Korean Circ J.  2012 Feb;42(2):136-139. 10.4070/kcj.2012.42.2.136.

A Case of Severe Aortic Valve Regurgitation Caused by an Ascending Aortic Aneurysm in a Young Patient With Autosomal Dominant Polycystic Kidney Disease and Normal Renal Function

• Kim J
• Kim SM
• Lee SY
• Lee HC
• Bae JW
• Hwang KK
• Kim DW
• Cho MC
• Byeon SJ
• Kim KB

• KMID: 1826389
• DOI: http://doi.org/10.4070/kcj.2012.42.2.136

Abstract

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.

Keyword

Aortic regurgitation; Aortic aneurysm, thoracic; Polycystic kidney, autosomal dominant

MeSH Terms

Adult
Aneurysm
Aorta, Abdominal
Aorta, Thoracic
Aortic Aneurysm
Aortic Aneurysm, Thoracic
Aortic Valve
Aortic Valve Insufficiency
Asian Continental Ancestry Group
Humans
Hypertension
Male
Polycystic Kidney, Autosomal Dominant
Stress, Psychological

Figure

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  Fig. 1 Chest X-ray of the patient. Mild cardiomegaly without pulmonary congestion was observed.

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  Fig. 2 Echocardiographic findings for the patient. A: left ventricular enlargement and systolic dysfunction was evident. B: markedly dilated aortic valve orifice and ascending thoracic aorta. C: severe aortic valve regurgitation was noticed on doppler echocardiography.

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  Fig. 3 Cardiac and abdominopelvic CT of the patient. A: dilated ascending thoracic aorta on cardiac CT. B: multiple sized cysts in liver and kidneys were compatible to autosomal dominant poly cystic kidney disease.

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  Fig. 4 Pathologic finding of excised aortic tissue. A: cystic medial necrosis, which is defined as mucoid material accumulation, was noticed in the aortic media (×200, H & E staining). B: the resected aorta showed medionecrosis of smooth muscle cells, change in muscle cell orientation, and elastic fragmentation (×200, elastic fiber staining). H & E: hematoxylin and eosin.

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