Evidence map›Paper›PMID 33435311›Full record

ReviewBiomedicines2021

Endothelial Dysfunction in Pulmonary Hypertension: Cause or Consequence?

Kondababu Kurakula, Valérie F E D Smolders, Olga Tura-Ceide, J Wouter Jukema, Paul H A Quax, Marie-José Goumans

Open access · goldAbstract readReview
In one paragraph

Review in Biomedicines, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 95 papers.

0numbers the graph read from it
0cells of the map it votes in
95citing papers in PubMed
13.4field-weighted citation impact, top 1% 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

95 citing papers in PubMed, 139 citations in OpenAlex.

  1. Article
  2. Association between vasoactive-inotropic score, clinical outcomes, defect size and laterality in congenital diaphragmatic hernia.Journal of perinatology : official journal of the California Perinatal Association · 2026
    Article
  3. Review
  4. Review
  5. Article
  6. Review
  7. Article
  8. Article
  9. Review
  10. Article
  11. Review
  12. Mechanisms Underlying Altitude-Induced and Group 3 Pulmonary Hypertension.International journal of molecular sciences · 2026
    Review
  13. Review
  14. Unveiling the Link Between COVID-19 and Pulmonary Hypertension.The Canadian journal of infectious diseases & medical microbiology = Journal canadien des maladies infectieuses et de la microbiologie medicale · 2026
    Review
  15. Review
  16. Article
  17. Article
  18. Review
  19. Increased lung [The European respiratory journal · 2025
    Article
  20. Article

35 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

6 authors at 2 institutions in 2 countries.

Kondababu KurakulaDepartment of Cell and Chemical Biology, Laboratory for CardioVascular Cell Biology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands.ORCID 0000-0002-9830-6344
Valérie F E D SmoldersDepartment of Surgery, Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, 2300 RC Leiden, The Netherlands.ORCID 0000-0002-9863-7581
Olga Tura-CeideDepartment of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, 08036 Barcelona, Spain.
J Wouter JukemaDepartment of Cardiology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands.ORCID 0000-0002-3246-8359
Paul H A QuaxDepartment of Surgery, Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, 2300 RC Leiden, The Netherlands.ORCID 0000-0002-6853-5760
Marie-José GoumansDepartment of Cell and Chemical Biology, Laboratory for CardioVascular Cell Biology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands.ORCID 0000-0001-9344-6746
Leiden University Medical Center · NLCentre for Biomedical Network Research on Rare Diseases · ES

Funding

a Miguel Servet grant from the Instituto de Salud Carlos III CP17/00114DCVA consortium grant PHAEDRA-IMPACTDutch Lung Foundation (Longfonds) grant 5.2.17.198J0European Commission Horizon 2020 research and innovation program under the MOGLYNET H2020-MSCA-ITN-EJD grant 675527Institute of Health Carlos III Spain, Catalan Society of Pneumology (SOCAP 2019) grants PI15/00582 and PI18/00960Leiden University Foundation grant W18378-2-32
6 · The paper itself

Abstract

Pulmonary arterial hypertension (PAH) is a rare, complex, and progressive disease that is characterized by the abnormal remodeling of the pulmonary arteries that leads to right ventricular failure and death. Although our understanding of the causes for abnormal vascular remodeling in PAH is limited, accumulating evidence indicates that endothelial cell (EC) dysfunction is one of the first triggers initiating this process. EC dysfunction leads to the activation of several cellular signalling pathways in the endothelium, resulting in the uncontrolled proliferation of ECs, pulmonary artery smooth muscle cells, and fibroblasts, and eventually leads to vascular remodelling and the occlusion of the pulmonary blood vessels. Other factors that are related to EC dysfunction in PAH are an increase in endothelial to mesenchymal transition, inflammation, apoptosis, and thrombus formation. In this review, we outline the latest advances on the role of EC dysfunction in PAH and other forms of pulmonary hypertension. We also elaborate on the molecular signals that orchestrate EC dysfunction in PAH. Understanding the role and mechanisms of EC dysfunction will unravel the therapeutic potential of targeting this process in PAH.

Indexed as

EndoMTendothelial dysfunctionepigeneticsinflammationpulmonary hypertensionTGF-βvasoactive factors

Identifiers

PMID33435311
PMCPMC7827874
OpenAlexW3118583265

What Socratic 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 Socratic graph.