OBJECTIVE Congenital heart disease (CHD) is a leading cause of premature death in infants and children. Currently limited data are available regarding lesion specific mortality over time. Our study aimed to describe pediatric mortality trends by CHD lesion in the United States. METHODS We conducted a 19 year analysis (1999 to 2017) of publicly available, deidentified multiple cause of death data compiled and produced by the National Center for Health Statistics. Analysis was stratified by CHD diagnosis and age using 3 age categories (infants, 1 to 4 years, and 5 to 17 years). Temporal trends of CHD mortality and the effect of contributing risk factors were analyzed by using joinpoint regression. RESULTS Mortality was highest for in infants for all CHD lesions, in particular for total anomalous pulmonary venous return. Significant declines in infant CHD mortality occurred for most other lesions. Contributing risk factors, including prematurity, extracardiac birth defects, and genetic conditions, occurred in 19% of infant CHD deaths and demonstrated worse mortality trends in the majority of lesions. Mortality rates remained highest for single ventricle lesions in all ages, with an infant mortality rate plateau in the later half of the study and progressive increasing mortality rates for children 5 to 17 years. CONCLUSIONS CHD mortality is decreasing for most lesions. Because of the heterogenicity of CHD lesions, there is expected variability in mortality trends by lesion and age group. Single ventricle lesions continue to contribute most heavily to premature death because of CHD demonstrated by significant increases in mortality rate for children aged 5 to 17 years.

中文翻译：

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儿童病变特异性先天性心脏病死亡率趋势：1999 年至 2017 年。

目的 先天性心脏病 (CHD) 是婴儿和儿童过早死亡的主要原因。目前关于病变特异性死亡率随时间变化的数据有限。我们的研究旨在描述美国 CHD 病变的儿科死亡率趋势。方法 我们对国家卫生统计中心编制和制作的公开可用的、去识别化的多死因数据进行了为期 19 年的分析（1999 年至 2017 年）。根据冠心病诊断和年龄使用 3 个年龄类别（婴儿、1 至 4 岁和 5 至 17 岁）对分析进行分层。使用连接点回归分析了冠心病死亡率的时间趋势和促成危险因素的影响。结果 对于所有 CHD 病变，婴儿的死亡率最高，特别是对于完全异常肺静脉回流。大多数其他病变导致婴儿 CHD 死亡率显着下降。促成危险因素，包括早产、心外出生缺陷和遗传条件，发生在 19% 的婴儿先天性心脏病死亡中，并且在大多数病变中表现出更差的死亡率趋势。所有年龄段的单心室病变死亡率仍然最高，婴儿死亡率在研究的后半段趋于平稳，5 至 17 岁儿童的死亡率逐渐增加。结论 大多数病变的冠心病死亡率正在下降。由于 CHD 病变的异质性，预期死亡率趋势会因病变和年龄组而异。