Meza James M, Hickey Edward, McCrindle Brian, Blackstone Eugene, Anderson Brett, Overman David, Kirklin James K, Karamlou Tara, Caldarone Christopher, Kim Richard, DeCampli William, Jacobs Marshall, Guleserian Kristine, Jacobs Jeffrey P, Jaquiss Robert
Division of Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario.
Division of Pediatric Cardiology, The Hospital for Sick Children, Toronto, Ontario.
Ann Thorac Surg. 2018 Jan;105(1):193-199. doi: 10.1016/j.athoracsur.2017.05.041. Epub 2017 Aug 25.
The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival.
The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factors for death after the Norwood and after S2P were identified. Based on the multivariable models, infants were stratified as low, intermediate, or high risk. Cumulative 2-year, post-Norwood survival was predicted. Optimal timing was determined using conditional survival analysis and plotted as 2-year, post-Norwood survival versus age at S2P.
A Norwood operation was performed in 534 neonates from 21 institutions. The S2P was performed in 71%, at a median age of 5.1 months (IQR: 4.3 to 6.0), and 22% died after Norwood. By 5 years after S2P, 10% of infants had died. For low- and intermediate-risk infants, performing S2P after age 3 months was associated with 89% ± 3% and 82% ± 3% 2-year survival, respectively. Undergoing an interval cardiac reoperation or moderate-severe right ventricular dysfunction before S2P were high-risk features. Among high-risk infants, 2-year survival was 63% ± 5%, and even lower when S2P was performed before age 6 months.
Performing S2P after age 3 months may optimize survival of low- and intermediate-risk infants. High-risk infants are unlikely to complete three-stage palliation, and early S2P may increase their risk of mortality. We infer that early referral for cardiac transplantation may increase their chance of survival.
二期姑息治疗（S2P）的时机对单心室姑息治疗后生存的影响尚不清楚。本研究调查了能使S2P前的损耗最小化并使S2P后的生存率最大化的S2P最佳时机。
使用先天性心脏病外科医生协会的严重左心室流出道梗阻队列。采用多阶段参数风险分析进行生存分析。确定了诺伍德手术（Norwood）后和S2P后死亡的独立风险因素。基于多变量模型，将婴儿分为低、中、高风险组。预测诺伍德手术后2年的累积生存率。使用条件生存分析确定最佳时机，并绘制为诺伍德手术后2年生存率与S2P时年龄的关系图。
来自21家机构的534例新生儿接受了诺伍德手术。71%的患儿进行了S2P，中位年龄为5.1个月（四分位间距：4.3至6.0），22%的患儿在诺伍德手术后死亡。到S2P后5年，10%的婴儿死亡。对于低风险和中风险婴儿，3个月龄后进行S2P的2年生存率分别为89%±3%和82%±3%。在S2P前进行心脏再次期间再次间隔再次手术或存在中度至重度右心室功能障碍是高风险特征。在高风险婴儿中，2年生存率为63%±5%，在6个月龄前进行S2P时生存率甚至更低。
3个月龄后进行S2P可能会优化低风险和中风险婴儿的生存。高风险婴儿不太可能完成三阶段姑息治疗，早期进行S2P可能会增加其死亡风险。我们推断，早期转诊进行心脏移植可能会增加他们的生存机会。
