Background Congenital heart surgery has improved the survival of patients with even the most complex defects, but the long-term survival after these procedures has not been fully described. Objectives Evaluate the long-term survival of patients (<21 years) operated for congenital heart defects. Methods This study used the Pediatric Cardiac Care Consortium (PCCC) data, a U.S.-based, multicenter registry of pediatric cardiac surgery. Survival analysis included 35,998 patients who survived their first congenital heart surgery at less than 21 years of age and had adequate identifiers for linkage with the National Death Index through 2014. Survival was compared to that in the general population using standardized mortality ratios (SMRs). Results After a median follow-up of 18 years (645,806 person-years), 3,191 deaths occurred with an overall SMR of 8.3 (95% CI: 8.0-8.7). The 15-year SMR decreased from 12.7 (95% CI: 11.9-13.6) in the early era (1982-1992) to 10.0 (95% CI: 9.3-10.8) in the late era (1998-2003). The SMR remained elevated even for mild forms of CHD such as patent ductus arteriosus (4.5) and atrial septal defects (4.9). The largest decreases in SMR occurred for patients with transposition of great arteries (early: 11.0 vs late: 3.8, p<0.05), complete atrioventricular canal (31.3 vs 15.3, p<0.05), and single ventricle (53.7; vs 31.3, p<0.05). Conclusions In this large U.S. cohort, long-term mortality after congenital heart surgery was elevated across all forms of CHD. Survival has improved over time, particularly for severe defects with significant changes in their management strategy, but still lags the general population.
Spector et al. (Tue,) studied this question.