Total artificial heart implantation in a young Marfan syndrome patient

Abstract

Total artificial heart is a durable option for severe biventricular failure and multiple valvular dysfunction as a bridge to transplant in a young patient with Marfan syndrome.

Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review

“…In contrast, there are several reports on end-stage heart failure necessitating heart transplantation in patients with MFS ( Table 3 ). In addition, heart failure is mentioned as one of the leading causes of death in MFS (see further) [ 15 , 16 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 ]. Whether myocardial impairment and development of heart failure in MFS is a primary intrinsic problem or a secondary, stressor-induced problem remains an unanswered question.…”

“…An association between prior aortic or valvular surgery and the development of heart failure as long-term complication in patients with MFS has also been suggested [ 48 , 52 , 53 , 54 ]. In several case reports and one case series describing patients with MFS undergoing orthotopic heart transplantation, almost all patients had a history of prior aortic or valvular surgery ( Table 3 ) [ 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 49 ]. Similarly, a study by Hetzer et al on a cohort of 421 patients with MFS who had undergone cardiac surgery reported cardiomyopathy in 11.2%.…”

Successful orthotopic heart transplantation in a patient with Marfan syndrome

“…Another possible explanation is that chronic immunosuppression may further reduce the integrity of the aortic connective tissue . For these reasons, some surgeons have advocated a prophylactic total aortic arch replacement at the time of orthotopic heart transplantation to prevent aortic complications . Others have advocated prophylactic wrapping of the remaining ascending aorta with a bovine pericardial patch, which may be an alternative to replacement of the remaining ascending aorta to prevent further dilatation of ascending aorta.…”

“…1,4 For these reasons, some surgeons have advocated a prophylactic total aortic arch replacement at the time of orthotopic heart transplantation to prevent aortic complications. 5 Others have advocated prophylactic wrapping of the remaining ascending aorta with a bovine pericardial patch, which may be an alternative to replacement of the remaining ascending aorta to prevent further dilatation of ascending aorta. Some patients with Marfan syndrome also have concomitant aortic diseases and several cases have been reported regarding successful combined orthotopic heart transplantation and replacement of the aorta.…”