Familial Aortic Dissection without Marfan Syndrome: A Report of Four Cases in a Family

(Department of Cardiovascular Surgery, Ishikawa Prefectural Central Hospital, Kanazawa, Japan)

Kenji Iino Masahiro Seki Kengo Kawakami
Naoki Sakakibara
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 4cases 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.
@Jpn. J. Cardiovasc. Surg. 33: 399-402 (2004)