Pentalogy of Cantrell with a Left Ventricular Diverticulum and Muscular VSD

Pentalogy of Cantrell (POC) is a rare congenital disorder characterized by defects of the anterior diaphragm, midline supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and heart. The low incidence and significant heterogeneity of POC make it difficult for most surgeons to gain consistent experience in treating these congenital heart defects. Here we describe the treatment of a patient with POC, Tetralogy of Fallot, left ventricular (LV) diverticulum, and a muscular ventricular septal defect (VSD). An innovative approach through a left ventricular diverticulotomy was utilized which provided excellent exposure for repair of the muscular VSD.