• Richard G Ohye, Julie V Schonbeck, Pirooz Eghtesady, Peter C Laussen, Christian Pizarro, Peter Shrader, Deborah U Frank, Eric M Graham, Kevin D Hill, Jeffrey P Jacobs, Kirk R Kanter, Joel A Kirsh, Linda M Lambert, Alan B Lewis, Chitra Ravishankar, James S Tweddell, Ismee A Williams, Gail D Pearson, and Pediatric Heart Network Investigators.
• University of Michigan Medical School, Ann Arbor, MI, USA. ohye@umich.edu
• J. Thorac. Cardiovasc. Surg.. 2012 Oct 1;144(4):907-14.

ObjectivesThe 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.MethodsThere 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.ResultsThe 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.ConclusionsIn 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.Copyright © 2012 The American Association for Thoracic Surgery. All rights reserved.

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