Kirklin J W, Blackstone E H, Shimazaki Y, Maehara T, Pacifico A D, Kirklin J K, Bargeron L M
Department of Surgery, University of Alabama, Birmingham School of Medicine 35294.
J Thorac Cardiovasc Surg. 1988 Jul;96(1):102-16.
Among 139 patients who underwent repair of tetralogy with pulmonary atresia, survival rates at 1 month and at 1, 5, 10, and 20 years were 85%, 82%, 76%, 69%, and 58%, respectively. The hazard function (instantaneous risk of dying) was greatest immediately after operation and declined thereafter, but a low constant hazard persisted for as long as the patients were followed up. Multivariately, the postrepair ratio between peak right ventricular and left ventricular pressures measured in the operating room provided the most information relative to the probability of death after repair, and cardiopulmonary bypass time the next. When morphologic abnormalities of the pulmonary circulation were considered in the multivariate analysis for risk factors for death, the size of the pulmonary arteries provided the most information, followed by the number of large aortopulmonary collateral arteries. The postrepair peak right ventricular/left ventricular pressure ratio was lower the day after operation than in the operating room in 65% of the patients in whom the measurements were made. Recurrent or residual ventricular septal defects necessitating rerepair occurred in four patients (3% of hospital survivors). Most surviving patients were in New York Heart Association class I at the time of follow-up.
Early, intermediate, and long-term survival is less good after repair of tetralogy with pulmonary atresia than after repair of tetralogy with pulmonary stenosis. This is related primarily to the greater prevalence of high peak right ventricular/left ventricular pressure ratio measured in the operating room in the former group. Both the postrepair peak right ventricular/left ventricular pressure ratio in the operating room and the probability of death are inversely related to the size of the pulmonary arteries and directly to the number of large aortopulmonary collateral arteries. This and inferences from other risk factors may be helpful in achieving better results in the future.
在139例接受肺动脉闭锁型法洛四联症修复术的患者中，1个月及1、5、10和20年的生存率分别为85%、82%、76%、69%和58%。风险函数（即时死亡风险）在术后即刻最高，随后下降，但在整个随访期间一直存在较低的恒定风险。多因素分析显示，手术室测量的右心室与左心室压力峰值的修复后比值提供了与修复后死亡概率最相关的信息，其次是体外循环时间。在死亡危险因素的多因素分析中考虑肺循环形态异常时，肺动脉大小提供的信息最多，其次是大的主肺动脉侧支动脉数量。在进行测量的患者中，65%的患者术后第一天的修复后右心室/左心室压力峰值比值低于手术室测量值。4例患者（占医院存活者的3%）出现复发性或残余室间隔缺损，需要再次修复。大多数存活患者在随访时纽约心脏协会心功能分级为I级。
肺动脉闭锁型法洛四联症修复术后的早期、中期和长期生存率低于肺动脉狭窄型法洛四联症修复术后。这主要与前一组在手术室测量的右心室/左心室压力峰值较高的患病率有关。手术室的修复后右心室/左心室压力峰值比值和死亡概率均与肺动脉大小呈负相关，与大的主肺动脉侧支动脉数量呈正相关。这一点以及其他危险因素的推论可能有助于未来取得更好的结果。
