Author + information
- Received December 20, 2017
- Revision received February 21, 2018
- Accepted March 6, 2018
- Published online May 21, 2018.
- Logan G. Spector, PhDa,
- Jeremiah S. Menk, MSb,
- Jessica H. Knight, PhDc,
- Courtney McCracken, PhDc,
- Amanda S. Thomas, MSPHc,
- Jeffrey M. Vinocur, MDd,
- Matthew E. Oster, MD, MPHc,
- James D. St Louis, MDe,
- James H. Moller, MDf and
- Lazaros Kochilas, MD, MSCRc,∗ (, )@EmoryMed
- aDepartment of Pediatrics, University of Minnesota, Minneapolis, Minnesota
- bBiostatistical Design and Analysis Center, University of Minnesota, Minneapolis, Minnesota
- cDepartment of Pediatrics, Emory University School of Medicine and Children’s Healthcare of Atlanta, Atlanta, Georgia
- dDepartment of Pediatrics, University of Rochester School of Medicine and Dentistry, Rochester, New York
- eDepartment of Pediatric Surgery, University of Missouri-Kansas City School of Medicine, Kansas City, Missouri
- fDepartment of Internal Medicine, University of Minnesota, Minneapolis, Minnesota
- ↵∗Address for correspondence:
Dr. Lazaros Kochilas, Department of Pediatrics, Emory University School of Medicine, 2835 Brandywine Road, Suite 300, Atlanta, Georgia 30341.
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.
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.
- Received December 20, 2017.
- Revision received February 21, 2018.
- Accepted March 6, 2018.
- 2018 American College of Cardiology Foundation
This article requires a subscription or purchase to view the full text. If you are a subscriber or member, click Login or the Subscribe link (top menu above) to access this article.