Successful orthotopic heart transplantation following total aortic replacement in a patient with Marfan syndrome: case report

Aortic dissection and aneurysm rupture are leading causes of death in patients with Marfan syndrome. Due to the risk of such vascular complications, the indication for heart transplantation in this population remains controversial. Previous reports, however, have demonstrated that heart transplantation in patients with Marfan syndrome can result in good long-term survival comparable to that in patients without Marfan syndrome. Here, we present a case of successful orthotopic heart transplantation after total aortic replacement in a patient with Marfan syndrome. A 41-year-old man with Marfan syndrome, who had been managed conservatively for type B aortic dissection, developed acute type A dissection involving both coronary arteries at age 30, resulting in myocardial infarction. He underwent aortic root replacement with a mechanical valve. Thereafter, he was repeatedly hospitalized for heart failure due to ischemic cardiomyopathy. Three years later, at age 33, he was referred to our institution for heart transplant registration because of progressively deteriorating cardiac function and frequent episodes of intractable lethal arrhythmias. He underwent replacement of the ascending aorta and aortic arch, along with bioprosthetic aortic valve replacement. His immediate postoperative course was complicated by hemodynamic decompensation, necessitating support with an extracorporeal left ventricular assist device. One year later, he underwent a thoracoabdominal aortic replacement and bridge-to-bridge implantation of a left ventricular assist device. Three years later, at age 37, he underwent replacement of the durable device because of a pump pocket infection. After 7 years on the transplantation list, he underwent successful orthotopic heart transplantation from a suitable donor at the age of 41. This rare case report describes a patient with Marfan syndrome who underwent orthotopic heart transplantation after total aortic replacement, followed by multiple surgical procedures, including extracorporeal and durable left ventricular assist device implantations, over an extended period. Although total aortic replacement was challenging in the context of complex coexisting cardiovascular disease, this approach enabled subsequent heart transplantation after mitigating the potential risk of Marfan syndrome-associated cardiovascular events.