Evidence map›Paper›PMID 32776846›Full record

ReviewCirculation2020

Fontan-Associated Liver Disease: Screening, Management, and Transplant Considerations.

Juliet Emamaullee, Ali N Zaidi, Thomas Schiano, Jeffrey Kahn, Pamela L Valentino, Ryan E Hofer, Timucin Taner, Joyce W Wald, Kim M Olthoff, John Bucuvalas and 1 more

Abstract readReview
In one paragraph

Review in Circulation, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 130 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
130citing papers in PubMed, 2 pooled it
–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

130 citing papers in PubMed, 2 syntheses or guidelines pooled it.

  1. Pooled it
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  3. Article
  4. Pharmacokinetics in the Fontan circulation: a multi-organ puzzle.European heart journal. Cardiovascular pharmacotherapy · 2026
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  20. Imaging findings in Fontan-associated liver disease: Results of a single center study.International journal of cardiology. Congenital heart disease · 2026
    Article

70 more citing papers are in PubMed but not listed here.

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

11 authors.

Juliet EmamaulleeLiver Transplant Center, Children's Hospital-Los Angeles, CA (J.E.).
Ali N ZaidiMount Sinai Cardiovascular Institute (A.N.Z.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
Thomas SchianoDivisions of Hepatology (T.S.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
Jeffrey KahnDivision of Gastrointestinal and Liver Diseases (J.K.), Keck School of Medicine, University of Southern California, Los Angeles.
Pamela L ValentinoSection of Gastroenterology and Hepatology, Department of Pediatrics, Yale University School of Medicine, New Haven, CT (P.L.V.).
Ryan E HoferDepartments of Anesthesiology and Perioperative Medicine (R.E.H.), Mayo Clinic, Rochester, MN.
Timucin TanerSurgery (T.T.), Mayo Clinic, Rochester, MN.
Joyce W WaldDivision of Cardiology, Department of Medicine (J.W.W.), University of Pennsylvania, Philadelphia, PA.
Kim M OlthoffDepartment of Surgery (K.M.O.), University of Pennsylvania, Philadelphia, PA.
John BucuvalasPediatric Hepatology (J.B.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.
Ryan FischerDepartment of Gastroenterology, Liver Care Center, Children's Mercy Kansas City, MO (R.F.).

Funding

Yale Clinical and Translational Science Award (U Component)UL1TR001863 · NCATS · YALE UNIVERSITY · PI John H. Krystal, LUCILA OHNO-MACHADO · 2016 to 2026
$102.9M
Structural and Functional Studies of the Uterine LymphaticsR01HL141857 · NHLBI · UNIVERSITY OF SOUTHERN CALIFORNIA · PI Young-Kwon Hong · 2018 to 2026
$3.6M
NCATS NIH HHS UL1 TR001863NHLBI NIH HHS R01 HL141857
6 · The paper itself

Abstract

Surgical innovation and multidisciplinary management have allowed children born with univentricular physiology congenital heart disease to survive into adulthood. An estimated global population of 70 000 patients have undergone the Fontan procedure and are alive today, most of whom are <25 years of age. Several unexpected consequences of the Fontan circulation include Fontan-associated liver disease. Surveillance biopsies have demonstrated that virtually 100% of these patients develop clinically silent fibrosis by adolescence. As they mature, there are increasing reports of combined heart-liver transplantation resulting from advanced liver disease, including bridging fibrosis, cirrhosis, and hepatocellular carcinoma, in this population. In the absence of a transplantation option, these young patients face a poor quality of life and overall survival. Acknowledging that there are no consensus guidelines for diagnosing and monitoring Fontan-associated liver disease or when to consider heart transplantation versus combined heart-liver transplantation in these patients, a multidisciplinary working group reviewed the literature surrounding Fontan-associated liver disease, with a specific focus on considerations for transplantation.

Indexed as

Fontan ProcedureLiver TransplantationAnimalsHeart TransplantationHumansLiver DiseasesPostoperative ComplicationsFontan procedureheart transplantationliver diseasesliver transplantation

Identifiers

PMID32776846
PMCPMC7422927

What OpenQuestion holds

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Registered trials

None linked

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.