• Shoji Haruta, Kazue Gunji, Toshiharu Kawamura, and Kenzo Hiroshima.
• Department of Cardiology, Tokyo Women's Medical University, Yachiyo Medical Center, Japan. Electronic address: haruta.shoji@twmu.ac.jp.
• J Natl Med Assoc. 2019 Oct 1; 111 (5): 563-568.

AbstractPolycystic kidney disease (PKD) is a multiple cystic disease involving both the kidneys. Some studies have reported cases of patients with PKD and concurrent aortic dissection; however, autopsy has been performed in only few of these cases. Here, we present the case of a 62-year-old male patient with PKD who showed generalized vascular degeneration, including aortic dissection. The patient had a family history of autosomal dominant PKD and was brought to our hospital because of cardiopulmonary arrest. He was diagnosed with Stanford type A aortic dissection and died on the same day, despite being under cardiopulmonary resuscitation. Autopsy detected multiple cysts in the kidneys, liver, pancreas, and testes. Moreover, multiple tears in the vascular wall of the splenic artery and superior mesenteric artery, including the aorta, were observed. The case findings indicate that patients with PKD may develop associated generalized vascular disease; however, development of cerebral aneurysms and aortic dissections with PKD is particularly serious. Therefore, suitable screening tests must be developed for the early diagnosis and disease characterization, thus, ensuring that the appropriate treatment is administered to the patients.Copyright © 2019 National Medical Association. Published by Elsevier Inc. All rights reserved.

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