Fontan-associated liver disease: Diagnosis, surveillance, and management

C de Lange, T Möller, H Hebelka - Frontiers in pediatrics, 2023 - frontiersin.org
The Fontan operation is a lifesaving procedure for patients with functional single-ventricle
congenital heart disease, where hypoplastic left heart syndrome is the most frequent …

A path FORWARD: development of a comprehensive multidisciplinary clinic to create health and wellness for the child and adolescent with a Fontan circulation

J Rychik, DJ Goldberg, E Rand, EE Mancilla… - Pediatric …, 2022 - Springer
Today, it is anticipated most individuals diagnosed with single-ventricle malformation will
survive surgical reconstruction through a successful Fontan operation. As greater numbers …

EASL-ERN position paper on liver involvement in patients with Fontan-type circulation

L Téllez, A Payancé, E Tjwa, MJ Del Cerro, L Idorn… - Journal of …, 2023 - Elsevier
Fontan-type surgery is the final step in the sequential palliative surgical treatment of infants
born with a univentricular heart. The resulting long-term haemodynamic changes promote …

Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force

G Perucca, C de Lange, S Franchi-Abella… - Pediatric …, 2021 - Springer
Abstract Since Francis Fontan first introduced the eponymous technique, the Fontan
procedure, this type of surgical palliation has allowed thousands of children affected by …

Associations of liver stiffness measured by ultrasound shear-wave elastography with portal hypertension and circulatory failure in individuals with Fontan circulation

BE Derinkuyu, JR Dillman, AM Lubert… - American Journal of …, 2024 - Am Roentgen Ray Soc
BACKGROUND. The Fontan operation palliates single-ventricle congenital heart disease
but causes hepatic congestion with associated progressive hepatic fibrosis. OBJECTIVE …

Single center experience with durable continuous flow single ventricle assist device: a viable option in Fontan circulatory failure

SE Fahnhorst, K Brandewie, T Perry… - ASAIO …, 2023 - journals.lww.com
Ventricular assist devices are increasingly used for patients with single ventricle physiology.
We describe the use of durable, continuous flow, single ventricular assist device (SVAD) …

Spectrum of Fontan-associated liver disease assessed by MRI and US in young adolescents

KJ Thrane, LSO Müller, KR Suther, KS Thomassen… - Abdominal …, 2021 - Springer
Abstract Purpose Patients with Fontan circulation are at risk of developing hepatic
fibrosis/cirrhosis. The mechanisms and disease development are unclear and early …

Large Regenerative Nodules and Focal Nodular Hyperplasia-Like Lesions: Definition, Pathogenesis, and Imaging Findings

PE Nolan, R Catania, CL Vendrami… - Radiologic …, 2022 - radiologic.theclinics.com
Hypervascular liver lesions, when not obviously a hemangioma, often cause concern and
diagnostic dilemma. One classic and generally benign type of hypervascular liver lesion to …

Relation of magnetic resonance elastography to Fontan circulatory failure in a cohort of pediatric and adult patients

SW Brayer, F Zafar, AM Lubert, AT Trout, JJ Palermo… - Pediatric …, 2021 - Springer
Elevated magnetic resonance elastography (MRE)-derived liver stiffness may be associated
with worse outcomes in people with Fontan circulation. We sought to evaluate the …

Management of patients with single-ventricle physiology across the lifespan: contributions from magnetic resonance and computed tomography imaging

CZ Lam, ET Nguyen, SJ Yoo, RM Wald - Canadian Journal of Cardiology, 2022 - Elsevier
Cardiovascular magnetic resonance (CMR) and cardiac computed tomography (CCT) are
robust cross-sectional imaging modalities that are increasingly being used to guide the …