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KMID : 0904020060220010030
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Journal of Korean Society for Vascular Surgery
2006 Volume.22 No. 1 p.30 ~ p.34
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Abdominal Aortic Aneurysm in Marfan’s Syndrome -Two Cases-
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김지훈/Kim JH
김태형/박순철/원용성/윤상섭/문인성/박장상/김승남/고용복/Kim TH/Park SC/Won YS/Yun SS/Moon IS/Park JS/Kim SN/Koh YB
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Abstract
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Marfan syndrome is an inherited autosomal dominant disorder of the connective tissues, and the afflicted patients present with abnormalities of the skeletal, ocular, and cardiovascular system. The progressive dilatation of the proximal aorta leading to dissection and rupture is the typical feature and this can be a lethal complication of this disease. The incidence of Marfan syndrome is estimated to be 1 in 10,000 in most racial and ethnic groups. We experienced two cases of aortic aneurysm in Marfan syndrome. The first case was 32-year-old woman. She was admitted because of sudden onset of a painful abdominal mass for 2 days. Aortic aneurysm was diagnosed by CT and an echocardiogram showed severe mitral regurgitation (area ratio: 13/15). She underwent successful abdominal aortic aneurysm (AAA) repair and was discharged on postoperative 14th day; she was then prepared for open cardiac surgery for mitral valve replacement. The second case was 19- year-old woman. She was admitted with a known abdominal aortic aneurysm that she’d had for 1.5 years. She also underwent abdominal aortic aneurysm (AAA) repair that was technically successful, but she died on postoperative 10th day due to sudden cardiac arrest.
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