp2 and endocardial Notch signaling drive presumptive ventricular endocardium to differentiate into valve endocardium. Understanding the molecular basis of valve development is instrumental to designing therapeutic strategies for congenital heart valve defects. © 2018. Published by The Company of Biologists Ltd.

Evolution of membrane oxygenator technology for utilization during pediatric cardiopulmonary bypass.

PubMed

Melchior, Richard W; Sutton, Steven W; Harris, William; Dalton, Heidi J

2016-01-01

The development of the membrane oxygenator for pediatric cardiopulmonary bypass has been an incorporation of ideology and technological advancements with contributions by many investigators throughout the past two centuries. With the pursuit of this technological achievement, the ability to care for mankind in the areas of cardiac surgery has been made possible. Heart disease can affect anyone within the general population, but one such segment that it can affect from inception includes children. Currently, congenital heart defects are the most common birth defects nationally and worldwide. A large meta-analysis study from 1930 to 2010 was conducted in review of published medical literature totaling 114 papers with a study population of 24,091,867 live births, and divulged a staggering incidence of congenital heart disease involving 164,396 subjects with diverse cardiac illnesses. The prevalence of these diseases increased from 0.6 per 1,000 live births from 1930-1934 to 9.1 per 1,000 live births after 1995. These data reveal an emphasis on a growing public health issue regarding congenital heart disease. This discovery displays a need for heightened awareness in the scientific and medical industrial community to accelerate investigative research on emerging cardiovascular devices in an effort to confront congenital anomalies. One such device that has evolved over the past several decades is the pediatric membrane oxygenator. The pediatric membrane oxygenator, in conjunction with the heart lung machine, assists in the repair of most congenital cardiac defects. Numerous children born with congenital heart disease with or without congestive heart failure have experienced improved clinical outcomes in quality of life, survival, and mortality as a result of the inclusion of this technology during their cardiac surgical procedure. The purpose of this review is to report a summary of the published medical and scientific literature related to development of the pediatric membrane oxygenator from its conceptual evolutionary stages to artificially supporting whole body perfusion in the modern pediatric cardiac surgical setting.

Association of US State Implementation of Newborn Screening Policies for Critical Congenital Heart Disease With Early Infant Cardiac Deaths.

PubMed

Abouk, Rahi; Grosse, Scott D; Ailes, Elizabeth C; Oster, Matthew E

2017-12-05

In 2011, critical congenital heart disease was added to the US Recommended Uniform Screening Panel for newborns, but whether state implementation of screening policies has been associated with infant death rates is unknown. To assess whether there was an association between implementation of state newborn screening policies for critical congenital heart disease and infant death rates. Observational study with group-level analyses. A difference-in-differences analysis was conducted using the National Center for Health Statistics' period linked birth/infant death data set files for 2007-2013 for 26 546 503 US births through June 30, 2013, aggregated by month and state of birth. State policies were classified as mandatory or nonmandatory (including voluntary policies and mandates that were not yet implemented). As of June 1, 2013, 8 states had implemented mandatory screening policies, 5 states had voluntary screening policies, and 9 states had adopted but not yet implemented mandates. Numbers of early infant deaths (between 24 hours and 6 months of age) coded for critical congenital heart disease or other/unspecified congenital cardiac causes for each state-month birth cohort. Between 2007 and 2013, there were 2734 deaths due to critical congenital heart disease and 3967 deaths due to other/unspecified causes. Critical congenital heart disease death rates in states with mandatory screening policies were 8.0 (95% CI, 5.4-10.6) per 100 000 births (n = 37) in 2007 and 6.4 (95% CI, 2.9-9.9) per 100 000 births (n = 13) in 2013 (for births by the end of July); for other/unspecified cardiac causes, death rates were 11.7 (95% CI, 8.6-14.8) per 100 000 births in 2007 (n = 54) and 10.3 (95% CI, 5.9-14.8) per 100 000 births (n = 21) in 2013. Early infant deaths from critical congenital heart disease through December 31, 2013, decreased by 33.4% (95% CI, 10.6%-50.3%), with an absolute decline of 3.9 (95% CI, 3.6-4.1) deaths per 100 000 births after states implemented mandatory screening compared with prior periods and states without screening policies. Early infant deaths from other/unspecified cardiac causes declined by 21.4% (95% CI, 6.9%-33.7%), with an absolute decline of 3.5 (95% CI, 3.2-3.8) deaths per 100 000 births. No significant decrease was associated with nonmandatory screening policies. Statewide implementation of mandatory policies for newborn screening for critical congenital heart disease was associated with a significant decrease in infant cardiac deaths between 2007 and 2013 compared with states without these policies.

Left-right asymmetry and cardiac looping: implications for cardiac development and congenital heart disease.

PubMed

Kathiriya, I S; Srivastava, D

2000-01-01

Proper morphogenesis and positioning of internal organs requires delivery and interpretation of precise signals along the anterior-posterior, dorsal-ventral, and left-right axes. An elegant signaling cascade determines left- versus right-sided identity in visceral organs in a concordant fashion, resulting in a predictable left-right (LR) organ asymmetry in all vertebrates. The complex morphogenesis of the heart and its connections to the vasculature are particularly dependent upon coordinated LR signaling pathways. Disorganization of LR signals can result in myriad congenital heart defects that are a consequence of abnormal looping and remodeling of the primitive heart tube into a multi-chambered organ. A framework for understanding how LR asymmetric signals contribute to normal organogenesis has emerged and begins to explain the basis of many human diseases of LR asymmetry. Here we review the impact of LR signaling pathways on cardiac development and congenital heart disease.

Folic acid supplementation and the occurrence of congenital heart defects, orofacial clefts, multiple births, and miscarriage.

PubMed

Bailey, Lynn B; Berry, Robert J

2005-05-01

Key research findings relative to the question of whether maternal use of folic acid before and during pregnancy reduces the chance that offspring will be born with a congenital heart defect or an orofacial cleft are reviewed in this paper. Observational studies in general support an association between maternal use of multivitamins containing folic acid and a reduction in the occurrence of congenital heart defects and orofacial clefts. Results from one randomized controlled trial (RCT) provide the strongest evidence that multivitamins prevent congenital heart defects, but this RCT did not provide evidence that multivitamins prevent orofacial clefts. In addition, most observational and interventional studies are not designed to detect an independent effect from folic acid. Early studies suggested that periconceptional multivitamin use was associated with an increased occurrence of both miscarriages and multiple births, which has resulted in a great deal of controversy about the safety of folic acid use during pregnancy. We also review reports that were designed to answer these questions with more definitive data. When more substantial evidence about the effect of periconceptional folic acid on the occurrence of congenital heart defects and orofacial clefts is reported, we will have additional support for promoting folic acid intervention programs. All women capable of becoming pregnant should continue to consume 400 mug/d of folic acid in addition to a healthy diet as advised.

From adolescents to adults with congenital heart disease: the role of transition.

PubMed

Moceri, Pamela; Goossens, Eva; Hascoet, Sebastien; Checler, Carine; Bonello, Béatrice; Ferrari, Emile; Acar, Philippe; Fraisse, Alain

2015-07-01

Improved surgical care during the last decades, together with advances in medical management, led to a remarkable increase in survival of patients with congenital heart disease (CHD). However, aging of the CHD population brings new challenges, and loss of follow-up of adolescents and adults with CHD is a major concern. It is crucial to optimize the transfer of patients with CHD from paediatric to adult health care services to prevent loss to follow-up. The transition process plays a central role in the future health and follow-up of the patient. The aim of this review is to explain and discuss the clinical impact of the transition process in adolescents with CHD. We will also discuss specific CHD adolescents' problems. Adolescence is a crucial phase for the formation of the personality. Understanding and acceptance of the responsibility for health at this stage through a transition process with a multidisciplinary team will determine the quality of future medical follow-up and probably limit psychosocial issues in their adult life. • Aging of the congenital heart disease population brings new challenges to the organisation of care. • Loss of follow-up is a major concern for patients with congenital heart disease. What is new: • The quality of a formal transition process during adolescence will determine future outcomes in patients with congenital heart disease.

Infant temperament and parental stress in 3-month-old infants after surgery for complex congenital heart disease.

PubMed

Torowicz, Deborah; Irving, Sharon Y; Hanlon, Alexandra L; Sumpter, Danica F; Medoff-Cooper, Barbara

2010-04-01

This study aimed to identify and compare differences in temperament and maternal stress between infants with complex congenital heart disease and healthy controls at 3 months of age. Study sample was drawn from an existing longitudinal study examining growth in infants with congenital heart disease when compared with healthy controls. Infant temperament and parental stress were measured in 129 mother-infant dyads. Inclusion criteria for infants with congenital heart disease were > or = 36-week postmenstrual age, > or = 2500 g at birth, surgery in first 6 weeks of life, and no major congenital anomalies or genetic syndromes. The Early Infancy Temperament Questionnaire and Parent Stress Index were the assessment tools used. Infants with single ventricular (SV) physiology were more negative in mood (F = 7.14, p < .001) and less distractible (F = 5.00, p < .008) than the biventricular physiology or Control (C) infant groups. The demands of care for infants with congenital heart disease were a source of stress when compared with Control infants (p < .05). Five of 6 subscales of the Child Domain were significant sources of stress in the SV group compared with biventricle and Control groups. Negative mood and difficulty to soothe were predictors for Child Domain and Total Life Stress in SV infants. The demands of parenting an irritable infant with SV physiology put these mothers at risk for high levels of stress. Results suggest the need for predischarge anticipatory guidance for parents to better understand and respond to the behavioral style of their infants, in particular, infants with SV physiology.

A Double-Blinded, Randomized, Placebo-Controlled Clinical Trial of Aminophylline to Prevent Acute Kidney Injury in Children following Congenital Heart Surgery with Cardiopulmonary Bypass

PubMed Central

Axelrod, David M.; Sutherland, Scott M.; Anglemyer, Andrew; Grimm, Paul C.; Roth, Stephen J.

2015-01-01

Objective Acute kidney injury (AKI) occurs commonly in children following congenital cardiac surgery with cardiopulmonary bypass (CPB) and has been associated with increased morbidity and mortality. Aminophylline, a methylxanthine nonselective adenosine receptor antagonist, has been effective in the management of AKI in certain populations. This study sought to determine if post-operative administration of aminophylline attenuates AKI in children undergoing congenital cardiac surgery with CPB. Design Single-center, double-blinded, placebo-controlled, randomized clinical trial (RCT). Setting Tertiary center, pediatric cardiovascular intensive care unit. Patients 144 children after congenital heart surgery with CPB. Interventions Seventy-two patients were randomized to receive aminophylline and 72 patients received placebo. Study drug was administered every six hours for 72 hours. Measurements and Main Results The primary outcome variable was development of any AKI, defined by the serum creatinine criteria of the Kidney Diseases: Improving Global Outcomes (KDIGO) criteria. Secondary outcomes included the development of severe AKI, time between CVICU admission and first successful extubation, percent fluid overload, total fluid balance, urine output, bioelectrical impedance, and serum neutrophil gelatinase-associated lipocalin (NGAL). The unadjusted rate and severity of AKI were not different between groups; 43/72 (60%) of the treatment group and 36/72 (50%) of the placebo group developed AKI (p=0.32). Stage 2/3 AKI occurred in 23/72 (32%) of the treatment group and 15/72 (21%) of the placebo group (p=0.18). Secondary outcome measures also demonstrated no significant difference between treatment and placebo groups. Aminophylline administration was safe; no deaths occurred in either group, and rates of adverse events were similar (14% in the treatment group versus 18% in the placebo group, p =0.30). Conclusions In this placebo-controlled RCT, we found no effect of aminophylline to prevent AKI in children recovering from cardiac surgery performed with CPB. Future study of pre-operative aminophylline administration to prevent AKI may be warranted. PMID:26669642

Specificity and effector mechanisms of autoantibodies in congenital heart block.

PubMed

Wahren-Herlenius, Marie; Sonesson, Sven-Erik

2006-12-01

Complete congenital atrio-ventricular (AV) heart block develops in 2-5% of fetuses of Ro/SSA and La/SSB autoantibody-positive pregnant women. During pregnancy, the Ro/SSA and La/SSB antibodies are transported across the placenta and affect the fetus. Emerging data suggest that this happens by a two-stage process. In the first step, maternal autoantibodies bind fetal cardiomyocytes, dysregulate calcium homestasis and induce apoptosis in affected cells. This step might clinically correspond to a first-degree heart block, and be reversible. La/SSB antibodies can bind apoptotic cardiomyocytes and thus increase Ig deposition in the heart. The tissue damage could, as a second step, lead to spread of inflammation in genetically pre-disposed fetuses, progressing to fibrosis and calcification of the AV-node and subsequent complete congenital heart block. Early intrauterine treatment of an incomplete AV-block with fluorinated steroids has been shown to prevent progression of the heart block, making it clinically important to find specific markers to identify the high-risk pregnancies.

Birth Defects & Other Health Conditions

MedlinePlus

... Congenital heart defects and CCHD Congenital syphilis Congenital Zika syndrome Coxsackie infection and your baby Cystic fibrosis ... families in our new awareness campaign video. GO Zika services near you Visit Zika Care Connect to ...

Cardiac haemangioma associated with a duct-dependent congenital heart disease in a newborn infant.

PubMed

Djordjevic, Stefan A; Glumac, Sofija; Kalanj, Jasna

2017-07-01

Cardiac haemangiomas are exceedingly rare; however, they can cause significant haemodynamic impairment and disturbances in heart rhythm. Rarely, cardiac tumours may also coexist with congenital heart lesions. We present an extremely unusual case of a cardiac haemangioma in the setting of complex transposition of the great arteries that caused functional tricuspid atresia. To our knowledge, this is the first such case described in the literature.

Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

PubMed Central

Mourato, Felipe Alves; Villachan, Lúcia Roberta R.; Mattos, Sandra da Silva

2014-01-01

OBJECTIVE: To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis. METHODS: Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7. RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age. CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension. PMID:25119745

Understanding age-based transition needs: Perspectives from adolescents and adults with congenital heart disease

USDA-ARS?s Scientific Manuscript database

The purpose of this study was to explore the transition process in congenital heart disease (CHD) care through the perceived needs and concerns of adolescents (pretransition) and the experiential insight from adults (post-transition), in order to inform future transition initiatives and information ...

Determining transition readiness in congenital heart disease: Assessing the utility of the Transition Readiness Questionnaire

USDA-ARS?s Scientific Manuscript database

The Transition Readiness Assessment Questionnaire (TRAQ) is a tool commonly used to assess transition readiness in adolescents with chronic diseases. It was previously validated in youth with special health care needs (YSHCN), but no patients with congenital heart disease (CHD) were included in the ...

Hypoplastic left heart syndrome (image)

MedlinePlus

Hypoplastic left heart syndrome is a congenital heart condition that occurs during the development of the heart in the ... womb. During the heart's development, parts of the left side of the heart (mitral valve, left ventricle ...

Fetal and neonatal mortality in patients with isolated congenital heart diseases and heart conditions associated with extracardiac abnormalities.

PubMed

Marantz, Pablo; Sáenz Tejeira, M Mercedes; Peña, Gabriela; Segovia, Alejandra; Fustiñana, Carlos

2013-10-01

Congenital malformations are a known cause of intrauterine death; of them, congenital heart diseases (CHDs) are accountable for the highest fetal and neonatal mortality rates. They are strongly associated with other extracardiac malformations and an early fetal mortality. Two hundred and twenty fves cases of CHDs are presented. Of them, 155 were isolated CHDs (group A) and 70 were associated with extracardiac malformations, chromosomal disorders, or genetic syndromes (group B). The overall mortality in group B was higher than that observed in group A (p <0.01). Prenatal mortality was similar in both groups: A: 8.4% (13 out of 155); B: 15.7% (11 out of 70). Postnatal mortality was A: 16.8% (26 out of 155) (p <0.01), OR: 0.52 (95% CI: 0.16-1.7); B: 32.9% (23 out of 70) (p <0.01), OR: 0.41 (95% CI: 0.20-0.83). Heart diseases associated with extracardiac abnormalities had a higher mortality rate than isolated congenital heart diseases in the period up to 60 weeks of postmenstrual age (140 days post-term). No differences were observed between both groups of patients in terms of prenatal mortality.

Current Interventional and Surgical Management of Congenital Heart Disease: Specific Focus on Valvular Disease and Cardiac Arrhythmias.

PubMed

Holst, Kimberly A; Said, Sameh M; Nelson, Timothy J; Cannon, Bryan C; Dearani, Joseph A

2017-03-17

Successful outcome in the care of patients with congenital heart disease depends on a comprehensive multidisciplinary team. Surgery is offered for almost every heart defect, despite complexity. Early mortality for cardiac surgery in the neonatal period is ≈10% and beyond infancy is <5%, with 90% to 95% of patients surviving with a good quality of life into the adult years. Advances in imaging have facilitated accurate diagnosis and planning of interventions and surgical procedures. Similarly, advances in the perioperative medical management of patients, particularly with intensive care, has also contributed to improving outcomes. Arrhythmias and heart failure are the most common late complications for the majority of defects, and reoperation for valvar problems is common. Lifelong surveillance for monitoring of recurrent or residual structural heart defects, as well as periodic assessment of cardiac function and arrhythmia monitoring, is essential for all patients. The field of congenital heart surgery is poised to incorporate new innovations such as bioengineered cells and scaffolds that will iteratively move toward bioengineered patches, conduits, valves, and even whole organs. © 2017 American Heart Association, Inc.

Presentation of obstructive left heart malformations in infancy.

PubMed Central

Abu-Harb, M; Wyllie, J; Hey, E; Richmond, S; Wren, C

1994-01-01

This study documents the presentation of acutely life threatening congenital heart disease in infancy in order to assess the performance of screening examinations soon after birth and at 6 weeks of age. All cases of obstructive left heart malformations presenting in infancy in one health region from 1987-91 were analysed retrospectively. Altogether, 120 infants presented with either hypoplastic left heart syndrome, interruption of the aortic arch, coarctation of the aorta, or aortic valve stenosis. Twelve became symptomatic or died within 24 hours. Thirty four had an abnormal neonatal examination, eight of whom were referred. Six more became symptomatic before discharge. Ninety four babies went home, 51 developed heart failure before 6 weeks, and another seven died without diagnosis. Twenty five of 36 (69%) reaching 6 weeks without diagnosis had a second examination which was abnormal in 17. Two babies died undiagnosed after 6 weeks and the other 18 presented at up to 11 months of age. The neonatal and 6 week examinations perform poorly as screening tests for congenital heart disease. A normal neonatal examination does not exclude life threatening congenital heart disease. Images PMID:7820713

[Risk factors for surgery of congenital heart disease in adults: twenty-two years of experience. Who should operate them?].

PubMed

Aroca, Angel; Polo, Luz; Pérez-Farinós, Napoleón; González, Ana E; Bret, Montserrat; Aguilar, Elizabeth; Oliver, José M

2014-01-01

To assess the association between mortality in surgery of congenital heart disease in adults, and factors related to patients and operations. Descriptive study of operations performed by specialized surgeons in congenital heart surgery (238), adult acquired surgery (117), and specialty residents (108). The association of mortality with surgical risk and complexity, specialization of surgeon, cardiopulmonary by-pass and aortic cross clamping was assessed fitting logistic regression models. A total of 463 operations were included (442 with cardiopulmonary by-pass) in the study performed between 1991 and 2012. Median age at surgery: 34; 52.8% were women. First surgery: 295, reoperation: 168. Median score of Aristotle was 6.8, with significantly higher complexity since 2001, after restructuring the Unit. Overall hospital mortality was 3.9%. Mortality was significantly associated to number of previous surgeries (OR: 5.02; 95%CI: 1.44-17.52), operations by acquired heart disease surgeons (OR: 3.53; 95%CI: 1.14-10.98), higher Aristotle (OR: 1,64; 95%CI: 1.18-2.29), and high cardiopulmonary by-pass time (OR: 1.13; 95%CI: 1.07-1.19). Surgery of congenital heart disease in adults has been performed with low mortality. High complexity interventions, prolonged cardiopulmonary by-pass times and multiple reoperations were associated to higher mortality. Participation of cardiac surgeons specialized in congenital heart disease is associated with better outcomes. Copyright © 2013 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

Jarcho-Levin syndrome presenting with diaphragmatic hernia.

PubMed

Onay, O S; Kinik, S T; Otgün, Y; Arda, I S; Varan, B

2008-08-01

Jarcho-Levin syndrome (spondylothoracic or spondylocostal dysostosis) is an eponym that is used to define individuals with a short neck, short trunk, and short stature and multiple vertebral anomalies. The prognosis is directly related to respiratory complications. Reported findings associated with Jarcho-Levin syndrome include congenital heart defects, abdominal wall malformations, genitourinary malformations, upper limb anomalies, and neural tube defects. We report on a 6-day-old girl who presented with an incomplete form of Jarcho-Levin syndrome with late-presenting congenital diaphragmatic hernia and congenital heart disease.

What Is Transesophageal Echocardiography?

MedlinePlus

... disease Heart attack Aortic aneurysm (AN-u-rism) Endocarditis (EN-do-kar-DI-tis) Cardiomyopathy (KAR-de- ... disease Congenital heart disease Heart attack Aortic aneurysm Endocarditis Cardiomyopathy Heart valve disease Injury to the heart ...

Handicapping Conditions Associated with the Congenital Rubella Syndrome.

ERIC Educational Resources Information Center

Vernon, McCay; And Others

1980-01-01

The authors discuss the incidence of impairments diagnosed among children with congenital rubella syndrome. Approximately 73 percent are hearing impaired, at least 35 percent have congenital heart disorders, and 33 percent have visual defects. (Author)

The Congenital Heart Surgeons Society Datacenter: unique attributes as a research organization.

PubMed

Caldarone, Christopher A; Williams, William G

2010-01-01

Over the last 25 years, the Congenital Heart Surgeons Society (CHSS) has evolved from an informal club to a mature organization. A central feature of the CHSS has been dedication to evaluating outcomes of congenital heart surgery across a wide array of clinical diagnoses. These research activities have been orchestrated through the CHSS Datacenter, which has developed a unique organizational structure that has strengths and weaknesses in comparison to other research organizational structures (e.g., prospective randomized trials, registries, etc). This review will highlight the unique attributes of the CHSS Datacenter with emphasis on the Datacenter's strengths and weaknesses in comparison to other organizational structures. Copyright (c) 2010 Elsevier Inc. All rights reserved.

Coping Behaviors of Parents with Children with Congenital Heart Disease.

ERIC Educational Resources Information Center

Strobino, Jane

The study addresses parental coping patterns of children with congenital heart disease in the state of Hawaii. Attention was given to geography and ethnicity as well as parental and child characteristics as factors impacting on the coping pattern. Telephone interviews with parents (N=32) obtained data concerning parent characteristics, their…

Emotional and Behavioural Problems in Children and Adolescents with Congenital Heart Disease

ERIC Educational Resources Information Center

Johnson, Beena; Francis, Johnson

2005-01-01

Major physical illnesses usually have an impact on the psychological well-being of any individual. An illness of early onset, with necessity of frequent diagnostic and therapeutic interventions can adversely affect the emotional balance and behavioural adaptation of children and adolescents. This is applicable for congenital heart disease,…

Effect of Congenital Heart Defects on Language Development in Toddlers with Down Syndrome

ERIC Educational Resources Information Center

Visootsak, J.; Hess, B.; Bakeman, R.; Adamson, L. B.

2013-01-01

Background: Down syndrome (DS, OMIM #190685) is the most commonly identified genetic form of intellectual disability with congenital heart defect (CHD) occurring in 50% of cases. With advances in surgical techniques and an increasing lifespan, this has necessitated a greater understanding of the neurodevelopmental consequences of CHDs. Herein, we…

Formative research to build mobile technologies that advance transitions of care for adolescents with congenital heart disease

USDA-ARS?s Scientific Manuscript database

Congenital heart diseases (CHDs) are the most common type of birth defects. Improvements in CHD care have led to ~1.4 million survivors reaching adulthood. Thus, successful transition and transfer from pediatric to adult care is crucial. Unfortunately, <30% of adults with CHD successfully transition...

Building mobile technologies to improve transitions of care in adolescents with congenital heart disease

USDA-ARS?s Scientific Manuscript database

Congenital heart diseases (CHDs) are the most common type of birth defects. Improvements in CHD care have led to roughly 1.4 million survivors reaching adulthood. This emerging "survivor" population are often palliated but not cured. Thus successful transition from pediatric to adult care for CHD pa...

Oral health status of children with congenital heart disease and the awareness, attitude and knowledge of their parents.

PubMed

Rai, Kavita; Supriya, S; Hegde, Amitha M

2009-01-01

The oral health status of children with congenital heart diseases and the parental awareness on maintaining good oral health and attitude towards preventive dental health measures were evaluated. A total of 170 children between the age group of 1-16 yrs belonging to both genders, with the history of congenital heart disease from Sree Chitra Tirunal Institute of Medical Science and Technology, Thiruvananthapuram and Narayana Hrudayalaya Institute of Medical Sciences, Bangalore were examined. Oral lesions and caries experience were recorded using modified WHO oral health assessment form. Oral hygiene of the children with congenital heart disease was found to be poor with tongue coating (50.6%), plaque (41.8%), calculus (35.3%), and caries (42.4%). Parental awareness on the importance of maintaining good oral hygiene, preventive dentistry, medicinal decay and its systemic effects has been found to be very poor. Dentistry should give priority to patients whose general health may be put at risk by poor dental health. Closer cooperation between Pediatrician, Pediatric Cardiologists and Pediatric Dentists could help improve dental care for these children.

Knowledge and Oral Health Attitudes among Parents of Children with Congenital Heart Disease.

PubMed

Suvarna, Reshma; Rai, Kavita; Hegde, Amitha M

2011-01-01

Congenital heart disease (CHD) is a devastating complex of diseases resulting from defects of development. It affects more than 1 of every 100 live births. Early preventive dental care should be adjusted to the special needs of these children in their first years of life. Knowledge of parental attitudes and experiences of dental care are therefore important. Aim : This study was done to assess the knowledge and attitudes among parents of children with congenital heart disease towards oral health and dental care. Materials and methods : Parents (n = 105) of children with congenital heart disease of an age ranging from 0 to 16 years were included in the study. A questionnaire was used to assess the knowledge and oral health attitudes. Results : The parents' knowledge was fair but the oral health attitudes were not very satisfactory. The parents in this study also recognized the importance of oral health for the well-being of rest of the body. Conclusion : The results of this study indicate that parents' and children's attitudes toward oral health and dental care need to be improved.

[The Relationships among Perceived Parental Bonding, Illness Perception, and Anxiety in Adult Patients with Congenital Heart Diseases].

PubMed

Shin, Nayeon; Jang, Youha; Kang, Younhee

2017-04-01

The purposes of this study were to identify the relationships among perceived parental bonding, illness perception, and anxiety and to determine the influences of perceived parental bonding and illness perception on anxiety in adult patients with congenital heart diseases. In this study a descriptive correlational design with survey method was utilized. The participants were 143 adult patients with congenital heart disease being cared for in the cardiology out-patient clinic of A medical center. Data were collected using the Parental Bonding Instrument, Illness Perception Questionnaire Revised Scale, and Cardiac Anxiety Questionnaire Scale. Data were analyzed using descriptive statistics, independent t-test, one-way ANOVA, Pearson correlation analysis, and hierarchial regression analyses. There showed significant positive relationships of anxiety with maternal overprotection, consequences, and personal control respectively. Among predictors, maternal overprotection (β=.45), consequence (β=.26), and personal control (β=-.03) had statistically significant influence on anxiety. Nursing interventions to decrease maternal overprotection and negative consequence, and to enhance personal control are essential to decrease the anxiety of adult patients with congenital heart diseases. © 2017 Korean Society of Nursing Science

Adult congenital heart disease imaging with second-generation dual-source computed tomography: initial experiences and findings.

