상행 대동맥 동맥류를 동반한 Marfan씨증후군 환자에서 생긴 대동맥 박리(Aortic Dissection) 1례

A Case of Dissection in Marfan Syndrome with Ascending Aortic Aneurysm

본원에서는 Marfan씨증후군과 대동맥류, 대동맥판 역류로 진단받은 환자가 1년후 흉통을 주소로 내원하여 대동맥 박리로 이행한 것으로 밝혀져 응급수술을 성공적으로 치룬 1례를 경험하였기에 보고하는 바이다.

The Marfans 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 aoritc aneurysm, aortic regurgitation, and Marfan syndrome, receiving successful operation.