Luis Téllez1, Enrique Rodríguez de Santiago1, Beatriz Minguez2, Audrey Payance3, Ana Clemente4, Anna Baiges5, Dalia Morales-Arraez6, Vincenzo La Mura7, Elba Llop8, Elena Garrido1, Elvira Garrido-Lestache9, Stephanie Tasayco2, Onorina Bruno10, Raquel Prieto11, Silvia Montserrat12, Mónica Pons2, Andreína Olavarría13, Laura Dos14, Antoine Legendre15, María Jesús Del Cerro9, Rafael Bañares4, Juan Carlos García-Pagán5, Pierre-Emmanuel Rautou3, Agustín Albillos16. 1. Servicio de Gastroenterología y Hepatología, Hospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá, Madrid, Spain. 2. Liver Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut of Research, CIBERehd, Universitat Autonoma de Barcelona, Barcelona, Spain. 3. Service d'Hépatologie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France. 4. Liver Unit, Hospital General Universitario Gregorio Marañón, Instituto de Investigación Sanitaria Gregorio Marañón, CIBERehd, Universidad Complutense, Madrid, Spain. 5. Barcelona Hepatic Hemodynamic lab, Liver Unit, Hospital Clínic, IDIBAPS, CIBERehd, Universidad de Barcelona, Barcelona, Spain. 6. Gastroenterology Department, University Hospital of the Canary Islands, La Laguna, Tenerife, Spain. 7. Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, U.O.C. Medicina Generale Emostasi e Trombosi, C.R.C. "A.M. e A. Migliavacca" per lo Studio e la Cura delle Malattie del Fegato and Dipartimento di Scienze Biomediche per la Salute, Università degli studi di Milano, Milano, Italy. 8. Servicio de Gastroenterología y Hepatología, Hospital Universitario Puerta de Hierro, Instituto de Investigación Sanitaria Puerta de Hierro, CIBERehd, Universidad Autónoma de Madrid, Madrid, Spain. 9. Servicio de Cardiología Infantil, Hospital Universitario Ramón y Cajal, IRYCIS, Universidad de Alcalá, Madrid, Spain. 10. Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France. 11. Department of Cardiology, Hospital General Universitario Gregorio Marañón, Instituto de Investigación Sanitaria Gregorio Marañón, CIBERCV, Universidad Complutense, Madrid, Spain. 12. Institut Clínic Cardio-Vascular (ICCV), Hospital Clínic, Universitat de Barcelona, Catalonia, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain; Centro de Investigacíon Biomédica en Red Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain. 13. Servicio de Radiodiagnóstico, Hospital Universitario Ramón y Cajal, Madrid, Spain. 14. Unitat Integrada de Cardiopaties Congènites de l'Adolescent i de l'Adult Vall d'Hebron-Sant Pau, Servicio de Cardiología, Hospital Universitario Vall d'Hebron, CIBERCV, Barcelona, Spain. 15. Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de référence Malformations Cardiaques Congénitales Complexes - M3C, Paris, France. 16. Servicio de Gastroenterología y Hepatología, Hospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá, Madrid, Spain. Electronic address: agustin.albillos@uah.es.
Abstract
BACKGROUND & AIMS: Fontan surgery is used to treat a variety of congenital heart malformations, and may lead to advanced chronic liver disease in the long-term. This study examines the prevalence, characteristics and predictors of liver nodules in patients following Fontan surgery. METHODS: This was a prospective, cross-sectional, observational study conducted at 8 European centres. Consecutive patients who had undergone Fontan surgery underwent blood tests, abdominal ultrasonography (US), transient elastography (Fibroscan®), echocardiography, haemodynamic assessments, and abdominal MRI/CT scan. The primary outcome measure was liver nodules detected in the MRI/CT scan. Predictors of liver nodules were identified by multivariate logistic regression. RESULTS: One hundred and fifty-two patients were enrolled (mean age 27.3 years). The mean time elapsed from surgery to inclusion was 18.3 years. Liver nodule prevalences were 29.6% (95% CI 23-37%) on US and 47.7% (95% CI 39-56%) on MRI/CT. Nodules were usually hyperechoic (76.5%), round-shaped (>80%), hyperenhancing in the arterial phase (92%) and located in the liver periphery (75%). The sensitivity and specificity of US were 50% (95% CI 38-62%) and 85.3% (95% CI 75-92%), respectively. Inter-imaging test agreement was low (adjusted kappa: 0.34). In the multivariate analysis, time since surgery >10 years was the single independent predictor of liver nodules (odds ratio 4.18; p = 0.040). Hepatocellular carcinoma was histologically diagnosed in 2 of the 8 patients with hypervascular liver nodules displaying washout. CONCLUSION: While liver nodules are frequent in Fontan patients, they may go unnoticed in US. Liver nodules are usually hyperechoic, hypervascular and predominantly peripheral. This population is at risk of hepatocellular carcinoma, the diagnosis of which requires confirmatory biopsy. LAY SUMMARY: Fontan surgery is the standard of care for many patients with univentricular congenital cardiopathies. Recent advances have improved the survival of Fontan patients, and nowadays most of them reach adulthood. In this setting, Fontan-associated liver disease (FALD) is increasingly recognised, and has become a significant prognostic factor. Liver nodules are considered a component of FALD yet their prevalence, imaging features and predictors have hardly been evaluated. In this study, we observed that liver nodules are frequent, typically hyperechoic, hypervascular and predominantly peripheral in patients with FALD. This population is at risk of hepatocellular carcinoma, the diagnosis of which must be confirmed by biopsy.
