Evidence map›Paper›PMID 40780770›Full record

ArticleJACC. Case reports2025

A 2-Decade Cardiac Magnetic Resonance Imaging Journey in Heritable Pulmonary Arterial Hypertension: Reversal With Sotatercept.

Bruno R Thal, Nils C Kremer, Sebastian Harth, Patrick Janetzko, Zvonimir A Rako, Simon Schäfer, Friedrich Grimminger, Hossein A Ghofrani, Werner Seeger, Khodr Tello

Abstract readCase Reports
In one paragraph

Article in JACC. Case reports, 2025. 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

10 authors.

Bruno R ThalDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany. Electronic address: https://twitter.com/BrunoRaphaeldD.
Nils C KremerDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany.
Sebastian HarthDepartment of Diagnostic and Interventional Radiology, Justus-Liebig-University Giessen, Giessen, Germany.
Patrick JanetzkoDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany.
Zvonimir A RakoDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany.
Simon SchäferDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany.
Friedrich GrimmingerDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany.
Hossein A GhofraniDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany; Department of Lung Development and Remodeling, Max Planck Institute for Heart and Lung Research, Bad Nauheim, Germany; Department of Medicine, Imperial College London, London, United Kingdom.
Werner SeegerDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany.
Khodr TelloDepartment of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany. Electronic address: Khodr.Tello@innere.med.uni-giessen.de.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundPulmonary arterial hypertension (PAH) is a progressive disease that leads to right heart failure. CASE SUMMARY: We report on the long-term follow-up of a male patient diagnosed at the age of 26 years with heritable PAH. Over 2 decades, he underwent several targeted therapies and repeated multimodal assessments, including cardiac magnetic resonance imaging (CMR). Despite a gradual clinical deterioration with right ventricular (RV) decompensation and an increase in pulmonary arterial pressure, the administration of sotatercept over 6 months led to a complete reversal of disease progression in terms of RV function and clinical status. DISCUSSION: This case demonstrates the evolving therapeutic landscape in PAH and the central role of CMR in the detailed assessment of RV function. Modern drugs can significantly modify the course of PAH. TAKE-HOME MESSAGE: Regular CMR examinations allow detailed monitoring of the disease course in PAH. Sotatercept can achieve impressive benefits even in late-stage PAH.

Indexed as

activin receptor ligand antagonistimagingpulmonary hypertensionright ventriclesotatercepttreatment

Identifiers

PMID40780770
PMCPMC12426550

What Socratic holds

Textmetadata
LicenceCC BY-NC-ND
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 Socratic graph.