Leier C V, Baker P B, Kilman J W, Wooley C F
Ann Intern Med. 1984 May;100(5):683-8. doi: 10.7326/0003-4819-100-5-683.
In a combined retrospective and prospective study, 11 patients with adult polycystic kidney disease were found to have one or more cardiac or aortic lesions. Seven patients had primary dilatation of the aortic root and annulus with aortic regurgitation. The severity of the aortic regurgitation necessitated aortic valve replacement in 2. Mitral regurgitation was present in 3 patients, of whom 2 had documented redundant mitral leaflets and ruptured chordae tendinae, and the third had mitral valve prolapse. Histologic analysis of available aortic and mitral valve tissue from these acquired lesions showed myxomatous degeneration with loss and disruption of collagen. Four patients had congenital bicuspid aortic valves with aortic regurgitation; 1 of these patients also had mild valvular stenosis, and 1 had coarctation of the aorta.
在一项回顾性与前瞻性相结合的研究中，发现11例成人多囊肾病患者存在一处或多处心脏或主动脉病变。7例患者有主动脉根部和瓣环原发性扩张并伴有主动脉瓣反流。2例患者因主动脉瓣反流严重而需要进行主动脉瓣置换。3例患者存在二尖瓣反流，其中2例记录有二尖瓣叶冗长和腱索断裂，第3例有二尖瓣脱垂。对这些后天性病变获取的主动脉瓣和二尖瓣组织进行组织学分析，显示为黏液样变性，伴有胶原蛋白丢失和破坏。4例患者有先天性二叶式主动脉瓣并伴有主动脉瓣反流；其中1例患者还伴有轻度瓣膜狭窄，1例有主动脉缩窄。