PubMed

Ghoshhajra, Brian B; Sidhu, Manavjot S; El-Sherief, Ahmed; Rojas, Carlos; Yeh, Doreen Defaria; Engel, Leif-Christopher; Liberthson, Richard; Abbara, Suhny; Bhatt, Ami

2012-01-01

Adult congenital heart disease patients present a unique challenge to the cardiac imager. Patients may present with both acute and chronic manifestations of their complex congenital heart disease and also require surveillance for sequelae of their medical and surgical interventions. Multimodality imaging is often required to clarify their anatomy and physiology. Radiation dose is of particular concern in these patients with lifelong imaging needs for their chronic disease. The second-generation dual-source scanner is a recently available advanced clinical cardiac computed tomography (CT) scanner. It offers a combination of the high-spatial resolution of modern CT, the high-temporal resolution of dual-source technology, and the wide z-axis coverage of modern cone-beam geometry CT scanners. These advances in technology allow novel protocols that markedly reduce scan time, significantly reduce radiation exposure, and expand the physiologic imaging capabilities of cardiac CT. We present a case series of complicated adult congenital heart disease patients imaged by the second-generation dual-source CT scanner with extremely low-radiation doses and excellent image quality. © 2012 Wiley Periodicals, Inc.

Materials and Manufacturing Technologies Available for Production of a Pediatric Bioabsorbable Stent

PubMed Central

Alexy, Ryan D.; Levi, Daniel S.

2013-01-01

Transcatheter treatment of children with congenital heart disease such as coarctation of the aorta and pulmonary artery stenosis currently involves the use of metal stents. While these provide good short term results, there are long term complications with their use. Children outgrow metal stents, obligating them to future transcatheter dilations and eventual surgical removal. A bioabsorbable stent, or a stent that goes away with time, would solve this problem. Bioabsorbable stents are being developed for use in coronary arteries, however these are too small for use in pediatric congenital heart disease. A bioabsorbable stent for use in pediatric congenital heart disease needs to be low profile, expandable to a diameter 8 mm, provide sufficient radial strength, and absorb quickly enough to allow vessel growth. Development of absorbable coronary stents has led to a great understanding of the available production techniques and materials such as bioabsorbable polymers and biocorrodable metals. Children with congenital heart disease will hopefully soon benefit from the current generation of bioabsorbable and biocorrodable materials and devices. PMID:24089660

Contraceptive Use and Unintended Pregnancy in Women With Congenital Heart Disease.

PubMed

Lindley, Kathryn J; Madden, Tessa; Cahill, Alison G; Ludbrook, Philip A; Billadello, Joseph J

2015-08-01

To identify patterns of contraceptive use and pregnancy in an academic adult congenital cardiology practice. In this cross-sectional study, from October 2013 through March 2014, 100 women with congenital heart disease aged 18-45 years were recruited from an academic congenital heart disease clinic and administered a survey regarding pregnancy history, contraception use, and understanding of pregnancy-related and contraceptive-related risk. The primary outcome was current use of long-acting reversible contraception, including intrauterine devices or subdermal implants. Of 83 sexually active women, 63 (75.9%, 95% confidence interval [CI] 65.3-85.1) reported currently using any contraceptive method, including 30 of 83 (36.1%, 95% CI 25.9-47.4) using tier I methods (typical-use failure rates of less than 1% per year) and 20 of 83 (24.1%, 95% CI 15.4-34.7) using tier II methods (typical-use failure rates of 6-12% per year). Nine of 83 (10.8%, 95% CI 5.1-19.6) reported currently using long-acting reversible contraception. Sixty-four of 141 total pregnancies (45.4%, 95% CI 31.9-58.9) were self-reported by participants as "unexpected" rather than "planned." Only one (1.6%, 95% CI 0-4.6) of the 64 unintended pregnancies occurred when the woman was using a tier I method of contraception at the time of conception. Most women with congenital heart disease of childbearing age are sexually active. The high incidence of unintended pregnancy in this group may be related to underuse of highly effective methods of contraception. Specific counseling on tier I methods may reduce unintended pregnancies in women with congenital heart disease. III.

Syndromic Hirschsprung's disease and associated congenital heart disease: a systematic review.

PubMed

Duess, Johannes W; Puri, Prem

2015-08-01

Hirschsprung's disease (HD) occurs as an isolated phenotype in 70% of infants and is associated with additional congenital anomalies or syndromes in approximately 30% of patients. The cardiac development depends on neural crest cell proliferation and is closely related to the formation of the enteric nervous system. HD associated with congenital heart disease (CHD) has been reported in 5-8% of cases, with septation defects being the most frequently recorded abnormalities. However, the prevalence of HD associated with CHD in infants with syndromic disorders is not well documented. This systematic review was designed to determine the prevalence of CHD in syndromic HD. A systematic review of the literature using the keywords "Hirschsprung's disease", "aganglionosis", "congenital megacolon", "congenital heart disease" and "congenital heart defect" was performed. Resulting publications were reviewed for epidemiology and morbidity. Reference lists were screened for additional relevant studies. A total of fifty-two publications from 1963 to 2014 reported data on infants with HD associated with CHD. The overall reported prevalence of HD associated with CHD in infants without chromosomal disorders was 3%. In infants with syndromic disorders, the overall prevalence of HD associated with CHD ranged from 20 to 80 % (overall prevalence 51%). Septation defects were recorded in 57% (atrial septal defects in 29%, ventricular septal defects in 32%), a patent ductus arteriosus in 39%, vascular abnormalities in 16%, valvular heart defects in 4% and Tetralogy of Fallot in 7%. The prevalence of HD associated with CHD is much higher in infants with chromosomal disorders compared to infants without associated syndromes. A routine echocardiogram should be performed in all infants with syndromic HD to exclude cardiac abnormalities.

Healthcare Disparities in Outcomes of a Metropolitan Congenital Heart Surgery Center: The Effect of Clinical and Socioeconomic Factors.

PubMed

Peterson, Jennifer K; Catton, Kirsti G; Setty, Shaun P

2018-04-01

The purpose of this study is to identify the impact of demographic, socioeconomic, and clinical factors on congenital heart surgery outcomes. This retrospective cohort study included 234 congenital heart surgery patients from 2011 through 2015, in a racially/ethnically diverse metropolitan children's hospital. Outcomes included length of stay (LOS), age at first echocardiogram, length of mechanical ventilation, and incidence of complications. Compared to others, black children underwent their first echocardiogram at a later age (median 23 versus 2 days, p = 0.014) and were more likely to be diagnosed with congenital heart disease in the emergency room (p = 0.026). Hispanic children were more likely to have major non-cardiac congenital anomalies (p = 0.045). Increased LOS during elective admissions was associated with higher surgical complexity (STAT category 4 and 5 Estimate 3.905 days, p = 0.001), compared to STAT category 1, and number of complications (Estimate = 2.306 days per complication, p < 0.001). Increased LOS in non-elective admissions was associated with the number of complex chronic conditions (Estimate = 15.446 days, p = 0.045) and the number of complications (Estimate = 11.591 days per complication, p < 0.001). However, in multivariate analysis, race and ethnicity was not associated with increased LOS or age at first echocardiogram. In this diverse setting, race/ethnicity was not associated with increased LOS, age at first echocardiogram, length of ventilation, or complications. Surgical complexity, chronic conditions, and complications were associated with increased LOS. We discuss some interventions to reduce disparities in congenital heart surgery outcomes.

Parental overprotection and heart-focused anxiety in adults with congenital heart disease.

PubMed

Ong, Lephuong; Nolan, Robert P; Irvine, Jane; Kovacs, Adrienne H

2011-09-01

The care of adult patients with congenital heart disease (CHD) is challenging from a mental health perspective, as these patients continue to face a variety of biopsychosocial issues that may impact emotional functioning. Despite these issues, there are limited data on the psychosocial functioning of adults with CHD, and there are no data on the impact of parental overprotection on heart-focused anxiety in this patient population. The aim of this study was to examine the relationships between patient recollections of parental overprotection and current heart-focused anxiety in adults with CHD. A cross-sectional sample of 190 adult patients with CHD (51% male; mean age = 32.28, SD = 11.86 years) completed validated measures of perceived parental overprotection (Parental Bonding Instrument) and heart-focused anxiety (Cardiac Anxiety Questionnaire). The results indicated that perceived parental overprotection (β = 0.19, p = 0.02) and heart defect complexity (β = 0.17, p = 0.03) were significantly related to heart-focused anxiety. Contrary to hypotheses, perceived parental overprotection did not vary as a function of heart defect complexity (F (2, 169) = 0.02, p = 0.98). Perceived parental overprotection and heart defect complexity are associated with heart-focused anxiety in adults with congenital heart disease. These results can inform the development of clinical interventions aimed at improving the psychosocial adjustment of this patient population.

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review

PubMed Central

HAO, YABIN; HONG, XU; ZHAO, XINYAN

2015-01-01

Abernethy malformation is a rare congenital malformation defined by an extrahepatic portosystemic shunt. The majority of affected patients are young (<18 years of age) and experience various symptoms, including vomiting, jaundice, dyspnea and coma. The current study presents a case of Abernethy malformation in an asymptomatic adult male patient. The patient exhibited congenital absence of the portal vein, congenital heart disease (postoperative ventricular septal defect status), and multiple liver lesions, confirmed to be focal nodular hyperplasia by biopsy. Ultrasonography and magnetic resonance imaging findings revealing the liver lesions, type II congenital absence of the portal vein and the portosystemic shunt are presented. In addition, the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder are investigated by reviewing 101 previously reported cases. PMID:25624897

[Congenital Heart Disease in Children with Down Syndrome: What Has Changed in the Last Three Decades?

PubMed

Dias, Filipa Mestre; Cordeiro, Susana; Menezes, Isabel; Nogueira, Graça; Teixeira, Ana; Marques, Marta; Abecasis, Miguel; Anjos, Rui

2016-10-01

The prevalence of Down syndrome has increased in the last 30 years; 55% of these children have congenital heart disease. A retrospective longitudinal cohort study; clinical data from 1982 to 2013 databases with the diagnosis of Down syndrome or trisomy 21 in a reference hospital in pediatric cardiology and cardiac surgery. to assess the progress in the last three decades of cardiological care given to children with Down syndrome and congenital heart disease. We studied 102 patients with Down syndrome and congenital heart disease subjected to invasive therapy: corrective or palliative cardiac surgery and therapeutic catheterization. The referral age was progressively earlier in patients referred in the first year of life. The most frequent diagnosis was complete atrioventricular sptal defect (41%). There was a trend towards increasingly early corrective surgery in patients under 12 months (p < 0.001). Since 2000, the large majority of patients were operated before reaching six months of age. The main cardiac complications were rhythm dysfunction and low output. More frequent noncardiac complications were pulmonary and infectious. The 30-day mortality rate was 3/102 cases (2.9%). Of patients in follow-up, 89% are in NYHA class I. The early surgical correction seen over the past 15 years follows the approach suggested in the literature. The observed 30-day mortality rate is overlapping international results. Patients with Down syndrome subjected to corrective surgery of congenital heart disease have an excellent long-term functional capacity.

Usefulness of cutting balloon angioplasty for the treatment of congenital heart defects.

PubMed

Kusa, Jacek; Mazurak, Magdalena; Skierska, Agnieszka; Szydlowski, Leslaw; Czesniewicz, Pawel; Manka, Lukasz

2018-01-01

Patients with complex congenital heart defects may have different hemodynamic prob-lems which require a variety of interventional procedures including angioplasty which involves using high-pressure balloons. After failure of conventional balloon angioplasty, cutting balloon angioplasty is the next treatment option available. The purpose of this study was to evaluate the safety and efficacy of cutting balloon angioplasty in children with different types of congenital heart defects. Cutting balloon angioplasty was performed in 28 children with different congenital heart defects. The indication for cutting balloon angioplasty was: pulmonary artery stenosis in 17 patients, creating or dilatation of interatrial communication in 10 patients, and stenosis of left subclavian artery in 1 patient. In the pulmonary arteries group there was a significant decrease in systolic blood pressure (SBP) in the proximal part of the artery from the average 74.33 ± 20.4 mm Hg to 55 ± 16.7 mm Hg (p < 0.001). Distal to the stenosis there was an increase in SBP from 19.8 ± 3.82 mm Hg to 30.3 ± ± 13.3 mm Hg (p = 0.04). This result remained constant in the follow-up. In atrial septal defect/fenestra-tion group, cutting balloon angioplasty was performed after an unsuccessful classic Rashkind procedure. After cutting balloon angioplasty there was a significant widening of the interatrial communication. Cutting balloon angioplasty is a feasible and effective treatment option in different con-genital heart defects.

Prevalence of HCV Infection in Adults with Congenital Heart Disease and Treatment with Direct Antiviral Agents.

PubMed

Gade, Ajay Reddy; Patel, Manisha; West, Donna R; Abrams, Gary A

2018-03-01

Hepatitis C virus (HCV) infection affects >3% of the US population, which over time can lead to cirrhosis and hepatocellular carcinoma. The lack of a reliable screening method for HCV before 1992 resulted in a higher prevalence of the virus in adults with congenital heart disease who underwent corrective surgery that required blood transfusions. Direct-acting antiviral agents such as sofosbuvir/ledipasvir have significantly increased the efficacy of HCV therapy, although use of these medications in adults with congenital heart disease has not been described. Ours was a retrospective study of 188 adults with congenital heart conditions who had cardiac surgery before 1992. These patients were screened for HCV using HCV antibody followed by HCV RNA if the screening test was positive. Of the 188 adults, 116 (43% male patients, 24-70 years) were screened for the HCV antibody, demonstrating that 104 individuals were negative and 12 subjects were positive for the virus. Subsequently, further testing for the presence of HCV demonstrated 11 of 12 were infected, with an overall prevalence of 9.5%. Five individuals chose to be treated with sofosbuvir/ledipasvir and 5 of 5 have successfully cleared the virus and are considered cured. Adults with congenital heart disease who underwent cardiac surgery before 1992 warrant being screened for HCV, and, if testing positive, may be considered for therapy using direct-acting antiviral agents with close monitoring for cardiac complications.

Illness Identity: A Novel Predictor for Healthcare Use in Adults With Congenital Heart Disease.

PubMed

Van Bulck, Liesbet; Goossens, Eva; Luyckx, Koen; Oris, Leen; Apers, Silke; Moons, Philip

2018-05-22

To optimize healthcare use of adults with congenital heart disease, all important predictors of healthcare utilization should be identified. Clinical and psychological characteristics (eg, age and depression) have been found to be associated with healthcare use. However, the concept of illness identity, which assesses the degree to which congenital heart disease is integrated into one's identity, has not yet been investigated in association with healthcare use. Hence, the purpose of the study is to examine the predictive value of illness identity for healthcare use. In this ambispective analytical observational cohort study, 216 adults with congenital heart disease were included. The self-reported Illness Identity Questionnaire was used to assess illness identity states: engulfment, rejection, acceptance, and enrichment. After 1 year, self-reported healthcare use for congenital heart disease or other reasons over the past 6 months was assessed including hospitalizations; visits to general practitioner; visits to medical specialists; and emergency room visits. Binary logistic and negative binomial regression analyses were conducted, adjusting for age, sex, disease complexity, and depressive and anxious symptoms. The more profoundly the heart defect dominated one's identity (ie, engulfment), the more likely this person was to be hospitalized (odds ratio=3.76; 95% confidence interval=1.43-9.86), to visit a medical specialist (odds ratio=2.32; 95% confidence interval=1.35-4.00) or a general practitioner (odds ratio=1.78; 95% confidence interval=1.01-3.17), because of their heart defect. Illness identity, more specifically engulfment, has a unique predictive value for the occurrence of healthcare encounters. This association deserves further investigation, in which the directionality of effects and the contribution of illness identity in terms of preventing inappropriate healthcare use should be determined. © 2018 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

[Prevalence of congenital heart diseases in Koranic schools (daara) in Dakar: a cross-sectional study based on clinical and echocardiographic screening in 2019 school children].

PubMed

Bodian, M; Ngaïdé, A A; Mbaye, A; Sarr, S A; Jobe, M; Ndiaye, M B; Kane, A D; Aw, F; Gaye, N D; Ba, F G; Bah, M B; Tabane, A; Dioum, M; Diagne, D; Diao, M; Diack, B; Sarr, M; Kane, A; Bâ, S A

2015-02-01

Congenital heart diseases are one of the major cardiovascular diseases in developing countries. Most prevalence studies were based on clinical examination of children with echocardiographic confirmation of suspected cases and underestimate its prevalence. The objective of this study was to investigate the prevalence of congenital heart disease in "daara" (Koranic schools) in the city of Dakar and its suburbs on the basis of clinical examination and Doppler echocardiography in school children. This cross-sectional survey was carried out from 9(th) August to 24(th) December 2011, and included a population of 2019 school children aged 5 to 18 years in 16 selected "daaras" under the Academic Inspectorate of Dakar and its suburbs. Anamnestic, clinical and echocardiographic data were recorded in a validated questionnaire. A p < 0.05 was considered to be statistically significant in bivariate analysis. 2 019 school children were included out of which 60.1% were male (sex-ratio: 0.66). The average age was 9.7 years (± 3.3 years). 18 cases of congenital heart diseases were detected being a prevalence of 8.9 per 1 000 (95 % CI: 1.8 to 7.9). This included 6 cases of inter-atrial septal aneurysm, 5 cases of peri-membranous ventricular septal defects, 4 cases of patent ductusarteriosus and 3 cases of tetralogy of Fallot. Factors correlated with the presence of congenital heart disease were ageless than 8 (p <0.001) and residence in the suburbs of Dakar (p <0.001). We also detected 10 cases of rheumatic valvular disease, a prevalence of 4.9 per 1 000 (95% CI: 2.4 to 9.1). Our study shows a high prevalence of congenital heart diseases, which is almost identical to the WHO estimates and that ultrasound screening is more sensitive than clinical screening. Reducing the prevalence of these diseases requires implementation of appropriate policies, focusing on awareness and early detection.

Brain angiogenic gene-expression in congenital heart disease.

PubMed

Sánchez, Olga; Ruiz-Romero, Aina; Domínguez, Carmen; Ferrer, Queralt; Ribera, Irene; Rodríguez-Sureda, Víctor; Alijotas, Jaume; Arévalo, Sílvia; Carreras, Elena; Cabero, Lluís; Llurba, Elisa

2017-12-05

To analyze potential differences in the expression of antiangiogenic and angiogenic factors and of genes associated with chronic hypoxia in cerebral tissue from euploid fetuses with congenital heart disease (CHD) and control fetuses. Cerebral tissue was obtained from 15 fetuses with CHD and 12 control fetuses undergoing termination of pregnancy. Expression profiles of the antiangiogenic soluble fms-like tyrosine kinase-1 (sFlt-1), the angiogenic vascular endothelial growth factor-A (VEGF-A) and placental growth factor (PlGF), and genes associated with chronic hypoxia were determined by real-time PCR in tissue from the frontal cortex and from basal ganglia-hypothalamus. sFlt-1 expression was 48% higher in the frontal cortex (p=0.0431) and 72% higher in the basal ganglia-hypothalamus (p=0.0369) of CHD fetuses than controls. VEGF-A expression was 60% higher in the basal ganglia-hypothalamus (p=0.0432) of CHD fetuses. The expression of hypoxia-inducible factor-2α (HIF-2α) in the basal ganglia-hypothalamus was 98% higher in CHD fetuses (p=0.0456). An overall dysregulation of angiogenesis with a net balance towards an antiangiogenic environment was observed in the cerebral tissue from fetuses with CHD, suggesting that these fetuses may have an intrinsic angiogenic impairment that could contribute to impaired brain perfusion and abnormal neurological development later in life. This article is protected by copyright. All rights reserved.

Brain volumes predict neurodevelopment in adolescents after surgery for congenital heart disease.

PubMed

von Rhein, Michael; Buchmann, Andreas; Hagmann, Cornelia; Huber, Reto; Klaver, Peter; Knirsch, Walter; Latal, Beatrice

2014-01-01

Patients with complex congenital heart disease are at risk for neurodevelopmental impairments. Evidence suggests that brain maturation can be delayed and pre- and postoperative brain injury may occur, and there is limited information on the long-term effect of congenital heart disease on brain development and function in adolescent patients. At a mean age of 13.8 years, 39 adolescent survivors of childhood cardiopulmonary bypass surgery with no structural brain lesions evident through conventional cerebral magnetic resonance imaging and 32 healthy control subjects underwent extensive neurodevelopmental assessment and cerebral magnetic resonance imaging. Cerebral scans were analysed quantitatively using surface-based and voxel-based morphometry. Compared with control subjects, patients had lower total brain (P = 0.003), white matter (P = 0.004) and cortical grey matter (P = 0.005) volumes, whereas cerebrospinal fluid volumes were not different. Regional brain volume reduction ranged from 5.3% (cortical grey matter) to 11% (corpus callosum). Adolescents with cyanotic heart disease showed more brain volume loss than those with acyanotic heart disease, particularly in the white matter, thalami, hippocampi and corpus callosum (all P-values < 0.05). Brain volume reduction correlated significantly with cognitive, motor and executive functions (grey matter: P < 0.05, white matter: P < 0.01). Our findings suggest that there are long-lasting cerebral changes in adolescent survivors of cardiopulmonary bypass surgery for congenital heart disease and that these changes are associated with functional outcome.

A Case Study of Infant Physiologic Response to Skin-to-Skin Contact After Surgery for Complex Congenital Heart Disease.

PubMed

Harrison, Tondi M; Ludington-Hoe, Susan

2015-01-01

Infants with complex congenital heart disease requiring surgical intervention within the first days or weeks of life may be the most seriously ill infants needing intensive nursing and medical care. Skin-to-skin contact (SSC) is well accepted and practiced as a positive therapeutic intervention in premature infants but is not routinely offered to infants in cardiac intensive care units. The physiologic effects of SSC in the congenital heart disease population must be examined before recommending incorporation of SSC into standard care routines. The purpose of this case study was to describe the physiologic response to a single session of SSC in an 18-day-old infant with hypoplastic left heart syndrome. Repeated measures of heart rate, respiratory rate, oxygen saturation, blood pressure, and temperature were recorded 30 minutes before SSC, during SSC (including interruptions for bottle and breast feedings), and 10 minutes after SSC was completed. All physiologic parameters were clinically acceptable throughout the 135-minute observation. This case study provides beginning evidence that SSC is safe in full-term infants after surgery for complex congenital heart disease. Further research with a larger sample is needed to examine the effects of SSC on infant physiology before surgery and earlier in the postoperative time period as well as on additional outcomes such as length of stay, maternal-infant interaction, and neurodevelopment.

Heart Transplant in Children: What a Primary Care Provider Needs to Know.

PubMed

Sehgal, Swati; Shea, Emily; Kelm, Lauren; Kamat, Deepak

2018-04-01

Heart transplantation is offered to children with heart failure that is not amenable to medical or surgical therapy. Indications for heart transplant include unrepairable congenital heart disease, failed palliation of congenital heart disease, and cardiomyopathies. There has been tremendous progress in this field since the first heart transplant was performed in 1967. Each year, approximately 500 pediatric heart transplants take place worldwide. Pediatric heart transplant survivors are living longer with their initial transplant. Many pediatric practitioners are faced with caring for these patients before as well as after the heart transplant and, therefore, they should be knowledgeable about basic principles related to heart transplant. There are certain complications that are unique to this population, and medication side-effects, evaluation and management of a febrile illness, opportunistic infections, vaccination, pregnancy, and exercise recommendations are areas that require special consideration. [Pediatr Ann. 2018;47(4):e172-e178.]. Copyright 2018, SLACK Incorporated.

Current Expectations for Cardiac Transplantation in Patients With Congenital Heart Disease.

PubMed

Kirklin, James K; Carlo, Waldemar F; Pearce, F Bennett

2016-11-01

Congenital heart disease accounts for 40% of pediatric heart transplants and presents unique challenges to the transplant team. Suitability for transplantation is defined in part by degree of sensitization, pulmonary vascular resistance, and hepatic reserves. The incremental transplant risk for patients with congenital heart disease occurs within the first 3 months, after which survival is equivalent to transplantation for cardiomyopathy. Single ventricle with prior palliation, and especially the failing Fontan, carry the highest risk for transplantation and are least amenable to bridging with mechanical circulatory support. More effective bridging to transplant with mechanical circulatory support will require improvements in the adverse event profile of available pumps and the introduction of miniaturized continuous flow technology. The major barriers to routine long-term survival are chronic allograft failure and allograft vasculopathy. Despite these many challenges, continuing improvements in the care of pediatric heart transplant patients have pushed the median posttransplant survival past 15 years for children and to 20 years for infants. © The Author(s) 2016.

The year 2012 in the European Heart Journal-Cardiovascular Imaging. Part II.

PubMed

Plein, Sven; Knuuti, Juhani; Edvardsen, Thor; Saraste, Antti; Piérard, Luc A; Maurer, Gerald; Lancellotti, Patrizio

2013-07-01

The part II of the best of the European Heart Journal - Cardiovascular Imaging in 2012 specifically focuses on studies of valvular heart diseases, heart failure, cardiomyopathies, and congenital heart diseases.

Preattentive processing of heart cues and the perception of heart symptoms in congenital heart disease.

PubMed

Karsdorp, Petra A; Kindt, Merel; Everaerd, Walter; Mulder, Barbara J M

2007-08-01

The present study was aimed at clarifying whether preattentive processing of heart cues results in biased perception of heart sensations in patients with congenital heart disease (ConHD) who are also highly trait anxious. Twenty-six patients with ConHD and 22 healthy participants categorized heart-related (heart rate) or neutral sensations (constant vibration) as either heart or neutral. Both sensations were evoked using a bass speaker that was attached on the chest of the participant. Before each physical sensation, a subliminal heart-related or neutral prime was presented. Biased perception of heart-sensations would become evident by a delayed categorization of the heart-related sensations. In line with the prediction, a combination of high trait anxiety and ConHD resulted in slower responses after a heart-related sensation that was preceded by a subliminal heart cue. Preattentive processing of harmless heart cues may easily elicit overperception of heart symptoms in highly trait anxious patients with ConHD.

Usefulness of plasma B-type natriuretic peptide to identify ventricular dysfunction in pediatric and adult patients with congenital heart disease.

PubMed

Law, Yuk M; Keller, Bradley B; Feingold, Brian M; Boyle, Gerard J

2005-02-15

The usefulness of B-type natriuretic peptide (BNP) levels to assess ventricular dysfunction in children and the congenital heart disease population remains largely unknown. We retrospectively analyzed 62 patients with or without known heart disease who had plasma BNP measured for the investigation of new or severity grading of known ventricular dysfunction. BNP levels were significantly higher in patients with ventricular dysfunction (mean 623 +/- 146 pg/ml, range 5 to 5,000) than in patients without ventricular dysfunction (mean 22 +/- 5 pg/ml, range 5 to 63; p <0.01). Using a cutoff of 40 pg/ml, BNP levels detected heart disease associated with ventricular dysfunction at a sensitivity of 85%, specificity of 81%, positive predictive value of 92%, and negative predictive value of 68%. The degree of BNP elevation was also associated with the severity of heart failure and high ventricular filling pressures. Plasma BNP elevation can be a reliable test in children and young adults with various kinds of congenital heart disease resulting in ventricular dysfunction.

