Pathogenetic significance of myxomatous degeneration in fenestration-related massive aortic regurgitation
Pathogenetic significance of myxomatous degeneration in fenestration-related massive aortic regurgitation
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DOI：
10.1253/circj.68.439
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发表时间：
2004-05-01
影响因子：
3.3
通讯作者：
Tsuda, M
中科院分区：
文献类型：
--
作者：
Akiyama, K;Hirota, J;Tsuda, M
研究背景主动脉瓣返流有多种机制，本研究探讨了其与主动脉瓣开窗术的临床病理关系。方法和结果6例男性患者接受了主动脉瓣置换术。其临床病理特征有许多相似之处。4例有主动脉瓣返流家族史。所有6例患者均显示主动脉瓣中度至重度粘液瘤样变性和主动脉瓣环扩大。4例患者有1-2条纤维索断裂，位于右冠状动脉尖。超声心动图检查发现3例有窗瓣断裂的脱垂瓣尖上附着异常纤维索，1例经食管超声心动图检查发现瓣尖连合处有完整的有窗瓣纤维索。结论因粘液瘤样变性导致主动脉瓣环扩大的广泛有窗瓣可引起慢性返流或纤维索断裂后突然恶化。日本人群中开孔相关主动脉瓣返流的发生率增加。男性患者的一个重要致病因素是主动脉瓣尖和瓣环的粘液瘤变性。
Background Aortic valvular regurgitation has several mechanisms and the present study investigated its clinicopathological correlations with aortic valve fenestration.Methods and Results Six male patients with massive regurgitation and enlarged fenestrations or ruptured fenestrated fibrous cords underwent aortic valve replacement. The clinicopathological features showed many similarities. Four cases had family histories of aortic regurgitation. All six patients showed moderate to severe myxomatous degeneration of the aortic valve and enlargement of aortic annulus. Four patients had 1-2 ruptured fibrous cords, located at the right coronary cusp. Echocardiographic examinations showed an abnormal fibrous cord attached to the prolapsing cusp in 3 cases with ruptured fenestrated valve and detailed examination by trans-esophageal echocardiography showed an intact fenestrated fibrous cord at the commissure in I case.Conclusion Extensively fenestrated cusps with an enlarged aortic annulus because of myxomatous degeneration can cause chronic regurgitation or sudden deterioration after rupture of the fibrous cord. There is an increasing incidence of fenestration-related aortic regurgitation in the Japanese population. An important pathogenetic factor in male patients is myxomatous degeneration of the aortic cusp and annulus.