HEART-TRANSPLANTATION IN PATIENTS WITH MARFANS-SYNDROME - A SURVEY OFATTITUDES AND RESULTS

Our institution performed an orthotopic heart transplantation in a pat ient with Marfan's syndrome. The immediate postoperative course was co mplicated with ultimate discharge; however, this patient died within 1 year as a result of an aortic dissection. On the basis of our experie nce, we surveyed all heart transplant centers listed with the North Am erican Transplant Coordinator Organization for other patients with Mar fan's syndrome who had been referred for transplantation. Nearly one t hird of responding centers evaluated a total of 30 patients with Marfa n's syndrome. Of these, only 13 were formally listed for a donor organ and 11 underwent heart transplantation with an operative mortality of 9.1% (n = 1). Posttransplantation morbidity was significant and inclu des a 40% (n = 4) incidence of thoracic aorta dissection (one fatal) i n operative survivors. There were three other non-dissection-related l ate deaths for an overall survival of 54.4% after a mean follow-up of 33.0 months. In this shared experience, it appears that the known vasc ular complications of Marfan's syndrome may diminish the anticipated r esults after heart transplantation. The reluctance to place these pati ents on heart transplant donor waiting lists can be justified.