BACKGROUND & AIMS: Fontan surgery is used to treat a variety of congenital heart malformations, and may lead to advanced chronic liver disease in the long-term. This study examines the prevalence, characteristics and predictors of liver nodules in patients following Fontan surgery. METHODS: This was a prospective, cross-sectional, observational study conducted at 8 European centres. Consecutive patients who had undergone Fontan surgery underwent blood tests, abdominal ultrasonography (US), transient elastography (Fibroscan®), echocardiography, haemodynamic assessments, and abdominal MRI/CT scan. The primary outcome measure was liver nodules detected in the MRI/CT scan. Predictors of liver nodules were identified by multivariate logistic regression. RESULTS: One hundred and fifty-two patients were enrolled (mean age 27.3 years). The mean time elapsed from surgery to inclusion was 18.3 years. Liver nodule prevalences were 29.6% (95% CI 23-37%) on US and 47.7% (95% CI 39-56%) on MRI/CT. Nodules were usually hyperechoic (76.5%), round-shaped (>80%), hyperenhancing in the arterial phase (92%) and located in the liver periphery (75%). The sensitivity and specificity of US were 50% (95% CI 38-62%) and 85.3% (95% CI 75-92%), respectively. Inter-imaging test agreement was low (adjusted kappa: 0.34). In the multivariate analysis, time since surgery >10 years was the single independent predictor of liver nodules (odds ratio 4.18; p = 0.040). Hepatocellular carcinoma was histologically diagnosed in 2 of the 8 patients with hypervascular liver nodules displaying washout. CONCLUSION: While liver nodules are frequent in Fontan patients, they may go unnoticed in US. Liver nodules are usually hyperechoic, hypervascular and predominantly peripheral. This population is at risk of hepatocellular carcinoma, the diagnosis of which requires confirmatory biopsy. LAY SUMMARY: Fontan surgery is the standard of care for many patients with univentricular congenital cardiopathies. Recent advances have improved the survival of Fontan patients, and nowadays most of them reach adulthood. In this setting, Fontan-associated liver disease (FALD) is increasingly recognised, and has become a significant prognostic factor. Liver nodules are considered a component of FALD yet their prevalence, imaging features and predictors have hardly been evaluated. In this study, we observed that liver nodules are frequent, typically hyperechoic, hypervascular and predominantly peripheral in patients with FALD. This population is at risk of hepatocellular carcinoma, the diagnosis of which must be confirmed by biopsy.
Authors: Charlotte de Lange; Karl Julius Thrane; Kristian S Thomassen; Oliver Geier; Bac Nguyen; Anders Tomterstad; Lil-Sofie Ording Müller; Erik Thaulow; Runar Almaas; Gaute Døhlen; Kathrine Rydén Suther; Thomas Möller Journal: Pediatr Radiol Date: 2020-10-09
Authors: Giulia Perucca; Charlotte de Lange; Stéphanie Franchi-Abella; Marcello Napolitano; Michael Riccabona; Damjana Ključevšek; Seema Toso; Jochen Herrmann; Samuel Stafrace; Kassa Darge; Maria Beatrice Damasio; Costanza Bruno; Magdalena Maria Woźniak; Luisa Lobo; Donald Ibe; Anne M Smets; Philippe Petit; Lil-Sofie Ording Müller Journal: Pediatr Radiol Date: 2021-10-15