Long-term Outcomes after Interventions for Congenital Heart Disease

项目总结/摘要 先天性心脏病（CHD）影响了近1%的美国（US）出生的儿童，其中许多儿童 他们需要心脏手术以避免死亡或严重发病。外科治疗进展 的冠心病导致冠心病幸存者人数不断增加，目前世界上有200多万冠心病患者。 美国，其中一半以上是成年人。虽然成年后的存活率对于CHD来说是极好的， 对于许多CHD类型的患者，死亡率只是在几十年内发生了变化，现在发生在成年期。 此外，随着年龄的增长，这些患者持续暴露于额外的心血管和非心血管疾病。 心血管疾病的发病率沿着增加，这些疾病的风险可能更高， 预期它们会产生协同损伤，导致额外的过早死亡。重要的是， 众所周知，教育程度，就业和社会关系，其中越来越多的 人口了解长期结果对于确定干预机会和 为这类资源利用率高的人群规划卫生保健服务。 我们的项目旨在填补与冠心病患者长期结局相关的重要空白， 儿科心脏护理联盟（PCCC）是一家大型的美国CHD干预登记中心， 国家死亡指数（NDI）。这种联系使我们能够组成一个队列，描述长期的 冠心病手术患者的死亡率。由于PCCC登记册在规模和范围上是无与伦比的， 该方法提供了美国最强大的数据源，以检查患者的结局轨迹 因CHD手术，现已达到成人年龄。然而，除了死亡率，患者的另一个重要结局是， CHD的各种干预措施的存活是终末期心力衰竭或肺功能衰竭的发展， 高血压导致需要器官移植。为了解决这个问题，我们补充了 数据集与器官获取和移植网络（OPTN）研究登记处联系， 获得关于在同一队列中进行移植和器官移植的信息。在下一阶段 在我们的项目中，死亡和移植数据都将更新到2021年，以评估与以下相关的特征： 存活率和移植率。此外，作为我们项目的一部分，我们将 调查患者及其兄弟姐妹，以确定疾病对长期医疗的影响， CHD幸存者与其兄弟姐妹和一般 并确定可能与这些结果相关的相关预后因素 这项研究的完成将提供一个现实和全面的预期结果后，先天性 心脏手术这些信息将对CHD人群的所有相关利益相关者有用， 促进患者及其家属、护理提供者、卫生保健管理人员和公共卫生的参与 改善这一弱势群体的结果。

Project Summary/Abstract Congenital heart diseases (CHD) affect nearly 1% of children born in the United States (US), with many of them requiring heart surgery to avoid mortality or significant morbidity. Advances in the surgical management of CHDs have led to a growing population of CHD survivors, with now over 2 million persons with CHD in the United States, more than half of whom are adults. While survival to adulthood is now excellent for CHD patients, for many CHD types the mortality has simply shifted a few decades and now occurs in adulthood. Moreover, with age, these patients are continuously exposed to additional cardiovascular and non- cardiovascular morbidities along with the general population, and risk for these morbidities is likely higher as they are expected to create synergistic injury leading to additional premature mortality. Importantly, little is known concerning educational attainment, employment, and social relationships among this growing population. Understanding long-term outcomes is important for identifying opportunities for intervention and planning for health care services in this population with high resource utilization. Our project aims to fill important gaps related to the long-term outcomes for patients with CHD by linking the Pediatric Cardiac Care Consortium (PCCC), a large US-based registry for interventions of CHDs, with the National Death Index (NDI). This linkage allowed us to constitute a cohort that describes the long-term mortality of patients with an operated CHD. Since the PCCC registry is unparalleled in its size and scope, this approach provides the most powerful data source in the US to examine the trajectory of outcomes for patients operated for CHD now reaching adult age. However, besides mortality another important outcome for patients surviving various interventions for CHD is the development of end stage cardiac failure or pulmonary hypertension resulting in the need for organ transplantation. To address this limitation, we supplemented this dataset with linkage with the Organ Procurement and Transplantation Network (OPTN) research registry to obtain information regarding listing for transplant and organ transplants in the same cohort. In the next phase of our project, both death and transplant data will be updated through 2021 to assess characteristics related to survival and transplant within this aging cohort. In addition, as part of the continuation of our project we will survey patients and their siblings to determine the effects of the disease on the long-term medical, neurocognitive, and psychosocial outcomes for survivors of CHD compared to their siblings and the general population and to identify relevant prognostic factors that may be associated with those outcomes Completion of this study will provide a realistic and comprehensive view of expected outcomes after congenital heart surgery. This information will be useful to all relevant stake holders for the population of CHD and will promote engagement of patients and their families, care providers, health care administrators and public health officers to improve outcomes in this vulnerable population.