The Evolution of an Adult Congenital Heart Surgery Program: The Emory System.

PubMed

Kogon, Brian; Rosenblum, Joshua; Alsoufi, Bahaaldin; Shashidharan, Subhadra; Book, Wendy

2017-01-01

The Emory Adult Congenital Heart (Emory University, Atlanta, GA) program was founded in 2001. In 2004, the surgical component transitioned from a pediatric facility to an adult facility. The aim of this article is characterize the program as a whole, outline changes in the program, and discuss the challenges of the transition process. Between 2001 and 2015, changes in program structure and personnel were evaluated. There has been significant growth of the program between 2001 and 2015. There are currently 19 half-day clinics per week, with 2,700 clinic visits per year. There are six cardiologists, three congenital cardiac surgeons, two sonographers, one advanced practice provider, and one social worker dedicated to the program. There are Accreditation Council for Graduate Medical Education-accredited adult congenital cardiology and congenital cardiac surgery fellowships. One thousand forty-four operations were performed between 2001 and 2015. There were 828 open-heart operations, of which 581 (70%) were re-operations. Over the study period, the number of yearly operations increased from 30 to 119, and the mean age at surgery increased from 22 to 35 years. Over time, more of the operations were performed at the adult hospital: increasing from 3% in 2001 to 82% in 2015, and more of the operations were performed by congenital cardiac surgeons: 87% (114 of 131) before the 2004 transition to 97% (881 of 913) afterward. The Emory Adult Congenital Heart program has undergone significant growth and change, including transition of the surgical component from the pediatric to the adult facility. While numerous obstacles have been overcome and great progress has been made, additional challenges remain. Copyright © 2017 Elsevier Inc. All rights reserved.

Dextrocardia

MedlinePlus

Cyanotic heart defect - dextrocardia; Congenital heart defect - dextrocardia; Birth defect - dextrocardia ... During the early weeks of pregnancy, the baby’s heart develops. Sometimes, it turns so that it points ...

Feasibility and observer reproducibility of speckle tracking echocardiography in congenital heart disease patients.

PubMed

Mokhles, Palwasha; van den Bosch, Annemien E; Vletter-McGhie, Jackie S; Van Domburg, Ron T; Ruys, Titia P E; Kauer, Floris; Geleijnse, Marcel L; Roos-Hesselink, Jolien W

2013-09-01

The twisting motion of the heart has an important role in the function of the left ventricle. Speckle tracking echocardiography is able to quantify left ventricular (LV) rotation and twist. So far this new technique has not been used in congenital heart disease patients. The aim of our study was to investigate the feasibility and the intra- and inter-observer reproducibility of LV rotation parameters in adult patients with congenital heart disease. The study population consisted of 66 consecutive patients seen in the outpatient clinic (67% male, mean age 31 ± 7.7 years, NYHA class 1 ± 0.3) with a variety of congenital heart disease. First, feasibility was assessed in all patients. Intra- and inter-observer reproducibility was assessed for the patients in which speckle tracking echocardiography was feasible. Adequate image quality, for performing speckle echocardiography, was found in 80% of patients. The bias for the intra-observer reproducibility of the LV twist was 0.0°, with 95% limits of agreement of -2.5° and 2.5° and for interobserver reproducibility the bias was 0.0°, with 95% limits of agreement of -3.0° and 3.0°. Intra- and inter-observer measurements showed a strong correlation (0.86 and 0.79, respectively). Also a good repeatability was seen. The mean time to complete full analysis per subject for the first and second measurement was 9 and 5 minutes, respectively. Speckle tracking echocardiography is feasible in 80% of adult patients with congenital heart disease and shows excellent intra- and inter-observer reproducibility. © 2013, Wiley Periodicals, Inc.

Re-entry using anatomically determined isthmuses: a curable ventricular tachycardia in repaired congenital heart disease.

PubMed

Kapel, Gijsbert F L; Reichlin, Tobias; Wijnmaalen, Adrianus P; Piers, Sebastiaan R D; Holman, Eduard R; Tedrow, Usha B; Schalij, Martin J; Stevenson, William G; Zeppenfeld, Katja

2015-02-01

Ventricular tachycardia (VT) is an important cause of late morbidity and mortality in repaired congenital heart disease. The substrate often includes anatomic isthmuses that can be transected by radiofrequency catheter ablation similar to isthmus block for atrial flutter. This study evaluates the long-term efficacy of isthmus block for treatment of re-entry VT in adults with repaired congenital heart disease. Thirty-four patients (49±13 years; 74% male) with repaired congenital heart disease who underwent radiofrequency catheter ablation of VT in 2 centers were included. Twenty-two (65%) had a preserved left and right ventricular function. Patients were inducible for 1 (interquartile range, 1-2) VT, median cycle length: 295 ms (interquartile range, 242-346). Ablation aimed to transect anatomic isthmuses containing VT re-entry circuit isthmuses. Procedural success was defined as noninducibility of any VT and transection of the anatomic isthmus and was achieved in 25 (74%) patients. During long-term follow-up (46±29 months), all patients with procedural success (18/25 with internal cardiac defibrillators) were free of VT recurrence but 7 of 18 experienced internal cardiac defibrillator-related complications. One patient with procedural success and depressed cardiac function received an internal cardiac defibrillator shock for ventricular fibrillation. None of the 18 patients (12/18 with internal cardiac defibrillators) with complete success and preserved cardiac function experienced any ventricular arrhythmia. In contrast, VT recurred in 4 of 9 patients without procedural success. Four patients died from nonarrhythmic causes. In patients with repaired congenital heart disease with preserved ventricular function and isthmus-dependent re-entry, VT isthmus ablation can be curative. © 2014 American Heart Association, Inc.

Post-mortem cytogenomic investigations in patients with congenital malformations.

PubMed

Dias, Alexandre Torchio; Zanardo, Évelin Aline; Dutra, Roberta Lelis; Piazzon, Flavia Balbo; Novo-Filho, Gil Monteiro; Montenegro, Marilia Moreira; Nascimento, Amom Mendes; Rocha, Mariana; Madia, Fabricia Andreia Rosa; Costa, Thais Virgínia Moura Machado; Milani, Cintia; Schultz, Regina; Gonçalves, Fernanda Toledo; Fridman, Cintia; Yamamoto, Guilherme Lopes; Bertola, Débora Romeo; Kim, Chong Ae; Kulikowski, Leslie Domenici

2016-08-01

Congenital anomalies are the second highest cause of infant deaths, and, in most cases, diagnosis is a challenge. In this study, we characterize patterns of DNA copy number aberrations in different samples of post-mortem tissues from patients with congenital malformations. Twenty-eight patients undergoing autopsy were cytogenomically evaluated using several methods, specifically, Multiplex Ligation-dependent Probe Amplification (MLPA), microsatellite marker analysis with a MiniFiler kit, FISH, a cytogenomic array technique and bidirectional Sanger sequencing, which were performed on samples of different tissues (brain, heart, liver, skin and diaphragm) preserved in RNAlater, in formaldehyde or by paraffin-embedding. The results identified 13 patients with pathogenic copy number variations (CNVs). Of these, eight presented aneuploidies involving chromosomes 13, 18, 21, X and Y (two presented inter- and intra-tissue mosaicism). In addition, other abnormalities were found, including duplication of the TYMS gene (18p11.32); deletion of the CHL1 gene (3p26.3); deletion of the HIC1 gene (17p13.3); and deletion of the TOM1L2 gene (17p11.2). One patient had a pathogenic missense mutation of g.8535C>G (c.746C>G) in exon 7 of the FGFR3 gene consistent with Thanatophoric Dysplasia type I. Cytogenomic techniques were reliable for the analysis of autopsy material and allowed the identification of inter- and intra-tissue mosaicism and a better understanding of the pathogenesis of congenital malformations. Copyright © 2016 Elsevier Inc. All rights reserved.

Recent advances in pediatric interventional cardiology

PubMed Central

2017-01-01

During the last 10 years, there have been major technological achievements in pediatric interventional cardiology. In addition, there have been several advances in cardiac imaging, especially in 3-dimensional imaging of echocardiography, computed tomography, magnetic resonance imaging, and cineangiography. Therefore, more types of congenital heart diseases can be treated in the cardiac catheter laboratory today than ever before. Furthermore, lesions previously considered resistant to interventional therapies can now be managed with high success rates. The hybrid approach has enabled the overcoming of limitations inherent to percutaneous access, expanding the application of endovascular therapies as adjunct to surgical interventions to improve patient outcomes and minimize invasiveness. Percutaneous pulmonary valve implantation has become a successful alternative therapy. However, most of the current recommendations about pediatric cardiac interventions (including class I recommendations) refer to off-label use of devices, because it is difficult to study the safety and efficacy of catheterization and transcatheter therapy in pediatric cardiac patients. This difficulty arises from the challenge of identifying a control population and the relatively small number of pediatric patients with congenital heart disease. Nevertheless, the pediatric interventional cardiology community has continued to develop less invasive solutions for congenital heart defects to minimize the need for open heart surgery and optimize overall outcomes. In this review, various interventional procedures in patients with congenital heart disease are explored. PMID:29042864

Recent advances in pediatric interventional cardiology.

PubMed

Kim, Seong-Ho

2017-08-01

During the last 10 years, there have been major technological achievements in pediatric interventional cardiology. In addition, there have been several advances in cardiac imaging, especially in 3-dimensional imaging of echocardiography, computed tomography, magnetic resonance imaging, and cineangiography. Therefore, more types of congenital heart diseases can be treated in the cardiac catheter laboratory today than ever before. Furthermore, lesions previously considered resistant to interventional therapies can now be managed with high success rates. The hybrid approach has enabled the overcoming of limitations inherent to percutaneous access, expanding the application of endovascular therapies as adjunct to surgical interventions to improve patient outcomes and minimize invasiveness. Percutaneous pulmonary valve implantation has become a successful alternative therapy. However, most of the current recommendations about pediatric cardiac interventions (including class I recommendations) refer to off-label use of devices, because it is difficult to study the safety and efficacy of catheterization and transcatheter therapy in pediatric cardiac patients. This difficulty arises from the challenge of identifying a control population and the relatively small number of pediatric patients with congenital heart disease. Nevertheless, the pediatric interventional cardiology community has continued to develop less invasive solutions for congenital heart defects to minimize the need for open heart surgery and optimize overall outcomes. In this review, various interventional procedures in patients with congenital heart disease are explored.

College Students with Congenital Heart Disease: A Critical Time for Transition

ERIC Educational Resources Information Center

Hardy, Rose Y.; Gurvitz, Michelle; Jackson, Jamie L.; May, Susan; Miller, Paula; Daskalov, Rachel; Foster, Elyse

2018-01-01

As medical and surgical advances improve, more young adults with congenital heart disease (CHD) are attending college. This case study illustrates some of the issues that these young adults may face as they attend college and discusses the role that college health practitioners can play in easing that transition. Participants: A case of a male…

"What I Wish You Knew": Social Barriers toward Physical Activity in Youth with Congenital Heart Disease (CHD)

ERIC Educational Resources Information Center

Moola, Fiona; Fusco, Caroline; Kirsh, Joel A.

2011-01-01

Despite the benefits of physical activity for youth with congenital heart disease (CHD), most patients are inactive. Although literature has addressed medical and psychological barriers to participation, little is known about the social barriers that youth encounter. This qualitative study explored sociocultural barriers to physical activity from…

Successful emergency cardiac pacing and permanent pacemaker insertion in a preterm 29-week gestation hydropic baby with congenital complete heart block.

PubMed

Beake, Matthew Jonathan; Bhole, Vinay; Johnston, Tracey; Rasiah, Shree Vishna

2015-02-01

A preterm 29-week gestation baby was delivered because of gross foetal hydrops secondary to congenital complete heart block. Despite a poor prognosis, she survived stabilisation and received emergency epicardial pacing followed by permanent pacemaker insertion on day 13, weighing 1.2 kg.

The global burden of congenital heart disease.

PubMed

Hoffman, Julien Ie

2013-05-01

Although the incidence of congenital heart disease (CHD) is similar worldwide, the burden of supporting these patients falls more heavily on countries with high fertility rates. In a country with a fertility rate of about eight per woman, the population has to support four times as many children with CHD as in a country with a fertility rate of two. Countries with the highest fertility rates tend to have the lowest incomes per capita, thus accentuating the disparity. Countries with high fertility rates have more children with congenital heart disease per wage earner. Improving local health services and controlling infectious diseases (diarrhoeal illness, rheumatic fever, measles, rotoviral infection) are important but are mere 'band-aids' compared to improving education, empowering women and reducing birth rates.

Cardiac Arrhythmias in Adults With Congenital Heart Disease: Scope, Specific Problems, and Management.

PubMed

Lindsay, Ian; Moore, Jeremy P

2015-12-01

Progressive advances in the field of congenital heart disease (CHD) have led to a dramatic rise in the number of affected patients surviving well into adulthood. Groundbreaking and innovative achievements in the medical, surgical, and interventional management of these patients have allowed them to lead full adult lives that have included raising families, completing degrees in higher education, and maintaining successful careers. As patients are living longer, arrhythmias have emerged as one of the most significant factors affecting their morbidity, mortality, and quality of life. This article aims to review the current understanding of arrhythmia within the adult congenital heart disease (ACHD) population, emphasizing recent advances and providing recommendations pertaining to the management of these patients.

The development of a congenital heart programme quality dashboard to promote transparent reporting of outcomes.

PubMed

Anand, Vijay; Cave, Dominic; McCrady, Heather; Al-Aklabi, Mohammed; Ross, David B; Rebeyka, Ivan M; Adatia, Ian

2015-12-01

In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children's Hospital.

Pictorial Review of Surgical Anatomy in Adult Congenital Heart Disease.

PubMed

De Cecco, Carlo N; Muscogiuri, Giuseppe; Madrid Pérez, José M; Eid, Marwen; Suranyi, Pal; Lesslie, Virginia W; Bastarrika, Gorka

2017-07-01

The survival rate of patients with congenital heart disease (CHD) has dramatically improved over the last 2 decades because of technological and surgical advances in diagnosis and treatment, respectively. The vast majority of CHD patients are, in fact, amenable to treatment by either device closure or surgery. Considering the wide spectrum of surgical procedures and complex native and derived anatomy, continuous and detailed follow-up is of paramount importance. Cardiac magnetic resonance and cardiac computed tomography angiography are the cornerstones of diagnosis and follow-up of CHD, allowing for comprehensive noninvasive assessment of the heart, coronary tree, and intrathoracic great vessels, along with both morphological and functional evaluation. The aim of this pictorial review is to provide an overview of the most common CHDs and their related surgical procedures as familiarity with the radiological findings of grown-up congenital heart disease patients is crucial for proper diagnostic and follow-up pathways.

[Adult congenital heart disease: Medical and psychosocial issues].

PubMed

Ladouceur, Magalie; Pontnau, Florence; Iserin, Laurence

2017-05-01

The population of adults with congenital heart disease (ACHD) is continuously increasing with now a higher prevalence than that of the pediatric population. This concerns above all complex congenital heart diseases. Heart failure is the primary cause of death followed by arrhythmia, which is very common in ACHD. A specialized follow-up by dedicated centers is significantly associated with an improvement of survival of ACHD patients compared to non-expert follow-up. Extracardiac disorders (liver, kidney, respiratory) are frequent and require an accurate and specific management. The psychosocial impact, particularly the professional difficulties, is common and may require implementation of appropriate measures to improve the patient social life. Unplanned pregnancy and/or a lack of information about contraception may induce severe cardiovascular complications in ACHD women. Education about contraceptive methods at adolescence and pre-conceptional counseling are requested in this population. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Transcatheter intervention for the treatment of congenital cardiac defects.

PubMed Central

Grifka, R G

1997-01-01

Cardiac catheterization has an illustrious history, originating in 1929 when Werner Forsmann, a surgical resident, performed a heart catheterization on himself. Transcatheter interventional procedures have been performed since the 1960s. The 1st intracardiac procedure to become standard therapy was a balloon atrial septostomy. Skeptics attacked this innovative procedure. However, the balloon septostomy procedure soon became the standard emergency procedure for certain congenital heart defects, and was the impetus for other investigators in the field of transcatheter intervention. We will discuss transcatheter treatment for congenital vascular stenoses and vascular occlusion. Images PMID:9456482

Nomenclature for congenital and paediatric cardiac disease: historical perspectives and The International Pediatric and Congenital Cardiac Code.

PubMed

Franklin, Rodney C G; Jacobs, Jeffrey Phillip; Krogmann, Otto N; Béland, Marie J; Aiello, Vera D; Colan, Steven D; Elliott, Martin J; William Gaynor, J; Kurosawa, Hiromi; Maruszewski, Bohdan; Stellin, Giovanni; Tchervenkov, Christo I; Walters Iii, Henry L; Weinberg, Paul; Anderson, Robert H

2008-12-01

Clinicians working in the field of congenital and paediatric cardiology have long felt the need for a common diagnostic and therapeutic nomenclature and coding system with which to classify patients of all ages with congenital and acquired cardiac disease. A cohesive and comprehensive system of nomenclature, suitable for setting a global standard for multicentric analysis of outcomes and stratification of risk, has only recently emerged, namely, The International Paediatric and Congenital Cardiac Code. This review, will give an historical perspective on the development of systems of nomenclature in general, and specifically with respect to the diagnosis and treatment of patients with paediatric and congenital cardiac disease. Finally, current and future efforts to merge such systems into the paperless environment of the electronic health or patient record on a global scale are briefly explored. On October 6, 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. In January, 2005, the International Nomenclature Committee was constituted in Canada as The International Society for Nomenclature of Paediatric and Congenital Heart Disease. This International Society now has three working groups. The Nomenclature Working Group developed The International Paediatric and Congenital Cardiac Code and will continue to maintain, expand, update, and preserve this International Code. It will also provide ready access to the International Code for the global paediatric and congenital cardiology and cardiac surgery communities, related disciplines, the healthcare industry, and governmental agencies, both electronically and in published form. The Definitions Working Group will write definitions for the terms in the International Paediatric and Congenital Cardiac Code, building on the previously published definitions from the Nomenclature Working Group. The Archiving Working Group, also known as The Congenital Heart Archiving Research Team, will link images and videos to the International Paediatric and Congenital Cardiac Code. The images and videos will be acquired from cardiac morphologic specimens and imaging modalities such as echocardiography, angiography, computerized axial tomography and magnetic resonance imaging, as well as intraoperative images and videos. Efforts are ongoing to expand the usage of The International Paediatric and Congenital Cardiac Code to other areas of global healthcare. Collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the representatives of the steering group responsible for the creation of the 11th revision of the International Classification of Diseases, administered by the World Health Organisation. Similar collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the International Health Terminology Standards Development Organisation, who are the owners of the Systematized Nomenclature of Medicine or "SNOMED". The International Paediatric and Congenital Cardiac Code was created by specialists in the field to name and classify paediatric and congenital cardiac disease and its treatment. It is a comprehensive code that can be freely downloaded from the internet (http://www.IPCCC.net) and is already in use worldwide, particularly for international comparisons of outcomes. The goal of this effort is to create strategies for stratification of risk and to improve healthcare for the individual patient. The collaboration with the World Heath Organization, the International Health Terminology Standards Development Organisation, and the healthcare industry, will lead to further enhancement of the International Code, and to its more universal use.

Association of periodontal disease, oral procedures, and other clinical findings with bacterial endocarditis in dogs.

PubMed

Peddle, Gordon D; Drobatz, Kenneth J; Harvey, Colin E; Adams, Allison; Sleeper, Meg M

2009-01-01

To identify risk factors potentially associated with the development of bacterial endocarditis in dogs and determine whether periodontal disease and surgical procedures (oral and nonoral) were associated with bacterial endocarditis. Retrospective case-control study. 76 dogs with (cases) and 80 dogs without (controls) bacterial endocarditis. Medical records were reviewed for information on signalment, physical examination findings, recent medical history, and results of echocardiography, clinicopathologic testing, and necropsy. None of the dogs with endocarditis had a history of undergoing any dental or oral procedure in the 3 months prior to the diagnosis of endocarditis, and no significant difference was found between groups with regard to the prevalence of oral infection. Dogs with endocarditis were significantly more likely to have undergone a nonoral surgical procedure that required general anesthesia in the preceding 3 months or to have developed a new heart murmur or a change in intensity of an existing heart murmur. Preexisting cardiac dis-ease (congenital or acquired) was not found to be a risk factor. Results did not provide any evidence of an association between bacterial endocarditis in dogs and either dental or oral surgical procedures or oral infection. Findings suggested that the routine use of prophylactic antimicrobial administration in dogs undergoing oral procedures needs to be reevaluated.

Coronary artery fistula

MedlinePlus

Congenital heart defect - coronary artery fistula; Birth defect heart - coronary artery fistula ... attaches to one of the chambers of the heart (the atrium or ventricle) or another blood vessel ( ...

Echocardiography findings in clinically confirmed congenital rubella syndrome cases seen at the University of Port Harcourt Teaching Hospital, Nigeria.

PubMed

Otaigbe, B E; Tabansi, P N; Agbedey, G O

2012-01-01

Congenital rubella syndrome (CRS) is known to affect thousands of children in the developing world because rubella vaccination is not available routinely in most of these countries. Among its many manifestations only congenital heart disease is life threatening. This study was undertaken to ascertain the cases of echocardiographic determined congenital heart disease in clinically confirmed CRS cases. Data of patients with clinically confirmed CRS seen over a period of 5 years in the Paediatric cardiology clinic of the University of Port Harcourt Teaching Hospital was retrieved and analysed. Seven cases (2.8 % of total cardiac cases) were seen. 6 (85.7%) cases had at least one murmur on auscultation. Patent ductus arteriosus was the commonest cardiac defect seen either in isolation or incombination with a VSD or ASD. Only one child had no cardiac defect. 4 (57.1%) of them had been admitted for heart failure at least once and 2 (28.6 %) were on anti-failure regimen, one of whom had cardiac surgery one month ago. In view of the fact that 6 (85.7%) of the patients with CRS had at least one congenital heart defect, we advocate routine echocardiography on patients with CRS to ensure early treatment and reduce mortality and morbidity.We also advocate that rubella vaccination be given routinely in developing countries.

Clinical features and outcome of cerebral abscess in congenital heart disease.

PubMed

Mehnaz, Atiq; Syed, Ahmed Umair; Saleem, Allana Salman; Khalid, Chishti N

2006-01-01

Cerebral abscess is a serious life threatening complication of several diseases. The objective of this study was to look at the clinical profile, microbiology and outcome of children with cerebral abscess having an underlying congenital heart disease as compared to other predisposing conditions. Thirty children aged less than 15 years were reviewed. There were 15 males and 15 females. The mean age of presentation was 5.6 +/- 4.4 years. The duration of illness at the time of admission was 17.6 +/- 24.6 days. Typically patients presented with fever, vomiting, headache and seizures. The predisposing conditions found were cyanotic congenital heart disease in 11 (37%) of children, meningitis in 6 (20%), septicemia in 7 (23%) and no underlying cause was found in 5 (17%) children. The most common microbe in children with cyanotic congenital heart disease was of the Streptococcus milleri group (52%). Computerized tomography confirmed the diagnosis and the most common location of the abscess was the parietal lobe of the cerebral hemisphere. All abscesses were large, more than 2 cm in diameter and were aspirated surgically. Excision was performed in 6 children. Five children expired, one due to a intracranial bleeding and the others due to severe cerebral edema and tentorial herniation. Complications were seen in 20 children and 16 had sequelae, hemiparesis in 11 and seizure disorder in 5. Brain abscess is a serious infection with poor outcome if diagnosed late. Delayed surgical drainage has high morbidity and mortality. The threshold for diagnosis should be low particularly in children with a predisposing condition like cyanotic congenital heart disease.

Cerebral oxygen saturation during the first 72h after birth in infants diagnosed prenatally with congenital heart disease.

PubMed

Mebius, Mirthe J; van der Laan, Michelle E; Verhagen, Elise A; Roofthooft, Marcus Tr; Bos, Arend F; Kooi, Elisabeth Mw

2016-12-01

Evidence suggests that hypoxic-ischemic brain injury in infants with congenital heart disease already occurs during early life. The aim of our study was, therefore, to assess the course of regional cerebral oxygen saturation (r c SO 2 ) and fractional tissue oxygen extraction (FTOE) during the first 72h after birth in infants with prenatally diagnosed duct-dependent congenital heart disease. In addition, we identified clinical parameters that were associated with r c SO 2 . We included 56 infants with duct-dependent congenital heart disease. We measured arterial oxygen saturation (SpO 2 ) and r c SO 2 during the first 72h after birth. Simultaneously, we calculated FTOE. We observed median r c SO 2 values of approximately 60%, a decreasing FTOE from 0.34 on day 1 to 0.28 on day 3 and stable preductal SpO 2 values around 90%. Several clinical variables were associated with r c SO 2 . In a multiple linear regression model only type of CHD and preductal SpO 2 were significant predictors of r c SO 2 during the first three days after birth. Infants with a duct-dependent pulmonary circulation had up to 12% lower r c SO 2 values than infants with a duct-dependent systemic circulation. We demonstrated that, during the first three days after birth, cerebral oxygen saturation is low in infants with duct-dependent congenital heart disease. Furthermore, this study provides preoperative reference values of r c SO 2 and FTOE in infants with duct-dependent CHD. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Pulmonary vascular volume ratio measured by cardiac computed tomography in children and young adults with congenital heart disease: comparison with lung perfusion scintigraphy.

PubMed

Goo, Hyun Woo; Park, Sang Hyub

2017-11-01

Lung perfusion scintigraphy is regarded as the gold standard for evaluating differential lung perfusion ratio in congenital heart disease. To compare cardiac CT with lung perfusion scintigraphy for estimated pulmonary vascular volume ratio in patients with congenital heart disease. We included 52 children and young adults (median age 4 years, range 2 months to 28 years; 31 males) with congenital heart disease who underwent cardiac CT and lung perfusion scintigraphy without an interim surgical or transcatheter intervention and within 1 year. We calculated the right and left pulmonary vascular volumes using threshold-based CT volumetry. Then we compared right pulmonary vascular volume percentages at cardiac CT with right lung perfusion percentages at lung perfusion scintigraphy by using paired t-test and Bland-Altman analysis. The right pulmonary vascular volume percentages at cardiac CT (66.3 ± 14.0%) were significantly smaller than the right lung perfusion percentages at lung perfusion scintigraphy (69.1 ± 15.0%; P=0.001). Bland-Altman analysis showed a mean difference of -2.8 ± 5.8% and 95% limits of agreement (-14.1%, 8.5%) between these two variables. Cardiac CT, in a single examination, can offer pulmonary vascular volume ratio in addition to pulmonary artery anatomy essential for evaluating peripheral pulmonary artery stenosis in patients with congenital heart disease. However there is a wide range of agreement between cardiac CT and lung perfusion scintigraphy.

Micro-economic impact of congenital heart surgery: results of a prospective study from a limited-resource setting.

