Bull C, Kostelka M, Sorensen K, de Leval M
Hospital for Sick Children, London, United Kingdom.
J Thorac Cardiovasc Surg. 1994 Feb;107(2):359-66.
The outcome in 135 patients with pulmonary atresia with intact ventricular septum was reviewed in terms of actuarial survival and "suitability" for eventual definitive repair (defined as having a tricuspid valve diameter larger than 2.4 standard deviations below the mean normal beyond the neonatal period). Of patients who underwent an initial closed valvotomy, 50% were dead, 22% were alive and suitable, and the remaining 28% were alive but unsuitable for definitive repair at 5 years. None of 66 patients who underwent primary shunt alone achieved suitability and 52% were dead at 5 years. No mode of neonatal closed valvotomy was consistently satisfactory: in only 10 of 26 survivors with serial measurements did the neonatal valvotomy alone achieve decompression to subsystemic pressures. Only half of the decompressed ventricles achieved growth of the tricuspid valve disproportionate to somatic growth as a result of the neonatal procedure. Strategies for the neonatal treatment of pulmonary atresia with intact ventricular septum should not rely on closed operations on the pulmonary valve to optimized right ventricular growth.
回顾了135例室间隔完整的肺动脉闭锁患者的预后情况，涉及精算生存率以及最终进行确定性修复的“适宜性”（定义为三尖瓣直径比新生儿期后的正常均值低2.4个标准差以上）。接受初次闭式瓣膜切开术的患者中，50%死亡，22%存活且适宜，其余28%存活但5年后不适合进行确定性修复。仅接受初级分流术的66例患者中，无一例达到适宜性，52%在5年后死亡。没有一种新生儿闭式瓣膜切开术模式始终令人满意：在26例有系列测量数据的幸存者中，仅10例单纯新生儿瓣膜切开术实现了减压至低于体循环压力。由于新生儿手术，只有一半减压后的心室实现了三尖瓣生长超过身体生长比例。室间隔完整的肺动脉闭锁新生儿治疗策略不应依赖于对肺动脉瓣进行闭式手术来优化右心室生长。
