Evidence map›Paper›PMID 42322353›Full record

ArticlePediatric cardiology2026

The FUEL FALD Study: Effects of Udenafil on Liver Stiffness and Fibrosis after Fontan.

Kurt R Schumacher, Jonathan Dillman, David J Goldberg, Mark Cartoski, Michael DiMaria, Benjamin Frischhertz, Salil Ginde, Bryan H Goldstein, Nagraj Kasi, Christine K Lee and 14 more

Abstract read
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In one paragraph

Article in Pediatric cardiology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

24 authors.

Kurt R SchumacherDepartment of Pediatrics, University of Michigan, Ann Arbor, USA. kurts@med.umich.edu.ORCID http://orcid.org/0000-0001-8659-736X
Jonathan DillmanDepartment of Pediatric Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, USA.
David J GoldbergChildren's Hospital of Philadelphia, Philadelphia, USA.
Mark CartoskiAlfred Dupont Children's Hospital, Wilmington, USA.
Michael DiMariaHCA HealthONE Rocky Mountain Children's at Presbyterian St. Luke's, Denver, USA.
Benjamin FrischhertzVanderbilt University, Nashville, USA.
Salil GindeChildren's Hospital of Wisconsin, Milwauke, USA.
Bryan H GoldsteinDepartment of Pediatrics, University of Pittsburgh Medical Center, Pittsburgh, USA.
Nagraj KasiMedical University of South Carolina, Charleston, USA.
Christine K LeeDepartment of Pediatrics, Boston Children's Hospital, Boston, USA.
Daniel H LeungDepartment of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine, Texas Children's Hospital, Houston, USA.
Chad Y MaoChildren's Healthcare of Atlanta, Atlanta, USA.
Brian W McCrindleHospital for Sick Children, University of Toronto, Toronto, USA.
Shaji MenonDepartment of Pediatrics, University of Pittsburgh Medical Center, Pittsburgh, USA.
Matthew O'ConnorChildren's Hospital of Philadelphia, Philadelphia, USA.
Alexander R OpotowskyDepartment of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, USA.
Joseph PalermoDepartment of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, USA.
Stephen M ParidonChildren's Hospital of Philadelphia, Philadelphia, USA.
Daniel PennyDepartment of Pediatrics, Division of Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston, USA.
Rahul RathodDepartment of Pediatric Cardiology, Boston Children's Hospital, Boston, USA.
Jonathan B WagnerDepartment of Pediatrics, University of Missouri-Kansas City School of Medicine, Kansas City, USA.
Angela YetmanDepartment of Pediatrics, University of Nebraska Medical Center, Omaha, USA.
Victor ZakCarelon Research, Wilmington, USA.
Mark RussellDepartment of Pediatrics, University of Michigan, Ann Arbor, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Fontan-associated liver disease (FALD) is a fibrotic liver disease in individuals with Fontan physiology. This study sought to determine the efficacy of treatment with udenafil, a phosphodiesterase type 5 inhibitor, for reducing liver congestion leading to a decreased liver stiffness and reduction in serologic biomarkers of liver fibrosis. In patients enrolling in the FUEL trial open label extension, liver stiffness measurements were obtained by ultrasound shear wave elastography (SWE) and magnetic resonance elastography (MRE); serum markers of liver fibrosis were measured by the Enhanced Liver Fibrosis (ELF) score. Change in liver stiffness and ELF score after 12 months of udenafil treatment were analyzed. In 88 subjects, 36 were previously treated with udenafil and 52 were udenafil-naïve. The cohort's baseline liver stiffness was elevated by SWE, mean 2.0 ± 0.4 m/sec (adult upper limit normal 1.5 m/sec), and MRE, mean 5.3 ± 1.2 kPa (adult upper limit normal 2.8 kPa). After 12 months of udenafil treatment, there was no change in SWE (post-treatment mean 1.9 ± 0.3 m/sec, p = 0.28) nor change in MRE (post-treatment mean 5.2 ± 0.97 kPa, p = 0.68). The cohort's baseline ELF score was elevated at 10.4 ± 1.0 according to values correlating with liver fibrosis in other diseases. ELF score decreased at 12 months to 9.8 ± 0.8 (p < 0.0001). Liver stiffness did not change following 12 months of udenafil treatment, but ELF scores significantly decreased. Further study is needed to understand the relationship of ELF score to age, but these results suggest the possibility that udenafil may alter the overall Fontan fibrotic milieu.

Indexed as

Congenital heart diseaseFontanFontan-associated liver diseasePDE5-inhibitorPulmonary vasodilator

Identifiers

PMID42322353

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.