PubMed

Raj, Manu; Paul, Mary; Sudhakar, Abish; Varghese, Anu Alphonse; Haridas, Aareesh Chittulliparamb; Kabali, Conrad; Kumar, Raman Krishna

2015-01-01

The microeconomic impact of surgery for congenital heart disease is unexplored, particularly in resource limited environments. We sought to understand the direct and indirect costs related to congenital heart surgery and its impact on Indian households from a family perspective. Baseline and first follow-up data of 644 consecutive children admitted for surgery for congenital heart disease (March 2013 - July 2014) in a tertiary referral hospital in Central Kerala, South India was collected prospectivelyfrom parents through questionnaires using a semi-structured interview schedule. The median age was 8.2 months (IQR: 3.0- 36.0 months). Most families belonged to upper middle (43.0%) and lower middle (35.7%) socioeconomic class. Only 3.9% of families had some form of health insurance. The median expense for the admission and surgery was INR 201898 (IQR: 163287-266139) [I$ 11989 (IQR: 9696-15804)], which was 0.93 (IQR: 0.52-1.49) times the annual family income of affected patients. Median loss of man-days was 35 (IQR: 24-50) and job-days was 15 (IQR: 11-24). Surgical risk category and hospital stay duration significantly predicted higher costs. One in two families reported overwhelming to high financial stress during admission period for surgery. Approximately half of the families borrowed money during the follow up period after surgery. Surgery for congenital heart disease results in significant financial burden for majority of families studied. Efforts should be directed at further reductions in treatment costs without compromising the quality of care together with generating financial support for affected families.

Choice and competition between adult congenital heart disease centers: evidence of considerable geographical disparities and association with clinical or academic results.

PubMed

Diller, Gerhard-Paul; Kempny, Aleksander; Piorkowski, Adam; Grübler, Martin; Swan, Lorna; Baumgartner, Helmut; Dimopoulos, Konstantinos; Gatzoulis, Michael A

2014-03-01

Although concentrating adult congenital heart disease services at high-volume centers has been widely advocated, the potential beneficial effects of competition and patient choice have received relatively little attention. We aimed to assess the degree of patient choice and competition between adult congenital heart disease units and to investigate whether competition indices correlate with clinical quality or research output. Competition between the 10 major adult congenital heart disease units in England was evaluated based on the Herfindahl-Hirschman Index, representing the sum of squared market shares of individual units. In addition, to account for geography and feasible access, we calculated spatial indices of competition based on travel time by road. These indices were correlated with 30-day mortality postpulmonary valve replacement in adult patients (as obtained from the National Central Cardiac Audit Database) and the aggregate research impact factors of individual centers. On a national level, a high level of competition without obvious dominant players was found (Herfindahl-Hirschman Index between 0.107 and 0.013). When accounting for geography, however, important disparities in patient choice and competition faced by individual centers emerged. The degree of local competition was correlated significantly with clinical outcomes and research output. In contrast, no association between center volume and outcome could be established. Beyond the usual focus on concentrating services at high-volume centers, the potentially beneficial effects of competition should not be ignored. Therefore, policymakers should consider fostering a competitive environment for adult congenital heart disease centers or at least avoiding creating government-granted monopolies in the field.

Impact of noncardiac congenital and genetic abnormalities on outcomes in hypoplastic left heart syndrome.

PubMed

Patel, Angira; Hickey, Edward; Mavroudis, Constantine; Jacobs, Jeffrey P; Jacobs, Marshall L; Backer, Carl L; Gevitz, Melanie; Mavroudis, Constantine D

2010-06-01

Hypoplastic left heart syndrome may coexist with noncardiac congenital defects or genetic syndromes. We explored the impact of such lesions on outcomes after staged univentricular palliation. Society of Thoracic Surgeons database 2002 to 2006: Children diagnosed with hypoplastic left heart syndrome who underwent stage 1 Norwood (n = 1,236), stage 2 superior cavopulmonary anastamosis (n = 702) or stage 3 Fontan (n = 553) procedures were studied. In-hospital mortality, postoperative complications, and length of stay were compared at each stage between those with and without noncardiac-genetic defects. Congenital Heart Surgeons' Society database 1994 to 2001: All 703 infants enrolled in the Congenital Heart Surgeons' Society critical left ventricular outflow tract obstruction study who underwent primary stage 1 palliation were reviewed. The impact of noncardiac defects-syndromes on survival was explored using multivariable parametric models with bootstrap bagging. Society of Thoracic Surgeons database: Stage 1 in-hospital mortality (26% vs 20%, p = 0.04) and mean postoperative length of stay (42 versus 31 days, p < 0.0001) were greater, and postoperative complications significantly more prevalent in infants with noncardiac-genetic defects. Congenital Heart Surgeons' Society database: Noncardiac-genetic defects were present in 55 (8%). Early hazard for death after Norwood was significantly worse in infants with noncardiac defects-syndromes (p = 0.008). Chromosomal defects (n = 14) were highly unfavorable: the early risk of death was doubled (10-year survival 25 +/- 9% vs 54 +/- 2%, p = 0.005). Turner syndrome accounted for the majority of chromosomal defects in this population (11 of 14, 79%). Mode of death was rarely attributable to the noncardiac-genetic defect. Survival in hypoplastic left heart syndrome is strongly influenced by the presence of noncardiac abnormalities. Strategies to improve mortality in infants with noncardiac abnormalities should be explored. Presence of chromosomal defects, especially Turner syndrome, should enter decision-management options for parents and physicians. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Tetralogy of Fallot

MedlinePlus

Tet; TOF; Congenital heart defect - tetralogy; Cyanotic heart disease - tetralogy; Birth defect - tetralogy ... The classic form includes four defects of the heart and its major blood vessels: Ventricular septal defect ( ...

Congenital and Acquired Valvular Heart Disease in Pregnancy.

PubMed

Goldstein, Sarah A; Ward, Cary C

2017-08-24

The number of pregnancies complicated by valvular heart disease is increasing. This review describes the hemodynamic effects of clinically important valvular abnormalities during pregnancy and reviews current guideline-driven management strategies. Valvular heart disease in women of childbearing age is most commonly caused by congenital abnormalities and rheumatic heart disease. Regurgitant lesions are well tolerated, while stenotic lesions are associated with a higher risk of pregnancy-related complications. Management of symptomatic disease during pregnancy is primarily medical, with percutaneous interventions considered for refractory symptoms. Most guidelines addressing the management of valvular heart disease during pregnancy are based on case reports and observational studies. Additional investigation is required to further advance the care of this growing patient population.

Congenital complete heart block in the newborn associated with maternal systemic lupus erythematosus and other connective tissue disorders.

PubMed Central

Hardy, J D; Solomon, S; Banwell, G S; Beach, R; Wright, V; Howard, F M

1979-01-01

Four babies with complete heart block associated with maternal systemic lupus erythematosus (SLE) are described, together with a 5th baby whose mother had serological abnormalities only. One baby had a rapidly fatal outcome, one has required digoxin for heart failure, and the remaining 3 are asymptomatic but remain in complete heart block. Additional manifestations were present in 2 of them. The spectrum of neonatal abnormalities that may occur in association with maternal SLE and related connective tissue disorders is discussed, together with the possible causes and the prognosis. We conclude that congenital heart block is more common than had previously been appreciated. Images Figure PMID:420526

Maternal reproductive history and the risk of isolated congenital malformations.

PubMed

Materna-Kiryluk, A; Więckowska, B; Wiśniewska, K; Borszewska-Kornacka, M K; Godula-Stuglik, U; Limon, J; Rusin, J; Sawulicka-Oleszczuk, H; Szwałkiewicz-Warowicka, E; Walczak, M

2011-03-01

We examined the relationship between maternal reproductive history and the newborn's risk of isolated congenital malformations in a large case-control cohort from the Polish Registry of Congenital Malformations. Congenital malformations were classified into four categories: isolated congenital heart defects (n=1673), isolated cleft palate (n=255), cleft lip with or without cleft palate (n=448) and renal agenesis (n=103). The case groups were compared with a shared group of 2068 controls recruited in the same time period and geographic area. Multivariable logistic regression was used to assess the risk associated with maternal gravidity and of previous miscarriages after accounting for maternal age and other potential risk factors. In unadjusted analyses, maternal gravidity was significantly associated with increased risk of all four classes of congenital malformations. After adjustment, a significant association persisted for congenital heart defects [odds ratio (OR)=1.22, [95% confidence interval (CI) 1.09, 1.36], P=0.0007] and cleft lip with or without cleft palate (OR=1.21, [95% CI 1.09, 1.36], P=0.0005). A similar trend existed for isolated cleft palate (OR=1.18, [95% CI 1.02, 1.37], P=0.03). There was no appreciable increase in the risk of congenital malformations associated with a maternal history of miscarriages, but a trend for a protective effect on the occurrence of cleft lip with or without cleft palate was observed (OR=0.72, [95% CI 0.52, 0.99], P=0.045). Based on our data, maternal gravidity represents a significant risk factor for congenital heart defects and cleft lip with or without cleft palate in the newborn infant. Our data do not support an increase in risk because of past history of miscarriages. © 2011 Blackwell Publishing Ltd.

Swarm intelligence applied to the risk evaluation for congenital heart surgery.

PubMed

Zapata-Impata, Brayan S; Ruiz-Fernandez, Daniel; Monsalve-Torra, Ana

2015-01-01

Particle Swarm Optimization is an optimization technique based on the positions of several particles created to find the best solution to a problem. In this work we analyze the accuracy of a modification of this algorithm to classify the levels of risk for a surgery, used as a treatment to correct children malformations that imply congenital heart diseases.

Basic imaging in congenital heart disease. 3rd Ed

DOE Office of Scientific and Technical Information (OSTI.GOV)

Swischuk, L.E.; Sapire, D.W.

1986-01-01

The book retains its previous format with chapters on embryology, plain film interpretation, classification of pulmonary vascular patterns, cardiac malpositions and vascular anomalies, and illustrative cases. The book is organized with an abundance of illustrative figures, diagrams, and image reproductions. These include plain chest radiographs, angiograms, echocardiograms, and MR images. The authors present the pathophysiology and imaging of congenital heart lesions.

Congenital mitral stenosis, subvalvular aortic stenosis, and congestive heart failure in a duck.

PubMed

Mitchell, Elizabeth B; Hawkins, Michelle G; Orvalho, Joao S; Thomas, William P

2008-06-01

A 2.6-year-old duck was evaluated for respiratory difficulty. On the basis of physical, radiographic and echocardiographic findings, a diagnosis of congestive heart failure secondary to congenital mitral stenosis and subvalvular aortic stenosis was made. The duck did not respond well to medical therapy and was euthanized. The diagnosis was confirmed at necropsy.

Long-Term Intellectual Functioning and School-Related Behavioural Outcomes in Children and Adolescents after Invasive Treatment for Congenital Heart Disease

ERIC Educational Resources Information Center

Spijkerboer, A. W.; Utens, E. M. W. J.; Bogers, A. J. J. C.; Verhulst, F. C.; Helbing, W. A.

2008-01-01

In this study, long-term intellectual functioning and school-related behavioural outcomes were assessed in a patient sample that underwent invasive treatment for congenital heart disease (ConHD) between 1990 and 1995. The Wechsler Intelligence Scale for Children-Revised was used to measure intellectual functioning and the Teacher's Report Form to…

Risk Factors for Gross Motor Dysfunction in Infants with Congenital Heart Disease

ERIC Educational Resources Information Center

Long, Suzanne H.; Eldridge, Bev J.; Galea, Mary P.; Harris, Susan R.

2011-01-01

Infants with congenital heart disease (CHD) that is severe enough to require early surgery are at risk for cognitive and motor delays, as well as musculoskeletal impairments, and are best managed by an interdisciplinary team during their hospital stay and after discharge. The purpose of this article is to review some of the risk factors associated…

Physical Activity and Sport Participation in Youth with Congenital Heart Disease: Perceptions of Children and Parents

ERIC Educational Resources Information Center

Moola, Fiona; Faulkner, Guy E. J.; Kirsh, Joel A.; Kilburn, Jennifer

2008-01-01

This study explored perceptions toward physical activity and sport in the lives of youth with congenital heart disease. Thirteen cardiac participants were interviewed in the presence of their parents, and a process of inductive analysis was conducted. Sport was not considered a valued pursuit despite the belief that it is essential for the…

Important considerations for the newborn: access to postdischarge newborn care, pulse oximetry screening for congenital heart disease, and circumcision.

PubMed

Pattishall, Amy E; Spector, Nancy D; McPeak, Katie E

2014-12-01

This article addresses three areas in which new policies and research demonstrate the opportunity to impact the health of neonates: access to postdischarge newborn care, pulse oximetry screening for congenital heart disease, and circumcision. Recent research has identified that child healthcare providers are not typically adhering to the recommended first newborn visit within 48 h of hospital discharge. Despite its benefits, cost-effectiveness, and the recommendation that routine screening for cyanotic congenital heart disease be added to the panel of universal newborn screening, adoption of this practice is variable. Evidence suggests a significant reduction in the transmission of HIV linked to circumcision, leading professional organizations to generate new policy statements on neonatal male circumcision. Pediatric healthcare providers should pay careful attention to the timing of the first newborn outpatient follow-up visit. Pulse oximetry screening for cyanotic congenital heart disease is specific, sensitive and meets criteria for universal screening, and providers should utilize well designed screening protocols. In addition, healthcare providers for newborns, especially those who perform circumcisions, should provide nonbiased, up-to-date information on the medical, financial, and ethical aspects of the procedure.

Mutations of the GATA4 and NKX2.5 genes in Chinese pediatric patients with non-familial congenital heart disease.

PubMed

Peng, Ting; Wang, Li; Zhou, Shu-Feng; Li, Xiaotian

2010-12-01

A number of mutations in GATA4 and NKX2.5 have been identified to be causative for a subset of familial congenital heart defects (CHDs) and a small number of sporadic CHDs. In this study, we evaluated common GATA4 and NKX2.5 mutations in 135 Chinese pediatric patients with non-familial congenital heart defects. Two novel mutations in the coding region of GATA4 were identified, namely, 487C >T (Pro163Ser) in exon 1 in a child with tetralogy of Fallot and 1220C >A (Pro407Gln) in exon 6 in a pediatric patient with outlet membranous ventricular septal defect. We also found 848C >A (Pro283Gln) in exon 2 of the NKX2.5 gene in a pediatric patient with ventricular septal defect, patent ductus arteriosus and aortic isthmus stenosis. None of the mutations was detected in healthy control subjects (n = 114). This study suggests that GATA4 and NKX2.5 missense mutations may be associated with congenital heart defects in pediatric Chinese patients. Further clinical studies with large samples are warranted.

[CLINICAL CHARACTERISTICS OF CONGENITAL HEART DISEASES ASSOCIATED WITH CONNECTIVE TISSUE DISPLASIA AT CHILDREN LIVING IN EAST REGION OF KAZAKHSTAN].

PubMed

Madiyeva, M; Rymbayeva, T

2017-11-01

The frequency of the combination of congenital heart defects (CHD) and connective tissue dysplasia remains poorly understood. And connective tissue dysplasia enhance severity the clinical of CHD. The aim of the study was to conduct a clinical and laboratory analysis of combinations of congenital heart defects and connective tissue dysplasia in children of Semey and to determine the risk for the development of these pathologies. The object of the study is the children of Semey (East Kazakhstan) aged 1-14 with congenital heart defects (CHD), with connective tissue dysplasia, healthy children and their mothers. Definition complex clinical and laboratory studies in children with CHD and connective tissue dysplasia, and their mothers. In children with CHD, the frequency of external and visceral signs of dysplasia was high. In 88.1% of cases in children with CHD was diagnosed 2-3 degrees of dysplasia. Was found difference in the microelement composition of blood serum and of hemostasis in children with CHD were expressed by hypofibrinogenemia, hypocalcemia, hypomagnesemia. Excess of the frequency of signs of dysplasia in mothers over the control group to consider dysplasia as a factor that influences the clinical of CHD.

Cardiovascular cast model fabrication and casting effectiveness evaluation in fetus with severe congenital heart disease or normal heart.

PubMed

Wang, Yu; Cao, Hai-yan; Xie, Ming-xing; He, Lin; Han, Wei; Hong, Liu; Peng, Yuan; Hu, Yun-fei; Song, Ben-cai; Wang, Jing; Wang, Bin; Deng, Cheng

2016-04-01

To investigate the application and effectiveness of vascular corrosion technique in preparing fetal cardiovascular cast models, 10 normal fetal heart specimens with other congenital disease (control group) and 18 specimens with severe congenital heart disease (case group) from induced abortions were enrolled in this study from March 2013 to June 2015 in our hospital. Cast models were prepared by injecting casting material into vascular lumen to demonstrate real geometries of fetal cardiovascular system. Casting effectiveness was analyzed in terms of local anatomic structures and different anatomical levels (including overall level, atrioventricular and great vascular system, left-sided and right-sided heart), as well as different trimesters of pregnancy. In our study, all specimens were successfully casted. Casting effectiveness analysis of local anatomic structures showed a mean score from 1.90±1.45 to 3.60±0.52, without significant differences between case and control groups in most local anatomic structures except left ventricle, which had a higher score in control group (P=0.027). Inter-group comparison of casting effectiveness in different anatomical levels showed no significant differences between the two groups. Intra-group comparison also revealed undifferentiated casting effectiveness between atrioventricular and great vascular system, or left-sided and right-sided heart in corresponding group. Third-trimester group had a significantly higher perfusion score in great vascular system than second-trimester group (P=0.046), while the other anatomical levels displayed no such difference. Vascular corrosion technique can be successfully used in fabrication of fetal cardiovascular cast model. It is also a reliable method to demonstrate three-dimensional anatomy of severe congenital heart disease and normal heart in fetus.

Spectrum of heart diseases in children: an echocardiographic study of 1,666 subjects in a pediatric hospital, Yaounde, Cameroon.

PubMed

Chelo, David; Nguefack, Félicitée; Menanga, Alain P; Ngo Um, Suzanne; Gody, Jean C; Tatah, Sandra A; Koki Ndombo, Paul O

2016-02-01

Children's health programs in Sub-Saharan Africa have always been oriented primarily to infectious diseases and malnutrition. We are witnessing in the early 21(st) century an epidemiological transition marked by the decline of old diseases and the identification of new diseases including heart disease. Therefore, it is necessary to describe the spectrum of these diseases in order to better prepare health workers to these new challenges. We conducted a cross-sectional study focused on heart disease diagnosed by echocardiography in children seen from January 2006 to December 2014 in a pediatric hospital of Yaounde. We collected socio-demographic data and the types of heart disease from registers, patients files as well as the electronic database of echocardiographic records. A total of 2,235 patients underwent echocardiographic examination during the study period including 1,666 subjects with heart disease. Congenital cardiopathies were found in 1,230 (73.8%) patients and acquired abnormalities in 429 (25.8%). Seven children (0.4%) had a combination of both types. Congenital heart defects (CHD) were dominated by ventricular septal defect (VSD). Acquired heart disease was mostly rheumatic valvulopathies. Dyspnea on exertion was the most frequent presenting complaint (87.6%). Discovery of a heart murmur was the principal clinical finding on physical examination (81.4%). The median age was 9 months for congenital heart disease and 132 months for acquired heart disease. As infectious diseases recede and the diagnostic facilities are improving, pediatric heart diseases occupy a more important position in the spectrum of pediatric diseases in our context. However, the ability to evoke the diagnosis remains unsatisfactory by the majority of health personnel and therefore needs to be improved. Apart from congenital heart diseases, the impact of acquired heart diseases, rheumatic valvulopathy being the highest ranking, is remarkable in pediatrics. Awareness of health personnel for better management of child tonsillitis is more than ever a necessity. This preventive attitude of rheumatic heart disease is the main attitude available in our disadvantaged economic environment.

A case study of infant physiologic response to skin-to-skin contact following surgery for complex congenital heart disease

PubMed Central

Harrison, Tondi M.; Ludington-Hoe, Susan

2014-01-01

Background Infants with complex congenital heart disease requiring surgical intervention within the first days or weeks of life may be the most seriously ill infants needing intensive nursing and medical care immediately after birth. Skin to skin contact (SSC) is well-accepted and practiced as a positive therapeutic intervention in premature infants, but is not routinely offered to infants in cardiac intensive care units. Physiologic effects of SSC in the congenital heart disease population must be examined before recommending incorporation of SSC into standard care routines. Objective The purpose of this case study was to describe the physiologic response to a single session of SSC in an 18-day-old infant with hypoplastic left heart syndrome. Methods Repeated measures of heart rate, respiratory rate, oxygen saturation, blood pressure, and temperature were recorded 30 minutes prior to SSC, during SSC (including interruptions for bottle and breast feedings), and 10 minutes after SSC was completed. Results All physiologic parameters were clinically acceptable throughout the 135-minute observation. Conclusion This case study provides beginning evidence that SSC is safe in full-term infants following surgery for complex congenital heart disease. Further research with a larger sample is needed to examine effects of SSC on infant physiology before surgery and earlier in the postoperative time period as well as on additional outcomes such as length of stay, maternal-infant interaction, and neurodevelopment. PMID:25325374

Reproduction and Survival After Cardiac Defect Repair

ClinicalTrials.gov

2016-02-17

Cardiovascular Diseases; Heart Diseases; Defect, Congenital Heart; Aortic Valve Stenosis; Transposition of Great Vessels; Ductus Arteriosus, Patent; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Down Syndrome; Tetralogy of Fallot; Pulmonic Stenosis; Coarctation of Aorta

If Your Child Has a Heart Defect (For Parents)

MedlinePlus

... congenital heart disease are at risk for bacterial endocarditis, an infection of the tissue that lines the ... who have artificial heart valves or have had endocarditis before. Most children with heart problems, however, do ...

Two-dimensional right ventricular strain by speckle tracking for assessment of longitudinal right ventricular function after paediatric congenital heart disease surgery.

PubMed

Karsenty, Clement; Hadeed, Khaled; Dulac, Yves; Semet, Florent; Alacoque, Xavier; Breinig, Sophie; Leobon, Bertrand; Acar, Philippe; Hascoet, Sebastien

2017-03-01

Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD). To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers. In this prospective single-centre study, RV peak systolic strain (RV-PSS) was measured using 2D speckle tracking in 37 consecutive children undergoing CHD surgery. Examinations were performed the day before surgery, a few hours after surgery and before discharge. Relationships with the z score of tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (TA Sa) were assessed. Median (interquartile range) age was 19 months (5-63); median weight was 9.2 kg (5.3-18.0). RV-PSS analysis was feasible in 92.9% (95% confidence interval [CI]: 86.0-97.1) of examinations. The coefficient of variation was 9.7% (95% CI: 7.4-11.9) for intraobserver variability and 15.1% (95% CI: 12.7-17.6) for interobserver variability. Correlations between RV-PSS and z score of TAPSE and TA Sa were strong (r=0.71, P<0.0001 and r=0.70, P<0.0001, respectively). RV-PSS was significantly reduced after surgery compared with baseline (-10.5±2.9% vs. -19.5±4.8%; P<0.0001) and at discharge (-13.5±4.0% vs. -19.5±4.8%; P<0.0001). Similar evolutions were observed with TAPSE and TA Sa (both P<0.0001). RV longitudinal strain by speckle tracking is a feasible and reproducible method of assessing perioperative evolution of RV function in children with CHD. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Interstitial deletion of 8q21-->22 associated with minor anomalies, congenital heart defect, and Dandy-Walker variant.

PubMed

Donahue, M L; Ryan, R M

1995-03-13

We describe an infant with a deletion of 8q21-->22 who had distinct clinical manifestations including minor facial anomalies, a congenital heart defect, a Dandy-Walker variant, and mild to moderate developmental delay. Her facial characteristics included small, wide-spaced eyes, asymmetric bilateral epicanthal folds, a broad nasal bridge, a "carp-shaped" mouth, micrognathia, and prominent, apparently low-set ears. Three other reports describe children with larger proximal deletions of 8q that include 8q21 and q22. These four children all have similar facial appearance. Of the others reported, one had a congenital heart defect and one had craniosynostosis. This case, in addition to the previously noted three cases, helps in delineating a recognizable syndrome.

Pediatric echocardiography: new developments and applications.

PubMed

Ge, Shuping

2013-04-01

In this Special Issue of the Journal, 6 review articles that represent the new developments and applications of echocardiography for diagnosis and assessment of congenital heart disease from fetus to adult are included. The goal is to provide an updated review of the evidence for the current and potential use of some of the new methodologies, i.e. fetal echocardiography, tissue Doppler imaging, strain imaging by speckle tracking imaging, ventricular synchrony, quantification using real time three-dimensional (3D) echocardiography, and 3D echocardiography for adults with congenital heart disease. We hope this effort will provide an impetus for more investigation and ultimately clinical application of these new methodologies to improve the care of those with congenital and acquired heart diseases in the pediatric population and beyond. © 2013, Wiley Periodicals, Inc.

75 FR 6671 - Agency Forms Undergoing Paperwork Reduction Act Review

Federal Register 2010, 2011, 2012, 2013, 2014

2010-02-10

... Project Congenital Syphilis (CS) Case Investigation and Report Form (CDC73.126), OMB No. 0920-0128, (exp... ``Congenital Syphilis (CS) Case Investigation and Report Form'' (CDC73.126). The CS Form is currently approved... Health Departments Congenital Syphilis (CS) 10 11 20/60 Case Investigation and Report. Territorial Health...

Intact Imaging of Human Heart Structure Using X-ray Phase-Contrast Tomography.

PubMed

Kaneko, Yukihiro; Shinohara, Gen; Hoshino, Masato; Morishita, Hiroyuki; Morita, Kiyozo; Oshima, Yoshihiro; Takahashi, Masashi; Yagi, Naoto; Okita, Yutaka; Tsukube, Takuro

2017-02-01

Structural examination of human heart specimens at the microscopic level is a prerequisite for understanding congenital heart diseases. It is desirable not to destroy or alter the properties of such specimens because of their scarcity. However, many of the currently available imaging techniques either destroy the specimen through sectioning or alter the chemical and mechanical properties of the specimen through staining and contrast agent injection. As a result, subsequent studies may not be possible. X-ray phase-contrast tomography is an imaging modality for biological soft tissues that does not destroy or alter the properties of the specimen. The feasibility of X-ray phase-contrast tomography for the structural examination of heart specimens was tested using infantile and fetal heart specimens without congenital diseases. X-ray phase-contrast tomography was carried out at the SPring-8 synchrotron radiation facility using the Talbot grating interferometer at the bending magnet beamline BL20B2 to visualize the structure of five non-pretreated whole heart specimens obtained by autopsy. High-resolution, three-dimensional images were obtained for all specimens. The images clearly showed the myocardial structure, coronary vessels, and conduction bundle. X-ray phase-contrast tomography allows high-resolution, three-dimensional imaging of human heart specimens. Intact imaging using X-ray phase-contrast tomography can contribute to further structural investigation of heart specimens with congenital heart diseases.

Central Venous Catheter Insertion Site and Colonization in Pediatric Cardiac Surgery

ClinicalTrials.gov

2017-11-04

Central Line-associated Bloodstream Infection (CLABSI); Central Venous Catheter Associated Bloodstream Infection; Heart; Surgery, Heart, Functional Disturbance as Result; Congenital Heart Disease; Newborn; Infection

Contemporary cardiac surgery for adults with congenital heart disease.

PubMed

Beurtheret, Sylvain; Tutarel, Oktay; Diller, Gerhard Paul; West, Cathy; Ntalarizou, Evangelia; Resseguier, Noémie; Papaioannou, Vasileios; Jabbour, Richard; Simpkin, Victoria; Bastin, Anthony J; Babu-Narayan, Sonya V; Bonello, Beatrice; Li, Wei; Sethia, Babulal; Uemura, Hideki; Gatzoulis, Michael A; Shore, Darryl

2017-08-01

Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery. This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified. Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE) <15 mm and female gender. Over a mean follow-up of 2.8±2.6 years, 46 patients died. Baseline predictors of total mortality were NYHA class ≥ III, TAPSE <15 mm and non-elective surgery. The number of sternotomies was not independently associated with neither early nor total mortality. At 10 years, probability of survival was 94%. NYHA class among survivors was significantly improved, compared with baseline. Contemporary cardiac surgery for ACHD performed at a single, tertiary reference centre with a multidisciplinary approach is associated with low mortality and improved functional status. Also, our findings emphasise the point that surgery should not be delayed because of reluctance to reoperate only. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Pediatric and neonatal cardiovascular pharmacology.

