Schmaltz A A, Schaefer M, Hentrich F, Neudorf U, Brecher A M, Asfour B, Urban A E
Klinik für Kinderkardiologie, Universitätsklinikum Essen, Hufelandstrasse 55, 45122 Essen, Germany.
Z Kardiol. 2004 Mar;93(3):194-200. doi: 10.1007/s00392-004-0015-2.
The development of aortic regurgitation (AI) is a rare but serious complication of subaortic ventricular septal defects (VSD). Over a period of 5 years we observed VSD-related AI in 24 patients, a frequency of 4.5% of all isolated VSD's encountered during that time frame. The location of the defects was in the infundibular septum in 59%, it was perimembranous in 25% and in the trabecular septum in 16%. Hemodynamically the defects were small except for 2 where the Q(p)/Q(s) ratio was > 2. Of the 24 pts, 16 had surgical closure of their VSD accompanied in 9 by aortic valvuloplasty. AI was caused by elongation or defect of the right coronary leaflet in 42%, of the noncoronary leaflet in 25% and a combination of both, in 8%. In 6 pts with infundibular VSD absence of part of the aortic valve ring above the defect was the underlying mechanism for AI. Postoperatively AI was improved to moderate in one pt and to none to trivial in 15. LV end-diastolic diameter decreased significantly in all pts operated. Pathogenetic mechanisms for the development of AI are a deficiency in the aorto-infundibular junction with prolaps of the right-or non-coronary leaflet, deficiency of the valve supporting structures including the valve ring as well as suction of the already elongated leaflet into the VSD with further damage to the antiregurgitant mechanism of the semilunar valve at risk. In perimembranous VSD's, late AI is probably related to turbulent flow through the adjacent LVOT. Surgical closure of isolated VSD's with a location immediately beneath the aortic valve is indicated regardless of their size to prevent the development of AI. If AI has occurred, VSD closure including aortic valvuloplasty improves the amount of regurgitation and normalizes LV enddiastolic dimension.
主动脉瓣反流（AI）的发生是室间隔缺损（VSD）的一种罕见但严重的并发症。在5年的时间里，我们观察到24例与VSD相关的AI，在该时间段内所有孤立性VSD中出现的频率为4.5%。缺损部位位于漏斗部间隔的占59%，位于膜周部的占25%，位于小梁部间隔的占16%。血流动力学上，除2例Q(p)/Q(s)比值>2外，其余缺损均较小。24例患者中，16例行VSD手术闭合，其中9例同时行主动脉瓣成形术。AI由右冠状动脉瓣叶延长或缺损导致的占42%，由无冠状动脉瓣叶导致的占25%，两者合并导致的占8%。6例漏斗部VSD患者中，缺损上方部分主动脉瓣环缺失是AI的潜在机制。术后，1例患者的AI改善为中度，15例患者的AI改善为无反流至微量反流。所有接受手术的患者左心室舒张末期直径均显著减小。AI发生的发病机制包括主动脉-漏斗部连接处不足伴右冠状动脉瓣叶或无冠状动脉瓣叶脱垂、瓣膜支撑结构（包括瓣环）不足，以及已延长的瓣叶被吸入VSD，进一步损害半月瓣的抗反流机制。在膜周部VSD中，晚期AI可能与通过相邻左心室流出道的湍流有关。无论孤立性VSD的大小如何，只要其位置紧邻主动脉瓣下方，均建议进行手术闭合，以防止AI的发生。如果已经发生AI，VSD闭合术（包括主动脉瓣成形术）可改善反流程度并使左心室舒张末期尺寸恢复正常。
