Orthotopic heart transplantation in patients with Marfan syndrome.

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.