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

BACKGROUND

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

CASE SUMMARY: 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.

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

TAKE-HOME MESSAGES: 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.