Abstract
We report the case of 2 brothers with autosomal dominant polycystic kidney disease (ADPKD) with acute aortic dissection. Case 1 (the older brother) had been undergoing kidney dialysis since age 48 years. On the development of acute aortic dissection (type A) at age 50, he underwent ascending aortic replacement. Case 2 (the younger brother) the development of acute aortic dissection (type A) at age 58, he underwent an emergency operation consisting of ascending aortic replacement and partial arch replacement. To the best of our knowledge, a case of brothers with ADPKD complicated by aortic dissection has not been reported in the literature. Hypertension resulting from abnormal vascular function due to genetic mutation and a fragile vascular structure caused by extracellular matrix abnormalities may contribute to aortic dissection in patients with ADPKD. Therefore, strict control of blood pressure is mandatory in patients with ADPKD.
