Successful orthotopic heart transplantation in a patient with Marfan syndrome

Abstract

Cardiovascular diseases represent the leading cause of mortality in patients with Marfan syndrome. Many treatments have been developed for patients with end‐stage heart failure, among which orthotopic heart transplantation remains the gold standard. We report a successful orthotopic heart transplantation for a Marfan syndrome patient in end‐stage heart failure.

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%.…”

Insuficiencia aórtica masiva con aneurisma de aorta gigante en síndrome de Marfan

“…De acuerdo a lo anterior el SM se puede presentar con cardiopatía primaria o secundaria (8) . En la insuficiencia cardíaca terminal en pacientes con SM se puede considerar el trasplante cardíaco, ya que es un tratamiento que en muy limitada experiencia ha mostrado eficacia en serie de casos (9) y en reportes (10,11) con sobrevida similar a los pacientes trasplantados sin SM; incluso en algunos pacientes se plantea el trasplante y el reemplazo profiláctico de la aorta ascendente (1) . Sin embargo el trasplante cardíaco debe considerarse en un contexto socioeconómico-cultural adecuado.…”