Shah Shawn, Asante-Korang Alfred, Ghazarian Sharon R, Stapleton Gary, Herbert Carrie, Decker Jamie, Almodovar Melvin C, Karl Tom R, Do Nhue L, Quintessenza James A, Mavroudis Constantine, Vricella Luca A, van Gelder Hugh M, Kartha Vyas, Alexander Plato, Carapellucci Jennifer, Krasnopero Diane, Hanson Jade, Amankwah Ernest, Roth Joeli, Jacobs Jeffrey P
1 Divisions of Cardiovascular Surgery, Cardiology, Cardiac Critical Care, Cardiac Anesthesia, Johns Hopkins All Children's Hospital, Johns Hopkins All Children's Hospital, Saint Petersburg, Tampa, and Orlando, FL, USA.
2 Health Informatics, Johns Hopkins All Children's Hospital, Saint Petersburg, FL, USA.
World J Pediatr Congenit Heart Surg. 2018 Sep;9(5):557-564. doi: 10.1177/2150135118782190.
This article reviews all patients who underwent heart transplantation (HTx) within a single institution (172 patients underwent 179 HTx [167 first-time HTxs, 10 second HTxs, 2 third HTxs]) to describe diagnostic characteristics, management protocols, and risk factors for mortality.
Descriptive analysis was performed for the entire cohort using mean, standard deviation, median, interquartile range, and overall range, as appropriate. Univariable and multivariable Cox proportional hazards models were performed to identify prognostic factors for outcomes over time. The primary outcome of interest was mortality, which was modeled by Kaplan-Meier analysis.
Median age at HTx was 263 days (range, 5 days to 24 years; mean = 4.63 ± 5.95 years; 18 neonates, 79 infants). Median weight at HTx was 7.5 kg (range, 2.2-113 kg; mean = 19.36 ± 23.54). Diagnostic categories were cardiomyopathy (n = 62), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 33), transplantation after cardiac surgery for HLHS or HLHS-related malformation (n = 17), non-HLHS congenital heart disease (n = 55), and retransplant (n = 12). Operative mortality was 10.1% (18 patients). Cumulative total follow-up is 1,355 years. Late mortality was 18.4% (33 patients). Overall Kaplan-Meier five-year survival was 76.2%. One hundred twenty-one patients are alive with a mean follow-up of 7.61 ± 6.46 years. No survival differences were seen among the five diagnostic subgroups ( P = .064) or between immunosensitized patients (n = 31) and nonimmunosensitized patients (n = 141; P = .422).
Excellent results are expected for children undergoing HTx with comparable results among diagnostic groups. Pretransplant mechanical circulatory support and posttransplant mechanical circulatory support are risk factors for decreased survival. Survival after transplantation for HLHS or HLHS-related malformation is better with primary HTx in comparison to HTx after prior cardiac surgery.
本文回顾了在单一机构接受心脏移植（HTx）的所有患者（172例患者接受了179次心脏移植[167例首次心脏移植，10例二次心脏移植，2例三次心脏移植]），以描述诊断特征、管理方案和死亡风险因素。
对整个队列进行描述性分析，酌情使用均值、标准差、中位数、四分位数间距和全距。进行单变量和多变量Cox比例风险模型以确定随时间变化的预后因素。感兴趣的主要结局是死亡率，通过Kaplan-Meier分析进行建模。
心脏移植时的中位年龄为263天（范围为5天至24岁；均值 = 4.63 ± 5.95岁；18例新生儿，79例婴儿）。心脏移植时的中位体重为7.5 kg（范围为2.2 - 113 kg；均值 = 19.36 ± 23.54）。诊断类别包括心肌病（n = 62）、因左心发育不全综合征（HLHS）或HLHS相关畸形进行的初次移植（n = 33）、因HLHS或HLHS相关畸形进行心脏手术后的移植（n = 17）、非HLHS先天性心脏病（n = 55）和再次移植（n = 12）。手术死亡率为10.1%（18例患者）。累计总随访时间为1355年。晚期死亡率为18.4%（33例患者）。总体Kaplan-Meier五年生存率为76.2%。121例患者存活，平均随访时间为7.61 ± 6.46年。五个诊断亚组之间（P = 0.064）或免疫致敏患者（n = 31）与非免疫致敏患者（n = 141；P = 0.422）之间未观察到生存差异。
接受心脏移植的儿童预期会有良好的结果，各诊断组之间结果相当。移植前机械循环支持和移植后机械循环支持是生存降低的风险因素。与先前心脏手术后的心脏移植相比，因HLHS或HLHS相关畸形进行初次心脏移植后的生存率更高。
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