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Somatic growth failure after the Fontan operation

Published online by Cambridge University Press:  19 August 2008

Mitchell I. Cohen ,
David M. Bush ,
Robert J. Ferry Jr ,
Thomas L. Spray ,
Thomas Moshang Jr ,
Gil Wernovsky  and
Victoria L. Vetter
Mitchell I. Cohen*
Affiliation:
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
David M. Bush
Affiliation:
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Robert J. Ferry Jr
Affiliation:
Endocrinology, and The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Thomas L. Spray
Affiliation:
Cardiothoracic Surgery and the Departments of Pediatrics and Surgery, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Thomas Moshang Jr
Affiliation:
Endocrinology, and The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Gil Wernovsky
Affiliation:
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
Victoria L. Vetter
Affiliation:
Divisions of Cardiology, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, USA
*
Mitchell I. Cohen, M.D., Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, 34th ' Civic Center Boulevard, Philadelphia, PA 19104. Tel: (215) 590-2230; Fax: (215) 590–3267; E-mail: cohenmi@email.chop.edu
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Abstract

Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings

Keywords

Congenital heart diseaseFontanfunctionally single ventriclegrowth
Type
Original Articles
Information
Cardiology in the Young , Volume 10 , Issue 5 , September 2000 , pp. 447 - 457
Copyright
Copyright © Cambridge University Press 2000

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