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

描述(申请人提供)：自20世纪40年代以来，先天性心脏病(CHD)的外科手术和其他介入和诊断技术的引入，为患者的生存开辟了一条超越其出生条件所强加的界限的道路。随着这些进步，大约85%-90%的先天性心脏病婴儿有望成年，在美国，修复或缓解先天性心脏病的成年人人数据信超过100万人。预计这些冠心病幸存者将经历与普通人群不同的发病率和死亡原因。由于冠心病患者命运的戏剧性变化是一个相对较新的事件，有关患者在冠心病干预后存活的较长期改变的病史的数据在很大程度上仍不清楚。我们的目标是研究冠心病干预后大量患者的死亡模式和死亡原因。我们将使用儿科心脏护理联盟(PCCC)的数据，该联盟是世界上最长的儿科心脏干预结果登记机构，也是唯一一个包括美国27个州48个中心的心脏、经导管和电生理程序数据的机构。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因。我们将把这两个数据集与可用的直接识别符联系起来，以确定已修复或缓解的冠心病患者的重要生命状态以及直接和潜在的死亡原因。合并后的数据集将被用来比较接受修复或缓解的冠心病患者1-30年的总体死亡率和特定原因死亡率与普通美国人以及最常见的冠心病的特定类型之间的死亡率。此外，我们将计算冠心病患者的直接和潜在死因，并将其与普通人群的数据进行比较。这项研究的完成将为冠心病修复或姑息治疗后的存活率提供有价值的数据。需要长期的数据来指导这一快速增长的人口的管理，并教育患者及其家人关于他们的预期结果和不同管理策略的比较有效性数据。此外，该项目可能会确定冠心病幸存者的致命疾病发展风险，它可能有助于了解和预防或最大限度地减少冠心病妇女的妊娠风险。这一知识可用于修改致病风险因素或增加对特定类别患者的针对性监测。

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.