Cause, Timing and Location of Death in the Single Ventricle Reconstruction Trial

• Type of Material: Article
• Authors: Richard G. Ohye, University of Michigan; Julie V. Schonbeck, New England Research Institutes; Pirooz Eghtesady, Cincinnati Children’s Medical Center; Peter C. Laussen, Children’s Hospital Boston; Christian Pizarro, Alfred I. duPont Hospital for Children; Peter Shrader, New England Research Institutes; Deborah U. Frank, University of Utah; Eric M. Graham, Medical University of South Carolina; Kevin D. Hill, Duke University; Jeffrey P. Jacobs, Congenital Heart Institute of Florida; Kirk R Kanter, Emory University; Joel A. Kirsh, Hospital for Sick Children; Linda M. Lambert, University of Utah; Alan B. Lewis, Children’s Hospital Los Angeles; Chitra Ravishankar, Children’s Hospital of Philadelphia; James S. Tweddell, Children’s Hospital of Wisconsin; Ismee A. Williams, Columbia University; Gail D. Pearson, National Heart, Lung, and Blood Institute
• Language: English
• Date: 2012-10-01
• Publisher: Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2012 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): https://www.jtcvs.org/article/S0022-5223(12)00888-4/fulltext
• Title of Journal or Parent Work: Journal of Thoracic and Cardiovascular Surgery
• ISSN: 0022-5223
• Volume: 144
• Issue: 4
• Start Page: 907
• End Page: 914
• Grant/Funding Information: This study was supported by U01 grants from the National Heart, Lung, and Blood Institute (Nos. HL068269, HL068270, HL068279, HL068281, HL068285, HL068292, HL068290, HL068288, and HL085057).
• Keywords: Cardiac & Cardiovascular Systems; Congenital heart surgery; Single ventricle; Congenital heart disease; Cardiovascular System & Cardiology; Life Sciences & Biomedicine; Science & Technology; Peripheral Vascular Disease
• Research Categories: Health Sciences, General; Health Sciences, Pathology; Health Sciences, Public Health

Abstract

Objectives: The Single Ventricle Reconstruction trial randomized 555 subjects with a single right ventricle undergoing the Norwood procedure at 15 North American centers to receive either a modified Blalock-Taussig shunt or right ventricle-to-pulmonary artery shunt. Results demonstrated a rate of death or cardiac transplantation by 12 months postrandomization of 36% for the modified Blalock-Taussig shunt and 26% for the right ventricle-to-pulmonary artery shunt, consistent with other publications. Despite this high mortality rate, little is known about the circumstances surrounding these deaths. Methods: There were 164 deaths within 12 months postrandomization. A committee adjudicated all deaths for cause and recorded the timing, location, and other factors for each event. Results: The most common cause of death was cardiovascular (42%), followed by unknown cause (24%) and multisystem organ failure (7%). The median age at death for subjects dying during the 12 months was 1.6 months (interquartile range, 0.6 to 3.7 months), with the highest number of deaths occurring during hospitalization related to the Norwood procedure. The most common location of death was at a Single Ventricle Reconstruction trial hospital (74%), followed by home (13%). There were 29 sudden, unexpected deaths (18%), although in retrospect, 12 were preceded by a prodrome. Conclusions: In infants with a single right ventricle undergoing staged repair, the majority of deaths within 12 months of the procedure are due to cardiovascular causes, occur in a hospital, and within the first few months of life. Increased understanding of the circumstances surrounding the deaths of these single ventricle patients may reduce the high mortality rate.