Marfan's syndrome: natural history and long-term follow-up of cardiovascular involvement

Abstract

A retrospective analysis was undertaken to define the natural history and long-term follow-up of a group of patients with Marfan's syndrome. Eighty-four patients were diagnosed between January 1959 and June 1987 as having Marfan's syndrome; 68% were male; their ages ranged from 2 to 67 years (mean 26.6). Sixteen patients constituted the early surgical group (those who underwent surgery before 1979; mean age 36.1 years). Nineteen patients constituted the late surgical group (surgery in 1979 or later; mean age 33.3 years). The nonsurgical group comprised 49 patients (mean age 19.3 years). Fifty-seven percent of the patients had a diastolic murmur and 38% had cardiomegaly at presentation. Fifty-seven percent underwent cardiac catheterization, which revealed aortic root dilation (85%), aortic regurgitation (73%), aortic dissection (33%) and mitral regurgitation (36%). Thirteen of the 19 patients in the late surgical group received a composite graft repair of the ascending aorta as compared with only 2 of the 16 in the early surgical group. Follow-up information was obtained on 81 (96%) of 84 patients; the follow-up time was 2 to 332 months (mean 99). Thirty-one of the 81 patients died at age 3 to 63 years (mean age 35 years); 87% of the known causes of death were related to the cardiovascular system. Sixty-one percent of deaths were the result of aortic dissection or rupture or sudden cardiac death. Of the 50 survivors, 98%, including all patients in the late surgical group, were in functional class I or II. Overall survival at 5, 10 and 15 years after operation was 78.4%, 57.1% and 49.5%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurovascular Complications of Marfan Syndrome

“…The close follow-up of aortic root diameter by serial ultrasound measurements, the use of prophylactic beta-blockers, and the availability of safer cardiovascular surgery in patients with Marfan syndrome may alter the natural history of associated neurovascular diseases in this patient population. [33][34][35][36][37] Marfan patients may be surviving longer and are now exposed to other potential causes for neurovascular disorders.…”

Segmental Aortic Stiffness in Children and Young Adults With Connective Tissue Disorders

“…3 These adverse events can be prevented by prophylactic surgical aortic root replacement. 4 The timing of surgical intervention is traditionally based on serial evaluation of aortic size.…”

Valve-sparing aortic root replacement in patients with Marfan syndrome—the Homburg experience

“…Life expectancy of patients with Marfan syndrome is reduced mostly due to the risk of aortic dissection or rupture (1).…”

“…Aortic root dilatation occurs in most patients with Marfan syndrome with secondary valve failure or aortic complications being frequent consequences (1). The traditional surgical approach has been composite replacement of aortic valve and root, first described in the 1960s (2).…”