Aortic Dissection in a Patient With Fontan Physiology: Complex Emergency Repair and Tailored Coronary Reconstruction

Aortic dissection in Fontan patients, likely driven by chronic systemic afterload and connective-tissue remodeling, is rare and carries high operative risk.

A 25-year-old man with pulmonary atresia and hypoplastic right ventricle after extracardiac Fontan presented with acute type A dissection involving the left main coronary artery, requiring emergency Bentall repair. The left main coronary artery was repaired and reimplanted, and the friable right coronary artery required saphenous vein posterior-wall reconstruction. Pre-bypass femoral cannulation and cooling enabled safe re-entry. Postoperative vasoplegia and ventricular dysfunction required short-term venoarterial extracorporeal membrane oxygenation, with decannulation at 36 hours.

A Fontan-tailored approach incorporating pre-bypass femoral cannulation, dedicated anesthetic management, and meticulous postoperative support enabled safe repair.

Aortic dilation and dissection can occur in Fontan survivors, requiring careful preoperative planning with peripheral cannulation, cooling, and coronary ostial cardioplegia. Complex coronary reconstruction, short-term venoarterial extracorporeal membrane oxygenation with early chest closure, and ongoing imaging surveillance are essential for management and long-term care.