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.

Public Health Relevance

With recent advancements in surgical repair and management of Congenital Heart Disease (CHD), 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 currently in the US. Despite advances in the surgical management, CHD remains a significant cause of societal burden in terms of mortality, long term morbidity, and health care resource utilization. Long-term mortality data is needed to guide the management of this rapidly growing population of patients, to educate patients and their families about expected outcomes and to provide comparative effectiveness data on different management strategies.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Research Project (R01)
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Study Section
Clinical and Integrative Cardiovascular Sciences Study Section (CICS)
Program Officer
Burns, Kristin
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Emory University
Schools of Medicine
United States
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