Surgical Repair of Tetralogy of Fallot and a Large Congenital Diaphragmatic Hernia in a 16-Week-Old Infant With Pentalogy of Cantrell and a Large Omphalocele: A Case Report

We present a case of surgical repair of tetralogy of Fallot complicated by a large congenital diaphragmatic hernia as part of the staged management of pentalogy of Cantrell (POC) in a full term 16-week-old infant. The diagnosis was made prenatally, and the patient was born with a large omphalocele, tetralogy of Fallot, and a presumed diaphragmatic hernia. A right ventricular outflow stent was placed at seven weeks of life due to hypoxia. Symptoms were temporarily alleviated but progressed, and the patient received full surgical repair, including repair of a large complex congenital diaphragmatic hernia, right ventricular to pulmonary artery conduit, and left pulmonary arterioplasty at 16 weeks of life. To our knowledge, this case report is among the first to describe the combined interventional and successful surgical management of one of the youngest reported cases of tetralogy of Fallot and POC. Parental consent and IRB approval were obtained.