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Hypoplastic left heart syndrome

Hypoplastic left heart syndrome(HLHS) refers to the abnormal development of the left-sided
cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In
addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as
well as mitral atresia or stenosis…………….>


Cantrell’s pentalogy

Pentalogy of Cantrell is a rare congenital malformation
characterized by midline defects, resulting from
defective development in the septum transversum. The
constellation of findings includes deficiency of the
diaphragmatic pericardium, lower sternum, anterior
diaphragm, supraumbilical abdominal wall often
allowing an omphalocele as well as an intrapericardial
diaphragmatic hernia, and a cardiac lesion, most often
a ventricular septal defect. In severe cases, the
heart herniates through the diaphragmatic defect,
causing thoracoabdominal ectopia cordis. Other
associated congenital cardiac lesions may include an
atrial septal defect, pulmonary valve stenosis, tetralogy
of Fallot, dextrocardia, anomalous pulmonary venous
connection, tricuspid atresia, and truncus arteriosus.


FETAL AND NEONATAL PRESENTATION OF NON-COMPACTED VENTRICULAR

Noncompaction of the ventricular myocardium (NCVM) is a rare cardiomyopathy characterized by
numerous, prominent trabeculations and deep intertrabecular recesses caused by arrest in myocardial embryogenesis.
This study reviews our experience with neonatal and fetal NCVM. In contrast to the past reports of fetal and
neonatal NCVM, all pts in this group improved, and survived infancy.