Therapeutic management of patients with Marfan syndrome: focus on cardiovascular involvement

CA NIENABER, Y VON KODOLITSCH - Cardiology in review, 1999 - journals.lww.com
Marfan syndrome is an autosomal dominant disorder of the connective tissues, with mutation
on the fibrillin-1 gene encoding for fibrillin, a major component of the extracellular
microfibrils. The prevalence of the syndrome is 7 to 17 in 100,000. The mean life expectancy
for untreated patients with Marfan syndrome is 32 years, with aortic dissection, aortic rupture,
or cardiac failure from mitral or aortic valve regurgitation as the predominant cause of death
in> 90% of patients. However, with optimal clinical management of patients with Marfan …
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