National Survival Estimates After Surgical Repair of Congenital Heart Disease

BACKGROUND: Survival estimates after congenital heart surgery (CHS) in the United States are essential for planning healthcare services for this growing, resource-intensive patient population. However, the absolute number of individuals living after interventions for congenital heart disease is unknown because nationally representative survival estimates are unavailable. Understanding postoperative survival from a representative national sample is a critical first step toward estimating the size and future healthcare needs of this population. This study aimed to develop nationally representative long-term survival estimates after CHS in the United States, focusing on benchmark procedures defined by the Society of Thoracic Surgeons. METHODS: We used 3 US data sets of children aged 0 to 20 years at the time of their benchmark CHS from 1998 to 2002 and surviving to hospital discharge. The Pediatric Cardiac Care Consortium (PCCC), a multi-institutional clinical registry, was matched to the National Death Index to determine mortality through December 2019. The Kids' Inpatient Database 2000, a nationally representative administrative claims data set, was used to derive age- and sex-specific weights. Kaplan-Meier curves were estimated from the PCCC-National Death Index cohort for benchmark operations, and Kids' Inpatient Database 2000 weights were applied. Unweighted PCCC survival estimates were also compared with those of Boston Children's Hospital, a large tertiary care center. RESULTS: Nationally representative survival curves were generated for 7 benchmark CHS from 7973 cases; 3802 (48%) were female, and 4505 (57%) were infants at the time of CHS. These weighted national survival curves closely mirrored the unweighted curves derived from PCCC-National Death Index. Unweighted survival estimates from PCCC also closely approximated those from BCH, with only modest differences observed for selected lesions. CONCLUSIONS: The weighted PCCC-National Death Index methodology provides the first nationally representative estimates of long-term survival after benchmark congenital heart disease in the United States. These estimates provide an important resource for healthcare planning and research for the growing population of individuals with congenital heart disease.