Birth Defects Monitoring Program, State Center for Health Statistics, Division of Public Health, North Carolina Department of Health and Human Services, Raleigh, North Carolina, USA.
Department of Epidemiology, Gillings School of Global Public Health, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
Birth Defects Res. 2024 Sep;116(9):e2394. doi: 10.1002/bdr2.2394.
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).
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.
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.
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.
严重先天性心脏缺陷（CCHD）与相当高的发病率和死亡率有关。本研究估计了非综合征性 CCHD 患儿的生存率，并通过 CCHD 严重程度（单心室或双心室功能）评估了感兴趣的暴露因素与生存率之间的关系。
本分析纳入了 1999 年至 2011 年间出生的 4380 例 CCHD 患儿（病例），并参加了国家出生缺陷预防研究，这是一项多地点、基于人群的主要出生缺陷病例对照研究。病例与州死亡档案相关联。使用非参数 Kaplan-Meier 生存函数估计整体和严重程度组（单心室/双心室）的 1 年和 5 年生存率，严重程度组按感兴趣的人口统计学和临床暴露变量分层。对数秩检验用于确定分层生存曲线是否等效。使用 Cox 比例风险模型估计生存率和 95%置信区间（CI），该模型调整了母亲年龄、教育程度、种族/族裔、研究地点和出生年份。
1 年和 5 年生存率分别为 85.8%（CI 84.7-86.8）和 83.7%（CI 82.5-84.9）。单心室 5 年生存率低于双心室病例生存率[65.3%（CI 61.7-68.5）与 89.0%（CI 87.8-90.1；p<0.001）]。在每个严重程度组中，临床因素（例如早产、低出生体重和复杂/多种缺陷）与生存率较低相关。社会人口学因素（非西班牙裔黑人种族/族裔、<高中学历、吸烟和低家庭收入）仅与双心室病例的生存率相关。
CCHD 患儿的死亡率主要发生在生命的第一年。单心室缺陷患儿的生存率较低，而健康的社会决定因素对预测双心室缺陷患儿的生存率最为重要。
