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.