Survival of Children With Hypoplastic Left Heart Syndrome.
Survival of Children With Hypoplastic Left Heart Syndrome.
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DOI：
10.1542/peds.2014-1427
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发表时间：
2015-10
期刊：
影响因子：
8
通讯作者：
Correa A
中科院分区：
文献类型：
--
作者：
Siffel C;Riehle-Colarusso T;Oster ME;Correa A
利用基于人群的数据研究左心发育不全综合征（HLHS）婴儿的生存率以及人口学和临床特征对生存率的潜在影响。1979年至2005年间出生的非综合征性HLHS婴儿（n = 212）是通过亚特兰大大都会先天性缺陷计划确定的。通过与生命记录的联系，确定了2009年的生命状态。我们根据选择的人口学和临床特征估计Kaplan-Meier生存率。到2009年，总生存率为24%，随着时间的推移显著提高：从1979-1984年的0%到1999-2005年的42%。第一周生存率为66%，第一年生存率为27%，前10年生存率为24%。出生体重过低、出生体重过低或早产的婴儿以及出生在高度贫困社区的婴儿的存活率明显较低。对于有手术干预信息的儿童（n = 88），总生存率为52%，早产儿的生存率（31%）明显低于足月婴儿（56%）。存活至1岁的儿童，长期生存率约为90%。近年来，亚特兰大市出生的非综合征型HLHS儿童到青春期的生存率显著提高，足月出生、出生体重正常或生活在低贫困社区的儿童生存率更高。从婴儿期到青春期的存活率很高。需要更好地了解越来越多的HLHS幸存者，以便为资源规划提供信息。
To examine the survival of infants with hypoplastic left heart syndrome (HLHS) and potential influence of demographic and clinical characteristics on survival using population-based data. Infants with nonsyndromic HLHS (n = 212) born between 1979 and 2005 were identified through the Metropolitan Atlanta Congenital Defects Program. Vital status was ascertained through 2009 based on linkage with vital records. We estimated Kaplan-Meier survival probabilities stratified by select demographic and clinical characteristics. The overall survival probability to 2009 was 24% and significantly improved over time: from 0% in 1979–1984 to 42% in 1999–2005. Survival probability was 66% during the first week, 27% during the first year of life, and 24% during the first 10 years. Survival of very low and low birth weight or preterm infants and those born in high-poverty neighborhoods was significantly poorer. For children with information on surgical intervention (n = 88), the overall survival was 52%, and preterm infants had significantly poorer survival (31%) compared with term infants (56%). For children who survived to 1 year of age, long-term survival was ~90%. Survival to adolescence of children with nonsyndromic HLHS born in metropolitan Atlanta has significantly improved in recent years, with those born full term, with normal birth weight, or in a low-poverty neighborhood having a higher survival probability. Survival beyond infancy to adolescence is high. A better understanding of the growing population of survivors with HLHS is needed to inform resource planning.