상행 대동맥 동맥류를 동반한 Marfan씨증후군 환자에서 생긴 대동맥 박리(Aortic Dissection) 1례
A Case of Dissection in Marfan Syndrome with Ascending Aortic Aneurysm
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The Marfan syndrome is a generalized connective tissue disease involving eye, musculoskeletal system, cardiovascular system, and inherited autosomal dominant with various expression type. The cardiovascular complications such as aortic aneurysm, aortic dissection, aortic regurgitation, mitral regurgitation and aortic dissection which usually occurs in previously normal sized aorta are poor prognostic factors. However, the aortic dissection which developed in patient with Marfan syndrome and aortic aneurysm was rare. We experienced one case of dissecting aneurysm in patient diagnosed as previous aortic aneurysm, aortic regurgitation, and Marfan syndrome, receiving successful operation
Abstract
The Marfan syndrome is a generalized connective tissue disease involving eye, musculoskeletal system, cardiovascular system, and inherited autosomal dominant with various expression type. The cardiovascular complications such as aortic aneurysm, aortic dissection, aortic regurgitation, mitral regurgitation and aortic dissection which usually occurs in previously normal sized aorta are poor prognostic factors. However, the aortic dissection which developed in patient with Marfan syndrome and aortic aneurysm was rare. We experienced one case of dissecting aneurysm in patient diagnosed as previous aortic aneurysm, aortic regurgitation, and Marfan syndrome, receiving successful operation