Japanese Journal of Cardiovascular Surgery
Online ISSN : 1883-4108
Print ISSN : 0285-1474
ISSN-L : 0285-1474
Familial Aortic Dissection without Marfan Syndrome
A Report of Four Cases in a Family
Kenji IinoMasahiro SekiKengo KawakamiNaoki Sakakibara
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Keywords: Marfan syndrome
JOURNAL FREE ACCESS

2004 Volume 33 Issue 6 Pages 399-402

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Abstract
Aortic dissection with multiple familial members is rare. It is commonly associated with Marfan syndrome. Several authors have reported familial aortic dissection without Marfan syndrome. We encountered 4 cases of aortic dissection in a family. The aortic dissection occurred in the mother and all of her children. No case had clinical manifestations of Marfan syndrome or other connective tissue disease. Histopathological examination of the aorta did not show cystic medial necrosis in 2 operated cases. Many members in the family had systemic arterial hypertension. The presence of multiple incidence of aortic dissection in one family suggests underlying connective tissue disease, irrespective of the absence of typical features of Marfan syndrome. Therefore we propose that other close relatives as well as the members with aortic dissection should be followed-up in the same way used for families with typical connective tissue disease like Marfan syndrome.
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© The Japanese Society for Cardiovascular Surgery
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