Somatic growth failure after the Fontan operation

Our study was designed to characterize the patterns of growth, in the mediu m term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation i n early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and h ypoxia. Repair of simple lesions appears to reverse the retardation in grow th. Palliation of the functionally single ventricular physiology with a sta ged 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 c hildren with functionally univentricular hearts followed primarily at our i nstitution who had completed a staged construction of the Fontan circulatio n between January 1990 and December 1995. Measurements were available on al l children prior to surgery, and annually for three years following the Fon tan operation. Data was obtained on siblings and parents for comparative pu rposes. The criterions of eligibility for inclusion were satisfied by 65 pa tients. The mean Z score for weight was -1.5 +/- 1.2 at the time of the hem i-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 Font an operation, but never normalized. The mean Z scores for height at the hem i-Fontan and Fontan operations were -0.67+/-1.1 and -0.89+/-1.2 respectivel y. At most recent follow-up, with a mean age of 6.1 +/- 1.3 years, and a me an 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 scor es for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are s ignificantly underweight and shorter than the general population and their siblings.