Evidence map›Paper›PMID 31727111›Full record

ArticleOrphanet journal of rare diseases2019

Systemic bevacizumab for high-output cardiac failure in hereditary hemorrhagic telangiectasia: an international survey of HHT centers.

Hanny Al-Samkari, Hasan A Albitar, Scott E Olitsky, Marianne S Clancy, Vivek N Iyer

Open access · goldAbstract read
In one paragraph

Article in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
29citing papers in PubMed, 2 pooled it
3.3field-weighted citation impact, top 8% of its field
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

29 citing papers in PubMed, 2 syntheses or guidelines pooled it, 41 citations in OpenAlex.

  1. Efficacy of bevacizumab in hereditary hemorrhagic telangiectasia: a systematic review and network meta-analysis.European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2025
    Pooled it
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  8. [A case of hereditary hemorrhagic telangiectasia treated with bevacizumab].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2025
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  11. Observational
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  17. Cardiac and Hemodynamic Manifestations of Hereditary Hemorrhagic Telangiectasia.The International journal of angiology : official publication of the International College of Angiology, Inc · 2022
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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

5 authors at 3 institutions in 1 country.

Hanny Al-SamkariDivision of Hematology, Massachusetts General Hospital, Harvard Medical School, Zero Emerson Place Suite 118 Office 112, Boston, MA, 02114, USA. hal-samkari@mgh.harvard.edu.ORCID 0000-0001-6175-1383
Hasan A AlbitarDepartment of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
Scott E OlitskyCure HHT, Monkton, MD, USA.
Marianne S ClancyCure HHT, Monkton, MD, USA.
Vivek N IyerDivision of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
Harvard University · USMayo Clinic · USMayo Clinic in Arizona · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundSystemic bevacizumab is a novel targeted anti-angiogenic therapy for high-output cardiac failure (HOCF) in hereditary hemorrhagic telangiectasia (HHT) but published data is limited. This survey-based study measured physician-reported safety, effectiveness and current treatment practices for systemic bevacizumab in HHT-HOCF.

methodsA 27-item survey was sent to center directors of 31 international HHT Centers of Excellence.

resultsResponse rate was 74% with centers reporting 150 total patients receiving systemic bevacizumab for HHT-HOCF. Approximately two-thirds of centers had treated ≥5 patients. All centers utilize a 5 mg/kg dose for induction treatment and most administer 6 doses (range, 4-6) every 2 weeks, although maintenance regimens varied considerably. Center directors reported bevacizumab to be effective, with 55% reporting significant improvement in cardiac index and HOCF symptoms in most patients treated with bevacizumab, although normalization of cardiac parameters was uncommon. Adverse events were uncommon with three-quarters of centers reporting adverse event rates < 10%. Discontinuation for adverse events or ineffectiveness was rare. Bevacizumab was typically administered by hematologists and pulmonologists (50 and 39% of centers, respectively), with highly variable thresholds for initiation. Although half the centers reported difficulty with the insurance approval process, 70% of centers were ultimately able to obtain coverage for most or all of their patients.

conclusionsSystemic bevacizumab is a widely-used therapy for HHT-HOCF with reasonable safety and effectiveness. HHT centers appear to vary considerably in maintenance treatment practices and disease severity thresholds for initiation of bevacizumab in HHT-related HOCF.

Indexed as

AdolescentAdultAgedAntineoplastic Agents, ImmunologicalBevacizumabFemaleHeart FailureHumansMaleMiddle AgedSurveys and QuestionnairesTelangiectasia, Hereditary HemorrhagicYoung AdultAntineoplastic Agents, ImmunologicalBevacizumabArteriovenous malformationBevacizumabHeart failureHereditary hemorrhagic telangiectasiaHHTHigh-output cardiac failureOsler-weber-Rendu

Identifiers

PMID31727111
PMCPMC6857247
OpenAlexW2985961843

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

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.