Ann Thorac Surg 2007;83:1696
© 2007 The Society of Thoracic Surgeons
Original Articles: Cardiovascular
Invited commentary
Valluvan Jeevanandam, MD
Cardiothoracic Surgery, The University of Chicago Medical Center, 5541 S Woodlawn Ave, Chicago, IL 60637
(Email: jeevan{at}uchicago.edu).
The authors [1] have retrospectively reviewed their experience with heart transplantation on recipients with Marfan syndrome. We believe this is the largest series reported from an individual center. A prior report by Kesler and colleagues [2] described a survey of transplant centers and detailed the course of 30 patients evaluated for transplant of which only 11 were eventually transplanted. The current report is very impressive in several regards. All transplant procedures were reoperation sternotomies (several multiple) as the recipients had manifestation of Marfan syndrome that required prior operative repair. There was only 1 early death due to complications from pneumonia. The surgical prowess of the team is certainly commendable. On long term follow-up, 1 patient died from a stroke and 3 others had vascular complications attributable to Marfan syndrome. The cause of the stroke is not documented. If it was caused by Marfan syndrome, then there is a 40% incidence of vascular complications in the series. Otherwise, there is a 30% incidence. There were 2 deaths caused by aortic rupture. This is consistent with the earlier report as previously mentioned.
Clearly there is an increased risk of mortality and morbidity caused by the underlying connective tissue disorder. There is an acceleration of pre-existing dissections and development of new lesions. This can be caused by immunosuppressive drugs, hypertension, normalization of dP/dT, and other causes. This mandates that all repairable lesions be addressed, if possible, prior to or early after transplant, and that these patients are followed very closely. Most of these patients are young and it is hard to deny them a transplant. They can be offered heart transplantation with close follow-up and a dedicated center that has the ability and surgical fortitude to take on very complex and unpredictable aortic procedures. New advances in endovascular repair will also assist in the management of these patients. Heart transplantation should not be considered a contraindication in patients with Marfan syndrome, but rather a higher risk procedure that mandates close follow-up with lifelong regular diagnostic imaging.
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References
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- Knosalla C, Weng Y-g, Hammerschmidt R, et al. Orthotopic heart transplantation in patients with Marfan syndrome Ann Thorac Surg 2007;83:1691-1696.[Abstract/Free Full Text]
- Kesler KA, Hanosh JJ, O’Donnell J, et al. Heart transplantation in patients with Marfan’s syndrome: a survey of attitudes and results J Heart & Lung Transplantation 1994;13:899-904.[Medline]
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