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Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection

Published online by Cambridge University Press:  24 July 2012

Andrew M. Atz*
Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America
Thomas G. Travison
New England Research Institutes, Watertown, Massachusetts, United States of America
Brian W. McCrindle
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Lynn Mahony
Department of Pediatrics, University of Texas Southwestern, Dallas, Texas, United States of America
Andrew C. Glatz
Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Aditya K. Kaza
Department of Surgery, University of Utah, Salt Lake City, Utah, United States of America
Roger E. Breitbart
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Steven D. Colan
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Jonathan R. Kaltman
National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, United States of America
Renee Margossian
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Sara K. Pasquali
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina, United States of America
Yanli Wang
New England Research Institutes, Watertown, Massachusetts, United States of America
Welton M. Gersony
Division of Pediatrics, Columbia University Medical Center, New York, United States of America
Correspondence to: Dr A. M. Atz, MD, Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, MSC 915, Room 601 Children's Hospital, Charleston, South Carolina 29425, United States of America. Tel: +1 843 792 3292; Fax: +1 843 792 1978; E-mail:



A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.


We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114).


A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome.


After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.

Original Articles
Copyright © Cambridge University Press 2012 

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