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[Abdominal aortic aneurysm and annuloaortic ectasia in siblings with Marfan syndrome]
M Koga1, H Toyohira, H Saigenji
1Second Department of Surgery, Kagoshima University Faculty of Medicine, Japan.
Kyobu Geka. the Japanese Journal of Thoracic Surgery
|March 1, 1997
Abstract:
A combination of annuloaortic ectasia (AAE) and abdominal aortic aneurysm (AAA) appeared in siblings with Marfan syndrome is presented. The patients were a 53-year-old female and her younger brother. The brother died of arrhythmia twenty years before while waiting for surgical repair of AAE. His AAA and Marfan syndrome were ascertained at autopsy. The female patient received surgical repair for AAA and AAE subsequently. Pathologic study of the resected aneurysm revealed cystic medial necrosis. A combination of AAE and AAA developed in two members of a family with Marfan syndrome is extremely rare.