Familial Aortic Dissection: A Report of Four Cases in Two Families / 日本心臓血管外科学会雑誌

ABSTRACT

There are rare reports of families with multiple members with aortic dissection in the absence of Marfan syndrome. We encountered four cases of aortic dissection in two families. The aortic dissection occurred in the mother and child of the first family and in sisters of the second family. All cases had systemic hypertension preoperatively and presented Stanford type A aortic dissection. All of them were operated successfully. None of them showed the characteristics of connective tissue disease affecting the skeletal, ocular, and cardiovascular system. However, many members of the two families had systemic hypertension and histopathological examination of the aorta showed cystic medial necrosis in all of the four cases. The present study suggests that the familial aortic dissection may be caused by weakness of the aortic wall related to heredity and systemic hypertension.