PubMed

Miller-Hoover, Suzan R

2003-01-01

Advances in cardiology, surgical techniques, postoperative care, and medications have improved the chances of long-term survival of the neonatal and pediatric patient with complex congenital cardiac anomalies. Rather than undergoing palliative repair, these children are now frequently taken to the operating room for complete repair. As complete repair becomes the norm, collaborative management and a thorough understanding of the pre and postoperative medications used become essential to the care of these patients. The nurse's ability to understand preop, postop, and management medications is enhanced by an understanding of the principles of cardiac anatomy and physiology, as well as developmental changes in cardiac function. All of these are reviewed. In addition, since the safe and effective administration of these drugs depends on the pediatric intensive care unit (PICU) and neonatal intensive care unit (NICU) nurse's thorough knowledge of these medications and their effects on the cardiovascular system, a brief review of these medications is presented. While new technology and techniques are improving survival rates for children with congenital heart anomalies, it is the postoperative care that these children receive that enhances the patient's survival even more.

Induced apnea enhances image quality and visualization of cardiopulmonary anatomic during contrastenhanced cardiac computerized tomographic angiography in children

PubMed Central

Chakravarthy, Murali; Sunilkumar, Gubbihalli; Pargaonkar, Sumant; Hosur, Rajathadri; Harivelam, Chidananda; Kavaraganahalli, Deepak; Srinivasan, Pradeep

2015-01-01

Objective: The purpose of our study was to determine the effect of induced apnea on quality of cardiopulmonary structures during computerized tomographic (CT) angiography images in children with congenital heart diseases. Methods: Pediatric patients with congenital heart defects undergoing cardiac CT angiography at our facility in the past 3 years participated in this study. The earlier patients underwent cardiac CT angiography without induced apnea and while, later, apnea was induced in patients, which was followed by electrocardiogram gated cardiac CT angiography. General anesthesia was induced using sleep dose of intravenous propofol. After the initial check CT, on request by the radiologist, apnea was induced by the anesthesiologist by administering 1 mg/kg of intravenous suxamethonium. Soon after apnea ensued, the contrast was injected, and CT angiogram carried out. CT images in the “apnea group” were compared with those in “nonapnea group.” After the completion of the procedure, the patients were mask ventilated with 100% oxygen till the spontaneous ventilation was restored. Results: We studied 46 patients, of whom 36 with apnea and yet another 10 without. The quality of the image, visualization of structures such as cardiac wall, outflow tracts, lung field, aortopulmonary shunts, and coronary arteries were analyzed and subjected to statistical analysis (Mann–Whitney U, Fischer's exact test and Pearson's Chi-square test). In the induced apnea group, overall image quality was considered excellent in 89% (n = 33) of the studies, while in the “no apnea group,” only 30% of studies were excellent. Absent or minimal motion artifacts were seen in a majority of the studies in apnea group (94%). In the nonapnea group, the respiratory and body motion artifacts were severe in 50%, moderate in 30%, and minimal in 20%, but they were significantly lesser in the apnea group. All the studied parameters were statistically significant in the apnea group in contrast to nonapnea group (P < 0.000). Conclusion: The image quality of cardiac CT angiography greatly improves, and motion artifact significantly decreases with the use of induced apnea in pediatrics patients being evaluated for congenital heart disease. This technique poses no additional morbidity of significance. PMID:25849686

Utilisation of three-dimensional printed heart models for operative planning of complex congenital heart defects.

PubMed

Olejník, Peter; Nosal, Matej; Havran, Tomas; Furdova, Adriana; Cizmar, Maros; Slabej, Michal; Thurzo, Andrej; Vitovic, Pavol; Klvac, Martin; Acel, Tibor; Masura, Jozef

2017-01-01

To evaluate the accuracy of the three-dimensional (3D) printing of cardiovascular structures. To explore whether utilisation of 3D printed heart replicas can improve surgical and catheter interventional planning in patients with complex congenital heart defects. Between December 2014 and November 2015 we fabricated eight cardiovascular models based on computed tomography data in patients with complex spatial anatomical relationships of cardiovascular structures. A Bland-Altman analysis was used to assess the accuracy of 3D printing by comparing dimension measurements at analogous anatomical locations between the printed models and digital imagery data, as well as between printed models and in vivo surgical findings. The contribution of 3D printed heart models for perioperative planning improvement was evaluated in the four most representative patients. Bland-Altman analysis confirmed the high accuracy of 3D cardiovascular printing. Each printed model offered an improved spatial anatomical orientation of cardiovascular structures. Current 3D printers can produce authentic copies of patients` cardiovascular systems from computed tomography data. The use of 3D printed models can facilitate surgical or catheter interventional procedures in patients with complex congenital heart defects due to better preoperative planning and intraoperative orientation.

Uses and limitations of registry and academic databases.

PubMed

Williams, William G

2010-01-01

A database is simply a structured collection of information. A clinical database may be a Registry (a limited amount of data for every patient undergoing heart surgery) or Academic (an organized and extensive dataset of an inception cohort of carefully selected subset of patients). A registry and an academic database have different purposes and cost. The data to be collected for a database is defined by its purpose and the output reports required for achieving that purpose. A Registry's purpose is to ensure quality care, an Academic Database, to discover new knowledge through research. A database is only as good as the data it contains. Database personnel must be exceptionally committed and supported by clinical faculty. A system to routinely validate and verify data integrity is essential to ensure database utility. Frequent use of the database improves its accuracy. For congenital heart surgeons, routine use of a Registry Database is an essential component of clinical practice. Copyright (c) 2010 Elsevier Inc. All rights reserved.

Infundibular pulmonic stenosis with intact ventricular septum: a report of 15 surgically corrected patients.

PubMed

Shyu, K G; Tseng, C D; Chiu, I S; Hung, C R; Chu, S H; Lue, H C; Tseng, Y Z; Lien, W P

1993-09-01

Infundibular pulmonic stenosis with intact ventricular septum of primary origin is an uncommon condition. We report 15 such patients (nine males and six females, aged 7-36 years) who had undergone surgical correction for the anomaly during the period between 1975 and 1992. The occurrence of this clinical setting represents 0.19% (15/7826) of all cardiac operations and 0.46% (15/3222) of congenital heart diseases undergoing surgical correction during that period of time. The lesion was of discrete fibromuscular hypertrophy of the infundibulum in all 15 patients. The presenting symptoms of most patients were exertional dyspnea and syncope; however, five patients with severe obstruction were asymptomatic. The peak systolic pressure gradient across the infundibulum ranged from 71 to 230 mmHg. There was only one operative death; the remainder had remained well following the surgery over a mean follow-up period of 35 months. Surgical correction for infundibular pulmonic stenosis is rewarding in the absence of heart failure.

Lifestyle Habits among Adult Congenital Heart Disease Patients in Malta.

PubMed

Caruana, Maryanne; Grech, Victor

2016-07-01

Adult congenital heart disease patients are prone to health risk behaviors like their peers. Such behaviors can have serious implications, particularly in patients with complex congenital lesions. We investigated patterns in tobacco smoking, alcohol consumption, substance misuse, dental reviews, and physical activity in Maltese patients. A questionnaire modeled on the 2008 European Health Interview Survey was offered to consecutive adult congenital heart disease outpatients, aged 16 years and over, between May 2013 and May 2014. Foreigners and patients with learning difficulties or cognitive impairment were excluded. Data on these habits was compared with that from 372 age-matched and sex-matched 2008 survey responders (general population cohort). There were 125 patient responders (65 males; mean age 30.64, SD 12.80 years). Adult congenital heart disease patients smoked less (patients 18.4%, general 32.0% P = .004) and had more regular dental reviews (patients 60.9%, general 45.2% P = .003) but were less physically active than the general population cohort (patients 30.6%, general 62.9% P < .001). The difference in smoking was only significant in patients born after the mid-1970s (younger) and females, while dental reviews were only better in younger and male patients. Male patients consumed alcohol more frequently (38.1%) than general population males (23.3%, P = .02). In the patient cohort, smoking was more likely with milder congenital lesions (OR 4.689, P = .007), substance misuse more likely in males (OR 5.703, P = .036) and with milder lesions (OR 5.405, P = .030) and alcohol consumption more likely in males (OR 11.697, P < .001). Although there is an overall lower uptake of some unhealthy habits by Maltese adult congenital heart disease patients, this is not consistent between sexes or age groups. Male and older patients and those with milder lesions might need more reinforcement of the advice given regarding lifestyle habits. All patients need targeted exercise prescription to ensure better physical activity levels. © 2016 Wiley Periodicals, Inc.

Providing Guidance for School Personnel Making Decisions in the Service of School Children with Congenital Heart Disease

ERIC Educational Resources Information Center

Roberts, Jillian; MacMath, Sheryl

2006-01-01

Due to improved medical procedures, more and more children with congenital heart disease are entering the school system. In order to help both school and health professionals involved in the education of children, we provide a brief review of the literature, review real-life dilemmas that school personnel face on a daily basis, and interpret the…

A tribute to francis fontan, MD, and guillermo kreutzer, MD.

PubMed

Mavroudis, Constantine

2012-04-01

At the Third Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery in June 2011 in Istanbul, Turkey, Professor Francis Fontan and Dr Guillermo Kreutzer were honored for their contributions to the world of pediatric and congenital heart surgery. The presentation included a video of each physician being interviewed about their clinical and historical experiences. The presentation and videos are summarized herein.

Life and Death of a Child with Down Syndrome and a Congenital Heart Condition: Experiences of Six Couples

ERIC Educational Resources Information Center

Reilly, Deirdre; Huws, Jaci; Hastings, Richard; Vaughan, Frances

2010-01-01

Individuals with Down syndrome are at increased risk of congenital heart conditions (CHCs), and mortality is higher in people with Down syndrome and a CHC than those without (J. C. Vis et al., 2009). As a consequence, parents of children with Down syndrome and a CHC are more likely to outlive their child. In this research, semistructured…

A review of the economics of adult congenital heart disease.

PubMed

Seckeler, Michael D; Thomas, Ian D; Andrews, Jennifer; Joiner, Keith; Klewer, Scott E

2016-01-01

Adults living with congenital heart disease (CHD) now outnumber children with the disease. Thanks to medical advances over the past 75 years, many of these fatal childhood heart problems have changed to chronic medical conditions. As the population of adults with CHD increases, they will require increasingly complex medical, surgical and catheter-based therapies. In addition, social burdens including education, employment and insurability, which increase the societal costs of adult CHD, are now being recognized for adults living with CHD. This review summarizes the available literature on the economics of adult CHD.

Early postoperative physical therapy for improving short-term gross motor outcome in infants with cyanotic and acyanotic congenital heart disease.

PubMed

Haseba, Sumihito; Sakakima, Harutoshi; Nakao, Syuhei; Ohira, Misaki; Yanagi, Shigefumi; Imoto, Yutaka; Yoshida, Akira; Shimodozono, Megumi

2018-07-01

We analysed the gross motor recovery of infants and toddlers with cyanotic and acyanotic congenital heart disease (CHD) who received early postoperative physical therapy to see whether there was any difference in the duration to recovery. This study retrospectively evaluated the influence of early physical therapy on postoperative gross motor outcomes of patients with CHD. The gross motor ability of patients with cyanotic (n = 25, average age: 376.4 days) and acyanotic (n = 26, average age: 164.5 days) CHD was evaluated using our newly developed nine-grade mobility assessment scale. Physical therapy was started at an average of five days after surgery, during which each patient's gross motor ability was significantly decreased compared with the preoperative level. Patients (who received early postoperative physical therapy) with cyanotic (88.0%) and acyanotic CHD (96.2%) showed improved preoperative mobility grades by the time of hospital discharge. However, patients with cyanotic CHD had a significantly prolonged recovery period compared to those with acyanotic CHD (p < .01). The postoperative recovery period to preoperative mobility grade was significantly correlated with pre-, intra-, and postoperative factors. Our findings suggested that infants with cyanotic CHD are likely at a greater risk of gross motor delays, the recovery of which might differ between infants with cyanotic and acyanotic CHD after cardiac surgery. Early postoperative physical therapy promotes gross motor recovery. Implications of Rehabilitation Infants and toddlers with cyanotic congenital heart disease are likely at greater risk of gross motor delays and have a prolonged recovery period of gross motor ability compared to those with acyanotic congenital heart disease. Early postoperative physical therapy for patients with congenital heart disease after cardiac surgery promoted gross motor recovery. The postoperative recovery period to preoperative mobility grade was affected by pre-, intra-, and postoperative factors. Rehabilitation experts should consider the risk of gross motor delays of patients with congenital heart disease after cardiac surgery and the early postoperative physical therapy to promote their gross motor recovery.

Double aortic arch

MedlinePlus

Aortic arch anomaly; Double arch; Congenital heart defect - double aortic arch; Birth defect heart - double aortic arch ... aorta is a single arch that leaves the heart and moves leftward. In double aortic arch, some ...

Cyanotic congenital heart disease and atherosclerosis.

PubMed

Tarp, Julie Bjerre; Jensen, Annette Schophuus; Engstrøm, Thomas; Holstein-Rathlou, Niels-Henrik; Søndergaard, Lars

2017-06-01

Improved treatment options in paediatric cardiology and congenital heart surgery have resulted in an ageing population of patients with cyanotic congenital heart disease (CCHD). The risk of acquired heart disease such as atherosclerosis increases with age.Previous studies have speculated whether patients with CCHD are protected against atherosclerosis. Results have shown that the coronary arteries of patients with CCHD are free from plaques and stenosis. Decreased carotid intima-media thickness and low total plasma cholesterol may indicate a reduced risk of later development of atherosclerosis. However, the evidence is still sparse and questionable, and a reasonable explanation for the decreased risk of developing atherosclerosis in patients with CCHD is still missing.This review provides an overview of what is known about the prevalence and potential causes of the reduced risk of atherosclerosis in patients with CCHD. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Cleft Palate, Retrognathia and Congenital Heart Disease in Velo-Cardio-Facial Syndrome: A Phenotype Correlation Study

PubMed Central

Friedman, Marcia A.; Miletta, Nathanial; Roe, Cheryl; Wang, Dongliang; Morrow, Bernice E.; Kates, Wendy R.; Higgins, Anne Marie; Shprintzen, Robert J.

2011-01-01

Objective Velo-cardio-facial syndrome (VCFS) is caused by a microdeletion of approximately 40 genes from one copy of chromosome 22. Expression of the syndrome is a variable combination of over 190 phenotypic characteristics. As of yet, little is known about how these phenotypes correlate with one another or whether there are predictable patterns of expression. Two of the most common phenotypic categories, congenital heart disease and cleft palate, have been proposed to have a common genetic relationship to the deleted T-box 1 gene (TBX1). The purpose of this study is to determine if congenital heart disease and cleft palate are correlated in a large cohort of human subjects with VCFS. Methods This study is a retrospective chart review including 316 Caucasian non-Hispanic subjects with FISH or CGH microarray confirmed chromosome 22q11.2 deletions. All subjects were evaluated by the interdisciplinary team at the Velo-Cardio-Facial Syndrome International Center at Upstate Medical University, Syracuse, NY. Each combination of congenital heart disease, cleft palates, and retrognathia was analyzed by chi square or Fisher exact test. Results For all categories of congenital heart disease and cleft palate or retrognathia no significant associations were found, with the exception of submucous cleft palate and retrognathia (nominal p=0.0325) and occult submucous cleft palate and retrognathia (nominal p=0.000013). Conclusions Congenital heart disease and cleft palate do not appear to be correlated in human subjects with VCFS despite earlier suggestions from animal models. Possible explanations include modification of the effect of TBX1 by genes outside of the 22q11.2 region that may further influence the formation of the palate or heart, or the presence of epigenetic factors that may effect genes within the deleted region, modifying genes elsewhere, or polymorphisms on the normal copy of chromosome 22. Lastly, it is possible that TBX1 plays a role in palate formation in some species, but not in humans. In VCFS, retrognathia is caused by an obtuse angulation of the skull base. It is unknown if the correlation between retrognathia and cleft palate in VCFS indicates a developmental sequence related to skull morphology, or direct gene effects of both anomalies. Much work remains to be done to fully understand the complex relationships between phenotypic characteristics in VCFS. PMID:21763005

Cleft palate, retrognathia and congenital heart disease in velo-cardio-facial syndrome: a phenotype correlation study.

PubMed

Friedman, Marcia A; Miletta, Nathanial; Roe, Cheryl; Wang, Dongliang; Morrow, Bernice E; Kates, Wendy R; Higgins, Anne Marie; Shprintzen, Robert J

2011-09-01

Velo-cardio-facial syndrome (VCFS) is caused by a microdeletion of approximately 40 genes from one copy of chromosome 22. Expression of the syndrome is a variable combination of over 190 phenotypic characteristics. As of yet, little is known about how these phenotypes correlate with one another or whether there are predictable patterns of expression. Two of the most common phenotypic categories, congenital heart disease and cleft palate, have been proposed to have a common genetic relationship to the deleted T-box 1 gene (TBX1). The purpose of this study is to determine if congenital heart disease and cleft palate are correlated in a large cohort of human subjects with VCFS. This study is a retrospective chart review including 316 Caucasian non-Hispanic subjects with FISH or CGH microarray confirmed chromosome 22q11.2 deletions. All subjects were evaluated by the interdisciplinary team at the Velo-Cardio-Facial Syndrome International Center at Upstate Medical University, Syracuse, NY. Each combination of congenital heart disease, cleft palates, and retrognathia was analyzed by Chi square or Fisher exact test. For all categories of congenital heart disease and cleft palate or retrognathia no significant associations were found, with the exception of submucous cleft palate and retrognathia (nominal p=0.0325) and occult submucous cleft palate and retrognathia (nominal p=0.000013). Congenital heart disease and cleft palate do not appear to be correlated in human subjects with VCFS despite earlier suggestions from animal models. Possible explanations include modification of the effect of TBX1 by genes outside of the 22q11.2 region that may further influence the formation of the palate or heart, or the presence of epigenetic factors that may effect genes within the deleted region, modifying genes elsewhere, or polymorphisms on the normal copy of chromosome 22. Lastly, it is possible that TBX1 plays a role in palate formation in some species, but not in humans. In VCFS, retrognathia is caused by an obtuse angulation of the skull base. It is unknown if the correlation between retrognathia and cleft palate in VCFS indicates a developmental sequence related to skull morphology, or direct gene effects of both anomalies. Much work remains to be done to fully understand the complex relationships between phenotypic characteristics in VCFS. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Effect of maternal age and cardiac disease severity on outcome of pregnancy in women with congenital heart disease.

PubMed

Furenäs, Eva; Eriksson, Peter; Wennerholm, Ulla-Britt; Dellborg, Mikael

2017-09-15

There is an increasing prevalence of women with congenital heart defects reaching childbearing age. In western countries women tend to give birth at a higher age compared to some decades ago. We evaluated the CARdiac disease in PREGnancy (CARPREG) and modified World Health Organization (mWHO) risk classifications for cardiac complications during pregnancies in women with congenital heart defects and analyzed the impact of age on risk of obstetric and fetal outcome. A single-center observational study of cardiac, obstetric, and neonatal complications with data from cardiac and obstetric records of pregnancies in women with congenital heart disease. Outcomes of 496 pregnancies in 232 women, including induced abortion, miscarriage, stillbirth, and live birth were analyzed regarding complications, maternal age, mode of delivery, and two risk classifications: CARPREG and mWHO. There were 28 induced abortions, 59 fetal loss, 409 deliveries with 412 neonates. Cardiac (14%), obstetric (14%), and neonatal (15%) complications were noted, including one maternal death and five stillbirths. The rate of cesarean section was 19%. Age above 35years was of borderline importance for cardiac complications (p=0.054) and was not a significant additional risk factor for obstetric or neonatal complications. Both risk classifications had moderate clinical utility, with area under the curve (AUC) 0.71 for CARPREG and 0.65 for mWHO on cardiac complications. Pregnancy complications in women with congenital heart disease are common but severe complications are rare. Advanced maternal age does not seem to affect complication rate. Existing risk classification systems are insufficient in predicting complications. Copyright © 2017 Elsevier B.V. All rights reserved.

[Value of indoleamine 2,3-dioxygenase in diagnosis of systemic inflammatory response syndrome after cardiopulmonary bypass in children with congenital heart disease].

PubMed

Wang, Xin; Li, Zong-Xiao; Wen, Yu-Peng; Chang, Cheng

2018-01-01

To study the value of indoleamine 2,3-dioxygenase (IDO) in the early diagnosis of systemic inflammatory response syndrome (SIRS) after cardiopulmonary bypass in children with congenital heart disease. A total of 90 children with congenital heart disease who underwent cardiopumonary bypass surgery between May 2012 and January 2016 were enrolled. According to the prsence or absence of SIRS after surgery, they were divided into SIRS group (n=43) and control group (n=47). Peripheral blood samples were collected before surgery, during surgery, and after surgery. Serum levels of IDO, C-reactive protein (CRP), and interleukin-6 (IL-6) were measured and compared between the two groups. The receiver operating characteristic (ROC) curve was used to evaluate their diagnostic efficiency. Compared with the control group, the SIRS group had higher serum CRP levels at 72 hours after surgery, higher IL-6 levels during surgery and at 72 hours after surgery, and higher IDO levels at 24 and 72 hours after surgery. IDO had a certain value in the diagnosis of SIRS at 24 hours after surgery with an area under the ROC curve (AUC) of 0.793, a specificity of 100%, and a sensitivity of 58.14%. CRP, IL-6, and IDO had a certain value in the diagnosis of SIRS at 72 hours after surgery. IDO had the highest diagnostic efficiency with an AUC of 0.927, a specificity of 95.74%, and a sensitivity of 76.74% at 72 hours after surgery. IL-6, CRP, and IDO have a certain value in the diagnosis of SIRS after surgery for congenital heart disease, and IDO has a higher diagnostic efficiency. IDO can predict the development of SIRS in children after surgery for congenital heart disease earlier.

Overweight and obesity in children with congenital heart disease: combination of risks for the future?

PubMed

Barbiero, Sandra Mari; D'Azevedo Sica, Caroline; Schuh, Daniela Schneid; Cesa, Claudia Ciceri; de Oliveira Petkowicz, Rosemary; Pellanda, Lucia Campos

2014-10-16

Children who have unhealthy lifestyles are predisposed to develop hypertension, dyslipidemia and other complications. The epidemic of obesity is also affecting children with congenital heart disease. The aim of this study is to estimate the prevalence of obesity and describe associated risk factors, including family history in children with congenital heart disease. A cross-sectional study with 316 children and adolescents with congenital heart disease seen in an outpatient clinic of a reference hospital. Collected sociodemographic data included family history of chronic disease, dietary habits, laboratory tests (total cholesterol, HDL and LDL/cholesterol, triglycerides, fasting glucose, CRP, hematocrit and hemoglobin), and anthropometric assessment. Anthropometric data of the caregivers was self-reported. The prevalence of excess weight was 26.9%. Altered levels of total cholesterol were observed in 46.9%, of HDL in 32.7%, LDL in 23.6% and of triglycerides levels in 20.0%. A higher frequency of family history of obesity (42.6%; p = 0.001), dyslipidemia (48.1%; p = <0.001), diabetes (47.4%; p = 0.002), hypertension (39.2%; p = 0.006) and ischemic disease (43.7%; p = 0.023), as well as significantly higher values of triglycerides (p = 0.017), glycemia (p = 0.004) and C-reactive protein (p = 0.002) were observed among patients with excess weight. The presence of modifiable risk factors and the variables associated to excess weight in this population was similar to that described in the literature for children without congenital disease. As these children already present the risks associated to heart disease, it is particularly important to promote a healthy lifestyle in this group.

Effects of eHealth physical activity encouragement in adolescents with complex congenital heart disease: The PReVaiL randomized clinical trial.

PubMed

Klausen, Susanne Hwiid; Andersen, Lars L; Søndergaard, Lars; Jakobsen, Janus Christian; Zoffmann, Vibeke; Dideriksen, Kasper; Kruse, Anne; Mikkelsen, Ulla Ramer; Wetterslev, Jørn

2016-10-15

To assess benefit and harms of adding an eHealth intervention to health education and individual counseling in adolescents with congenital heart disease. Randomized clinical trial. Denmark. A total of 158 adolescents aged 13-16years with no physical activity restrictions after repaired complex congenital heart disease. PReVaiL consisted of individually tailored eHealth encouragement physical activity for 52weeks. All patients received 45min of group-based health education and 15min of individual counseling involving patients' parents. The primary outcome was maximal oxygen uptake (VO2 peak) at 52weeks after randomization. The secondary outcome was physical activity. Exploratory outcomes were generic and disease-specific questionnaires. In the intervention group, 58 patients (72%) completed the final test, but of those, only 46 (57%) fulfilled the compliance criteria of using the eHealth application for at least 2 consecutive weeks. In the control group, 61 patients (79%) completed both exercise tests. Adjusted for baseline values, the difference between the intervention group and the control group in mean VO2 peak at 1year was -0.65ml·kg(-1)·min(-1) (95% CI -2.66 to 1.36). Between-group differences at 1year in physical activity, generic health-related quality of life, and disease-specific quality of life were not statistically significant. Adding a tailored eHealth intervention to health education and individual counseling did not affect outcomes among adolescents with congenital heart disease. Our results do not support the use of this eHealth intervention in adolescents with complex congenital heart disease. Clinical trials.gov identifier: NCT01189981. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Experiences and Outcomes of Transition from Pediatric to Adult Health Care Services for Young People with Congenital Heart Disease: A Systematic Review.

PubMed

Heery, Emily; Sheehan, Aisling M; While, Alison E; Coyne, Imelda

2015-01-01

This review synthesizes the empirical literature on outcomes and experiences of transfer and transition from pediatric to adult care for young people with congenital heart disease. A systematic review of papers published between January 2001 and May 2013 that examined outcomes or experiences of transfer and transition among young people with congenital heart disease was conducted. Data were extracted by two independent reviewers with the outcomes data combined using narrative synthesis and the experiences data integrated using thematic synthesis. Thirteen papers were included in the review: six reported outcomes following transfer, six reported experiences of transfer and transition, and one reported both outcomes and experiences. The review data indicate that high proportions of young people were lost to follow-up or experienced long gaps in care after leaving pediatric cardiology. Factors that protected against loss to follow-up or lapse in care included: beliefs that specialized adult care was necessary; poorer health status; attendance at pediatric appointments without parents; and pediatric referral to an adult congenital heart disease center. Data on experiences highlighted that many young people were unconcerned about transition, but lacked knowledge about their condition and were insufficiently prepared for transfer. In terms of adult services, many young people desired continuity in the quality of care, youth-oriented facilities, a personalized approach, and for their parents to remain involved in their care, but in a secondary, supportive capacity. In conclusion, the high proportions of young people lost to follow-up highlight the need for formal transition programs, which ensure a planned and coordinated transfer. Patients with congenital heart disease need education throughout adolescence about the implications of their condition, the differences between pediatric and adult services, and self-care management. © 2015 Wiley Periodicals, Inc.

Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI).

PubMed

Lopes, Antonio Augusto; Barst, Robyn J; Haworth, Sheila Glennis; Rabinovitch, Marlene; Al Dabbagh, Maha; Del Cerro, Maria Jesus; Ivy, Dunbar; Kashour, Tarek; Kumar, Krishna; Harikrishnan, S; D'Alto, Michele; Thomaz, Ana Maria; Zorzanelli, Leína; Aiello, Vera D; Mocumbi, Ana Olga; Santana, Maria Virginia T; Galal, Ahmed Nasser; Banjar, Hanaa; Tamimi, Omar; Heath, Alexandra; Flores, Patricia C; Diaz, Gabriel; Sandoval, Julio; Kothari, Shyam; Moledina, Shahin; Gonçalves, Rilvani C; Barreto, Alessandra C; Binotto, Maria Angélica; Maia, Margarida; Al Habshan, Fahad; Adatia, Ian

2014-06-01

Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

[Pulmonary hypertension associated with congenital heart disease and Eisenmenger syndrome].

PubMed

Calderón-Colmenero, Juan; Sandoval Zárate, Julio; Beltrán Gámez, Miguel

2015-01-01

Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis. The progressive rise in pulmonary vascular resistance and increased pressures in the right heart provocated reversal of the shunt may arise with the development of Eisenmenger' syndrome the most advanced form de Pulmonary arterial hypertension associated with congenital heart disease. The prevalence of Pulmonary arterial hypertension associated with CHD has fallen in developed countries in recent years that is not yet achieved in developing countries therefore diagnosed late as lack of hospital infrastructure and human resources for the care of patients with CHD. With the development of targeted medical treatments for pulmonary arterial hypertension, the concept of a combined medical and interventional/surgical approach for patients with Pulmonary arterial hypertension associated with CHD is a reality. We need to know the pathophysiological factors involved as well as a careful evaluation to determine the best therapeutic strategy. Copyright © 2014 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

Dynamic three-dimensional display of common congenital cardiac defects from reconstruction of two-dimensional echocardiographic images.

PubMed

Hsieh, K S; Lin, C C; Liu, W S; Chen, F L

1996-01-01

Two-dimensional echocardiography had long been a standard diagnostic modality for congenital heart disease. Further attempts of three-dimensional reconstruction using two-dimensional echocardiographic images to visualize stereotypic structure of cardiac lesions have been successful only recently. So far only very few studies have been done to display three-dimensional anatomy of the heart through two-dimensional image acquisition because such complex procedures were involved. This study introduced a recently developed image acquisition and processing system for dynamic three-dimensional visualization of various congenital cardiac lesions. From December 1994 to April 1995, 35 cases were selected in the Echo Laboratory here from about 3000 Echo examinations completed. Each image was acquired on-line with specially designed high resolution image grazmber with EKG and respiratory gating technique. Off-line image processing using a window-architectured interactive software package includes construction of 2-D ehcocardiographic pixel to 3-D "voxel" with conversion of orthogonal to rotatory axial system, interpolation, extraction of region of interest, segmentation, shading and, finally, 3D rendering. Three-dimensional anatomy of various congenital cardiac defects was shown, including four cases with ventricular septal defects, two cases with atrial septal defects, and two cases with aortic stenosis. Dynamic reconstruction of a "beating heart" is recorded as vedio tape with video interface. The potential application of 3D display of the reconstruction from 2D echocardiographic images for the diagnosis of various congenital heart defects has been shown. The 3D display was able to improve the diagnostic ability of echocardiography, and clear-cut display of the various congenital cardiac defects and vavular stenosis could be demonstrated. Reinforcement of current techniques will expand future application of 3D display of conventional 2D images.

Infant health after heart surgery.

PubMed

2016-11-08

Background The number of infants who survive initial surgery for complex congenital heart disease (CHD), such as hypoplastic left heart syndrome, is increasing, but they are often left with residual complex health needs.

Normal ranges for fetal electrocardiogram values for the healthy fetus of 18-24 weeks of gestation: a prospective cohort study.

PubMed

Verdurmen, Kim M J; Lempersz, Carlijn; Vullings, Rik; Schroer, Christian; Delhaas, Tammo; van Laar, Judith O E H; Oei, S Guid

2016-08-17

The fetal anomaly ultrasound only detects 65 to 81 % of the patients with congenital heart disease, making it the most common structural fetal anomaly of which a significant part is missed during prenatal life. Therefore, we need a reliable non-invasive diagnostic method which improves the predictive value for congenital heart diseases early in pregnancy. Fetal electrocardiography could be this desired diagnostic method. There are multiple technical challenges to overcome in the conduction of the fetal electrocardiogram. In addition, interpretation is difficult due to the organisation of the fetal circulation in utero. We want to establish the normal ranges and values of the fetal electrocardiogram parameters in healthy fetuses of 18 to 24 weeks of gestation. Women with an uneventful singleton pregnancy between 18 and 24 weeks of gestation are asked to participate in this prospective cohort study. A certified and experienced sonographist performs the fetal anomaly scan. Subsequently, a fetal electrocardiogram recording is performed using dedicated signal processing methods. Measurements are performed at two institutes. We will include 300 participants to determine the normal values and 95 % confidence intervals of the fetal electrocardiogram parameters in a healthy fetus. We will evaluate the fetal heart rate, segment intervals, normalised amplitude and the fetal heart axis. Three months postpartum, we will evaluate if a newborn is healthy through a questionnaire. Fetal electrocardiography could be a promising tool in the screening program for congenital heart diseases. The electrocardiogram is a depiction of the intimate relationship between the cardiac nerve conduction pathways and the structural morphology of the fetal heart, and therefore particularly suitable for the detection of secondary effects due to a congenital heart disease (hypotrophy, hypertrophy and conduction interruption).

Clinical implications of eicosapentaenoic acid/arachidonic acid ratio (EPA/AA) in adult patients with congenital heart disease.

PubMed

Kanoh, Miki; Inai, Kei; Shinohara, Tokuko; Tomimatsu, Hirofumi; Nakanishi, Toshio

2017-12-01

Recent studies showed that a low ratio between the levels of eicosapentaenoic acid and those of arachidonic acid (EPA/AA) is associated with higher incidence of coronary artery disease and poor prognosis of heart failure, arrhythmia, and cardiac sudden death. However, the clinical implications of EPA/AA in adult patients with congenital heart disease remain unclear. We aimed to assess the prognostic value of EPA/AA regarding cardiac events in adult patients with congenital heart disease. We measured the serum levels of eicosapentaenoic acid and arachidonic acid in 130 adult patients (median age, 31 years) stratified into two groups according to their EPA/AA (low, ≤0.22; high, >0.22). We prospectively analyzed the association between EPA/AA and incidence of cardiac events during a mean observation period of 15 months, expressed in terms of hazard ratio (HR) with 95% confidence interval (95% CI). In the subgroup of patients with biventricular circulation (2VC) (n = 76), we analyzed the same clinical endpoints. In our study population, EPA/AA was not associated with the incidence of arrhythmic events (HR, 1.52; 95% CI, 0.82-2.85; p = 0.19), but low EPA/AA was a predictor of heart failure hospitalization (HR, 2.83; 95% CI, 1.35-6.30; p < 0.01). Among patients with 2VC, an EPA/AA of ≤0.25 was associated with a significantly higher risk of arrhythmic events (HR, 2.55; 95% CI, 1.11-6.41; p = 0.03) and heart failure hospitalization (HR, 5.20; 95% CI, 1.78-18.1; p < 0.01). EPA/AA represents a useful predictor of cardiac events in adult patients with congenital heart disease.

A Double-Blinded, Randomized, Placebo-Controlled Clinical Trial of Aminophylline to Prevent Acute Kidney Injury in Children Following Congenital Heart Surgery With Cardiopulmonary Bypass.

PubMed

Axelrod, David M; Sutherland, Scott M; Anglemyer, Andrew; Grimm, Paul C; Roth, Stephen J

2016-02-01

Acute kidney injury occurs commonly in children following congenital cardiac surgery with cardiopulmonary bypass and has been associated with increased morbidity and mortality. Aminophylline, a methylxanthine nonselective adenosine receptor antagonist, has been effective in the management of acute kidney injury in certain populations. This study sought to determine whether postoperative administration of aminophylline attenuates acute kidney injury in children undergoing congenital cardiac surgery with cardiopulmonary bypass. Single-center, double-blinded, placebo-controlled, randomized clinical trial. Tertiary center, pediatric cardiovascular ICU. A total of 144 children after congenital heart surgery with cardiopulmonary bypass. Seventy-two patients were randomized to receive aminophylline and 72 patients received placebo. Study drug was administered every 6 hours for 72 hours. The primary outcome variable was the development of any acute kidney injury, defined by the serum creatinine criteria of the Kidney Diseases: Improving Global Outcomes. Secondary outcomes included the development of severe acute kidney injury, time between cardiovascular ICU admission and first successful extubation, percent fluid overload, total fluid balance, urine output, bioelectrical impedance, and serum neutrophil gelatinase-associated lipocalin. The unadjusted rate and severity of acute kidney injury were not different between groups; 43 of 72 (60%) of the treatment group and 36 of 72 (50%) of the placebo group developed acute kidney injury (p = 0.32). Stage 2/3 acute kidney injury occurred in 23 of 72 (32%) of the treatment group and 15 of 72 (21%) of the placebo group (p = 0.18). Secondary outcome measures also demonstrated no significant difference between treatment and placebo groups. Aminophylline administration was safe; no deaths occurred in either group, and rates of adverse events were similar (14% in the treatment group vs 18% in the placebo group; p = 0.30). In this placebo-controlled randomized clinical trial, we found no effect of aminophylline to prevent acute kidney injury in children recovering from cardiac surgery performed with cardiopulmonary bypass. Future study of preoperative aminophylline administration to prevent acute kidney injury may be warranted.

Risk of congenital anomalies in relation to the uptake of trihalomethane from drinking water during pregnancy

PubMed Central

Grazuleviciene, Regina; Kapustinskiene, Violeta; Vencloviene, Jone; Buinauskiene, Jurate; Nieuwenhuijsen, Mark J

2013-01-01

Objectives Congenital anomalies have been inconsistently associated with maternal crude estimated exposure to drinking water trihalomethane (THM). We investigated the relationship between individual THM uptake during the first trimester of pregnancy and congenital anomalies. Methods We estimated maternal THM uptake for 3074 live births using residential tap water concentrations, drinking water ingestion, showering and bathing, and uptake factors of THM in the blood. Multiple logistic regression was used to investigate the association of THM exposure with congenital anomalies. Results We observed no statistically significant relationships between congenital anomalies and the total THM internal dose. We found little indication of a dose-response relationship for brominated THM and congenital heart anomalies. The relationship was statistically significant for bromodichloromethane (BDCM) (OR=2.16, 95% CI 1.05 to 4.46, highest vs lowest tertile) during the first month of pregnancy. During the first trimester of pregnancy, the probability of developing heart anomalies increased for every 0.1 μg/d increase in the BDCM and for every 0.01 μg/d increase in the internal dibromochloromethane (DBCM) dose (OR 1.70, 95% CI 1.09 to 2.66, and OR 1.25, 95% CI 1.01 to 1.54, respectively). A dose-response relationship was evident for musculoskeletal anomalies and DBCM exposure during the first and second months of pregnancy, while BDCM exposure tended to increase the risk of urogenital anomalies. Conclusions This study shows some evidence for an association between the internal dose of THM and the risk of congenital anomalies. In particular, increased prenatal exposure to brominated THM might increase the risk of congenital heart and musculoskeletal anomalies. PMID:23404756

Content validation of the infant malnutrition and feeding checklist for congenital heart disease: a tool to identify risk of malnutrition and feeding difficulties in infants with congenital heart disease.

PubMed

St Pierre, Astrid; Khattra, Parveen; Johnson, Mandy; Cender, Laurie; Manzano, Sandra; Holsti, Liisa

2010-10-01

Infants with congenital heart disease (CHD) have a high prevalence of feeding difficulties and malnutrition. Early intervention decreases morbidity and long-term developmental deficits. The purpose of this study was to develop and establish the content validity of a screening checklist to identify infants with CHD at risk of feeding difficulties or inadequate nutritional intake for timely referral to a feeding specialist or dietitian. The Delphi method was used, and expert participants reached consensus on 24 risk indicators. This study is the first step in establishing the validity and reliability of a screening tool for early intervention of feeding difficulties and inadequate nutritional intake in infants with CHD. Copyright © 2010 Elsevier Inc. All rights reserved.

IGF-I replacement therapy in children with congenital IGF-I deficiency (Laron syndrome) maintains heart dimension and function.

PubMed

Scheinowitz, Mickey; Feinberg, Micha S; Laron, Zvi

2009-06-01

Untreated patients with congenital growth hormone deficiency (GHD) and IGF-I deficiency are characterized not only by dwarfism but also by acromicria and organomicria, such as the heart. We assessed cardiac dimensions and function in very young patients with Laron syndrome (LS) undergoing IGF-I replacement therapy. Two to seven echocardiographic measurements were performed during IGF-I replacement therapy on male (n=4) and female (n=4) LS -patients, mean+/-SD age of 7.1+/-3.6 years (range 1.6-11.6 years), weight 16.1+/-9.7 kg, and height 89.9+/-18.5 cm. As aged- and gender-matched controls served 44 healthy children, age: 8.7+/-5.5 years, weight: 36.1+/-22.4 kg, and height: 129.7+/-33.1cm. Data of LS patients were normalized to body surface area and compared to the control group as well as nomograms of normal echocardiographic parameters for this age group. Left ventricular diastolic and systolic dimensions (LVDD/ LVSD, mm) and LV mass (gr) were significantly smaller in boys and girls with IGF-I treated LS compared with controls while the shortening fraction (%) and intraventricular septum thickness (mm) were similar. When compared with standard values for this age group, all treated LS patients were within 1 standard deviation of the mean. IGF-I therapy of young patients with Laron syndrome maintain LV dimensions and function within the normal range of aged-matched controls.

Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

PubMed

Giannakoulas, G; Vasiliadis, K; Frogoudaki, A; Ntellos, C; Tzifa, A; Brili, S; Manginas, A; Papaphylactou, M; Parcharidou, D; Kampouridis, N; Pitsis, A; Chamaidi, A; Kolios, M; Papadopoulos, G; Douras, A; Davlouros, P; Ntiloudi, D; Karvounis, H; Kalangos, A; Tsioufis, C; Rammos, S

2017-10-15

The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation. Copyright © 2017 Elsevier B.V. All rights reserved.

A Survey of Congenital Heart Disease and Other Organic Malformations Associated with Different Types of Orofacial Clefts in Eastern China

PubMed Central

Sun, Ting; Tian, Hua; Wang, Changqian; Yin, Ping; Zhu, Yaqin; Chen, Xianghua; Tang, Zhengde

2013-01-01

Background A high incidence of orofacial clefts is reported in China, but no data has shown the relation between cleft types and the incidence of other defects so far. The aim of this study is to assess the incidence of congenital heart diseases and other organic defects associated with different types of orofacial clefts. Methodology and Principal Findings All children with orofacial clefts, which were sought out from the Health Information System of Shanghai Ninth People's Hospital between 1st Jan 2009 and 30th Dec 2011, were enrolled in this study. All subjects underwent a thorough examination and grouped by the cleft phenotype. The numbers and types of other organic defects were recorded and analyzed statistically using SPSS 17.0. Of 2180 cases reported as having orofacial clefts, 657 (30.1%) had other congenital abnormalities, which were significantly more common in cleft palate (47.9% (329/687)) than that in cleft lip (10.6% (80/755)) or cleft lip and palate (33.6% (248/738)) (P<0.01). In subgroups, unilateral cleft lip and palate had a statistically higher incidence of associated abnormalities than bilateral cleft lip and palate (P<0.01). The most common malformation was congenital heart disease, which counted 45.1% (296/657) of all malformations. Disorders of the central nervous system (14.3%(94/657)) and Skeletal anomalies (13.1%(86/657)) were also frequently associated. Additionally, the most common defect in heart was atrial septal defect, which was 39.7% (118/296) of all congenital heart diseases. Conclusions and Significance As the high incidence of heart defects and other organic abnormalities in the children with cleft palate in Eastern China, special attention should be paid to them and echocardiography should be a proposed examination in the evaluation of children with cleft palate before any surgical correction being executed. PMID:23349958

A survey of congenital heart disease and other organic malformations associated with different types of orofacial clefts in Eastern China.

PubMed

Sun, Ting; Tian, Hua; Wang, Changqian; Yin, Ping; Zhu, Yaqin; Chen, Xianghua; Tang, Zhengde

2013-01-01

A high incidence of orofacial clefts is reported in China, but no data has shown the relation between cleft types and the incidence of other defects so far. The aim of this study is to assess the incidence of congenital heart diseases and other organic defects associated with different types of orofacial clefts. All children with orofacial clefts, which were sought out from the Health Information System of Shanghai Ninth People's Hospital between 1(st) Jan 2009 and 30(th) Dec 2011, were enrolled in this study. All subjects underwent a thorough examination and grouped by the cleft phenotype. The numbers and types of other organic defects were recorded and analyzed statistically using SPSS 17.0. Of 2180 cases reported as having orofacial clefts, 657 (30.1%) had other congenital abnormalities, which were significantly more common in cleft palate (47.9% (329/687)) than that in cleft lip (10.6% (80/755)) or cleft lip and palate (33.6% (248/738)) (P<0.01). In subgroups, unilateral cleft lip and palate had a statistically higher incidence of associated abnormalities than bilateral cleft lip and palate (P<0.01). The most common malformation was congenital heart disease, which counted 45.1% (296/657) of all malformations. Disorders of the central nervous system (14.3%(94/657)) and Skeletal anomalies (13.1%(86/657)) were also frequently associated. Additionally, the most common defect in heart was atrial septal defect, which was 39.7% (118/296) of all congenital heart diseases. As the high incidence of heart defects and other organic abnormalities in the children with cleft palate in Eastern China, special attention should be paid to them and echocardiography should be a proposed examination in the evaluation of children with cleft palate before any surgical correction being executed.

Validation of the grown-ups with congenital heart disease score.

PubMed

Hörer, Jürgen; Roussin, Régine; LeBret, Emanuel; Ly, Mohamed; Abdullah, Jarrah; Marzullo, Rafaella; Pabst von Ohain, Jelena; Belli, Emre

2018-06-01

Adults with congenital heart disease in need of heart surgery frequently present with significant comorbidity. Furthermore, additional technical difficulties often related to redo operations increase the risk for postoperative mortality and morbidity. Hence, next to the type of the procedure, additional procedure-dependent and procedure-independent factors have to be considered for risk evaluation. The recently proposed grown-ups with congenital heart disease (GUCH) mortality and morbidity scores account for these additional risk factors. We sought to validate their predictive power in a large population operated in a single centre. Data of all consecutive patients aged 18 years or more, who underwent surgery for congenital heart disease between 2005 and 2016, were collected. Mortality was defined as hospital mortality or mortality within 30 days following surgery. Morbidity was defined as occurrence of one or more of the following complications: renal failure requiring dialysis, neurologic deficit persisting at discharge, atrioventricular block requiring permanent pacemaker implantation, mechanical circulatory support, phrenic nerve injury and unplanned reoperation. The discriminatory power of the GUCH scores was assessed using the area under the receiver operating characteristics curve (c-index, including 95% CI). Eight hundred and twenty-four operations were evaluated. Additional procedure-dependent and procedure-independent factors, as defined in the GUCH scores, were present in 165 patients (20.0%) and 544 patients (66.0%), respectively. Hospital mortality and morbidity was 3.4% and 10.0%, respectively. C-index for GUCH mortality score was 0.809 (0.742-0.877). C-index for GUCH morbidity score was 0.676 (0.619-0.734). We could confirm the good predictive power of the GUCH mortality score for postoperative mortality in a large population of adults with congenital heart disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Search for Rare Copy-Number Variants in Congenital Heart Defects Identifies Novel Candidate Genes and a Potential Role for FOXC1 in Patients With Coarctation of the Aorta.

PubMed

Sanchez-Castro, Marta; Eldjouzi, Hadja; Charpentier, Eric; Busson, Pierre-François; Hauet, Quentin; Lindenbaum, Pierre; Delasalle-Guyomarch, Béatrice; Baudry, Adrien; Pichon, Olivier; Pascal, Cécile; Lefort, Bruno; Bajolle, Fanny; Pezard, Philippe; Schott, Jean-Jacques; Dina, Christian; Redon, Richard; Gournay, Véronique; Bonnet, Damien; Le Caignec, Cédric

2016-02-01

Congenital heart defects are the most frequent malformations among newborns and a frequent cause of morbidity and mortality. Although genetic variation contributes to congenital heart defects, their precise molecular bases remain unknown in the majority of patients. We analyzed, by high-resolution array comparative genomic hybridization, 316 children with sporadic, nonsyndromic congenital heart defects, including 76 coarctation of the aorta, 159 transposition of the great arteries, and 81 tetralogy of Fallot, as well as their unaffected parents. We identified by array comparative genomic hybridization, and validated by quantitative real-time polymerase chain reaction, 71 rare de novo (n=8) or inherited (n=63) copy-number variants (CNVs; 50 duplications and 21 deletions) in patients. We identified 113 candidate genes for congenital heart defects within these CNVs, including BTRC, CHRNB3, CSRP2BP, ERBB2, ERMARD, GLIS3, PLN, PTPRJ, RLN3, and TCTE3. No de novo CNVs were identified in patients with transposition of the great arteries in contrast to coarctation of the aorta and tetralogy of Fallot (P=0.002; Fisher exact test). A search for transcription factor binding sites showed that 93% of the rare CNVs identified in patients with coarctation of the aorta contained at least 1 gene with FOXC1-binding sites. This significant enrichment (P<0.0001; permutation test) was not observed for the CNVs identified in patients with transposition of the great arteries and tetralogy of Fallot. We hypothesize that these CNVs may alter the expression of genes regulated by FOXC1. Foxc1 belongs to the forkhead transcription factors family, which plays a critical role in cardiovascular development in mice. These data suggest that deregulation of FOXC1 or its downstream genes play a major role in the pathogenesis of coarctation of the aorta in humans. © 2015 American Heart Association, Inc.

Maximising the clinical use of exercise gaseous exchange testing in children with repaired cyanotic congenital heart defects: the development of an appropriate test strategy.

PubMed

McManus, A; Leung, M

2000-04-01

Implicit in deciding upon an exercise test strategy to elucidate cardiopulmonary function in children with congenital heart disease are appropriate application of gas exchange techniques and the significance of the data collected to the specific congenital heart disorder. Post-operative cardiopulmonary responses to exercise in cyanotic disorders are complex and, despite a large body of extant literature in paediatric patients, there has been much difficulty in achieving quality and consistency of data. Maximal oxygen uptake is widely recognised as the best single indicator of cardiopulmonary function and has therefore been the focus of most clinical exercise tests in children. Many children with various heart anomalies are able to exercise to maximum without adverse symptoms, and it is essential that test termination is based on the same criteria for these children. Choosing appropriate, valid indicators of maximum in children with congenital heart disease is beset by difficulties. Such maximal intensity exercise testing procedures have been challenged on the grounds that they do not give a good indication of cardiopulmonary function that is relevant to real life situations. Furthermore, they are prone to much interindividual variability and error in the definition of maximal exertion. Alternative strategies have been proposed which focus upon dynamic submaximal and kinetic cardiopulmonary responses, which are thought to be less dependent on maximal voluntary effort and more suited to the daily activity patterns of children. These methods are also not without problems. Variability in anaerobic threshold measurements and controversy regarding its physiological meaning have been debated. It is recommended that an appropriate cardiopulmonary exercise gas exchange test strategy, which provides clinically useful information for children with cyanotic congenital heart disease, should include both maximal and submaximal data. The inclusion of oxygen uptake kinetics and ventilatory data are encouraged, since they may allow the distinction between a pulmonary, cardiovascular or inactivity related exercise limitation.

Tachyarrhythmia in patients with congenital heart disease: inevitable destiny?

PubMed

Teuwen, C P; Taverne, Y J H J; Houck, C; Götte, M; Brundel, B J J M; Evertz, R; Witsenburg, M; Roos-Hesselink, J W; Bogers, A J J C; de Groot, N M S

2016-03-01

The prevalence of patients with congenital heart disease (CHD) has increased over the last century. As a result, the number of CHD patients presenting with late, postoperative tachyarrhythmias has increased as well. The aim of this review is to discuss the present knowledge on the mechanisms underlying both atrial and ventricular tachyarrhythmia in patients with CHD and the advantages and disadvantages of the currently available invasive treatment modalities.

Embolization of Uterine Arteriovenous Malformations Associated with Cyanotic Congenital Heart Disease

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wijesekera, N. T., E-mail: n.wijesekera@doctors.net.uk; Padley, S. P.; Kazmi, F.

2009-09-15

Uterine arteriovenous malformation (AVM) is a rare cause of vaginal bleeding and miscarriage. We report two cases of uterine AVMs in patients with a history of complex congenital heart disease, an association that has not been previously described. Both patients were treated by selective uterine artery embolization, a minimally invasive therapy that has revolutionized the management of uterine AVMs, thus offering an alternative to conventional hysterectomy.

Quality of life following paediatric heart transplant: are age and activity level factors?

PubMed

Parent, John J; Sterrett, Lauren; Caldwell, Randall; Darragh, Robert; Schamberger, Marcus; Murphy, Debbie; Ebenroth, Eric

2015-03-01

We evaluated whether quality of life correlates to age and activity in children following heart transplantation. In addition, quality of life in children following heart transplantation was compared with previously reported values in children with congenital heart disease. Quality of life remains an important aspect of therapy. The Pediatric Quality of Life Inventory Generic Core Scales and Cardiac Module were administered to 14 children who had previously undergone heart transplantation. Patients wore a pedometer for 7 days to assess daily activity. The age at assessment was 13.1±1.9 years. The patients were 7.1±5.7 years post heart transplantation. There was a negative correlation between age at first heart transplantation and emotional (r=-0.64; p<0.05) and school function (r=-0.57; p<0.05). A negative correlation between patient's age at assessment and perceived physical appearance existed (r=-0.53; p<0.05). Daily steps negatively correlated with cognitive (r=-0.58; p<0.05), physical (r=-0.63; p<0.05), emotional (r=-0.62; p<0.05), and school function (r=-0.66; p<0.01). Heart transplantation patients reported better scores for treatment and symptoms (p<0.05) but lower physical health scores (p<0.01) than those with moderate congenital heart disease. Paediatric heart transplantation patients reported overall similar quality of life as patients with moderate congenital heart disease. Children receiving heart transplants at an older age may require additional emotional and educational support. Heart transplantation patients with higher activity levels may be more aware of their physical, emotional, and cognitive limitations, and thus score lower on these quality of life indicators.

