49例儿童先天性主动脉瓣狭窄的外科治疗

Surgical repair for congenital aortic stenosis in children： results of 49 cases

目的回顾性总结先天性主动脉瓣狭窄患儿手术治疗的效果及经验。方法2006年2月至2011年11月，共收治49例先天性主动脉瓣狭窄患儿，男29例，女20例；年龄1个月-14岁；体质量3．2～47．0kg。轻度狭窄2例，中度狭窄21例，重度狭窄26例。术前跨瓣压差45—123mmHg（1mmHg=0．133kPa），平均（74．9±20．4）mmHg。单纯主动脉瓣狭窄14例，伴有中-重度反流4例。伴其他心内畸形33例，包括室间隔缺损、房间隔缺损、动脉导管未闭、主动脉缩窄、左心室流出道狭窄、二尖瓣瓣上环等。行主动脉瓣交界切开术31例，主动脉瓣成形术9例，主动脉瓣置换术2例，Ross手术1例，Ross．Konno手术2例，Konno＋主动脉瓣置换术4例。术后随访2～55个月，平均20个月。评估患儿心功能、主动脉瓣跨瓣压差及瓣膜反流程度。结果死亡1例，生存患儿术后心功能良好，左心室射血分数0．69±0．10，短轴缩短分数0．38±0．09。术后跨瓣压差20～73mmHg，平均（38．6±15．8）mmHg，较术前降低（36．2±18．3）mmHg（P〈0．001）。40例患儿行主动脉瓣交界切开和成形，术后主动脉瓣反流程度为无或轻微8例，轻度25例，中度7例。1例主动脉瓣交界切开术后因切开处复粘连再次行交界切开术。结论先天性主动脉瓣狭窄的患儿如无明显反流，可行主动脉瓣交界切开术或同时行主动脉瓣成形术，如果反流中度以上，则需根据患儿年龄等情况选择合适的手术方法。

Objective To studiy the surgical outcomes of children with congenital aortic stenosis （AS） , and summarized the experience of surgical procedures. Methods From February 2006 to November 2011, a total of 49 consecutive children with AS underwent surgical treatments. Twenty-nine patients were male and 20 patients were female. The median age was 17 months （ 1 month to 14 years） , and median weight was 15.6 kg （3.2 -47.0 kg）. Peak AS gradients was （74. 9 ±20. 4） mmHg （45 -123 mm Hg） before surgery. Fourteen patients had isolated congenital AS, 4 had combined moderate-to-severe aortic insufficiency （AI）. Thirty-Three patients had associated cardiac anomalies, including ventricular septal defect, atrial septal defect, patent ductus ateriosus, coarctation of aorta, subaortic stenosis, and supravalvular mitral stenosis. Thirty-one pa- tients underwent aortic valvotomy, 9 valvuloplasty, 2 Aortic valve replacement （AVR）, 1 Ross procedure, 2 Ross-Konno pro- cedure, and 4 Konno/AVR. Median follow-up was 20 months （2 -55 months）. Clinical and echocardiographic follow-up data were analyzed. Results There was 1 postoperative death who died of heart failure due to severe mitral valve insuffciency. Lat- est follow-up data showed that the survivals performed in satisfactory cardiac function. Left ventricular ejection fraction （LVEF） was 0.69 ± 0.10, and fraction shortening （FS） was 0.38 ± 0.09. Peak AS gradient＇s decreased significantly after surgery to （38.6 ± 15.8 ） mm Hg （20 - 73 mm Hg）, P 〈 0. 001. One patient needed reintervention because of severe recurrence of ad- hesion in the commissure 3 months after the previous surgery. Totally 31 patients underwent simple valvotomy procedure. Post- operative AI was none or trace in 5, mild in 20, and moderate in 6. Two surgical techniques were used to repair the aortic valve according to the anatomy of the valve and the cause of insufficiency, including commissuroplasty in 6 and pericarial casp exten- sion in 3. Nine patients underwent valvuloplasty procedure, post-operative AI was none or trace in 3, mild in 5, and moderate in 1. We put more attention to the 7 patients who had moderate AI valvotomy or valvuloplasty, and the latest follow-up showed that these patients were in good condition and there was no deterioration the degree of AI. Conclusion Long-term outcomes show that aortic valvotomy and valvuloplasty are reliable and effective approaches for congenital AS in children. When moder- ate-to-severe AI exists, other procedures such as Ross, Ross-Konno, AVR, or Konno/AVR are preferred.