Abstract 18091: Growth and Puberty in Fontan Survivors (GAPS study): A Multicenter Cross-sectional Study

Abstract

Introduction: Chronic diseases may result in growth impairment and delayed puberty that contribute to psychosocial maladjustment. There are no data on the prevalence of short stature or delayed puberty in children and adolescents after Fontan operation, a cohort characterized by chronic low cardiac output. Methods: This was a cross-sectional study of 299 Fontan patients (8-18 years) from 11 Pediatric Heart Network centers. We collected demographic data, anthropometric measurements and Tanner stage using a validated self-assessment questionnaire. Anthropometric measurements and pubertal stage were compared to United States normative data. Short stature was defined as height <5% and abnormal BMI as <5% or >95%. Delayed puberty was defined as failure to reach a stage of development at an age greater than the median age in the subsequent Tanner stage. Comparisons were made between study population and contemporary normal population data. Results: Of the 299 subjects [42% female, median age at enrollment 13.9 years (IQR: 11.3, 16.1)], 98 (33%) had hypoplastic left ventricle and 24 (8%) had heterotaxy syndrome. Median age at Fontan was 3 years (IQR: 2, 4). PLE was present in 16 subjects (5%). Fontan survivors had a higher prevalence of short stature relative to normative data (20% vs. 5%, p<0.0001) and an increased prevalence of abnormal BMI (18% vs. 10%, p<0.0001). Abnormal BMI were split between low BMI (43%) and high BMI (57%). Both males (58%) and females (58%) had delay in ≥1 Tanner stage parameter with at least 2 yr differences between Fontan patients and population norms for most parameters (Figure). Conclusion: Compared to the normal population, Fontan survivors have a 4-fold increase in the prevalence of short stature and nearly 2 fold abnormality in in BMI. Delayed puberty was common in both genders. As these factors may have a negative psychosocial impact, routine screening and management of short stature and delayed puberty should be a priority in Fontan survivors.