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Ann Thorac Surg 2007;83:1691-1695
© 2007 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Orthotopic Heart Transplantation in Patients With Marfan Syndrome

^a Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany
^b Institute of Medical Immunology, Charité University Medicine Berlin, Berlin, Germany

Accepted for publication January 11, 2007.

^_* Address correspondence to Dr Knosalla, Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, D-13353 Berlin, Germany (Email: knosalla{at}dhzb.de).

Presented at the Poster Session of the Forty-second Annual Meeting of The Society of Thoracic Surgeons, Chicago, IL, Jan 30 – Feb 1, 2006.

Background: Due to the risk of vascular complications, the indication for heart transplantation (HTx) in patients with Marfan syndrome and end-stage heart disease remains controversial. We analyzed the results of such patients who underwent HTx at our institution.

Methods: Ten patients with Marfan syndrome (median age 36, range 19 to 56 years) underwent HTx between March 1986 and December 2005. The primary vascular manifestation of Marfan syndrome was type-A aortic dissection in three patients and ascending aortic aneurysm in seven patients. All patients had undergone cardiovascular operations prior to transplantation. All had refractory heart failure (New York Heart Association class IV) before transplantation. Three patients underwent transplantation after ventricular assist device (VAD) support (left VAD, n = 2; biventricular assist device, n = 1).

Results: There were no perioperative deaths. Two patients died of causes unrelated to Marfan disease (pneumonia on day 27, n = 1; stroke on day 102, n = 1). One patient died due to type-B dissection 3.8 years posttransplantation and one due to rupture of an aortic arch aneurysm after 12.1 years. Two patients underwent thoracoabdominal aortic replacement for chronic dissection 14 and 20 months posttransplantation, respectively. Kaplan-Meier survival rate was 80% at 1 year and 64% at 10 years. The Kaplan-Meier freedom from reoperation was 100% at 1 year and 62.5% at 10 years.

Conclusions: Heart transplantation in patients with Marfan syndrome results in good long-term survival, similar to that of patients without Marfan syndrome. Close follow-up and timely operation of aortic pathologies is mandatory. Reluctance to place these patients on a heart transplant waiting list appears not to be justified.

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Invited commentary

Valluvan Jeevanandam
Ann. Thorac. Surg. 2007 83: 1696. [Extract] [Full Text] [PDF]

This article has been cited by other articles:

				M. G. Keane and R. E. Pyeritz Medical Management of Marfan Syndrome Circulation, May 27, 2008; 117(21): 2802 - 2813. [Full Text] [PDF]

				V. Jeevanandam Invited commentary Ann. Thorac. Surg., May 1, 2007; 83(5): 1696 - 1696. [Full Text] [PDF]