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.
Thanks for posting this video – really amazing case!
This type of case is indeed rare. The video is concise and to the point. The CPB was short due to good planning. An informative and amazing case.