Long-Term Mortality After Congenital Heart Surgery

Study Questions:

What is the long-term survival for patients <21 years who have undergone surgery for congenital heart defects?


A retrospective cohort study with prospectively collected data was performed. The study made use of the Pediatric Cardiac Care Consortium (PCCC registry), which was linked with the National Death Index (NDI) for long-term mortality data. Survival was compared to that of the general population using standardized mortality ratios (SMRs), which was assessed over the entire study period, as well as by surgical era.


The survival analysis included 35,998 patients who survived their first congenital heart surgery and had adequate identifiers for linkage with the NDI through 2014. After a median follow-up of 18 years, 3,191 deaths occurred, with an overall SMR of 8.3 (95% confidence interval [CI], 8-8.7). The 15-year SMR was highest in the early surgical era (1982-1992) at 12.7 (95% CI, 11.9-13.6) and lowest in the most recent surgical era (1998-2008) at 10.0 (95% CI, 9.3-10.8). The SMR was elevated even for mild congenital heart disease with a SMR of 3.4 for patent ductus arteriosus and 3.1 for atrial septal defect over the course of the study period.


The authors concluded that long-term mortality was elevated across all forms of congenital heart disease as compared with the general population. Survival has improved across the spectrum of congenital heart defects, particularly for complex lesions, although it continues to lag behind the general population.


Most large-scale, long-term studies of outcomes of congenital heart surgery have been performed in countries with national health systems (generally in Scandinavia) and the ability to link data from cardiac surgery and diagnosis data to national survival registries. This study made use of the Pediatric Cardiac Care Consortium (PCCC), a US-based multicenter registry, which has been estimated to capture 10-30% of infant operations (Vinocur JM, et al. Circ Cardiovasc Qual Outcomes 2012;5:577-9). The study showed increased mortality risk even for patients with mild disease (atrial septal defect and patent ductus arteriosus) who would not be expected to have significant residua. While the study demonstrated progressive improvement in survival in more recent eras across the spectrum of diagnoses, the most improvement was seen in patients with complex disease. For patients with more mild disease (likely with less room for improvement), a plateau was seen in outcomes in the most recent surgical era. While the PCCC is an important data set for long-term outcomes in congenital heart disease, the ultimate goal should be a national, all-inclusive registry in the United States.

Keywords: Cardiac Surgical Procedures, Child, Ductus Arteriosus, Patent, Heart Defects, Congenital, Heart Septal Defects, Atrial, Infant, Outcome Assessment, Health Care, Pediatrics, Survival Analysis

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