We report 12 patients with ventricular noncompaction who were echocardiographically identified at our institution since 1991. The mean age at presentation was 3.5 years. Five patients had isolated noncompaction. Three of them had subnormal left ventricular systolic function at presentation. Noncompaction was associated with complex congenital heart defect in 3 patients. Four patients had simple...

We present an interactive algorithm to segment the heart chambers and epicardial surfaces, including the great vessel walls, in pediatric cardiac MRI of congenital heart disease. Accurate whole-heart segmentation is necessary to create patient-specific 3D heart models for surgical planning in the presence of complex heart defects. Anatomical variability due to congenital defects precludes fully...

A short axis echocardiographic cut of the heart from the subcostal approach was used to study the atrioventricular junction in 47 infants and children with congenital heart disease and 20 with normal hearts. Examination of the diastolic openings of both atrioventricular valves was able to establish normal developments of the valves and annuli even when this was found in cases of complex congeni...

Ectopia cordis (EC) is a rare malformation due to failure of maturation of the midline mesodermal components of the chest and abdomen. It can be defined as a complete or partial displacement of the heart outside the thoracic cavity. It comprises 0.1% of congenital heart diseases. Common cardiac anomalies associated with EC are ventricular septal defect, atrial septal defect, and tetralogy of Fa...

Coronary artery anomalies include the anomalies of origin, termination, and structure or course. Coronary artery fistulas (CAFs) are classified as the anomalies of termination and are considered a major congenital anomaly and are in the subgroup of acyanotic heart disease.1 • Atrial septal defect • Ventricular septal defect • Patent ductus arteriosus • Aortic stenosis • Pulmonary stenosis • Par...

Background: Down syndrome is the most common congenital chromosomal anomaly and occurs in about 1-10:1.000 live births globally. Various reports stated an increasing survival rate because of advanced medical surgical care. The highest mortality children takes place first three years life with its comorbidities being heart disease gastrointestinal defect. Low birth weight was also more compared ...

BACKGROUND The occurrence of a congenital heart defect has long been thought to have a multifactorial basis, but the evidence is indirect. Complex trait analysis could provide a more nuanced understanding of congenital heart disease. METHODS AND RESULTS We assessed the role of genetic and environmental factors on the incidence of ventricular septal defects (VSDs) caused by a heterozygous Nkx2...

Congenital heart defects are the most common malformation, and are the foremost causes of mortality in the first year of life. Among congenital heart defects, conotruncal defects represent about 20% and are severe malformations with significant morbidity. Insulin gene enhancer protein 1 (ISL1) has been considered a candidate gene for conotruncal heart defects based on its embryonic expression p...