Regional Cardiovascular Center, Division of Cardiology, Department of Internal Medicine, Chungbuk National University School of Medicine, Cheongju, Korea.
Korean Circ J. 2012 Feb;42(2):136-9. doi: 10.4070/kcj.2012.42.2.136. Epub 2012 Feb 27.
Aortic aneurysm is one several well-known cardiovascular complications in patients with autosomal dominant polycystic kidney disease (ADPCKD). Commonly affected site of aortic aneurysm and its related dissection in ADPCKD is abdominal aorta. Long standing hypertension, haemodialysis, old age are closely related with discovering of aortic aneurysm and dissection in ADPCKD. However, thoracic aortic aneurysms and its related severe aortic regurgitations (ARs) are rare in younger patients suffering from ADPCKD, especially ones who have normal renal function. Here, we report a case involving a 27-year-old Asian male patient with severe AR due to an ascending aneurysm of the thoracic aorta associated with ADPCKD. The patient had normal renal function without Marfan's habitus. The AR and thoracic aortic aneurysm were corrected surgically.
主动脉瘤是常染色体显性多囊肾病(ADPCKD)患者几种已知的心血管并发症之一。ADPCKD 患者中,主动脉瘤及其相关夹层常见的受累部位是腹主动脉。长期高血压、血液透析、高龄与 ADPCKD 患者中发现的主动脉瘤和夹层密切相关。然而,在年轻的 ADPCKD 患者中,尤其是那些肾功能正常的患者,胸主动脉瘤及其相关严重主动脉瓣反流(AR)较为罕见。在此,我们报告了 1 例因 ADPCKD 引起的升主动脉瘤导致严重 AR 的 27 岁亚洲男性患者。该患者肾功能正常,无马凡氏体型。通过手术矫正了 AR 和胸主动脉瘤。