Thomas G Wilson1, Yves d'Udekem2, David S Winlaw3, Rachael L Cordina4, David S Celermajer4, Gavin R Wheaton5, Andrew Bullock6, Thomas L Gentles7, Robert G Weintraub8, Robert N Justo9, Leeanne E Grigg10, Dorothy J Radford11, Winita Hardikar12, Michael Cheung13, Timothy M Cain14, Padma Rao14, Stephen I Alexander15, Julian Ayer3, Charlotte Verrall15, Karin Du Plessis16, Janina Chapman16, Kathryn Rice7, Judith Barry7, Diana Zannino16, Ajay J Iyengar17. 1. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia. 2. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia; Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia. Electronic address: yves.dudekem@rch.org.au. 3. The Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia; Department of Paediatrics, University of Sydney, Sydney, Australia. 4. Department of Paediatrics, University of Sydney, Sydney, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. 5. Department of Cardiology, Women's and Children's Hospital, Adelaide, Australia. 6. Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Australia. 7. Greenlane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand. 8. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia; Department of Cardiology, The Royal Children's Hospital, Melbourne, Australia. 9. Queensland Paediatric Cardiac Service, Lady Cilento Children's Hospital, Brisbane, Queensland, Australia. 10. Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Australia. 11. Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Australia; Faculty of Medicine, University of Queensland, Brisbane, Australia. 12. Department of Gastroenterology, The Royal Children's Hospital, Melbourne, Australia. 13. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Cardiology, The Royal Children's Hospital, Melbourne, Australia. 14. Medical Imaging Department, The Royal Children's Hospital, Melbourne, Australia. 15. The Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia. 16. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia. 17. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia; Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia.
Abstract
BACKGROUND: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. METHODS: 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). RESULTS: 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). CONCLUSIONS: 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.
BACKGROUND: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. METHODS: A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99mDTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). RESULTS: 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). CONCLUSIONS: 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.
Authors: Peter Kramer; Anastasia Schleiger; Marie Schafstedde; Friederike Danne; Johannes Nordmeyer; Felix Berger; Stanislav Ovroutski Journal: Front Cardiovasc Med Date: 2022-03-10
Authors: Evi Ritmeester; Veerle A Veger; Jelle P G van der Ven; Gabrielle M J W van Tussenbroek; Carine I van Capelle; Floris E A Udink Ten Cate; Willem A Helbing Journal: Front Cardiovasc Med Date: 2022-03-22