Familial Type A Aortic Dissection in Two Sisters within One Week in a Family without Marfan’s Syndrome, Suggestive of Familial Aortic Aneurysm

We describe a large family in which 13 members over two generations developed aortic dissection or aortic aneurysm at a young age. Two sisters of this family presented with type A aortic dissection within one week with no previous medical history, Marfan’s syndrome or other connective tissue disorders. Their 2D echocardiographic images were unique with floating tissue in the false lumen. Hereditary disorders, familial aortic aneurysms cause the individuals to develop an enlargement of the aorta. The aneurysm may occur anywhere, dilation at the level of the ascending aorta or the sinus of Valsalva, and it may progress over time to eventually tear or rupture. In both of our cases, the intimal flap started from the annulus and extended to the abdominal aorta, with severe aortic regurgitation. The Bentall procedure was performed for both of our patients; one of them died during the operation due to massive hemorrhage, but the other one tolerated surgery well. All their family members were subsequently screened; there was no medical history of Marfan’s syndrome or other connective tissue diseases. Echocardiography, however, showed varying degrees of dilatation of the ascending aorta in most of the first and second-degree relatives with mild to moderate AI, suggestive of familial aortic aneurysms (Iranian Heart Journal 2007; 8 (4): 57-59).