Component A: Surveillance of CHDs Among Children, Adolescents and Adults in Utah - This project aims at developing in Utah a statewide, population-based surveillance for individuals aged 1-45 years with congenital heart defects (CHD) that is able evaluate and track over time their health (mortality, comorbidities), their patterns of healthcare utilization (e.g., hospitalizations, emergency room visits), and other selected health and non-health related outcomes. These indicators will be assessed in relations to demographic and clinical factors, such as socio-economic status, race/ethnicity, rural/urban residence, as well as CHD type and severity. The time period is 2008 through 2017. Prior multiyear projects funded by CDC have piloted methods and generated initial data. This project builds on the prior efforts in two ways: it focuses on those 1-45 years of age while expanding the range of outcomes and the time period of assessment (2008-2017 vs. 2011-2013). We will demonstrate capacity to access, link, and use data from the birth defects surveillance program and congenital cardiac clinical databases to identify eligible cases with CHDs. These case-finding sources will provide the gold standard diagnostic information on CHD. Data collection and linkages will help establish the vital status of cases, enhance case-based health and non-health information, geocode residence; and create a standardized dataset. Specifically we will demonstrate capacity to access, link, and use data from state vital records and the National Death Index (NDI), healthcare claims database(s), clinics, health providers, hospital discharge, payers, and other sources. We will link identified cases to NDI data to establish vital status; link identified cases to birth certificates and to additional existing data sources to collect standardized data elements documented between 1/1/2008-12/31/2017 (e.g. all healthcare claims occurring between 2008 and 2017).We will explore methods to understand in- and out-migration in catchment area of identified CHD cases during the surveillance period. We will calculate age-specific mortality, assess major causes of death, evaluate patterns of healthcare utilization, and assess long term outcomes (including non-health outcomes such as employment status), overall and by subgroups of interest (e.g., by CHD type and severity, by demographic factors). Building on the case–finding sources, we will leverage the linkages maintained at the Utah Population Database to multiple Utah data sources, including Enterprise Data Warehouses of the University of Utah (UofU) and Intermountain Healthcare, and multiple demographic databases at the state of Utah (e.g., vital records, marriage and divorce records, voter registration, driver's license, workforce services). The core study group includes experienced epidemiologists, data analysts, and clinicians (medical geneticists and adult and pediatric cardiologist) from the UofU. This group has significant experience with this type of data and study, having now nearly completed a four-year study on a similar topic, funded by the same agency (CDC). Statement of Significance: CHDs are a major clinical and public health concern because of the their frequency (approximately one in 100 babies is born with a heart defect in the US) and their health impact (major cause of infant mortality due to birth defects, and among the top ten most costly birth defects). With better treatment, people with CHDs live longer and better lives. However, there are significant gaps in knowledge of the overall impact after infancy, in terms of morbidity, mortality, disability, health resource utilization, quality of life, and employment. This study aims at filling such gaps in a systematic fashion, efficiently leveraging available data systems combined with the cognitive expertise of clinicians, epidemiologists, and data scientists. Utah is uniquely positioned for this study because of its system of linked administrative and medical records.
