Heart transplantation in patients with Marfan's syndrome

A survey of attitudes and results

Kenneth Kesler, J. J. Hanosh, Jacqueline O'Donnell, S. Faust, Mark Turrentine, Y. Mahomed, John Brown

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)899-904
Number of pages6
JournalJournal of Heart and Lung Transplantation
Volume13
Issue number5
StatePublished - 1994

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Marfan Syndrome
Heart Transplantation
Dissection
Tissue Donors
Transplants
Waiting Lists
Thoracic Aorta
Blood Vessels
Survivors
Transplantation
Surveys and Questionnaires
Organizations
Morbidity
Survival
Mortality
Incidence

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Transplantation

Cite this

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abstract = "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.",
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AU - Kesler, Kenneth

AU - Hanosh, J. J.

AU - O'Donnell, Jacqueline

AU - Faust, S.

AU - Turrentine, Mark

AU - Mahomed, Y.

AU - Brown, John

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N2 - 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.

AB - 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.

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