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Acute Aortic Valvular Insufficiency Due to Isolated Myxomatous Degeneration

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Department of Thoracic and Cardiovascular Surgery and Department of Pathology, The Medical College of Wisconsin; Milwaukee, Wisconsin

Ann Intern Med. 1978;88(6):807-808. doi:10.7326/0003-4819-88-6-807
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Aortic valvular insufficiency in Marfan's syndrome is ordinarily due to aortic dilatation (1). The aortic media shows degeneration of elastic fibers and replacement with mucopolysaccharide; the aortic valve usually lacks these findings, although leaflet redundancy, fenestration, and rupture are recognizable. Aortic pathology may be similar in the forme fruste of Marfan's where the noncardiovascular manifestations of mesodermal dystrophy are variable. Isolated myxomatous degeneration of the aortic valve is rare, however, and in the only three reported cases, perforations of valve leaflets caused insufficiency. (2, 3). We report herein the only known case of acute aortic insufficiency occurring without perforation in


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