Evidence map›Paper›PMID 39939060›Full record

ArticleChest2025

Ineffectiveness of Sotatercept Therapy in a Patient With Heritable Pulmonary Arterial Hypertension Associated With a Previously Unreported Missense Variant in GDF2, the Gene for Bone Morphogenic Protein-9.

David Langleben, Lyda Lesenko, Benjamin D Fox, Shaun Eintracht, William D Foulkes, David S Rosenblatt

Abstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Chest, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing 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

1 citing paper in PubMed.

  1. Review
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

6 authors.

David LanglebenCenter for Pulmonary Vascular Disease, Division of Cardiology, Jewish General Hospital, McGill University, Montreal, QC, Canada; Lady Davis Institute for Medical Research, Jewish General Hospital, McGill University, Montreal, QC, Canada. Electronic address: david.langleben@mcgill.ca.
Lyda LesenkoCenter for Pulmonary Vascular Disease, Division of Cardiology, Jewish General Hospital, McGill University, Montreal, QC, Canada.
Benjamin D FoxDivision of Pulmonary Medicine, Yitzhak Shamir Hospital and Tel Aviv University, Tzrifin, Israel.
Shaun EintrachtDivision of Medical Biochemistry, Jewish General Hospital, McGill University, Montreal, QC, Canada.
William D FoulkesLady Davis Institute for Medical Research, Jewish General Hospital, McGill University, Montreal, QC, Canada; Division of Medical Genetics, Jewish General Hospital, McGill University, Montreal, QC, Canada.
David S RosenblattDivision of Medical Genetics, Jewish General Hospital, McGill University, Montreal, QC, Canada.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Pulmonary arterial hypertension (PAH) frequently is associated with an imbalance in antiproliferative bone morphogenic protein-2 receptor signaling and proproliferative type-II activin receptor signaling, favoring the latter. Sotatercept is an activin ligand trap that reduces the dominant detrimental activin signaling and provides clinical benefit. We report a patient with heritable PAH in whom sotatercept had neither positive nor negative effects; we relate that fact to his PAH being caused by a previously unreported variant of unknown significance (c.1276T>C, p.[Cys426Arg]) in the GDF2 gene. GDF2 encodes bone morphogenic protein type-9, the presence of which is required for proper functioning of the pulmonary microvasculature. Low levels of functionally active bone morphogenic protein type-9 contribute to PAH. As we enter an era of precision medicine for patients with PAH with increasingly costly therapies, genetic screening may direct appropriate therapy and limit the use of expensive but likely ineffective therapies.

Indexed as

Familial Primary Pulmonary HypertensionGrowth Differentiation Factor 2Pulmonary Arterial HypertensionHumansMutation, MissenseGDF2 protein, humanGrowth Differentiation Factor 2activinbone morphogenic proteinpulmonary arterial hypertensionsotatercepttelangiectasiavariant

Identifiers

PMID39939060
PMCPMC11867889

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.