目的 观察心内直视镶嵌封堵肌部多发室间隔缺损并同期纠治心脏伴发畸形的临床疗效。方法 40例肌部多发室间隔缺损伴其他心脏畸形患者，于体外循环下行心内直视镶嵌封堵室间隔缺损并同期纠治心脏畸形，分析手术结果与随访情况。结果 手术死亡3例，病死率7.5%。3例患者术后发生肺动脉高压危象，经治疗痊愈；所有患者均无明显残余分流。术后随访6个月～3年，无死亡病例；17例封堵器边缘残留少量左向右分流，3例二尖瓣整形残留轻度反流。结论 心内直视镶嵌封堵肌部多发室间隔缺损操作简便、安全，可缩短体外循环时间，并同期纠治心脏合并畸形，中长期疗效较佳。

Objective To observe the clinical outcomes of hybrid approach under direct vision in management of multiple muscular ventricular septal defects with associated cardiac malformations. Methods Forty patients with multiple muscular ventricular septal defects and associated cardiac malformations were enrolled, and hybrid approach under direct vision and cardiac malformation correction were performed with cardiopulmonary bypass. The results of operation and follow up were analyzed. Results Three patients died during operation, with the mortality of 7.5%. Pulmonary hypertension crisis occurred in 3 patients, and all were cured. No obvious residual shunt was observed in all patients. Patients were followed up for 6 months to 3 years, and no death occurred. Clinically insignificant residual shunt took place in 17 patients, and mild residual regurgitation was observed in 3 patients undergoing mitral valvuloplasty. Conclusion Hybrid approach under direct vision in management of multiple muscular ventricular septal defects, which can shorten the time of cardiopulmonary bypass and correct associated cardiac malformations at the same time, is convenient and safe, and may yield favorable mid-term and long-term outcomes.