The aim is To evaluate growth in a population of patients with Fontan circulation.

We performed a cross-sectional evaluation of patients followed in our multidisciplinary Fontan clinic from January 2011 through August 2015. We reviewed the historical data, anthropometry, clinical, and laboratory studies and performed a bivariate and multivariate analysis of factors associated with height z score.

Patients (n = 210) were included in the study at median age 11.07 years (8.3, 14.73 years) (43% female); 138 (65%) had a dominant right systemic ventricle and 92 (44%) hypoplastic left heart syndrome. The median age at completion of Fontan circulation was 31 months (7.6, 135.8 months). The median height z score was −0.58 (−1.75, 0.26). Twenty-five (12%) had a current or past history of protein-losing enteropathy (PLE). The median height z score for those with a current or past history of PLE was −2.1 (−2.46, 1.24). Multivariate analysis revealed positive associations between height z score and body mass index z score, time since Fontan, mid-parental height, dominant systemic ventricle type, and serum alkaline phosphatase. Height correlated negatively with a known genetic syndrome, PLE, use of stimulant or oral steroid medication. Children with Fontan circulation have mild deficits in height, with greater deficits in those with PLE. Height z score improves with time postsurgery. Improving weight, leading to improved body mass index, maybe a modifiable factor that improves growth in those who are underweight. Biochemical markers may be helpful screening tests for high-risk groups in whom to intensify interventions.

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