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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 ,
Thomas G. Travison ,
Brian W. McCrindle ,
Lynn Mahony ,
Andrew C. Glatz ,
Aditya K. Kaza ,
Roger E. Breitbart ,
Steven D. Colan ,
Jonathan R. Kaltman  and
Renee Margossian
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Andrew M. Atz*
Affiliation:
Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America
Thomas G. Travison
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Brian W. McCrindle
Affiliation:
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Lynn Mahony
Affiliation:
Department of Pediatrics, University of Texas Southwestern, Dallas, Texas, United States of America
Andrew C. Glatz
Affiliation:
Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Aditya K. Kaza
Affiliation:
Department of Surgery, University of Utah, Salt Lake City, Utah, United States of America
Roger E. Breitbart
Affiliation:
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Steven D. Colan
Affiliation:
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Jonathan R. Kaltman
Affiliation:
National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, United States of America
Renee Margossian
Affiliation:
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States of America
Sara K. Pasquali
Affiliation:
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina, United States of America
Yanli Wang
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Welton M. Gersony
Affiliation:
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: atzam@musc.edu
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Abstract

Background

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.

Methods

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).

Results

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.

Conclusions

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.

Keywords

Congenital heart diseaseFontanfunctionally univentricular heartquality of life
Type
Original Articles
Information
Cardiology in the Young , Volume 23 , Issue 3 , June 2013 , pp. 335 - 343
Copyright
Copyright © Cambridge University Press 2012 

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