Heart transplantation in patients with Marfan’s syndrome

Abstract

Due to the risk of vascular complications the indication for heart transplantation in patients with Marfan’s syndrome and end stage heart disease remains controversial. We analysed the results of such patients who underwent heart transplantation at our institution ven patients with Marfan’s syndrome (median age 38, range 22 – 56 years) underwent HTx between March 1986 and August 2004. The primary vascular manifestation of Marfan’s syndrome was type-A aortic dissection in 3 and ascending aortic aneurysm in 4 patients. All patients had cardiovascular operations prior to transplantation (ascending aortic replacement (n=7), mitral valve replacement or repair (n=3), thoracoabdominal aortic replacement (n=2), fenestration of type-B dissection (n=1)). All had refractory heart failure (NYHA IV) before transplantation. Two patients underwent transplantation after VAD support.