Di Donato R M, Jonas R A, Lang P, Rome J J, Mayer J E, Castaneda A R
Department of Cardiovascular Surgery, Children's Hospital, Boston, MA 02115.
J Thorac Cardiovasc Surg. 1991 Jan;101(1):126-37.
Our experience with the arterial switch operation for transposition of the great arteries has confirmed the attainability of excellent results with elective neonatal surgery. Up to this time, we have repaired tetralogy of Fallot during the neonatal period only when symptoms, either severe persistent cyanosis or cyanotic spells, have been present. This review assesses the results of such nonelective neonatal correction of tetralogy between 1973 and 1988. Twenty-seven neonates with either symptomatic tetralogy of Fallot or symptomatic tetralogy of Fallot with valvar pulmonary atresia underwent repair. Mean age at repair was 8 +/- 8.4 days and mean weight was 3.0 +/- 0.7 kg. Unsatisfactory palliative shunts had previously been placed elsewhere in four patients. Twenty-five transannular patches and two conduits were used for reconstruction of the right ventricular outflow tract. There were five deaths in the hospital, three of which were due to avoidable technical problems. All deaths occurred in patients with pulmonary artery (Nakata) index less than 150 mm2/m2. One premature child weighing 2.3 kg displayed an absent pulmonary valve-like syndrome after repair and died late of respiratory complications caused by aneurysmal branch pulmonary arteries. Actuarial survival at 5 years was 74%. There was a single rapidly declining hazard phase for death, with the hazard approaching zero at 1 1/2 years after repair. Actuarial freedom from need for reoperation was 76% at 5 years. Postoperative catheterization of 15 long-term survivors showed right ventricular pressure less than 70% systemic in 13 cases. All patients are symptomatically well and functioning in sinus rhythm 1 to 15 years after repair (mean, 5 +/- 4 years). This experience with neonates with symptoms suggests that, if mortality is lower in the absence of symptoms, elective repair of tetralogy of Fallot could be reasonably undertaken during the first months of life.
我们在大动脉转位的动脉调转手术方面的经验证实，择期新生儿手术能够取得优异的效果。截至目前，我们仅在新生儿期出现严重持续性青紫或青紫发作等症状时才对法洛四联症进行修复。本综述评估了1973年至1988年间此类非择期新生儿法洛四联症矫正术的结果。27例有症状的法洛四联症或有症状的法洛四联症合并肺动脉瓣闭锁的新生儿接受了修复手术。修复时的平均年龄为8±8.4天，平均体重为3.0±0.7千克。此前有4例患者在其他部位进行了效果不佳的姑息性分流术。25个跨环补片和2个管道用于右心室流出道重建。住院期间有5例死亡，其中3例是由于可避免的技术问题。所有死亡均发生在肺动脉（中田）指数小于150mm²/m²的患者中。1例体重2.3千克的早产儿修复后出现无肺动脉瓣样综合征，后期死于肺动脉分支动脉瘤引起的呼吸并发症。5年时的精算生存率为74%。死亡有一个单一的快速下降危险阶段，修复后1.5年时危险接近零。5年时无需再次手术的精算自由度为76%。对15例长期存活者进行术后心导管检查发现，13例患者的右心室压力低于体循环压力的70%。所有患者术后症状改善良好，修复后1至15年（平均5±4年）均维持窦性心律。对有症状新生儿的这一经验表明，如果无症状时死亡率较低，那么在出生后的头几个月对法洛四联症进行择期修复是合理的。
