Orthotopic heart transplantation for Fontan failure: experience and treatment strategy-a case report

Orthotopic heart transplantation after the Fontan operation presents technical surgical challenges due to the connection of systemic veins to pulmonary arteries and well-developed systemic-to-pulmonary collateral arteries. The altered anatomy and hemodynamics necessitate extensive vascular reconstruction. We report a successful orthotopic heart transplantation with three years of ventricular assist device (VAD) support in a child who had undergone the Fontan operation.

A 10-year-old boy had undergone extracardiac total cavopulmonary connection (18 mm expanded polytetrafluoroethylene conduit) at 2 years of age for a large ventricular septal defect, straddling tricuspid valve, and mitral stenosis. Following the Fontan operation, his systemic ventricular function gradually deteriorated. At 7 years of age, a Berlin Heart EXCOR® Pediatric VAD was implanted due to progressive heart failure, and he was listed for heart transplantation. Three years later, a heart transplant was performed. Cardiopulmonary bypass was established via cervical cannulation before re-sternotomy. The superior vena cava and extracardiac conduit were detached from the pulmonary artery. The pulmonary artery was reconstructed from hilum to hilum with a large bovine pericardial patch. Well-developed systemic-to-pulmonary collaterals caused excessive left atrial return; therefore, the left atrial anastomosis was performed under deep hypothermic circulatory arrest. The systemic veins were reconstructed with bicaval anastomosis, and inferior vena caval continuity was restored by leaving a short segment of the previous conduit. The procedure was completed without complications. Postoperative recovery was uneventful, and the patient was discharged on day 35.

This case illustrates a successful approach to orthotopic heart transplantation in a child with failing Fontan circulation supported by a VAD. Reconstruction of the pulmonary artery using a large pericardial patch and restoration of bicaval continuity were key to overcoming complex anatomical challenges.