Abstract 18648: Derangements in Hepatic Structure and Renal Function are Associated With Longer Duration of Follow-up After Fontan Completion
Introduction: In Fontan survivors, systemic venous hypertension may lead to liver and renal dysfunction. The incidence of hepatic & renal impairment, the best method of diagnosis, and their predisposing factors are unclear.
Methods: A total of 151 participants from a binational registry of 1,535 patients underwent hepatic evaluation using ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest) and standard laboratory testing, and renal evaluation using ultrasound, 99m-Tc-DTPA measurement of glomerular filtration rate (GFR), and urine albumin:creatinine ratio (ACR). Cut-off values of 10 kPa (FibroScan) and 0.49 (FibroTest) were used to define ≥F2/4 fibrosis. Proteinuria was defined as ACR ≥2.5 in males and ≥3.5 in females.
Results: Mean age and time since Fontan were 19.6 ± 9.2 and 14.0 ± 7.5 years, respectively. Fibrosis (≥F2/4) was present on FibroScan in 117/133 (88%), on FibroTest in 54/118 (46%), and on both in 40/104 (38%). Fibrosis was observed on ultrasound in 44/108 (41%), and no patient was identified to have hepatocellular carcinoma. Renal impairment was mild (GFR 60-89) in 43/130 (33%) and moderate (GFR 30-59) in 3/130 (2%). Proteinuria was detected in 54/121 participants (45%). FibroTest score was correlated with FibroScan value (r=0.248, p=0.01) and ACR (r=0.127, p=0.004). Independent predictors of increased FibroTest score (≥F2) included male gender (OR 2.52, 95% CI: 1.21-5.40, p=0.014) and time since Fontan (per year; OR 1.05, 95% CI: 1.03-1.08, p=0.034). No significant correlation between GFR and FibroScan or FibroTest was observed. Longer duration of time since Fontan was correlated with reduced GFR (1.2mL/min/1.73m2/year if proteinuria was present), and increased FibroScan and FibroTest values (r=-0.267, p=0.002; r=0.212, p=0.014; and r=0.227, p=0.013, respectively).
Conclusions: In this cross-sectional study, abnormalities in liver structure and renal function were common, and severity were associated with longer duration of time after Fontan. Proteinuria was present in almost half of patients. Prevention of end-organ dysfunction should be the focus of future research.
Author Disclosures: T.G. Wilson: None. Y. d’Udekem: Other Research Support; Significant; Yves d’Udekem is a NHMRC Clinician Practitioner Fellow (1082186). Consultant/Advisory Board; Significant; Yves d’Udekem is consultant for MSD and Actelion. D.S. Winlaw: None. R.L. Cordina: None. D.S. Celermajer: None. G.R. Wheaton: None. A. Bullock: Consultant/Advisory Board; Significant; Andrew Bullock reports consulting fees from Actelion. T.L. Gentles: None. R.G. Weintraub: Consultant/Advisory Board; Significant; Robert Weintraub serves on an advisory board for Actelion. R.N. Justo: None. L.E. Grigg: None. D.J. Radford: None. W. Hardikar: None. M. Cheung: None. T.M. Cain: None. P. Rao: None. S.I. Alexander: None. D. Zannino: None. A.J. Iyengar: None.
- © 2016 by American Heart Association, Inc.