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

• Type of Material: Article
• Authors: Matthew Oster, Emory University; Jessica H. Knight, Emory University; Divya Suthar, Emory University; Omayma Amin, Morehouse School of Medicine; Lazaros Kochilas, Emory University
• Language: English
• Date: 2018-12-04
• Publisher: LIPPINCOTT WILLIAMS & WILKINS
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2018 American Heart Association, Inc.
• Final Published Version (URL): http://dx.doi.org/10.1161/CIRCULATIONAHA.118.036821
• Title of Journal or Parent Work: CIRCULATION
• Volume: 138
• Issue: 23
• Start Page: 2718
• End Page: 2720
• Grant/Funding Information: This study was supported by National Heart, Lung, and Blood Institute R01 HL122392 and NIH CTSA Award UL1TR000114
• Keywords: Science & Technology; survival analysis; congenital; thoracic surgery; hypoplastic left heart syndrome; heart defects; heart transplantation; Cardiac & Cardiovascular Systems; heart ventricles; Life Sciences & Biomedicine; Peripheral Vascular Disease; mortality; Cardiovascular System & Cardiology; TRENDS
• Research Categories: Biology, Anatomy; Health Sciences, Medicine and Surgery; Health Sciences, Health Care Management

Abstract

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.