Disease · EFO_0022954
chronic thromboembolic pulmonary hypertension
6 sourcesdiseases-2026-09-18-0.1.0
Cite this record
ConductScience Library. chronic thromboembolic pulmonary hypertension. Diseases, record EFO_0022954. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/EFO_0022954
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Disease
Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.
- Name
- chronic thromboembolic pulmonary hypertension
- Open Targets ID
- EFO_0022954
- Other names
- CTEPH
- Highest stage of its drugs
- Approval, for 1 of its 6 drugs, from PMDA
Source Open Targets
Drugs6
The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.
| Drug | ChEMBL ID | Type | Open Targets' stage | Stage reported by | Reports | Of them trials | Trials shown here | Source |
|---|---|---|---|---|---|---|---|---|
| RIOCIGUAT | CHEMBL2107834 | Small molecule | Approval | PMDA | 13 | 12 | 12 | Drugs and targets |
| MACITENTAN | CHEMBL2103873 | Small molecule | Phase 3 | ClinicalTrials.gov | 3 | 3 | 3 | Drugs and targets |
| AMBRISENTAN | CHEMBL1111 | Small molecule | Phase 3 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| BOSENTAN | CHEMBL957 | Small molecule | Phase 3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| TREPROSTINIL SODIUM | CHEMBL561157 | Small molecule | Phase 3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| ATORVASTATIN | CHEMBL1487 | Small molecule | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
Targets7
The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.
| Target | Protein | Its drugs that act on it | Mechanisms | Highest stage of those drugs | Gene in Genes | Source |
|---|---|---|---|---|---|---|
| EDNRA | Endothelin receptor, ET-A/ET-B | 3 | Endothelin receptor, ET-A/ET-B antagonist | Phase 3 | https://conductscience.org/library/genes/1909 | Drugs and targets |
| EDNRB | Endothelin receptor, ET-A/ET-B | 3 | Endothelin receptor, ET-A/ET-B antagonist | Phase 3 | https://conductscience.org/library/genes/1910 | Drugs and targets |
| GUCY1A1 | Soluble guanylate cyclase | 1 | Soluble guanylate cyclase positive allosteric modulator | Approval | https://conductscience.org/library/genes/2982 | Drugs and targets |
| GUCY1A2 | Soluble guanylate cyclase | 1 | Soluble guanylate cyclase positive allosteric modulator | Approval | https://conductscience.org/library/genes/2977 | Drugs and targets |
| GUCY1B1 | Soluble guanylate cyclase | 1 | Soluble guanylate cyclase positive allosteric modulator | Approval | https://conductscience.org/library/genes/2983 | Drugs and targets |
| HMGCR | 3-hydroxy-3-methylglutaryl-coenzyme A reductase | 1 | HMG-CoA reductase inhibitor | Phase 2 | https://conductscience.org/library/genes/3156 | Drugs and targets |
| PTGIR | Prostacyclin receptor | 1 | Prostanoid IP receptor agonist | Phase 3 | https://conductscience.org/library/genes/5739 | Drugs and targets |
Trials19
Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.
