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新生儿期先天性心脏病的初次移植:长期结果。

Primary Transplantation for Congenital Heart Disease in the Neonatal Period: Long-term Outcomes.

机构信息

Department of Cardiothoracic Surgery, Loma Linda University, Loma Linda, California.

Department of Cardiothoracic Surgery, Loma Linda University, Loma Linda, California.

出版信息

Ann Thorac Surg. 2019 Dec;108(6):1857-1864. doi: 10.1016/j.athoracsur.2019.06.022. Epub 2019 Jul 27.

DOI:10.1016/j.athoracsur.2019.06.022
PMID:31362016
Abstract

BACKGROUND

Primary transplantation was developed in the 1980s as an alternative therapy to palliative reconstruction of uncorrectable congenital heart disease. Although transplantation achieved more favorable results, its utilization has been limited by the availability of donor organs. This review examines the long-term outcomes of heart transplantation in neonates at our institution.

METHODS

The institutional pediatric heart transplant database was queried for all neonatal heart transplants performed between 1985 and 2017. Follow-up was obtained from medical records and an annually administered questionnaire. Overall survival and time to development of complications were estimated using the Kaplan Meier method. Univariate and multivariate analyses were performed to identify independent predictors of survival.

RESULTS

Heart transplantation was performed in 104 neonates. Median age was 17 days. Hypoplastic left heart syndrome (classic or variant) was the primary diagnosis in 77.8% of patients. Survival at 10 years and 25 years was 73.9% and 55.8%, respectively. At 20 years, freedom from allograft vasculopathy and lymphoproliferative disease was 72.0% and 81.9%, respectively. Freedom from re-transplantation was 81.4% at 20 years. Eight patients (7.6%) developed end-stage renal disease. By multivariate analysis, lower glomerular filtration rate and allograft vasculopathy were the only significant predictors of death.

CONCLUSIONS

Neonatal heart transplantation remains a durable therapy with very acceptable long-term survival. Children transplanted in the newborn period have the potential to reach adulthood with minimal need for reintervention.

摘要

背景

原发性移植术于 20 世纪 80 年代作为一种替代疗法被开发出来,用于姑息性矫正无法纠正的先天性心脏病。尽管移植术取得了更有利的结果,但由于供体器官的可用性有限,其应用受到了限制。本研究旨在回顾本中心新生儿心脏移植的长期结果。

方法

通过查询机构内儿科心脏移植数据库,检索了 1985 年至 2017 年期间所有接受心脏移植的新生儿病例。通过病历和每年进行的问卷调查获得随访信息。采用 Kaplan-Meier 法估计总生存率和发生并发症的时间。采用单变量和多变量分析确定生存的独立预测因素。

结果

共对 104 例新生儿进行了心脏移植。中位年龄为 17 天。左心发育不全综合征(经典型或变异型)是 77.8%患者的主要诊断。10 年和 25 年的生存率分别为 73.9%和 55.8%。20 年时,同种异体移植血管病和淋巴增生性疾病的无复发率分别为 72.0%和 81.9%。20 年时的无再次移植率为 81.4%。8 例(7.6%)患者发展为终末期肾病。多变量分析显示,肾小球滤过率较低和同种异体移植血管病是死亡的唯一显著预测因素。

结论

新生儿心脏移植仍然是一种持久有效的治疗方法,具有非常可接受的长期生存率。在新生儿期接受移植的儿童有潜力在成年后无需再次干预。

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