Trends in Long-Term Mortality After Congenital Heart Surgery

Abstract

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: The purpose of this study was to evaluate the long-term survival of patients (age <21 years) who were operated on for congenital heart defects (CHDs).

Methods: This study used the Pediatric Cardiac Care Consortium 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 <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% confidence interval [CI]: 8.0 to 8.7). The 15-year SMR decreased from 12.7 (95% CI: 11.9 to 13.6) in the early era (1982 to 1992) to 10.0 (95% CI: 9.3 to 10.8) in the late era (1998 to 2003). The SMR remained elevated even for mild forms of CHD such as patent ductus arteriosus (SMR 4.5) and atrial septal defects (SMR 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 behind the general population.

Keywords: children’s health; congenital heart defects; mortality; outcomes research.

Figure 1. Standardized mortality ratios (SMRs) of patients discharged alive after their initial CHD surgery compared with the age-, sex-, and year-matched US general population

A) SMRs of the cumulative PCCC cohort and separately for the subgroup without chromosomal defects, for males/females and for each severity group. B) SMRs by individual lesions.

Figure 2. Long-term survival of patients after discharge from initial congenital heart surgery

A) with/without a chromosomal (chr) abnormality, B) by sex, C) by era of initial surgery and D) by severity.

Central Illustration. Survival after initial operation for a congenital heart defect by severity of the condition

A) Survival to hospital discharge, B) conditional survival post- discharge from initial surgery and C) cumulative long-term survival including in-hospital and post-discharge survival. Patients whose CHD could not be classified into a severity category are not included here (N=3,029).