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Author Notes:

Matthew Oster, MD, MPH, Children’s Healthcare of Atlanta, Sibley Heart Center Cardiology, 2835 Brandywine Rd, Suite 400, Atlanta, GA 30341, Fax: 770-488-9477, Phone: 404-256-2593, osterm@kidsheart.com. Twitter handles: Matthew Oster: @mattoster, Children’s Healthcare of Atlanta: @ChildrensATL, Sibley Heart Center Cardiology: @SibleyHeart

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 possible.

Disclosures: None.


Research Funding:

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


  • Science & Technology
  • Life Sciences & Biomedicine
  • Cardiac & Cardiovascular Systems
  • Peripheral Vascular Disease
  • Cardiovascular System & Cardiology
  • heart defects
  • congenital
  • heart transplantation
  • heart ventricles
  • hypoplastic left heart syndrome
  • mortality
  • survival analysis
  • thoracic surgery

Long-Term Outcomes in Single-Ventricle Congenital Heart Disease: Importance of Ventricular Morphology


Journal Title:



Volume 138, Number 23


, Pages 2718-2720

Type of Work:

Article | Post-print: After Peer Review


Long-term survival for children with congenital heart disease (CHD) has been improving over time, particularly among those with single-ventricle CHD.1, 2 Among this population, it is unproven whether having a systemic left ventricle (LV) confers any long-term survival advantage as compared to having a systemic right ventricle (RV). By using a large, multi-center surgical registry, we aimed to compare long-term transplant-free survival of individuals with single-ventricle CHD by systemic ventricular morphology. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium (PCCC) linked with the United States National Death Index and Organ Procurement and Transplant Network through 2014.3 We included subjects with single-ventricle CHD who were born 1982–2003 and were treated surgically at <1 year of age at a PCCC center (excluding those whose primary surgery was heart transplant). The primary outcome was the composite transplant/mortality prior to 2015, assessed at varying timepoints: in-hospital prior to discharge following first surgery, following discharge from first surgery up to 20 years, in-hospital prior to discharge following Fontan, and following discharge from Fontan up to 15 years. Transplant-free survival was assessed by ventricular morphology using unadjusted Kaplan-Meier survival curves and adjusted Cox hazards models with relevant covariates, including a random effect for treatment center. The University of Minnesota and Emory University IRBs have approved the use of this database for research purposes with a waiver of informed consent.

Copyright information:

© 2018 American Heart Association, Inc.

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