完全性房室间隔缺损合并法洛四联症或右心室双出口的双心室矫治

Biventricular repair of complete artioventricular septal defect associated with tetralogy of Fallot or double right ventricular outlets

目的总结完全性房室间隔缺损合并法洛四联症或右心室双出口的外科治疗效果和经验.方法 2013年4月至2017年6月,收治完全性房室间隔缺损合并法洛四联症或右心室双出口患儿10例,男7例,女3例;年龄2个月~13岁,中位年龄6个月;体质量3.7~23.6 kg,中位值6.3 kg.术中均行一期根治术,改良单片法矫治房室间隔缺损3例,改良双片法6例,双片法1例.结果无手术死亡.术后患儿ICU停留2~5天,术后住院7~10天.随访16~65个月,无死亡和再次手术,所有患儿一般情况好,复查超声心动图均未提示左右心室流出道梗阻,左侧房室瓣功能正常2例,轻度反流6例,中度反流1例,重度反流1例.结论完全性房室间隔缺损合并法洛四联症或右心室双出口的外科治疗结果满意,远期需进一步随访.

Objective To summarize the surgical results and experience of patients with complete atrioventricular septal defect associated with tetralogy of Fallot or double right ventricular outlets. Methods From April 2013 to June 2017, 10 patients with complete atrioventricular septal defect associated with tetralogy of Fallot or double right ventricular outlets underwent biventricular repair at Guangzhou Women and Children Medical Center. Seven were male, and 3 were female. The age and body weight at surgery was 2 months to 13 years and 3.7-23.6 kg. Repair was performed with modified one-patch technique in 3 patients, modified two-patch technique in 6 patients, two-patch technique in 1 patient. Results There was no hospital mortality. The ICU stay and hospital stay after operation were 2~5 days and 7~10 days. The follow-up duration was 16 to 65 months. All patients were alive and free from left ventricular outlet obstruction. The left atrioventricular valve function were normal in 2 patients, mild regurgitation in 6 patients, moderate regurgitation in 1 patient and severe regurgitation in 1 patient. Conclusion The outcomes of biventricular repair for patients with complete atrioventricular septal defect associated with Tetralogy of Fallot or double right ventricular outlets were satisfied, and long-term follow-up was demanded.