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

BACKGROUND: 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. CASE SUMMARY: 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. DISCUSSION: This case highlights progressive aortopathy after Fontan palliation and the importance of multidisciplinary management. TAKE-HOME MESSAGE: Multidisciplinary management is effective to treat acute aortic dissection complicated by coronary occlusion in complex patients with multiple prior surgeries for congenital heart disease.