@Article{chd.12836,
AUTHOR = {Paolo D’Ambrosio, Derek Tran, Charlotte E. Verrall, Chantal Attard, Maria Fiatarone Singh, Julian Ayer, Yves d’Udekem, Stephen Twigg, David S. Celermajer, Rachael Cordina},
TITLE = {Prevalence and risk factors for low bone density in adults with a Fontan circulation},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {14},
YEAR = {2019},
NUMBER = {6},
PAGES = {987--995},
URL = {http://www.techscience.com/schd/v14n6/38904},
ISSN = {3071-1738},
ABSTRACT = {<b>Objective and Patients:</b> This study aimed to characterize bone mineral density abnormalities and pathophysiological associations in young adults living with a Fontan
circulation.<br/>
<b>Design:</b> Participants underwent bone mineral density measurement using dual‐energy X‐ray absorptiometry and serum biochemical analysis, cardiopulmonary exercise and strength testing and transthoracic echocardiography.<br/>
<b>Results:</b> In our cohort (n = 28), 29% had osteopenic‐range bone mineral density and
one patient was osteoporotic (average hip t score: −0.6 ± 1.1; spine t score: −0.6 ± 0.9).
Four patients (14%) had z scores < −2.0. Parathyroid hormone levels were increased
compared with laboratory median (6.1 ± 3.5 vs 4 pmol/L, P = .01) and 27% had 25‐
hydroxy‐vitamin D < 50 nmol/L. 25‐hydroxy‐vitamin D negatively correlated with
parathyroid hormone (ρ = −0.53, P = .01) suggesting secondary hyperparathyroidism.
Atrioventricular valve systolic to diastolic duration ratio, an echocardiographic measure of diastolic dysfunction, inversely correlated with hip t and z scores (P < .01). Hipt scores were positively associated with oxygen saturations (ρ = 0.45, P = .05) and 
tended to be inversely associated with parathyroid hormone levels (ρ = −0.44, P = .07) 
and N‐Terminal pro b‐type natriuretic peptide (ρ = −0.42, P = .08).<br/>
<b>Conclusions:</b> Many young adults with a Fontan circulation have abnormal bone
mineral density. The underlying pathophysiology is likely multifactorial. Possible
contributors include secondary hyperparathyroidism, hypoxemia, diastolic cardiac
dysfunction and neurohormonal activation. As low bone mineral density is clinically
relevant and potentially treatable, assessment of bone mineral density should be part
of routine care in this cohort.},
DOI = {10.1111/chd.12836}
}