@Article{chd.12597,
AUTHOR = {Shaji C. Menon, Ragheed Al-Dulaimi, Brian W. McCrindle, David J. Goldberg, Ritu Sachdeva, Bryan H. Goldstein, Thomas Seery, Karen C. Uzark, Anjali Chelliah, Ryan Butts, Heather Henderson, Tiffanie Johnson, Richard V. Williams},
TITLE = {Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: A multicenter cross-sectional study},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {13},
YEAR = {2018},
NUMBER = {3},
PAGES = {463--469},
URL = {http://www.techscience.com/schd/v13n3/39003},
ISSN = {3071-1738},
ABSTRACT = {<b>Introduction:</b> We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors.<br/>
<b>Methods:</b> 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.<br/>
<b>Results:</b> 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.<br/>
<b>Conclusion:</b> 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.},
DOI = {10.1111/chd.12597}
}