All Cause Mortality 1-30 Years After Interventions for Congenital Heart Diseases

描述（由申请人提供）：自20世纪40年代以来，先天性心脏病（CHD）的手术和其他介入和诊断技术的引入为患者的生存开辟了道路，使其超越了出生条件所施加的界限。随着这些进展，预计约85-90%的CHD婴儿将进入成年期，并且据信美国患有修复或缓解的CHD的成人人数超过1，000，000。预计这些CHD幸存者的发病率和死亡原因与一般人群不同。由于CHD患者命运的巨大变化是相对较新的事件，因此关于CHD干预后存活患者的长期改变病史的数据在很大程度上仍然未知。我们的目的是研究冠心病介入治疗后的死亡率模式和死亡原因。我们将使用来自儿科心脏护理联盟（PCCC）的数据，PCCC是世界上最长的儿科心脏介入治疗结局登记研究，也是唯一一个包括来自美国27个州48个中心的心脏、经导管和电生理手术数据的登记研究。该项目利用了这一独特的临床数据集和国家死亡指数（NDI），这是美国最准确的死亡登记，包括死亡证明上列出的死亡原因。我们将把这两个数据集与可用的直接标识符联系起来，以确定患有修复或缓解的CHD的个体的重要状态以及直接和潜在的死亡原因。合并数据集将用于比较患有修复或缓解的CHD的个体与一般美国人群以及最常见CHD的特定类型之间的1-30年总体和原因特异性死亡率。此外，我们将计算冠心病患者的直接和潜在死亡原因，并将其与普通人群的数据进行比较。这项研究的完成将提供有关冠心病修复或缓解后生存率的有价值的数据。需要长期数据来指导这一快速增长的人群的管理，并教育患者及其家属了解不同管理策略的预期结果和比较有效性数据。此外，该项目可能会确定CHD幸存者中存在发展风险的致命疾病，并可能有助于了解和预防或最大限度地减少CHD女性的妊娠相关风险。这些知识可用于修改促成风险因素或增加对特定类别患者的目标监测。

DESCRIPTION (provided by applicant): Since the 1940s, the introduction of surgical and other interventional and diagnostic techniques for congenital heart diseases (CHD) opened the way for the survival of patients beyond the boundaries imposed by the condition with which they were born. With these advancements, about 85-90% of infants with CHD are expected to reach adulthood and the number of adults with repaired or palliated CHD is believed to exceed 1,000,000 in the US. These CHD survivors are expected to experience different morbidity and causes of death than the general population. Since the dramatic change in the fate of patients with CHD is a relatively recent event, data about the longer term altered history of patients surviving interventions for CHD remains largely unknown. We aim to study mortality patterns and causes of death in a large cohort of patients after interventions for CHD. We will use data from the Pediatric Cardiac Care Consortium (PCCC), the longest standing registry of outcomes for pediatric cardiac interventions in the world and the only one including data from cardiac, trans- catheter and electrophysiologic procedures from 48 centers in 27 U.S. states. The project leverages this unique clinical dataset and the National Death Index (NDI) which is the most accurate registry of death in the U.S. and includes causes of death as listed on the death certificate. We will link the two datasets with available direct identifiers to determine vital staus and immediate and underlying cause of death in individuals with repaired or palliated CHD. The combined dataset will be used to compare the 1-30 year overall and cause- specific mortality for individuals with repaired or palliated CHD to the general U.S. population and between specific types of the most common CHD. In addition, we will calculate immediate and underlying causes of death for patients with CHDs and compare them with data from the general population. Completion of this study will provide valuable data about survival after repair or palliation of CHD. Long-term data is needed to guide the management of this rapidly growing population and educate patients and their families about their expected outcomes and comparative effectiveness data of different management strategies. In addition, the project may identify fatal conditions at risk for development in survivors with CHD and it may help to understand and prevent or minimize pregnancy associated-risks in women with CHD. This knowledge can be used to modify contributing risk factors or increase targeted surveillance for specific categories of patients.