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Functional state of patients with heterotaxy syndrome following the Fontan operation

Published online by Cambridge University Press:  26 November 2007

Andrew M. Atz ,
Meryl S. Cohen ,
Lynn A. Sleeper ,
Brian W. McCrindle ,
Minmin Lu ,
Ashwin Prakash ,
Roger E. Breitbart ,
Richard V. Williams ,
Charlie J. Sang  and
Gil Wernovsky
Andrew M. Atz*
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America
Meryl S. Cohen
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Lynn A. Sleeper
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Brian W. McCrindle
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Canada
Minmin Lu
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Ashwin Prakash
Affiliation:
Department of Pediatrics, Division of Cardiology, Columbia University Medical Center, New York, New York, United States of America
Roger E. Breitbart
Affiliation:
Department of Cardiology, Children’s Hospital Boston, Boston, Massachusetts, United States of America
Richard V. Williams
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Utah, Salt Lake City, Utah, United States of America
Charlie J. Sang
Affiliation:
Department of Pediatrics, Division of Cardiology, Duke University Medical Center, Durham, North Carolina, United States of America
Gil Wernovsky
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: Andrew M Atz, Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, Box 250915, Room 601 Children’s Hospital, Charleston, South Carolina 29425, USA. Tel: 843 792 3292; Fax: 843 792 3284; E-mail: atzam@musc.edu
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Abstract

Background

Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation.

Methods

In the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy.

Results

Patients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p = 0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p = 0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p = 0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p = 0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy.

Conclusions

The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

Keywords

Situs ambiguusisomerism of atrial appendagesfunctionally univentricular heartheart rhythm
Type
Original Article
Information
Cardiology in the Young , Volume 17 , Supplement S4 , September 2007 , pp. 44 - 53
Copyright
Copyright © Cambridge University Press 2007

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