Kesler K A, Hanosh J J, O'Donnell J, Faust S, Turrentine M W, Mahomed Y, Brown J W
Indiana University School of Medicine, Department of Surgery, Indianapolis 46202.
J Heart Lung Transplant. 1994 Sep-Oct;13(5):899-904.
Our institution performed an orthotopic heart transplantation in a patient with Marfan's syndrome. The immediate postoperative course was complicated with ultimate discharge; however, this patient died within 1 year as a result of an aortic dissection. On the basis of our experience, we surveyed all heart transplant centers listed with the North American Transplant Coordinator Organization for other patients with Marfan's syndrome who had been referred for transplantation. Nearly one third of responding centers evaluated a total of 30 patients with Marfan'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 includes a 40% (n = 4) incidence of thoracic aorta dissection (one fatal) in operative survivors. There were three other non-dissection-related late 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 vascular complications of Marfan's syndrome may diminish the anticipated results after heart transplantation. The reluctance to place these patients on heart transplant donor waiting lists can be justified.
我们机构为一名患有马凡氏综合征的患者进行了原位心脏移植。术后即刻病程复杂，最终出院；然而，该患者在1年内因主动脉夹层死亡。基于我们的经验，我们对北美移植协调组织列出的所有心脏移植中心进行了调查，以了解其他被转诊进行移植的马凡氏综合征患者的情况。近三分之一的回复中心共评估了30例马凡氏综合征患者。其中，只有13例正式列入供体器官等待名单，11例接受了心脏移植，手术死亡率为9.1%（n = 1）。移植后发病率较高，手术幸存者中胸主动脉夹层发生率为40%（n = 4）（1例死亡）。另有3例与夹层无关的晚期死亡，平均随访33.0个月后总体生存率为54.4%。在这一共同经验中，马凡氏综合征已知的血管并发症似乎可能降低心脏移植后的预期效果。不愿将这些患者列入心脏移植供体等待名单是有道理的。
