Title

Growth in Children with a Fontan Circulation.

Authors

Edna E Mancilla, Children's Hospital of Philadelphia, Philadelphia, PAFollow
Benjamin Zielonka
Jeffrey D Roizen, Children's Hospital of Philadelphia, Philadelphia, PAFollow
Kathryn M Dodds, Children's Hospital of Philadelphia, Philadelphia, PAFollow
Elizabeth B Rand, Children's Hospital of Philadelphia, Philadelphia, PAFollow
Jennifer R Heimall, Children's Hospital of Philadelphia, Philadelphia, PAFollow
Feiyan Chen, Children's Hospital of Philadelphia, Philadelphia, PA
Chao Wu, Children's Hospital of Philadelphia, Philadelphia, PA
David J Goldberg, Children's Hospital of Philadelphia, Philadelphia, PAFollow
Jack Rychik, Children's Hospital of Philadelphia, Philadelphia, PAFollow

Publication Title

The Journal of pediatrics

Document Type

Article

PubMed ID

33887332

Abstract

OBJECTIVE: To evaluate growth in a population of patients with Fontan circulation.

STUDY DESIGN: 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 bivariate and multivariate analysis of factors associated with height z score.

RESULTS: 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. Median age at completion of Fontan circulation was 31 months (7.6, 135.8 months). Median height z score was -0.58 (-1.75, 0.26). Twenty-five (12%) had current or past history of protein-losing enteropathy (PLE). Median height z score for those with 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 known genetic syndrome, PLE, use of stimulant or oral steroid medication.

CONCLUSIONS: 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, may be 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.

Keywords

Adolescent, Body Height, Body Weight, Child, Cross-Sectional Studies, Female, Fontan Procedure, Growth and Development, Heart Ventricles, Humans, Male, Protein-Losing Enteropathies, Retrospective Studies

DOI

10.1016/j.jpeds.2021.04.019

Publication Date

8-1-2021

Citation

Mancilla, E., Zielonka, B., Roizen, J., Dodds, K., Rand, E., Heimall, J., Chen, F., Wu, C., Goldberg, D., & Rychik, J. (2021). Growth in Children with a Fontan Circulation.. The Journal of pediatrics, 235, 149-155. https://doi.org/10.1016/j.jpeds.2021.04.019