Pentalogy of Cantrell associated with hypoplastic left heart syndrome and herniation of the ventricular mass into the abdominal cavity

Objective: A term female with the diagnosis pentalogy of Cantrell associated with hypoplastic left heart syndrome was born without complication. A significant portion of the sternal body was hypoplastic. The right ventricle was found to be herniated into the abdominal cavity. Method: The patient underwent an uncomplicated repair of an omphalocele. The infant then underwent an uncomplicated modified Norwood procedure. The right ventricular mass was reduced into the thoracic cavity prior to the Norwood. Results: Because of inability to close the anterior chest wall, a skin allograft was employed to cover the heart. The child died of fungal sepsis at 3 months of life. Conclusions: Repair of these complex constellation of defects, although successful technically, should take into account long-term morbidity that precludes a positive outcome.