Surgical Repair of Cantrell’s Pentalogy With a Left Ventricular Diverticulum

Cantrell’s pentalogy is a rare association of congenital defects. A 9-month-old boy was referred to the authors for management of Cantrell’s pentalogy. On physical examination, there was a pulsatile midline epigastric mass compatible with a supraumbilical hernia, pulsatile in its superior portion. The association of these midline defects confirmed the complete form of Cantrell’s pentalogy.

Echocardiography showed a large membranous ventricular septal defect (VSD) and a left ventricular diverticulum protruding into the supraumbilical hernia. Cardiac catheterization confirmed the diagnosis. Computed tomography confirmed the supraumbilial hernia, a lower sternal cleft, and anterior diaphragm and pericardial defect. Cardiac magnetic resonance imaging showed a thick pouch of myocardial tissue arising from the apex of the left ventricle (LV), forming an LV diverticulum. This video describes the surgical repair, with a VSD patch closure and diverticulum resection, and primary repair using interrupted sutures secured with Teflon felt strips. The postoperative course was uneventful, with rapid extubation and discharge. The patient underwent a second-stage repair of the supraumbilical hernia, not shown in this video.