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  • Hepatic and renal end-organ...
    Wilson, Thomas G.; d'Udekem, Yves; Winlaw, David S.; Cordina, Rachael L.; Celermajer, David S.; Wheaton, Gavin R.; Bullock, Andrew; Gentles, Thomas L.; Weintraub, Robert G.; Justo, Robert N.; Grigg, Leeanne E.; Radford, Dorothy J.; Hardikar, Winita; Cheung, Michael; Cain, Timothy M.; Rao, Padma; Alexander, Stephen I.; Ayer, Julian; Verrall, Charlotte; Du Plessis, Karin; Chapman, Janina; Rice, Kathryn; Barry, Judith; Zannino, Diana; Iyengar, Ajay J.

    International journal of cardiology, 12/2018, Letnik: 273
    Journal Article

    Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). Mean age and time since Fontan were 19.8 ± 9.3 and 14.1 ± 7.6 years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (r = 0.24, p = 0.015) and ACR (r = 0.29, p = 0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, p = 0.002). Renal impairment was mild (mGFR 60–89 ml/min/1.73 m2) in 46/131 (35%) and moderate (mGFR 30–59 ml/min/1.73 m2) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (β = 0.89, 95% CI 0.54–1.25, p = 0.002) and decreased mGFR (β = −0.77, 95% CI −1.29–0.24, p = 0.005). In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion. •In the second decade after the Fontan procedure, abnormalities in hepatic and renal structure and function are common.•Close to two-thirds of patients have ultrasonographic evidence of hepatic fibrosis.•Almost 50% of patients have an elevated serum-based hepatic fibrosis (FibroTest) score.•More than 50% of patients have either a reduced glomerular filtration rate or microalbuminuria.•Hepatic and renal structure and function should be monitored for potential impacts on outcomes after Fontan completion.