Original Investigation
Trends in Long-Term Mortality After Congenital Heart Surgery

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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.

Key Words

children’s health
congenital heart defects
mortality
outcomes research

Abbreviations and Acronyms

CAVC
complete atrioventricular canal
CHD
congenital heart defect
CHS
congenital heart surgery
d-TGA
dextro-transposition of the great arteries
NDI
National Death Index
PCCC
Pediatric Cardiac Care Consortium
SMR
Standardized Mortality Ratio

Cited by (0)

This study was supported by National Heart, Lung, and Blood Institute R01 HL122392 and National Institutes of Health CTSA Award UL1TR000114. Dr. Menk’s spouse is an employee of Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Listen to this manuscript's audio summary by JACC Editor-in-Chief Dr. Valentin Fuster.