Two-Stage Total Cardioaortic Replacement for End-Stage Heart and Aortic Disease in Marfan Syndrome: Case Report and Review of the Literature

A 24-year-old man with Marfan syndrome underwent mitral valve repair for prolapse at age 13. He sustained an acute type A aortic dissection at age 20 and underwent aortic root/ascending aortic replacement with a mechanical valved conduit. He initially did well after the latter procedure, but end-stage heart disease developed 4 years later, apparently secondary to primary cardiomyopathy. Pre-transplant evaluation revealed residual chronic dissection with aneurysmal dilatation of the distal ascending aorta, transverse arch, and descending thoracic aorta. He underwent combined orthotopic heart transplantation (OHT) and total arch replacement (stage I elephant trunk procedure). Subsequently, he underwent extent II thoracoabdominal aneurysm repair, leaving no residual aortic disease. The 2 procedures resulted in total cardioaortic replacement, thus definitively managing his cardiomyopathy and aortic disease resulting from Marfan syndrome. The operative strategies employed represent a novel approach in this clinical setting. This report emphasizes that patients with this disease should not be denied potentially life-saving OHT on the basis of concomitant aortic disease, but rather should be treated in centers offering expertise in both areas of surgical therapy.