Life Expectancy and Causes of Death in the Marfan Syndrome

Abstract The Marfan syndrome is a dominantly inherited disorder of connective tissue with multisystem involvement. The cardiac complications, particularly aortic dilatation, dissection and rupture and involvement of the aortic and mitral valves, lead to a greatly reduced life expectancy. This poor survival was demonstrated in a series of 257 patients with the Marfan syndrome. The average age at death for the 72 deceased patients was 32 years. Cardiac problems led to 52 of the 56 deaths of known cause, with aortic dilatation and its complications accounting for 80 per cent of these. Congestive heart failure, myocardial infarction and bacterial endocarditis were other cardiac causes of death. In patients with early aortic changes treatment with propranolol or reserpine is suggested. These agents decrease myocardial contractility and diminish the pulsatile flow stress on the weakened aorta.