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Author Notes:

Address for correspondence: Lazaros Kochilas, MD, MSCR, Department of Pediatrics, Emory University School of Medicine, 2835 Brandywine Road, Suite 300, Atlanta, Georgia 30341, Telephone: 404-785-0930, Fax: 770-488-9431, lazaros.kochilas@emory.edu, Twitter: @EmoryMed

We thank the program directors and data collection coordinators from the participating PCCC centers; without their effort and dedication, this work could not have been completed.

Susan Anderson and Brian Harvey were especially instrumental in the management of PCCC and initial linkage of this cohort with mortality information.

We also thank Alvin Chin, MD for critically reviewing the manuscript.

No relevant disclosures.

Subject:

Research Funding:

This study was supported by National Heart, Lung, and Blood Institute R01 HL122392 and NIH CTSA Award UL1TR000114.

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Cardiac & Cardiovascular Systems
  • Cardiovascular System & Cardiology
  • children's health
  • congenital heart defects
  • mortality
  • outcomes research
  • PEDIATRIC CARDIAC-SURGERY
  • ATRIOVENTRICULAR SEPTAL-DEFECT
  • SINGLE-VENTRICLE PALLIATION
  • ARTERIAL SWITCH OPERATION
  • FOLLOW-UP
  • GREAT-ARTERIES
  • CORRECTED TRANSPOSITION
  • FONTAN PROCEDURE
  • TEMPORAL TRENDS
  • WORKING GROUP

Trends in Long-Term Mortality After Congenital Heart Surgery

Tools:

Journal Title:

Journal of the American College of Cardiology

Volume:

Volume 71, Number 21

Publisher:

, Pages 2434-2446

Type of Work:

Article | Post-print: After Peer Review

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.

Copyright information:

© 2018 American College of Cardiology Foundation

This is an Open Access work distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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