Mitroflow DL Post Approval Study- North America

ClinicalTrials.gov

2017-12-04

Aortic Stenosis; Aortic Regurgitation; Aortic Valve Insufficiency; Heart Valve Diseases; Cardiovascular Abnormalities; Cardiovascular Diseases; Congenital Abnormalities; Heart Diseases; Pathological Conditions, Anatomical

The role of the Intra‐aortic balloon pump in supporting children with acute cardiac failure

PubMed Central

Collison, Sathiakar Paul; Dagar, Kulbhusan Singh

2007-01-01

Acute heart failure occurs in children following the operative correction of a congenital anomaly, as an acute change in a child with a congenital anomaly, or in a structurally normal heart with acute myocarditis. Acute heart failure in children justifies aggressive treatment because of the high potential for complete recovery. The options for providing mechanical support to the failing heart in a child include extracorporeal membrane oxygenation, left ventricular assist devices and the use of the intra‐aortic balloon pump (IABP). The principles of intra‐aortic balloon pump usage are described, and the literature regarding the indications and outcome of its use in children is reviewed. PMID:17488858

Report of the National Heart, Lung, and Blood Institute Working Group: An Integrated Network for Congenital Heart Disease Research

PubMed Central

Pasquali, Sara K.; Jacobs, Jeffrey P.; Farber, Gregory K.; Bertoch, David; Blume, Elizabeth D.; Burns, Kristin M.; Campbell, Robert; Chang, Anthony C.; Chung, Wendy K.; Riehle-Colarusso, Tiffany; Curtis, Lesley H.; Forrest, Christopher B.; Gaynor, William J.; Gaies, Michael G.; Go, Alan S.; Henchey, Paul; Martin, Gerard R.; Pearson, Gail; Pemberton, Victoria L.; Schwartz, Steven M.; Vincent, Robert; Kaltman, Jonathan R.

2016-01-01

The National Heart, Lung, and Blood Institute convened a Working Group in January 2015 to explore issues related to an integrated data network for congenital heart disease (CHD) research. The overall goal was to develop a common vision for how the rapidly increasing volumes of data captured across numerous sources can be managed, integrated, and analyzed to improve care and outcomes. This report summarizes the current landscape of CHD data, data integration methodologies used across other fields, key considerations for data integration models in CHD, and the short- and long-term vision and recommendations made by the Working Group. PMID:27045129

Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.

PubMed

Baruteau, Alban-Elouen; Pass, Robert H; Thambo, Jean-Benoit; Behaghel, Albin; Le Pennec, Solène; Perdreau, Elodie; Combes, Nicolas; Liberman, Leonardo; McLeod, Christopher J

2016-09-01

Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities. Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block. • Prevalence of congenital heart block of 1 per 15,000 to 20,000 live births. AV block is defined as congenital if diagnosed in utero, at birth, or within the first month of life, whereas childhood AV block is diagnosed between the first month and the 18th year of life. As a result of several different etiologies, congenital and childhood atrioventricular block may occur in an entirely structurally normal heart or in association with concomitant congenital heart disease. Cardiac pacing is indicated in symptomatic patients and has several prophylactic indications in asymptomatic patients to prevent sudden death. • Autoimmune, congenital AV block is associated with a high neonatal mortality rate and development of dilated cardiomyopathy in 5 to 30 % cases. What is New: • Several genes including SCN5A have been implicated in autosomal dominant forms of familial progressive cardiac conduction disorders. • Leadless pacemaker technology and gene therapy for biological pacing are promising research fields. In utero percutaneous pacing appears to be at high risk and needs further development before it can be adopted into routine clinical practice. Cardiac resynchronization therapy is of proven value in case of pacing-induced cardiomyopathy.

December 2014 HeartWeek issue of cardiology in the young: highlights of HeartWeek 2014: diseases of the cardiac valves from the foetus to the adult.

PubMed

Jacobs, Jeffrey P

2014-12-01

This December Issue of Cardiology in the Young represents the 12th annual publication generated from the two meetings that compose "HeartWeek in Florida". "HeartWeek in Florida", the joint collaborative project sponsored by the Cardiac Center at the Children's Hospital of Philadelphia, Pennsylvania, together with Johns Hopkins All Children's Heart Institute of Saint Petersburg, Florida, averages over 1000 attendees every year and is now recognised as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of cardiac disease in the foetus, neonate, infant, child, and adult. "HeartWeek in Florida" combines the International Symposium on Congenital Heart Disease, organised by All Children's Hospital and Johns Hopkins Medicine and entering its 15th year, with the Annual Postgraduate Course in Pediatric Cardiovascular Disease, organised by The Children's Hospital of Philadelphia and entering its 18th year. This December, 2014 Issue of Cardiology in the Young features highlights of Johns Hopkins All Children's Heart Institute's 14th Annual International Symposium on Congenital Heart Disease, which was held at the Renaissance Vinoy Resort & Golf Club, Saint Petersburg, Florida, from 15-18 February, 2014. This Symposium was co-sponsored by The American Association for Thoracic Surgery (AATS) and had as its special focus " Diseases of the Cardiac Valves from the Fetus to the Adult ". We acknowledge the tremendous contributions made to paediatric and congenital cardiac care by Duke Cameron and Joel Brenner, and therefore we dedicate this December, 2014 HeartWeek Issue of Cardiology in the Young to them. Duke Cameron is Professor of Surgery at Johns Hopkins University and Cardiac Surgeon-in-Charge at The Johns Hopkins Hospital. Joel Brenner is Professor of Pediatrics at Johns Hopkins University and Director of the Taussig Heart Center at Bloomberg Children's Center, The Johns Hopkins Hospital. Together, Joel and Duke lead the proud paediatric and congenital cardiac programme at The Johns Hopkins Hospital.

Embolization of Collateral Vessels Using Mechanically Detachable Coils in Young Children with Congenital Heart Disease

DOE Office of Scientific and Technical Information (OSTI.GOV)

Sato, Y.; Ogino, H.; Hara, M.

2003-11-15

Our objective was to evaluate the usefulness of embolizing collateral vessels using mechanically detachable coils (MDCs) in children aged 3 years or younger with congenital heart disease. The subjects were 8 children with congenital heart disease featuring collateral vessels (age 18 days-3 years): 3 with a single ventricle, 2 with the tetralogy of Fallot, 2 with pulmonary atresia, and 1 with a ventricular septal defect. The embolized vessels were the major aortopulmonary collateral artery (MAPCA) in 5 patients, the persistent left superior vena cava in 2, and the coronary arteriovenous fistula in 1. A 4 or a 5 F cathetermore » was used as the guiding device, and embolization was performed using MDCs and other conventional coils introduced through the microcatheter. One patient had growth of new MAPCAs after embolization, and these MAPCAs were also embolized with MDCs. Thus, a total of 9 embolization procedures were performed in 8 patients. Complete occlusion of the collateral vessels was achieved in 8 of 9 procedures (89%). Seven of 8 patients (88%) had uneventful courses after embolization, and MDC procedures appeared to play important roles in avoiding coil migration and achievement of safe coil embolization. One patient who underwent MAPCA embolization showed no improvement in heart function and died 2 months and 19 days later. Embolization of collateral vessels using MDCs in young children with congenital heart disease can be an effective procedure and a valuable adjunct to surgical management.« less

Paediatric cardiac rehabilitation in congenital heart disease: a systematic review.

PubMed

Tikkanen, Ana Ubeda; Oyaga, Ainhoa Rodriguez; Riaño, Olga Arroyo; Álvaro, Enrique Maroto; Rhodes, Jonathan

2012-06-01

Advances in medical and surgical care have contributed to an important increase in the survival rates of children with congenital heart disease. However, survivors often have decreased exercise capacity and health-related issues that affect their quality of life. Cardiac Rehabilitation Programmes have been extensively studied in adults with acquired heart disease. In contrast, studies of children with congenital heart disease have been few and of limited scope. We therefore undertook a systematic review of the literature on cardiac rehabilitation in children with congenital heart disease to systematically assess the current evidence regarding the use, efficacy, benefits, and risks associated with this therapy and to identify the components of a successful programme. We included studies that incorporated a cardiac rehabilitation programme with an exercise training component published between January, 1981 and November, 2010 in patients under 18 years of age. A total of 16 clinical studies were found and were the focus of this review. Heterogeneous methodology and variable quality was observed. Aerobic and resistance training was the core component of most studies. Diverse variables were used to quantify outcomes. No adverse events were reported. Cardiac Rehabilitation Programmes in the paediatric population are greatly underutilised, and clinical research on this promising form of therapy has been limited. Questions remain regarding the optimal structure and efficacy of the programmes. The complex needs of this unique population also mandate that additional outcome measures, beyond serial cardiopulmonary exercise testing, be identified and studied.

[Neurological and psychomotor development of foetuses and children with congenital heart disease--causes and prevalence of disorders and long-term prognosis].

PubMed

Herberg, U; Hövels-Gürich, H

2012-06-01

Children with severe congenital heart defects (CHD) requiring open heart surgery in the first year of life are at high risk for developing neurological and psychomotor abnormalities. Depending on the type and severity of the CHD, between 15 and over 50% of these children have deficits, which are usually confined to distinct domains of development, although formal intelligence tends to be normal. Children with mild CHD, who comprise the majority of congenital heart defects, have a far better developmental prognosis than those with complex CHD. This review concentrates on the impact of severe CHD on the developing brain of the foetus and infant. It also provides a summary of recent clinical and neuroimaging studies, and an overview of the long-term neurological prognosis. Advanced neuroimaging modalities indicate that, related to altered cerebral blood flow and oxygenation, foetuses with severe CHD show delayed third trimester brain maturation and increased vulnerability for hypoxic injury. Morphological and neurological abnormalities are present before surgery, commonly affecting the white matter. In the long-term, impaired neurological and developmental outcomes are related to the combination of prenatal, perinatal and additional perioperative risk factors. Therefore, new therapeutic approaches aim to optimise the intra- and perinatal management of foetuses and newborns with congenital heart defects. Identification and avoidance of risk factors, early neurodevelopmental assessment and therapy may optimise the long-term outcome in this high-risk population. © Georg Thieme Verlag KG Stuttgart · New York.

Ciona as a Simple Chordate Model for Heart Development and Regeneration

PubMed Central

Evans Anderson, Heather; Christiaen, Lionel

2016-01-01

Cardiac cell specification and the genetic determinants that govern this process are highly conserved among Chordates. Recent studies have established the importance of evolutionarily-conserved mechanisms in the study of congenital heart defects and disease, as well as cardiac regeneration. As a basal Chordate, the Ciona model system presents a simple scaffold that recapitulates the basic blueprint of cardiac development in Chordates. Here we will focus on the development and cellular structure of the heart of the ascidian Ciona as compared to other Chordates, principally vertebrates. Comparison of the Ciona model system to heart development in other Chordates presents great potential for dissecting the genetic mechanisms that underlie congenital heart defects and disease at the cellular level and might provide additional insight into potential pathways for therapeutic cardiac regeneration. PMID:27642586

Healthy Heart Quizzes

MedlinePlus

... More Healthy Heart Quizzes Updated:Oct 30,2017 Cardiovascular Conditions • Conditions Home • Arrhythmia and Atrial Fibrillation • Cardiac Arrest • Cardiac Rehab • Cardiomyopathy • Cardiovascular Conditions of Childhood • Cholesterol • Congenital Heart Defects • Diabetes • ...

Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease.

PubMed

Mantegazza, Valentina; Apostolo, Anna; Hager, Alfred

2017-07-01

Recently, the number of patients with congenital heart diseases reaching adulthood has been progressively increasing in developed countries, and new issues are emerging: the evaluation of their capacity to cope with physical activity and whether this knowledge can be used to optimize medical management. A symptom-limited cardiopulmonary exercise test has proven to be an essential tool, because it can objectively evaluate the functional cardiovascular capacity of these patients, identify the pathological mechanisms of the defect (circulatory failure, shunts, and/or pulmonary hypertension), and help prescribe an individualized rehabilitation program when needed. The common findings on cardiopulmonary exercise testing in patients with congenital heart diseases are a reduced peak [Formula: see text]o 2 , an early anaerobic threshold, a blunted heart rate response, a reduced increase of Vt, and an increased [Formula: see text]e/[Formula: see text]co 2 . All these measures suggest common pathophysiological abnormalities: (1) a compromised exercise capacity from anomalies affecting the heart, vessels, lungs, or muscles; (2) chronotropic incompetence secondary to cardiac autonomic dysfunction or β-blockers and antiarrhythmic therapy; and (3) ventilatory inefficiency caused by left-heart failure with pulmonary congestion, pulmonary hypertension, pulmonary obstructive vascular disease, or cachexia. Most of these variables also have prognostic significance. For these patients, cardiopulmonary exercise testing allows evaluation and decisions affecting lifestyle and therapeutic interventions.

Detection of congenital heart defects throughout pregnancy; impact of first trimester ultrasound screening for cardiac abnormalities.

PubMed

Eleftheriades, Makarios; Tsapakis, Elsa; Sotiriadis, Alexandros; Manolakos, Emmanouil; Hassiakos, Demetrios; Botsis, Demetrios

2012-12-01

To evaluate prospectively the efficacy to screen for congenital heart defects (CHD) during the first trimester nuchal translucency (NT) ultrasound examination by assessing the four chambers' view of fetal heart. Pregnancies that were examined prospectively by ultrasound in the first trimester (11th-14th week), the second (19th-24th week) and third trimester were included in the study. 3774 fetuses were examined and fetal heart was assessed during the NT scan by examining the four chambers view. Detailed echocardiography was performed during the anomaly and growth scans. Diagnosis of congenital heart defects (CHD) was further confirmed by a fetal cardiologist. The four chambers view was obtained in 99.52% of the cases. CHD were diagnosed in 29 fetuses (0.77%). Thirteen cases (44.8%) were detected during the 11-13 weeks' scan, 14 cases (48.3%) during the anomaly scan, 1 CHD (3.5%) during the third trimester scan and 1 case (3.5%) postpartum. Assessment of the four chambers of fetal heart early in pregnancy was feasible and allowed the detection of 45% of CHD. Additional parameters of fetal cardiac anatomy during the NT scan may further improve the detection rate providing pregnancy management information early in the first trimester.

Exercise in children with common congenital heart lesions: balancing benefits with risks.

PubMed

Halliday, Melanie; Selvadurai, Hiran; Sherwood, Megan; Fitzgerald, Dominic A

2013-10-01

Children with corrected common congenital heart lesions are often withheld from regular exercise by their parents. While there are some modest risks with exercise, they should be seen in perspective, and the life-long benefits of regular exercise on general health, mood and well-being should be emphasised. © 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Pediatric Electrocardiographic Imaging (ECGI) Applications

PubMed Central

Silva, Jennifer N. A.

2014-01-01

Summary Noninvasive electrocardiographic imaging (ECGI) has been used in pediatric and congenital heart patients to better understand their electrophysiologic substrates. In this article we focus on the 4 subjects related to pediatric ECGI: 1) ECGI in patients with congenital heart disease and Wolff-Parkinson-White syndrome, 2) ECGI in patients with hypertrophic cardiomyopathy and pre-excitation, 3) ECGI in pediatric patients with Wolff-Parkinson-White syndrome, and 4) ECGI for pediatric cardiac resynchronization therapy. PMID:25722754

Role of CT in Congenital Heart Disease.

PubMed

Rajiah, Prabhakar; Saboo, Sachin S; Abbara, Suhny

2017-01-01

Congenital heart diseases (CHD) are being increasingly encountered in cardiac imaging due to improved outcomes from surgical and interventional techniques. Imaging plays an important role in the evaluation of CHD, both prior to and after surgeries and interventions. Computed tomography (CT) has several advantages in the evaluation of these disorders, particularly its high spatial resolution, multi-planar reconstruction capabilities at sub-millimeter isotropic resolution, good temporal resolution, wide field of view, and rapid turnaround time, which minimizes the need for sedation and anesthesia in young children or children with disabilities. With modern scanners, images can be acquired as fast as within one heartbeat. Although there is a risk of ionizing radiation, the radiation dose can be minimized by using several dose reduction strategies. There is a risk of contrast nephrotoxicity in patients with renal dysfunction. In this article, we will review the role of CT in the evaluation of several congenital heart diseases, both in children and adults.

Interstitial deletion of 8q21{yields}22 associated with minor anomalies, congenital heart defect, and Dandy-Walker variant

DOE Office of Scientific and Technical Information (OSTI.GOV)

Donahue, M.L.; Ryan, R.M.

1995-03-13

We describe an infant with a deletion of 8q21{yields}22 who had distinct clinical manifestations including minor facial anomalies, a congenital heart defect, a Dandy-Walker variant, and mild to moderate developmental delay. Her facial characteristics included small, wide-spaced eyes, asymmetric bilateral epicanthal folds, a broad nasal bridge, a {open_quotes}carp-shaped{close_quotes} mouth, micrognathia, and prominent, apparently low-set ears. Three other reports describe children with larger proximal deletions of 8q that include 8q21 and q22. These four children all have similar facial appearance. Of the others reported, one had a congenital heart defect and one had craniosynostosis. This case, in addition to the previouslymore » noted three cases, helps in delineating a recognizable syndrome. 12 refs., 3 figs., 1 tab.« less

Bartonella endocarditis in complex congenital heart disease.

PubMed

Hoffman, Risa M; AboulHosn, Jamil; Child, John S; Pegues, David A

2007-01-01

Bartonella species are an important cause of culture-negative endocarditis, with recognized risk factors of alcoholism, homelessness, cat exposure, and pre-existing valvular disease. We report a case of Bartonella henselae endocarditis in a 36-year-old woman with complex congenital heart disease who presented with a 7-month history of hemolytic anemia, leukocytoclastic vasculitis, and recurrent fevers. Transesophageal echocardiogram revealed vegetations on the patient's native aortic valve and in the right ventricular to pulmonary artery conduit and associated bioprosthetic valve. Diagnosis of B. henselae was confirmed with serum antibody and polymerase chain reaction (PCR) testing and tissue stains. The patient was treated successfully with surgical resection and prolonged antimicrobial therapy with ceftriaxone, gentamicin, and doxycycline. A review of the literature suggests prosthetic valves and complex congenital heart disease are risk factors for Bartonella endocarditis, and a high index of suspicion with antibody and PCR testing can expedite diagnosis and improve outcomes.

[Heart transplantation for the treatment of isolated left ventricular myocardial noncompaction. First case in Mexico].

PubMed

Zetina-Tun, Hugo Jesús; Careaga-Reyna, Guillermo; Galván-Díaz, José; Sánchez-Uribe, Magdalena

Myocardial noncompaction of the left ventricle is a congenital cardiomyopathy characterised by left ventricular hypertrabeculation and prominent intertrabecular recesses. The incidence ranges from 0.15% to 2.2%. Clinical manifestations include heart failure, arrhythmias, and stroke. Prognosis is fatal in most cases. Heart transplantation is a therapeutic option for this cardiomyopathy, and few had been made worldwide. The case is presented of a 20 year-old male with noncompacted myocardium of the left ventricle, who had clinical signs of heart failure. His functional class was IV on the New York Heart Association scale. He was successfully transplanted. Its survival to 15 months is optimal in class I New York Heart Association, and endomyocardial biopsies have been reported without evidence of acute rejection. It is concluded that heart transplantation modified the natural history and improved survival in patients with this congenital heart disease. Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.

Usefulness of 64-detector computed tomography in the diagnosis and management of patients with congenital heart disease.

PubMed

Bret-Zurita, Montserrat; Cuesta, Emilio; Cartón, Antonio; Díez, Jesús; Aroca, Ángel; Oliver, José M; Gutiérrez-Larraya, Federico

2014-11-01

Although congenital heart defects are the most common major congenital abnormalities, the associated mortality has been decreasing due to improvements in their diagnosis and treatment. We assessed the usefulness of 64-multidetector computed tomography in the diagnosis and management of these patients. This 5-year observational, analytical, retrospective, cohort study included a total of 222 tomographic studies of patients with congenital heart disease. Computed tomography scans were read twice and medical records were reviewed. We assessed the complexity of the disease, patient, and radiological technique, and evaluated the contribution of new data in relation to clinical suspicion and diagnostic change. A confidence interval was set at 95% and a P value of<.05 was used as the cutoff for statistical significance. In 35.1% of patients, the treatment procedure was performed after computed tomography without other tests. Additional diagnostic catheterization was performed in 12.5% of patients. There were new findings in 77% of patients (82.9% with complex disease), which prompted a change in patient management in 35.6%. All unexpected reports described new findings. No significant differences were found by age, sex, study period, urgency of the test order, patient complexity, or difficulty of the technique. Use of 64-detector computed tomography yields good diagnostic performance in congenital heart disease, prompts changes in management in more than one-third of patients, and reveals new findings in relation to the presumed diagnosis in 77% of patients. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

Congenital heart disease in Liverpool: 1960--69.

PubMed

Kenna, A P; Smithells, R W; Fielding, D W

1975-01-01

The incidence of congenital heart disease (C.H.D.) in Liverpool from 1960 to 1969 inclusive has been determined from the Liverpool Congenital Abnormalities Registry with a follow-up period of 3 to 12 years. The incidence is 6-6 per 1000 total births and this probably represents a very small degree of under-reporting. There is no consistent seasonal variation in the incidence of any of the main congenital heart lesions. In general, infants with C.H.D. tend to be of lower birth weight and born after shorter gestation than controls. This is most conspicuous with patent ductus arteriosus (P.D.A.). Females preponderate in P.D.A. and males in transposition. There is probably also a male preponderance in coarctation and aortic stenosis. Fallot's tetralogy is associated with increased maternal age and parity. Pregnancies leading to the birth of a baby with C.H.D. are complicated by threatened abortion more frequently than are controls. The concordance rate for C.H.D. in twins is low. Monozygotic twins are more liable to C.H.D. than are dizygotic twins. The incidence of C.H.D. in the siblings of affected propositi is 2-3 times that expected. Affected sibs often have the same lesion. About 20 per cent of infants with C.H.D. have associated major defects notably monogolism and defects of the alimentary, skeletal, genito-urinary and nervous systems. These are responsible for the early death of about one quarter of all infants born with C.H.D. The data presented here suggest that environmental rather than genetic factors are predominantly responsible for congenital heart disease.

ECG (image)

MedlinePlus

The electrocardiogram (ECG, EKG) is used extensively in the diagnosis of heart disease, ranging from congenital heart disease in ... and myocarditis in adults. Several different types of electrocardiogram exist.

The Total Artificial Heart in End-Stage Congenital Heart Disease.

PubMed

Villa, Chet R; Morales, David L S

2017-01-01

The development of durable ventricular assist devices (VADs) has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD), despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc.) and physiologic constraints (diastolic dysfunction). The total artificial heart (TAH), which has right and left sided pumps that can be arranged in a variety of orientations, can accommodate the anatomic variation present in CHD patients. This review provides an overview of the potential use of the TAH in patients with CHD.

The Total Artificial Heart in End-Stage Congenital Heart Disease

PubMed Central

Villa, Chet R.; Morales, David L. S.

2017-01-01

The development of durable ventricular assist devices (VADs) has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD), despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc.) and physiologic constraints (diastolic dysfunction). The total artificial heart (TAH), which has right and left sided pumps that can be arranged in a variety of orientations, can accommodate the anatomic variation present in CHD patients. This review provides an overview of the potential use of the TAH in patients with CHD. PMID:28536530

Clinical Experience With the Subcutaneous Implantable Cardioverter-Defibrillator in Adults With Congenital Heart Disease.

PubMed

Moore, Jeremy P; Mondésert, Blandine; Lloyd, Michael S; Cook, Stephen C; Zaidi, Ali N; Pass, Robert H; John, Anitha S; Fish, Frank A; Shannon, Kevin M; Aboulhosn, Jamil A; Khairy, Paul

2016-09-01

Sudden cardiac death is a major contributor to mortality for adults with congenital heart disease. The subcutaneous implantable cardioverter-defibrillator (ICD) has emerged as a novel tool for prevention of sudden cardiac death, but clinical performance data for adults with congenital heart disease are limited. A retrospective study involving 7 centers over a 5-year period beginning in 2011 was performed. Twenty-one patients (median 33.9 years) were identified. The most common diagnosis was single ventricle physiology (52%), 9 palliated by Fontan operation and 2 by aortopulmonary shunts: d-transposition of the great arteries after Mustard/Senning (n=2), tetralogy of Fallot (n=2), aortic valve disease (n=2), and other biventricular surgery (n=4). A prior cardiac device had been implanted in 7 (33%). The ICD indication was primary prevention in 67% and secondary in 33% patients. The most common reason for subcutaneous ICD placement was limited transvenous access for ventricular lead placement (n=10) followed by intracardiac right-to-left shunt (n=5). Ventricular arrhythmia was induced in 17 (81%) and was converted with ≤80 Joules in all. There was one implant complication related to infection, not requiring device removal. Over a median follow-up of 14 months, 4 patients (21%) received inappropriate and 1 (5%) patient received appropriate shocks. There was one arrhythmic death related to asystole in a single ventricle patient. Subcutaneous ICD implantation is feasible for adults with congenital heart disease patients. Most candidates have single ventricle heart disease and limited transvenous options for ICD placement. Despite variable anatomy, this study demonstrates successful conversion of induced ventricular arrhythmia and reasonable rhythm discrimination during follow-up. © 2016 American Heart Association, Inc.

Ventricular arrhythmias and sudden cardiac death in adults with congenital heart disease.

PubMed

Khairy, Paul

2016-11-01

Remarkable gains in survival have led to an unprecedented number of adults with congenital heart disease. Arrhythmias collectively comprise the most common complication encountered. Recognising the unique issues and challenges involved in managing arrhythmias in adults with congenital heart disease and the consequential decisions surrounding sudden death prevention, expert societies have proposed evidence-based recommendations. On the whole, acute ventricular arrhythmias are managed according to general cardiology guidelines, while taking into consideration congenital heart disease-specific issues, such as positioning of patches or paddles according to location of the heart. Implantable cardioverter-defibrillators (ICDs) are indicated for secondary prevention in patients with sustained ventricular tachycardia or resuscitated cardiac arrest in the absence of a reversible cause. Pharmacological therapy and catheter ablation can be effective in reducing recurrent ICD shocks. Risk-benefit assessment for primary prevention ICDs is a major challenge. Although a clearer picture has emerged of the high-risk patient with tetralogy of Fallot, ICD indications for those with systemic right ventricles or univentricular hearts remain contentious. Challenges to ICD implantation include obstructed veins, conduits and baffles, atrioventricular valve disease and intracardiac shunts. In selected patients, customised systems with epicardial and/or subcutaneous coils may represent a viable solution. Alternatively, the subcutaneous ICD is an attractive option for patients in whom transvenous access is not feasible or desirable and in whom bradycardia and antitachycardia pacing features are not essential. Continued advances in risk stratification and device technologies carry the potential to further improve efficacy and safety outcomes in this growing population of patients. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Congenital intrahepatic portosystemic shunt--an incidental rare anomaly.

PubMed

Singh, Kunwarpal; Kapoor, Aprajita; Kapoor, Atul; Gupta, Kamini; Mahajan, Goldaa

2006-12-01

Congenital intrahepatic shunts are rare anomalies that are usually incidental in a child who undergoes an ultrasound examination for some other reasons. Early diagnosis is important, because the condition can lead to hepatic encephalopathy and hypoglycemia. Author's would like to describe the findings of one such patient diagnosed to be having congenital intrahepatic shunt and discuss the clinical importance of this condition.

In Vitro Simulation and Validation of the Circulation with Congenital Heart Defects

PubMed Central

Figliola, Richard S.; Giardini, Alessandro; Conover, Tim; Camp, Tiffany A.; Biglino, Giovanni; Chiulli, John; Hsia, Tain-Yen

2010-01-01

Despite the recent advances in computational modeling, experimental simulation of the circulation with congenital heart defect using mock flow circuits remains an important tool for device testing, and for detailing the probable flow consequences resulting from surgical and interventional corrections. Validated mock circuits can be applied to qualify the results from novel computational models. New mathematical tools, coupled with advanced clinical imaging methods, allow for improved assessment of experimental circuit performance relative to human function, as well as the potential for patient-specific adaptation. In this review, we address the development of three in vitro mock circuits specific for studies of congenital heart defects. Performance of an in vitro right heart circulation circuit through a series of verification and validation exercises is described, including correlations with animal studies, and quantifying the effects of circuit inertiance on test results. We present our experience in the design of mock circuits suitable for investigations of the characteristics of the Fontan circulation. We use one such mock circuit to evaluate the accuracy of Doppler predictions in the presence of aortic coarctation. PMID:21218147

Nutrition and growth in congenital heart disease: a challenge in children.

