Kardiovascul�re Manifestationsformen des Marfan Syndroms

Cardiac Pathology of Marfan’s Syndrome

“…The most common site of rupture is within the first few centimeters above the sinuses of Valsalva (1.3, 8, 13, 17, 31). Typically the young adult (8,10,13,17) is stricken with this complication. However, the pediatric age group is not exempt.…”

“…It follows that in Marfan patients with evidence of cardiovascular disease a high mortality can be expected, with aortic rupture as the leading single cause of death. In 80% of the patients dissection occurs acutely (13), resulting in aortic rupture and death. The most common site of rupture is within the first few centimeters above the sinuses of Valsalva (1.3, 8, 13, 17, 31).…”

“…The incidence of ascending aortic aneurysms in 119 patients with Marfan's syndrome is 65% (7,13,16). Murdoch's (18) series includes 56 patients with a known cause of death: 20 of these (36%) died of dissection or rupture of an aortic aneurysm; an additional 56% died either of congestive failure, during cardiac surgery or of other cardiac causes.…”

“…Summarizing 67 autopsies of patients with Marfan's syndrome, Kiihl and Fricke (13) reported an incidence of 66% of aneurysms of the ascending aorta. 36% of these aneurysms will eventually dissect and mpture (18), resulting in death.…”

“…93% of the patients died of cardiovascular causes at a mean age of 32 years (18). Two major review articles (6,13) extensively describe the various aspects of this disease.…”