Open Access

ARTICLE

Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: A multicenter cross-sectional study

Shaji C. Menon¹, Ragheed Al-Dulaimi¹, Brian W. McCrindle², David J. Goldberg³, Ritu Sachdeva⁴, Bryan H. Goldstein⁵, Thomas Seery⁶, Karen C. Uzark⁷, Anjali Chelliah⁸, Ryan Butts⁹, Heather Henderson¹⁰, Tiffanie Johnson¹¹, Richard V. Williams¹

1 University of Utah and Primary Children’s Hospital, Salt Lake City, Utah, USA
2 The Hospital for Sick Children, Toronto, Ontario, Canada
3 Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
4 Emory University School of Med, Atlanta, Georgia, USA
5 Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
6 Texas Children’s Hospital, Houston, Texas, USA
7 University of Michigan, Ann Arbor, Michigan, USA
8 Columbia University Medical Center, New York, New York, USA
9 Medical University of South Carolina, Charleston, South Carolina, USA
10 Duke University School of Medicine, Durham, North Carolina, USA
11 Riley Hospital for Children, Indianapolis, Indiana, USA

* Corresponding Author: Shaji C. Menon, MD, Primary Children’s Medical Hospital, 81 N Mario Capecchi Drive, Salt Lake City, UT 84113, USA. Email:

Congenital Heart Disease 2018, 13(3), 463-469. https://doi.org/10.1111/chd.12597

Abstract

Introduction: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors.
Methods: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL). Mixed effects regression modeling adjusting for clustering by center was used to evaluate factors associated with abnormal anthropometry and delayed puberty and associations with QoL.
Results: Of the 299 subjects, 42% were female. The median enrollment age was 13.9 years, and the median age at Fontan was 3 years. Fontan survivors had a higher prevalence of short stature relative to normative data (20% vs 5%, P < .0001) and an increased prevalence of abnormal BMI (16% vs 10%, P < .0001) (low [43%] and high [57%]). Fontan subjects, both males (58%) and females (58%), had a delay of 1.5‒2 years in 1 Tanner stage parameter compared to normal population. There was no association between delayed puberty and QoL. Abnormal anthropometry was associated with lower overall (62.3 ± 17.3 vs 72.5 ± 16.6; P < .001) and physical appearance scores (72.2 ± 27.4 vs 79.8 ± 21.5; P < .01). Lower exercise capacity was associated with abnormal anthropometry and >2 surgeries before Fontan was associated with delayed puberty. Lower family income (<$25 000) and hypoplastic left heart syndrome were associated with lower QoL.
Conclusion: Compared to the normal population, Fontan survivors have high prevalence of short stature, abnormal BMI and delayed puberty. Abnormal anthropometry, but not delayed puberty, was associated with lower overall QoL and perceived physical appearance scores. Routine screening for abnormal anthropometry, especially in HLHS and in lower socioeconomic status families, should be considered to allow interventions, which might ameliorate the negative psychosocial impact.

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