Cardiovascular Manifestations of Marfan Syndrome

Koh, Kwang Kon; Hyon, Min Su; Lim, Ha Jin; Kim, Cheol Ho; Oh, Byung Hee; Park, Young Bae; Choi, Yun Shik; Seo, Jung Don; Lee, Young Woo

Abstract

From march, 1983 to May, 1987, we experienced 17 patients of Marfan syndrome. The clinical profile and course of 17 patients of Marfan syndrome were reviewed. 1) Their ages ranged from 13 to 50 years with a mean age of 30 years and 16 patients were male and 1 patient was female. 2) musculoskeletal features were present in 14(88%) of the 16 examined. Ocular features were found in 5(42%) of 12 examined. Familial features were present in 7(47%) of 15 examined. 3) Among cardiovascular manifestations dissecting aneurysm was present in 10(59%) patients, aortic annuloectasia in 15(94%), MR in 4(24%) and MVP in 1(6%). 4) Modified Bentall operation was performed to 8 patients. Two of them died. The other 8 patients took a conservative medical therapy. Four of them died.