Multidisciplinary Treatment for a Fontan Patient With Acute Aortic Dissection and Left Coronary Artery Occlusion

Acute aortic dissection complicated by coronary malperfusion is a life-threatening emergency. Although aortic root degeneration is a well-recognized complication in adults with congenital heart disease, aortic dissection causing external compression of the left main coronary artery is rare and requires prompt intervention.

A 36-year-old man with a surgical history of Fontan palliation presented with severe chest pain. He was diagnosed with acute aortic dissection with coronary occlusion by coronary angiography and computed tomography. A staged multidisciplinary strategy was undertaken: emergent percutaneous coronary intervention with left main trunk stent placement, followed by surgical aortic root replacement with a mechanical valve. The patient was discharged uneventfully.

This case highlights progressive aortopathy after Fontan palliation and the importance of multidisciplinary management.

Multidisciplinary management is effective to treat acute aortic dissection complicated by coronary occlusion in complex patients with multiple prior surgeries for congenital heart disease.