Acute Aortic Dissection Associated with Adult Autosomal Dominant Polycystic Kidney Disease: Three Case Reports and a Review

Abstract

Adult autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disorder responsible for 8% to 10% of cases of end-stage chronic renal failure. The extrarenal complications of ADPKD kidney include cardiovascular disorders (such as mitral valve prolapse syndrome, bicuspid aortic valve, aortic coarctation, aortic aneurysm, and aortic dissection), intracranial aneurysms, diverticulum of colon, hepatic cysts, inguinal hernia, and urinary calculus. Acute aortic dissection (AAD) associated with ADPKD is life-threatening. There are few reports of ADPKD with AAD. To the best of our knowledge, only 9 cases have been found, including at autopsy, in Japan. We have treated 121 patients with AAD in the last 5 years, and 3 of these 121 patients also had ADPKD in the intensive care unit of Nippon Medical School. We report clinical features of cases of ADPKD with AAD on the basis of 12 cases, including 9 previously reported cases and our 3 cases. AAD requires urgent antihypertensive therapy, which often aggravates renal function. Priority should be given to antihypertensive therapy in emergent cases. In fact, 2 of our patients required hemodialysis owing to antihypertensive therapy for AAD. Close cooperation between cardiologists and nephrologists is essential.