Pentalogy of Cantrell associated with hypoplastic left heart syndrome

Pentalogy of Cantrell is a rare anomaly characterized by midline closure defects, including a defect in the lower sternum, supraumbilical abdominal wall defect, deficiency of the anterior portion of the diaphragm, deficiency in the diaphragmatic portion of the pericardium with free communication between the pericardial and peritoneal cavities, and congenital heart defects. The long-term prognosis for children with this anomaly depends to a great extent on the complexity of the associated congenital heart defect. We describe the previously unreported association of pentalogy of Cantrell with hypoplastic left heart syndrome.