National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA (K.F.D., C.E.R., M.E.O., S.L.F.).
Department of Epidemiology, Fay W. Boozman College of Public Health and the Arkansas Center for Birth Defects Research and Prevention, University of Arkansas for Medical Sciences, Little Rock (W.N.N.).
Circulation. 2023 Aug 15;148(7):575-588. doi: 10.1161/CIRCULATIONAHA.123.064400. Epub 2023 Jul 4.
Limited population-based information is available on long-term survival of US individuals with congenital heart defects (CHDs). Therefore, we assessed patterns in survival from birth until young adulthood (ie, 35 years of age) and associated factors among a population-based sample of US individuals with CHDs.
Individuals born between 1980 and 1997 with CHDs identified in 3 US birth defect surveillance systems were linked to death records through 2015 to identify those deceased and the year of their death. Kaplan-Meier survival curves, adjusted risk ratios (aRRs) for infant mortality (ie, death during the first year of life), and Cox proportional hazard ratios for survival after the first year of life (aHRs) were used to estimate the probability of survival and associated factors. Standardized mortality ratios compared infant mortality, >1-year mortality, >10-year mortality, and >20-year mortality among individuals with CHDs with general population estimates.
Among 11 695 individuals with CHDs, the probability of survival to 35 years of age was 81.4% overall, 86.5% among those without co-occurring noncardiac anomalies, and 92.8% among those who survived the first year of life. Characteristics associated with both infant mortality and reduced survival after the first year of life, respectively, included severe CHDs (aRR=4.08; aHR=3.18), genetic syndromes (aRR=1.83; aHR=3.06) or other noncardiac anomalies (aRR=1.54; aHR=2.53), low birth weight (aRR=1.70; aHR=1.29), and Hispanic (aRR=1.27; aHR=1.42) or non-Hispanic Black (aRR=1.43; aHR=1.80) maternal race and ethnicity. Individuals with CHDs had higher infant mortality (standardized mortality ratio=10.17), >1-year mortality (standardized mortality ratio=3.29), and >10-year and >20-year mortality (both standardized mortality ratios ≈1.5) than the general population; however, after excluding those with noncardiac anomalies, >1-year mortality for those with nonsevere CHDs and >10-year and >20-year mortality for those with any CHD were similar to the general population.
Eight in 10 individuals with CHDs born between1980 and 1997 survived to 35 years of age, with disparities by CHD severity, noncardiac anomalies, birth weight, and maternal race and ethnicity. Among individuals without noncardiac anomalies, those with nonsevere CHDs experienced similar mortality between 1 and 35 years of age as in the general population, and those with any CHD experienced similar mortality between 10 and 35 years of age as in the general population.
关于美国先天性心脏病（CHD）患者的长期生存情况，基于人群的信息有限。因此，我们评估了基于人群的美国 CHD 患者从出生到青年期（即 35 岁）的生存模式及其相关因素。
通过 3 个美国出生缺陷监测系统，确定了 1980 年至 1997 年出生的 CHD 患者，通过与死亡记录相链接，确定了截至 2015 年的死亡患者及其死亡年份。Kaplan-Meier 生存曲线、婴儿死亡率（即生命第一年死亡）的调整风险比（aRR）和生命第二年以后的生存的 Cox 比例风险比（aHR）用于估计生存概率和相关因素。标准化死亡率比将 CHD 患者的婴儿死亡率、1 年以上死亡率、10 年以上死亡率和 20 年以上死亡率与普通人群的估计值进行了比较。
在 11695 例 CHD 患者中，总体 35 岁生存率为 81.4%，无合并非心脏畸形的患者生存率为 86.5%，存活至生命第一年的患者生存率为 92.8%。分别与婴儿死亡率和生命第二年以后的生存率降低相关的特征包括严重 CHD（aRR=4.08；aHR=3.18）、遗传综合征（aRR=1.83；aHR=3.06）或其他非心脏畸形（aRR=1.54；aHR=2.53）、低出生体重（aRR=1.70；aHR=1.29）以及西班牙裔（aRR=1.27；aHR=1.42）或非西班牙裔黑人（aRR=1.43；aHR=1.80）母亲种族和民族。CHD 患者的婴儿死亡率（标准化死亡率比=10.17）、1 年以上死亡率（标准化死亡率比=3.29）和 10 年以上及 20 年以上死亡率（两者的标准化死亡率比均≈1.5）均高于普通人群；然而，在排除非心脏畸形患者后，非严重 CHD 患者的 1 年以上死亡率和任何 CHD 患者的 10 年以上及 20 年以上死亡率与普通人群相似。
1980 年至 1997 年出生的 CHD 患者中，80%存活至 35 岁，其严重程度、非心脏畸形、出生体重以及母亲种族和民族存在差异。在无非心脏畸形的患者中，非严重 CHD 患者的 1 至 35 岁死亡率与普通人群相似，任何 CHD 患者的 10 至 35 岁死亡率与普通人群相似。
