Survival of Children With Critical Congenital Heart Defects in the National Birth Defects Prevention Study

IF 1.6 4区医学 Q4 DEVELOPMENTAL BIOLOGY Birth Defects Research Pub Date : 2024-09-11 DOI:10.1002/bdr2.2394

Nina E. Forestieri, Andrew F. Olshan, Matthew E. Oster, Elizabeth C. Ailes, Michael P. Fundora, Sarah C. Fisher, Charles Shumate, Paul A. Romitti, Rebecca F. Liberman, Wendy N. Nembhard, Suzan L. Carmichael, Tania A. Desrosiers, The National Birth Defects Prevention Study

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Abstract

Background

Critical congenital heart defects (CCHDs) are associated with considerable morbidity and mortality. This study estimated survival of children with nonsyndromic CCHDs and evaluated relationships between exposures of interest and survival by CCHD severity (univentricular or biventricular function).

Methods

This analysis included 4380 infants with CCHDs (cases) born during 1999–2011 and enrolled in the National Birth Defects Prevention Study, a multisite, population-based case–control study of major birth defects. Cases were linked to state death files. Nonparametric Kaplan–Meier survival functions were used to estimate 1- and 5-year survival probabilities overall and by severity group (univentricular/biventricular) stratified by demographic and clinical exposure variables of interest. The log-rank test was used to determine whether stratified survival curves were equivalent. Survival and 95% confidence intervals (CIs) were also estimated using Cox proportional hazards modeling adjusted for maternal age, education, race/ethnicity, study site, and birth year.

Results

One- and five-year survival rates were 85.8% (CI 84.7–86.8) and 83.7% (CI 82.5–84.9), respectively. Univentricular 5-year survival was lower than biventricular case survival [65.3% (CI 61.7–68.5) vs. 89.0% (CI 87.8–90.1; p < 0.001)]. Clinical factors (e.g. preterm birth, low birthweight, and complex/multiple defects) were associated with lower survival in each severity group. Sociodemographic factors (non-Hispanic Black race/ethnicity, <high school education, smoking, and lower household income) were only associated with survival among biventricular cases.

Conclusions

Mortality among children with CCHDs occurred primarily in the first year of life. Survival was lower for those with univentricular defects, and social determinants of health were most important in predicting survival for those with biventricular defects.

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全国出生缺陷预防研究中先天性心脏病患儿的存活率

背景严重先天性心脏缺陷（CCHD）与相当高的发病率和死亡率有关。本研究估算了非综合征先天性心脏病患儿的存活率，并根据先天性心脏病的严重程度（单心室或双心室功能）评估了相关暴露与存活率之间的关系。方法该分析包括 1999-2011 年间出生的 4380 名患有 CCHD 的婴儿（病例），他们都参加了全国出生缺陷预防研究（National Birth Defects Prevention Study），该研究是一项针对重大出生缺陷的多地点、基于人群的病例对照研究。病例与州死亡档案相关联。采用非参数 Kaplan-Meier 生存函数估算了总体的 1 年和 5 年生存概率，以及根据人口统计学和临床暴露变量分层的严重程度组（单心室/双心室）的生存概率。对数秩检验用于确定分层生存曲线是否相同。此外，还根据产妇年龄、教育程度、种族/民族、研究地点和出生年份，使用 Cox 比例危险度模型估算了存活率和 95% 置信区间 (CI)。结果一年和五年存活率分别为 85.8%（CI 84.7-86.8）和 83.7%（CI 82.5-84.9）。单心室病例的 5 年存活率低于双心室病例[65.3% (CI 61.7-68.5) vs. 89.0% (CI 87.8-90.1; p <0.001)]。临床因素（如早产、低出生体重和复杂/多重缺陷）与各严重程度组中较低的存活率有关。社会人口因素（非西班牙裔黑人种族/人种、高中教育程度、吸烟和较低的家庭收入）仅与双心室病例的存活率有关。结论 CCHD患儿的死亡主要发生在出生后的第一年。单心室缺损患儿的存活率较低，而健康的社会决定因素对预测双心室缺损患儿的存活率最为重要。

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来源期刊

Birth Defects Research Medicine-Embryology

CiteScore

3.60

自引率

9.50%

发文量

153

期刊介绍： The journal Birth Defects Research publishes original research and reviews in areas related to the etiology of adverse developmental and reproductive outcome. In particular the journal is devoted to the publication of original scientific research that contributes to the understanding of the biology of embryonic development and the prenatal causative factors and mechanisms leading to adverse pregnancy outcomes, namely structural and functional birth defects, pregnancy loss, postnatal functional defects in the human population, and to the identification of prenatal factors and biological mechanisms that reduce these risks. Adverse reproductive and developmental outcomes may have genetic, environmental, nutritional or epigenetic causes. Accordingly, the journal Birth Defects Research takes an integrated, multidisciplinary approach in its organization and publication strategy. The journal Birth Defects Research contains separate sections for clinical and molecular teratology, developmental and reproductive toxicology, and reviews in developmental biology to acknowledge and accommodate the integrative nature of research in this field. Each section has a dedicated editor who is a leader in his/her field and who has full editorial authority in his/her area.