Growth in Children with a Fontan Circulation

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Growth and Development

Background:

• Fontan circulation is a complex surgical palliation for single-ventricle congenital heart defects.
• Growth abnormalities are a known concern in this patient population.
• Long-term outcomes and factors influencing growth require ongoing investigation.

Purpose of the Study:

• To evaluate growth patterns in a cohort of patients with Fontan circulation.
• To identify factors associated with height z-score in this population.
• To explore potential modifiable factors for improving growth.

Main Methods:

• Cross-sectional study of 210 patients followed in a multidisciplinary Fontan clinic (2011-2015).
• Review of historical data, anthropometry, clinical, and laboratory studies.
• Bivariate and multivariate analyses to determine factors associated with height z-score.

Main Results:

• Median height z-score was -0.58; 12% had a history of protein-losing enteropathy (PLE), with significantly lower height z-scores (-2.1).
• Positive associations with height z-score included BMI z-score, time since Fontan, mid-parental height, dominant ventricle type, and serum alkaline phosphatase.
• Negative correlations were found with genetic syndromes, PLE, and stimulant or oral steroid use.

Conclusions:

• Patients with Fontan circulation exhibit mild height deficits, more pronounced in those with PLE.
• Height z-score demonstrates improvement over time post-Fontan surgery.
• Optimizing body mass index and utilizing biochemical markers may aid in managing growth and identifying high-risk individuals.