J Korean Soc Vasc Surg.  2006 May;22(1):30-34.

Abdominal Aortic Aneurysm in Marfan's Syndrome: Two Cases

• Kim JH
• Kim TH
• Park SC
• Won YS
• Yun SS
• Moon IS
• Park JS
• Kim SN
• Koh YB

Abstract

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.

Keyword

Marfan's syndrome; Abdominal aortic aneurysm

MeSH Terms

Adult
Aorta
Aortic Aneurysm
Aortic Aneurysm, Abdominal*
Cardiovascular System
Connective Tissue
Death, Sudden, Cardiac
Dilatation
Ethnic Groups
Female
Humans
Incidence
Marfan Syndrome*
Mitral Valve
Mitral Valve Insufficiency
Rupture
Thoracic Surgery