婴儿早期法洛四联症的外科治疗

目的总结婴儿早期法洛四联症（TOF）外科治疗经验,探讨TOF早期根治术的相关问题。方法回顾性分析上海市儿童医院2008年6月至2010年8月收治21例婴儿早期（〈6个月）TOF患者的临床资料,其中男14例,女7例;年龄4.86±1.15个月;体重6.84±1.33 kg。均经心脏彩色超声心动图确诊,有4例行CT或磁共振成像（MRI）或右心导管造影术。McGoon比值1.86±0.41,肺动脉指数（PAI）142.54±59.46 mm2/m2。经右心房（19例）或右心室（2例）自体心包补片连续缝合修补室间隔缺损;对肺动脉瓣环Z值〈-1者采用心包补片跨瓣扩大成形（18例）;对接近或基本达到正常Z值者保留瓣环,用心包补片分别扩大右心室流出道（RVOT）和肺总动脉（3例）。结果术后第15 d因心力衰竭死亡1例;1例术后第2 d拔除气管内插管后出现喉头水肿,再插管辅助通气,3 d后顺利撤机;其余患者术后均顺利恢复。18例获得随访,随访9.89±6.47个月,心功能改善（Ross分级Ⅰ~Ⅱ级）;随访心脏超声心动图提示：RVOT压差为21.20±12.27 mm Hg（8.10~45.14 mm Hg）;肺动脉瓣反流（PI）轻度10例,中度5例,无重度患者;2例早期残余室间隔缺损已闭合。与术后早期相比,RVOT压差和PI程度差异均无统计学意义（P〉0.05）,右心功能良好。结论婴儿早期行TOF根治术可取得良好的手术效果;经右心房矫正心内畸形,并保留肺动脉瓣环有利于术后心功能保护。

Surgical Intervention for Tetralogy of Fallot in Early Infancy

Objective To summarize the experiences of surgical intervention for tetralogy of Fallot（TOF） in early infancy and to discuss the relevant issues about primary treatment procedures in the period.Methods We retrospectively analyzed the clinical operative information of 21 patients in their early infancy（less than 6 months） with TOF treated in Children＇s Hospital of Shanghai from June 2008 to August 2010.There were 14 males and 7 females with a mean age of 4.86±1.15 months and a mean body weight of 6.84±1.33 kg.All patients were diagnosed by heart color Doppler ultrasound.Four patients underwent CT or magnetic resonance imaging（MRI） or right heart catheter arteriography examination.The McGoon ratio was 1.86±0.41 and the pulmonary artery index（PAI） was 142.54±59.46 mm2/m2.The ventricular septal defect（VSD） was closed with autologous pericardium using continuous sutures through right atrium（19 cases） or right ventricle（2 cases）.Transannular repair was performed when pulmonary valve annulus was one standard deviation less than the normal Z value（18 cases）.If the annulus diameter approached or reached the normal Z value,the valve annulus was preserved and pericardium was used to enlarge the right ventricular outflow tract（RVOT） and the main pulmonary artery（3 cases）. Results There was one death due to heart failure on the 15th day after operation,one patient had acute laryngeal edema after removal of endotracheal intubation on the second day after operation,and received re-intubation and assisted ventilation for three days.All the other patients recovered well.Eighteen patients were followed up for 9.89±6.47 months.Their heart functions were in modified Ross class I or II.Echocardiography during the follow-up showed that RVOT pressure was 21.20±12.27 mm Hg（8.10-45.14 mm Hg）;pulmonary incompetence（PI） was mild in 10 cases,moderate in 5 cases,and no severe PI occurred.Two cases of residual VSD were spontaneously closed.Compared with the early postoperative period,RVOT pressure and PI levels were not significantly different（P0.05）.Right heart function was good.Conclusion Early complete repair of TOF yields good surgical results.Transatrial repair of intracardiac pathology and retaining pulmonary valve annulus can be safely applied to yield good postoperative right ventricular function.