回顾总结1岁以下婴儿法洛四联症(tetralogy of Fallot,TOF)根治术的经验。

1999年1月至2005年8月，58例1岁以下TOF患儿的根治手术。合并房间隔缺损8例、动脉导管未闭3例、冠状动脉异常3例、肺动脉闭锁2例、21-三体综合征2例，既往行改良B-T分流术2例。术中发现室间隔缺损(ventricular septal defect, VSD)嵴下型56例，干下型2例。VSD经右心室修补48例，经右房修补4例，经右房、右室共同修补6例。VSD开窗2例，右心室流出道跨瓣环补片39例。

平均体外循环时间为95. 5 min，主动脉阻断时间为53. 5 min。平均呼吸机辅助通气时间为30 h，重症监护室(intensive care unit, ICU)平均停留时间为4 d，术后平均住院14 d。延迟关胸3例，术后早期死亡2例(3. 4%)，死亡原因分别为严重心排血量低和顽固性交界性心动过速。术后早期主要并发症包括：心排血量低(5例)，肾衰竭(4例)，室上性、交界性心动过速(2例)，Ⅲ°房室传导阻滞(AVB)(1例)。随访3个月至5年，均恢复良好，心功能1〜2级。

婴儿期TOF根治术死亡率低，术后早中期随访并发症少，心功能良好。TOF可常规在婴儿期进行根治。

The purpose of this study was to evaluate the outcome following complete repair of tetralogy of Fallot(TOF)under one year of age.

58 infants with TOF undergoing were completely repaired between January 1999 and August 2005 were reviewed. The concomitant lesion included atrial septal defect in 8 patients, patent ductus arteriosus in 3 patients and abnormal coronary arteries in 3 patients, Down's syndrome in 2 patients. 2 patients had a previous modified B-T shunt. The site of ventricular septal defect(VSD)was perimembranous in 56 patients and subarterial in 2. 2 patients had VSD repair with fenestration. Correction was accomplished through ventriculotomy(48 patients), atriotomy(4 patients)or transatrial-transventricular approach(6 patients). A transannular patch was inserted in 39 patients.

The mean bypass and aortic cross-clamp time were 95. 5 and 53. 5 minutes respectively. The mean ventilation time, ICU stay and postoperative hospital stay were 30 hours, 4 days and 14 days respectively. Delayed sternum closure was performed in 3 patients. There were 2 early deaths(mortality 3. 4%)and no late death. Early postoperative complication included low cardiac output(5 patients), renal failure(4 patients), supraventricular, junctional ectopic tachycardia(2 patients), complete heart block(1 patient), etc. Follow-up was available from 3 months to 5 years postoperatively. All the patients alive were doing well with NYHA functional class I to Ⅱ .

Early complete repair of TOF in infancy is associated with acceptable mortality and morbidity, as well as excellent heart function during early and mid-term follow-up. Elective complete repair of TOF under one year of age is recommended.