Abstract 19714: Growth Parameters, Bone Mineral Density and Secondary Hyperparathyroidism After Fontan Operation: Results From the Multidisciplinary Clinic at the CHOP Single Ventricle Survivorship Program
Background/Objectives: Height and lean mass deficits are reported in patients with single ventricle physiology after Fontan operation (FO). Our objective is to determine growth, areal bone mineral density (aBMD) and related outcomes in a large population of patients with single ventricle physiology after FO.
Methods: Cross sectional evaluation through chart review of patients followed in our multidisciplinary Single Ventricle Survivorship Program clinic from Jan. 2011 through Aug. 2015. Standardized evaluation includes anthropometry, biochemistry and bone densitometry (DXA).
Results: 210 patients were included in the study at mean age of 11.8 ± 4.8 yrs (90 female, 120 male); 65% had dominant right ventricle and 44% hypoplastic left heart syndrome. Median age at FO was 31 mos (7.6-135.8); 25 had current or a history of protein losing enteropathy (PLE). Mean height Z score was -0.73 ± 1.35 after excluding patients with genetic syndromes, and -0.65 ± 1.42 after excluding patients with PLE. Mean height Z score for those with PLE was -1.99 ± 1.02. Overall mean weight and BMI Z scores were -0.64 ± 1.54 and -0.14 ± 1.16 respectively. Height adjusted (HA) whole body less head (WBLH) BMD Z score was -1.18 ± 1 for those without PLE, and -2.4 ± 0.58 for those with PLE. HA Lumbar Spine (LS) BMD Z score was -0.22 ±1.02 for those without PLE and -1.29 ± 1.42 for those with PLE. Laboratory values in those without PLE were significant for mean parathyroid hormone (PTH) 55.5 ± 35.4 pg/mL (normal range = 9-52) and 25 OH Vitamin D 31.7 ± 11.7 ng/mL. Overall PTH was above normal in 47% and 13% had low Vitamin D (<20 ng/mL), with higher PTH values in those with PLE (high PTH in 84%). Serum albumin, calcium and alkaline phosphatase were markedly lower in those with PLE. Height Z scores correlated with dominant right ventricle. WBLH BMD Z scores correlated with PTH and LS BMD Z scores correlated with calcium, independent of dominant ventricle.
Conclusions: Children after FO have significant deficits in height, BMD and commonly exhibit secondary hyperparathyroidism. Greater abnormalities are observed in those with PLE. These findings are part of a myriad of increasingly recognized end-organ consequences that define a “Fontan circulatory syndrome” and require further investigation as to etiology and treatment.
Author Disclosures: E.E. Mancilla: None. B. Zielonka: None. J. Brodsky: None. J. Roizen: None. B. Zemel: None. D.J. Goldberg: None. K.M. Dodds: None. J. Rychik: None.
- © 2016 by American Heart Association, Inc.