• H Harada, Y Ichimiya, K Kamata, T Kazui, and S Komatsu.
• Department of Thoracic and Cardiovascular Surgery, Hokkaido Prefectural Hospital, Kushiro, Japan.
• Nihon Kyobu Geka Gakkai Zasshi. 1990 Sep 1; 38 (9): 1497-500.

AbstractThe occurrence of aortic dissection in 2 or more family members is rare. Such occurred, however, in the brothers and elder sister described herein. Case 1: A 54-year-old male had chronic Type I dissecting aortic aneurysm with severe aortic insufficiency and abdominal aortic aneurysm. Case 2: A 57-year-old female had chronic Type II dissecting aortic aneurysm with severe aortic insufficiency. Case 3: A 49-year-old male had chronic Type IIb dissecting aortic aneurysm. Two cases (Case 1 & 3) of them had surgical interventions. The histology showed cystic medial necrosis of aorta. Neither of these 3 patients or other family members had skeletal or ocular features of the Marfan syndrome. The metacarpal indices of them were well within normal. Their parents are cousins. It is, therefore, likely that the occurrence of dissecting aneurysm in the above three cases was due to an underlying hereditary disease. Thus familial dissecting aneurysm is suggested.

11 keywords...

hide…