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Somatic growth failure after the Fontan operation
M I Cohen1, D M Bush, R J Ferry
1Division of Cardiology, The Children' Hospital of Philadelphia and The University of Pennsylvania School of Medicine, 19104, USA. cohenmi@email.chop.edu
Insights
Children with single ventricle hearts undergoing staged Fontan palliation show improved but persistently low weight and height Z scores. These growth deficits remain significant compared to siblings and the general population.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Growth and Development
Background:
- Congenital cardiac malformations often lead to growth failure.
- Staged Fontan palliation aims to reduce hypoxemia and ventricular overload in functionally univentricular hearts.
- The impact of this approach on somatic growth requires further investigation.
Purpose of the Study:
- To characterize the medium-term growth patterns of children with functionally univentricular hearts after staged Fontan palliation.
- To assess somatic growth following hemi-Fontan and modified Fontan operations.
Main Methods:
- Retrospective review of 65 patients with functionally univentricular hearts who completed staged Fontan circulation between 1990-1995.
- Growth parameters (weight, height) were measured pre-operatively and annually for three years post-Fontan.
- Comparison with sibling and parental growth data.
Main Results:
- Mean weight Z score improved post-Fontan but remained below normal (-0.91 +/- 0.99).
- Mean height Z scores at follow-up were significantly lower than parents and siblings (-1.15 +/- 1.2).
- Persistent underweight and short stature were observed in this cohort.
Conclusions:
- Children palliated with staged Fontan operations for single ventricle physiology exhibit significant and persistent growth deficits.
- Growth retardation remains a concern despite successful surgical palliation.
- Further strategies may be needed to optimize growth in this population.
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.