PubMed

Medoff-Cooper, Barbara; Ravishankar, Chitra

2013-03-01

Growth failure secondary to feeding problems after complex neonatal cardiac surgery is well documented, but not well understood. The purpose of this review is to describe feeding and growth pattern in children with congenital heart defects. Nearly half of the infants with univentricular heart defects require supplementation with nasogastric or gastrostomy tube at discharge from neonatal surgery. Feeding challenges contribute to parental stress, and persist beyond infancy. These infants are 'stunted' with both weight and height being below normal. Nearly a quarter of these infants meet the definition of 'failure to thrive' in the first year of life. Short stature is a significant problem for many of these children, and has an impact on neurodevelopmental outcomes. A structured nutritional program can have a positive impact on growth in the interstage period prior to the superior cavopulmonary connection. Optimizing nutritional intake has been targeted as a key component of the National Pediatric Cardiology Quality Improvement Collaborative. This initiative has enabled the development of best practices that have the potential to mitigate poor growth in children with congenital heart defects.

[Congenital heart disease in adulthood].

PubMed

Baumgartner, Helmut; Däbritz, Sabine

2008-03-15

While a few decades ago only a minority of patients, particularly of those with complex congenital heart disease, could reach adulthood, progress of pediatric cardiology and cardiac surgery allows now the survival of the majority. Thus, adult cardiology is faced with a new challenging patient population. Since only a few congenital heart defects can be cured, regular follow-up during adult life is of major importance. Residual as well as consequently developed lesions must be recognized. Optimal timing of surgery or catheter intervention is necessary to provide the best long-term outcome. Despite optimal treatment part of the patients will develop long-term complications such as arrhythmias, pulmonary hypertension and, eventually, heart failure. Acute complications such as arrhythmias, aortic dissection or rupture, endocarditis, cerebral events due to embolism, bleeding or abscesses, and pulmonary embolism or bleeding must be recognized early and treated appropriately. Management of noncardiac surgery, pregnancy and delivery can be challenging. Another task is counseling regarding exercise and sports, choice of profession, driving and insurance issues. Finally, psychosocial issues must be taken into account for appropriate care of this special patient group.

Regional Variations in the Prevalence of Major Congenital Malformations in Quebec: The Importance of Fetal Growth Environment.

PubMed

Zhao, Jin-Ping; Sheehy, Odile; Bérard, Anick

2015-01-01

Congenital anomalies are the consequence of a complex interaction between genetic predisposition and fetal environment. Based on the Congenital Anomalies Surveillance in Canada Report, between 1998 and 2007 the rate of congenital heart defects in Quebec was significantly higher than the Canadian average; no data on the overall prevalence of congenital anomalies for Quebec or data on regional variations in any province are available. To estimate the prevalence of major congenital malformations (MCMs) in all of the 17 administrative regions of Quebec. Using data from the Quebec Pregnancy Cohort, we included infants if they were born between January 1, 1998 and December 31, 2008. MCMs were identified within the infant's first year of life using validated ICD-9 and ICD-10 codes. The rate of MCMs was calculated and stratified on Quebec's administrative regions. Among 152,353 eligible infants, the prevalence of MCMs was 36.6 (all rates were reported as per 1,000 live births). The regions with the highest rate of MCMs were Lanaudière (48.1), Laval (45.8), and Mauricie (45.1). Regions with the lowest rate were Outaouais (13.4), Côte-Nord (19.1), Abitibi-Témiscamingue (27.5), Gaspésie-îles-de-la-Madeleine (27.9), and Saguenay-Lac-Saint-Jean (28.9). Congenital heart defects (10.3) and musculoskeletal anomalies (12.6) were the most common. Laval had the highest rate of heart defects (16.1), and Lanaudière had the highest rate of musculoskeletal anomalies (22.0). The central regions of Quebec had high rate of MCMs, whereas the relatively genetically homogenous peripheral regions of Quebec had lower rate of MCM, suggesting the importance of fetal growth environment in the etiology of MCMs in Quebec.

The Critically Ill Infant with Congenital Heart Disease.

PubMed

Strobel, Ashley M; Lu, Le N

2015-08-01

This article presents an approach for identification of infants with congenital heart disorders. These disorders are difficult to diagnose because of the complexity and variety of cardiac malformations; additionally presentation can be complicated by age-dependent physiology. By compiling data from the history and the physical examination, the emergency physician can identify lesion category and initiate stabilization procedures. Critical congenital cardiac lesions can be classified as left-sided obstructive ductal dependent, right-sided obstructive ductal dependent, and shunting or mixing. The simplified approach categorizes infants with these lesions respectively as "pink," "blue," or "gray." The emergency provider can provide life-saving stabilization until specialized care can be obtained. Copyright © 2015 Elsevier Inc. All rights reserved.

Interrupted Aortic Arch Associated with Absence of Left Common Carotid Artery: Imaging with MDCT

DOE Office of Scientific and Technical Information (OSTI.GOV)

Onbas, Omer; Olgun, Hasim; Ceviz, Naci

2006-06-15

Interrupted aortic arch (IAA) is a rare severe congenital heart defect defined as complete luminal and anatomic discontinuity between ascending and descending aorta. Although its association with various congenital heart defects has been reported, absence of left common carotid artery (CCA) in patients with IAA has not been reported previously. We report a case of IAA associated with the absence of left CCA which was clearly shown on multidetector-row spiral CT.

Xenopus as a Model Organism for Birth Defects – Congenital Heart Disease and Heterotaxy

PubMed Central

Duncan, Anna R.; Khokha, Mustafa K.

2016-01-01

Congenital heart disease is the leading cause of birth defects, affecting 9 out of 1000 newborns each year. A particularly severe form of congenital heart disease is heterotaxy, a disorder of left-right development. Despite aggressive surgical management, patients with heterotaxy have poor survival rates and severe morbidity due to their complex congenital heart disease. Recent genetic analysis of affected patients has found novel candidate genes for heterotaxy although their underlying mechanisms remain unknown. In this review, we discuss the importance and challenges of birth defects research including high locus heterogeneity and few second alleles that make defining disease causality difficult. A powerful strategy moving forward is to analyze these candidate genes in a high-throughput human disease model. Xenopus is ideal for these studies. We present multiple examples demonstrating the power of Xenopus in discovery new biology from the analysis of candidate heterotaxy genes such as GALNT11, NEK2 and BCOR. These genes have diverse roles in embryos and have led to a greater understanding of complex signaling pathways and basic developmental biology. It is our hope that the mechanistic analysis of these candidate genes in Xenopus enabled by next generation sequencing of patients will provide clinicians with a greater understanding of patient pathophysiology allowing more precise and personalized medicine, to help them more effectively in the future. PMID:26910255

Cognitive and attentional functioning in adolescents and young adults with Tetralogy of Fallot and d-transposition of the great arteries.

PubMed

Murphy, Lexa K; Compas, Bruce E; Reeslund, Kristen L; Gindville, Melissa C; Mah, May Ling; Markham, Larry W; Jordan, Lori C

2017-01-01

The objective of this study is to investigate cognitive and attentional function in adolescents and young adults with operated congenital heart disease. Previous research has indicated that children with congenital heart disease have deficits in broad areas of cognitive function. However, less attention has been given to survivors as they grow into adolescence and early adulthood. The participants were 18 non-syndromic adolescents and young adults with tetralogy of Fallot and d-transposition of the great arteries that required cardiac surgery before the age of 5 years, and 18 healthy, unaffected siblings (11-22 years of age for both groups). Cases with congenital heart disease and their siblings were administered Wechsler Intelligence scales and reported attention problems using the Achenbach System of Empirically Based Assessments. Cases were compared to both healthy siblings and established norms. Cases performed significantly lower than siblings on full scale IQ and processing speed, and significantly lower than norms on perceptual reasoning. Cases also reported more attention problems compared to both siblings and norms. Effect sizes varied with medium-to-large effects for processing speed, perceptual reasoning, working memory, and attention problems. Findings suggest that neurocognitive function may continue to be affected for congenital heart disease survivors in adolescence and young adulthood, and that comparisons to established norms may underestimate neurocognitive vulnerabilities.

Experiences of informational needs and received information following a prenatal diagnosis of congenital heart defect

PubMed Central

Bergman, Gunnar; Wadensten, Barbro; Mattsson, Elisabet

2016-01-01

Abstract Objective To explore the need for information and what information was actually received following prenatal diagnosis of a congenital heart defect, in a country where termination of pregnancy beyond 22 weeks of gestation is not easily possible because of legal constraints. Methods Twenty‐six Swedish‐speaking pregnant women (n = 14) and partners (n = 12) were consecutively recruited for semi‐structured telephone interviews following the prenatal diagnosis of a congenital heart defect. Data were analyzed using content analysis. Results Although high satisfaction with the specialist information was described, the information was considered overwhelming and complex. Objective, honest, and detailed information about multiple subjects were needed, delivered repeatedly, and supplemented by written information/illustrations. Eighteen respondents had used the Internet to search for information and identified issues involving searching difficulties, low quality, and that it was too complex, insufficient, or unspecific. Those who terminated their pregnancy criticized that there was a lack of information about termination of pregnancy, both from health professionals and online sources, resulting in unanswered questions and unpreparedness. Conclusion Individuals faced with a prenatal diagnosis of a congenital heart defect need individualized and repeated information. These needs are not all adequately met, as individuals are satisfied with the specialist consultation but left with unanswered questions regarding pregnancy termination. © 2016 The Authors. Prenatal Diagnosis published by John Wiley & Sons, Ltd. PMID:26991536

Elevated serum levels of ghrelin and TNF-α in patients with cyanotic and acyanotic congenital heart disease.

PubMed

Zhang, Sai; Guo, Gong-Liang; Yang, Li-Li; Sun, Li-Qun

2017-04-01

The levels of ghrelin and tumor necrosis factor alpha (TNF-α) are considered biological markers of congenital heart diseases (CHD). The present meta-analysis was conducted to investigate the clinical significance of serum levels of ghrelin and TNF-α in children with (CHD). Chinese and English scientific literature databases were searched to retrieve published studies relevant to ghrelin, TNF-α and CHD. Manual search was additionally employed to identify other relevant studies from cross-references. The retrieved studies were screened on the basis of our stringent inclusion and exclusion criteria to select high quality case-control studies for meta-analysis. We initially retrieved 108 published studies (20 in Chinese and 88 in English) from database searches. Finally, 6 case-control studies (5 in English and 1 in Chinese) were enrolled in our meta-analysis, and contained a total of 160 cyanotic congenital heart disease (CCHD) patients and 215 acyanotic congenital heart disease (ACHD) patients, along with 162 healthy controls. The results of meta-analysis showed that serum levels of ghrelin and TNF-α in CCHD or ACHD children were significantly higher than those in healthy controls. Our meta-analysis results showed that serum levels of ghrelin and TNF-α are elevated in children with CHD, and could be used as effective biologic markers in early diagnosis of CHD.

Experiences of informational needs and received information following a prenatal diagnosis of congenital heart defect.

PubMed

Carlsson, Tommy; Bergman, Gunnar; Wadensten, Barbro; Mattsson, Elisabet

2016-06-01

To explore the need for information and what information was actually received following prenatal diagnosis of a congenital heart defect, in a country where termination of pregnancy beyond 22 weeks of gestation is not easily possible because of legal constraints. Twenty-six Swedish-speaking pregnant women (n = 14) and partners (n = 12) were consecutively recruited for semi-structured telephone interviews following the prenatal diagnosis of a congenital heart defect. Data were analyzed using content analysis. Although high satisfaction with the specialist information was described, the information was considered overwhelming and complex. Objective, honest, and detailed information about multiple subjects were needed, delivered repeatedly, and supplemented by written information/illustrations. Eighteen respondents had used the Internet to search for information and identified issues involving searching difficulties, low quality, and that it was too complex, insufficient, or unspecific. Those who terminated their pregnancy criticized that there was a lack of information about termination of pregnancy, both from health professionals and online sources, resulting in unanswered questions and unpreparedness. Individuals faced with a prenatal diagnosis of a congenital heart defect need individualized and repeated information. These needs are not all adequately met, as individuals are satisfied with the specialist consultation but left with unanswered questions regarding pregnancy termination. © 2016 John Wiley & Sons, Ltd. © 2016 John Wiley & Sons, Ltd.

Glucose inhibits cardiac muscle maturation through nucleotide biosynthesis.

PubMed

Nakano, Haruko; Minami, Itsunari; Braas, Daniel; Pappoe, Herman; Wu, Xiuju; Sagadevan, Addelynn; Vergnes, Laurent; Fu, Kai; Morselli, Marco; Dunham, Christopher; Ding, Xueqin; Stieg, Adam Z; Gimzewski, James K; Pellegrini, Matteo; Clark, Peter M; Reue, Karen; Lusis, Aldons J; Ribalet, Bernard; Kurdistani, Siavash K; Christofk, Heather; Nakatsuji, Norio; Nakano, Atsushi

2017-12-12

The heart switches its energy substrate from glucose to fatty acids at birth, and maternal hyperglycemia is associated with congenital heart disease. However, little is known about how blood glucose impacts heart formation. Using a chemically defined human pluripotent stem-cell-derived cardiomyocyte differentiation system, we found that high glucose inhibits the maturation of cardiomyocytes at genetic, structural, metabolic, electrophysiological, and biomechanical levels by promoting nucleotide biosynthesis through the pentose phosphate pathway. Blood glucose level in embryos is stable in utero during normal pregnancy, but glucose uptake by fetal cardiac tissue is drastically reduced in late gestational stages. In a murine model of diabetic pregnancy, fetal hearts showed cardiomyopathy with increased mitotic activity and decreased maturity. These data suggest that high glucose suppresses cardiac maturation, providing a possible mechanistic basis for congenital heart disease in diabetic pregnancy.

Glucose inhibits cardiac muscle maturation through nucleotide biosynthesis

PubMed Central

Nakano, Haruko; Minami, Itsunari; Braas, Daniel; Pappoe, Herman; Wu, Xiuju; Sagadevan, Addelynn; Vergnes, Laurent; Fu, Kai; Morselli, Marco; Dunham, Christopher; Ding, Xueqin; Stieg, Adam Z; Gimzewski, James K; Pellegrini, Matteo; Clark, Peter M; Reue, Karen; Lusis, Aldons J; Ribalet, Bernard; Kurdistani, Siavash K; Christofk, Heather; Nakatsuji, Norio

2017-01-01

The heart switches its energy substrate from glucose to fatty acids at birth, and maternal hyperglycemia is associated with congenital heart disease. However, little is known about how blood glucose impacts heart formation. Using a chemically defined human pluripotent stem-cell-derived cardiomyocyte differentiation system, we found that high glucose inhibits the maturation of cardiomyocytes at genetic, structural, metabolic, electrophysiological, and biomechanical levels by promoting nucleotide biosynthesis through the pentose phosphate pathway. Blood glucose level in embryos is stable in utero during normal pregnancy, but glucose uptake by fetal cardiac tissue is drastically reduced in late gestational stages. In a murine model of diabetic pregnancy, fetal hearts showed cardiomyopathy with increased mitotic activity and decreased maturity. These data suggest that high glucose suppresses cardiac maturation, providing a possible mechanistic basis for congenital heart disease in diabetic pregnancy. PMID:29231167

Congenital and maternal syphilis in the capital of Brazil.

PubMed

Muricy, Carmen Lucia; Pinto Júnior, Vitor Laerte

2015-01-01

​This study aimed to describe the epidemiology of congenital and maternal syphilis in the Brazilian Federal District in 2010. ​ ​A retrospective descriptive study was conducted on the basis of the cases recorded in the System of Notifiable Disease Information. ​The study population comprised 133 cases of congenital syphilis; of these, 116 (52.6%) mothers received prenatal care, and 70 (60.4%) were diagnosed with syphilis during pregnancy. Only 1 mother was adequately treated, and 100 (75.2%) of the pregnant women's partners did not undergo treatment for syphilis. ​ Although mothers attended prenatal care, not all were diagnosed during pregnancy or received adequate treatment for syphilis, as their partners did not undergo treatment for syphilis.

77 FR 16844 - National Heart, Lung, and Blood Institute; Notice of Closed Meeting

Federal Register 2010, 2011, 2012, 2013, 2014

2012-03-22

... Emphasis Panel; Resource-related Application in Congenital Heart Diseases (R24). Date: April 17, 2012. Time...; 93.837, Heart and Vascular Diseases Research; 93.838, Lung Diseases Research; 93.839, Blood Diseases... DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Heart, Lung, and...

Clinical and molecular cytogenetic studies in ring chromosome 5: report of a child with congenital abnormalities.

PubMed

Basinko, Audrey; Giovannucci Uzielli, Maria Luisa; Scarselli, Gloria; Priolo, Manuela; Timpani, Giuseppina; De Braekeleer, Marc

2012-02-01

We report here a child with a ring chromosome 5 (r(5)) associated with facial dysmorphology and multiple congenital abnormalities. Fluorescent in situ hybridization (FISH) using bacterial artificial chromosome (BAC) clones was performed to determine the breakpoints involved in the r(5). The 5p deletion extended from 5p13.2-3 to 5pter and measured 34.61 Mb (range: 33.7-35.52 Mb) while the 5q deletion extended from 5q35.3 to 5qter and measured 2.44 Mb (range: 2.31-2.57 Mb). The patient presented signs such as microcephaly, hypertelorism, micrognathia and epicanthal folds, partially recalling those of a deletion of the short arm of chromosome 5 and the "cri-du-chat" syndrome. The most striking phenotypic features were the congenital heart abnormalities which have been frequently reported in deletions of the distal part of the long arm of chromosome 5 and in rings leading to a 5q35-5qter deletion. However, the NKX2-5 gene, which has been related to congenital heart defects, was not deleted in our patient, nor presumably to some other patients with 5q35.3-5qter deletion. We propose that VEGFR3, deleted in our patient, could be a candidate gene for the congenital heart abnormalities observed. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Primary prophylaxis of venous thromboembolism in children.

PubMed

Cole, Catherine H

2010-06-01

Venous thromboembolism (VTE) is rare in children and young adolescents, and occurs predominantly in those with congenital heart disease in whom guidelines exist for VTE prophylaxis. For other paediatric patients, the rarity of the event makes writing an evidence-based clinical practice guideline difficult because each of the known risk factors contributes only a small increase in risk. Thrombophilia screening is controversial because few results assist with prediction of likely thrombosis and may not alter recommendations for prophylaxis. Recent publications highlight the importance of non-pharmacological prevention of VTE in children and adolescents undergoing surgery and the importance of liaison among surgeon, anaesthetist and haematologist. This annotation was written with the aim of collating current evidence for VTE prophylaxis and emphasising the need for further research in vulnerable subgroups.

Childhood recurrent pneumonia caused by endobronchial sutures: A case report.

PubMed

Zan, Yiheng; Liu, Hanmin; Zhong, Lin; Qiu, Li; Tao, Qingfen; Chen, Lina

2017-01-01

Recurrent pneumonia is defined as more than two episodes of pneumonia in one year or three or more episodes anytime in life. Common clinical scenarios leading to recurrent pneumonia include anatomical abnormalities of respiratory tract, immunodeficiency, congenital heart diseases, primary ciliary dyskinesia, etc. A school-aged girl suffered from 1-2 episodes of pneumonia each year after trachea connection and lung repair operation resulted from an accident of car crash. Bronchoscopy revealed the sutures twisted with granulation in the left main bronchus and the patient's symptoms relieved after removal of the sutures. Here we report for the first time that surgical suture was the cause of recurrent pneumonia. This case indicates that children with late and recurrent onset of pneumonia should undergo detailed evaluation including bronchoscopy.

Improving pacemaker therapy in congenital heart disease: contractility and resynchronization.

PubMed

Karpawich, Peter P

2015-01-01

Designed as effective therapy for patients with symptomatic bradycardia, implantable cardiac pacemakers initially served to improve symptoms and survival. With initial applications to the elderly and those with severe myocardial disease, extended longevity was not a major concern. However, with design technology advances in leads and generators since the 1980s, pacemaker therapy is now readily applicable to all age patients, including children with congenital heart defects. As a result, emphasis and clinical interests have advanced beyond simply quantity to quality of life. Adverse cardiac effects of pacing from right ventricular apical or epicardial sites with resultant left bundle branch QRS configurations have been recognized. As a result, and with the introduction of newer catheter-delivered pacing leads, more recent studies have focused on alternative or select pacing sites such as septal, outflow tract, and para-bundle of His. This is especially important in dealing with pacemaker therapy among younger patients and those with congenital heart disease, with expected decades of artificial cardiac stimulation, in which adverse myocellular changes secondary to pacing itself have been reported. As a correlate to these alternate or select pacing sites, applications of left ventricular pacing, either via the coronary sinus, intraseptal or epicardial, alone or in combination with right ventricular pacing, have gained interest for patients with heart failure. Although cardiac resynchronization pacing has, to date, had limited clinical applications among patients with congenital heart disease, the few published reports do indicate potential benefits as a bridge to cardiac transplant. Copyright © 2015 Elsevier Inc. All rights reserved.

Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?

PubMed Central

Attenhofer Jost, Christine H.; Schmidt, Dörthe; Huebler, Michael; Balmer, Christian; Noll, Georg; Caduff, Rosmarie; Greutmann, Matthias

2013-01-01

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger's syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future. PMID:23577237

Prevalence of overweight and obesity among patients with congenital and acquired heart disease in Kocaeli, Turkey.

PubMed

Babaoğlu, Kadir; Deveci, Murat; Kayabey, Özlem; Altun, Gürkan; Binnetoğlu, Köksal

2015-03-01

Childhood obesity has increased in the last half of the century. The aim of this study was to evaluate the frequency of obesity in the children with congenital or acquired heart disease. A total of 1410 children were assessed in this study. The study population was composed of 518 children (289 boys, 229 girls) as control group and 892 children (477 boys, 415 girls) as heart disease group. Patients were grouped into four categories: (I) "Clinic control subjects"; (II) "mild heart disease" that has not been treated with either surgical or catheter intervention; (III) congenital heart disease treated with surgical and/or catheter intervention; and (IV) "arrhythmias". A body mass index ⩾85th percentile was defined as overweight, ⩾95th percentile as obese, and <5th percentile was defined as underweight. We did not detect any association between heart disease and obesity. There was no difference in the rates of overweight, obesity, and underweight between the healthy control subjects and patients with heart disease (8.1%, 13.3%, and 5.0%; 9.0%, 10.7%, and 4.7%, respectively, p=0.145). All subgroups had a similar prevalence of underweight, overweight, and obesity as the healthy control population. Within the heart disease population, the overall prevalence rates for overweight, obesity, and underweight were similar between the boys and girls. Obesity is a common problem in children with heart disease, at least in general population. It is an important additional risk factor for long-term cardiovascular morbidity and mortality in children with heart disease. Precautions to prevent obesity should be a part of paediatric cardiologist's examination.

Ambient air pollution and congenital heart defects in Lanzhou, China

NASA Astrophysics Data System (ADS)

Jin, Lan; Qiu, Jie; Zhang, Yaqun; Qiu, Weitao; He, Xiaochun; Wang, Yixuan; Sun, Qingmei; Li, Min; Zhao, Nan; Cui, Hongmei; Liu, Sufen; Tang, Zhongfeng; Chen, Ya; Yue, Li; Da, Zhenqiang; Xu, Xiaoying; Huang, Huang; Liu, Qing; Bell, Michelle L.; Zhang, Yawei

2015-07-01

Congenital heart defects are the most prevalent type of birth defects. The association of air pollution with congenital heart defects is not well understood. We investigated a cohort of 8969 singleton live births in Lanzhou, China during 2010-2012. Using inverse distance weighting, maternal exposures to particulate matter with diameters ≤10 μm (PM10), nitrogen dioxide (NO2), and sulfur dioxide (SO2) were estimated as a combination of monitoring station levels for time spent at home and in a work location. We used logistic regression to estimate the associations, adjusting for maternal age, education, income, BMI, disease, folic acid intake and therapeutic drug use, and smoking; season of conception, fuel used for cooking and temperature. We found significant positive associations of Patent Ductus Arteriosus (PDA) with PM10 during the 1st trimester, 2nd trimester and the entire pregnancy (OR 1st trimester = 3.96, 95% confidence interval (CI): 1.36, 11.53; OR 2nd trimester = 3.59, 95% CI: 1.57, 8.22; OR entire pregnancy = 2.09, 95% CI: 1.21, 3.62, per interquartile range (IQR) increment for PM10 (IQR = 71.2, 61.6, and 27.4 μg m-3, respectively)), and associations with NO2 during 2nd trimester and the entire pregnancy (OR 2nd trimester = 1.92, 95% CI: 1.11, 3.34; OR entire pregnancy = 2.32, 95% Cl: 1.14, 4.71, per IQR increment for NO2 (IQR = 13.4 and 10.9 μg m-3, respectively)). The associations for congenital malformations of the great arteries and pooled cases showed consistent patterns. We also found positive associations for congenital malformations of cardiac septa with PM10 exposures in the 2nd trimester and the entire pregnancy, and SO2 exposures in the entire pregnancy. Results indicate a health burden from maternal exposures to air pollution, with increased risk of congenital heart defects.

Current Trends in Racial, Ethnic, and Healthcare Disparities Associated with Pediatric Cardiac Surgery Outcomes

PubMed Central

Peterson, Jennifer K.; Chen, Yanjun; Nguyen, Danh V.; Setty, Shaun P.

2017-01-01

Objective Despite overall improvements in congenital heart disease outcomes, racial and ethnic disparities have continued. The purpose of this study is to examine the effect of race and ethnicity, as well as other risk factors on congenital heart surgery length of stay and in-hospital mortality. Design From the 2012 Healthcare Cost and Utilization Project Kids Inpatient Database (KID), we identified 13,130 records with Risk Adjustment in Congenital Heart Surgery complexity score-eligible procedures. Multivariate logistic and linear regression modeling with survey weights, stratification and clustering was used to examine the relationships between predictor variables and length of stay as well as in-hospital mortality. Results No significant mortality differences were found among all race and ethnicity groups across each age group. Black neonates and black infants had a longer length of stay (neonatal Estimate = 8.73 days, p = .0034; infant Estimate 1.10 days, p = 0.0253), relative to whites. Government-sponsored insurance was associated with increased odds of neonatal mortality (odds ratio = 1.51, p = .0055), increased length of stay in neonates (Estimate = 4.26 days, p = .0009) and infants (Estimate = 1.52 days, p = .0181), relative to private insurance. Government-sponsored insurance was associated with increased number of chronic conditions, which were also associated with increased LOS (estimate 8.39 days, p < 0.001 in neonates; estimate 3.60 days, p < 0.001 in infants; estimate 1.87 days, p < 0.001 children). Conclusions Racial/ethnic disparities in congenital heart surgical outcomes may be changing compared to previous studies using the KID database. Increased length of stay in children with government-sponsored insurance may reflect expansion of individual states government-sponsored insurance eligibility criteria for children with complex chronic medical conditions. These findings warrant cautious optimism regarding racial and ethnic disparities in congenital heart surgery outcomes. PMID:28544396