| Trial | Title | Phase | Participants | Results first posted | Conditions | Drugs | Linked by | Source |
|---|---|---|---|---|---|---|---|---|
| NCT00855465 | A Study to Evaluate Efficacy and Safety of Oral BAY63-2521 in Patients With CTEPH. | Phase 3 | 262 | 2014-03-11 | Pulmonary Hypertension | RIOCIGUAT | Its record names the disease | ClinicalTrials.gov |
| NCT00910429 | BAY63-2521 - Long-term Extension Study in Patients With Chronic Thromboembolic Pulmonary Hypertension | Phase 3 | 237 | 2020-10-22 | Pulmonary Hypertension | RIOCIGUAT | Its record names the disease | ClinicalTrials.gov |
| NCT00319111 | Bosentan in Patients With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) | Phase 3 | 151 | 2012-12-31 | Pulmonary Hypertension | BOSENTAN | Its record names the disease | ClinicalTrials.gov |
| NCT01416636 | Efficacy and Tolerability of Subcutaneously Administered Treprostinil Sodium in Patients With Severe (Non-operable) Chronic Thromboembolic Pulmonary Hypertension (CTREPH) | Phase 3 | 105 | 2019-06-10 | Non-operable Chronic Thromboembolic Pulmonary Hypertension | TREPROSTINIL SODIUM | Its record names the disease | ClinicalTrials.gov |
| NCT05339087 | Efficacy and Safety of Riociguat in Incipient Pulmonary Vascular Disease as an Indicator for Early PAH | Phase 2 | 35 | 2026-01-21 | Pulmonary Vascular Disorder, Primary Pulmonary Hypertension, Systemic Sclerosis, Other Systemic Involvement of Connective Tissue | RIOCIGUAT | Its record names the disease | ClinicalTrials.gov |
| NCT01884675 | Ambrisentan for Inoperable Chronic Thromboembolic Pulmonary Hypertension. | Phase 3 | 33 | 2015-12-16 | Hypertension | AMBRISENTAN | Its record names the disease | ClinicalTrials.gov |
| NCT01894022 | A Open Label Study to Assess the Long-term Safety, Tolerability and Efficacy of Ambrisentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) | Phase 3 | 19 | 2017-06-08 | Hypertension | AMBRISENTAN | Its record names the disease | ClinicalTrials.gov |
| NCT02117791 | Prospective, Non-interventional, Multi-center Post-authorization Safety Study of Riociguat for Chronic Thromboembolic Pulmonary Hypertension (CTEPH ) | 1,298 | Hypertension, Pulmonary | RIOCIGUAT | Its record names the disease | ClinicalTrials.gov | ||
| NCT00615823 | Atorvastatin in Pulmonary Hypertension | Phase 2 | 220 | Hypertension, Pulmonary | ATORVASTATIN | Its record names the disease | ClinicalTrials.gov | |
| NCT02545465 | A Study to Understand the Treatment Patterns in Patients With Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension During a Switch of Treatment to Adempas in Real-life Clinical Practice | 125 | Hypertension, Pulmonary | RIOCIGUAT | Its record names the disease | ClinicalTrials.gov |
1 to 10 of 19
See all 19 trialsComparisons in its trials16
Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.
| Comparison | Group 1, with the outcome | Group 2, with the outcome | Participants | Trial | Source |
|---|---|---|---|---|---|
| Riociguat (Adempas, BAY63-2521)_individual Dose Titration vs Placebo: All Caused Mortality | 2 of 173 | 3 of 88 | 261 | NCT00855465 | Trial comparisons |
| Riociguat (Adempas, BAY63-2521)_individual Dose Titration vs Placebo: serious adverse events | 34 of 173 | 14 of 88 | 261 | NCT00855465 | Trial comparisons |
| Riociguat-Former Riociguat 1.0-2.5 mg vs Riociguat-Former Placebo: Number of Participants With Death | 22 of 155 | 13 of 82 | 237 | NCT00910429 | Trial comparisons |
| Former Riociguat 1.0-2.5 mg vs Former Placebo: deaths from any cause | 22 of 155 | 13 of 82 | 237 | NCT00910429 | Trial comparisons |
| Former Riociguat 1.0-2.5 mg vs Former Placebo: serious adverse events | 96 of 155 | 56 of 82 | 237 | NCT00910429 | Trial comparisons |
| High Dose vs Low Dose: Number of Participants With Clinical Worsening | 7 of 53 | 12 of 52 | 105 | NCT01416636 | Trial comparisons |
| High Dose vs Low Dose: deaths from any cause | 2 of 53 | 1 of 52 | 105 | NCT01416636 | Trial comparisons |
| High Dose vs Low Dose: serious adverse events | 9 of 53 | 10 of 52 | 105 | NCT01416636 | Trial comparisons |
| Riociguat vs Placebo: deaths from any cause | 0 of 17 | 0 of 18 | 35 | NCT05339087 | Trial comparisons |
| Riociguat vs Placebo: serious adverse events | 2 of 17 | 1 of 18 | 35 | NCT05339087 | Trial comparisons |
1 to 10 of 16
See all 16 comparisons in its trialsPapers from its trials26
The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.
Data dates
The date of each source this release read.
Sources 6
- ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
- ChEMBL, through Open TargetsCC BY-SA 3.0
- ClinicalTrials.govNo license stated
- NLM's MeSHNLM's terms
- Open TargetsCC0 1.0
- PubMedNLM's terms
Each source's terms
- ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
- ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- ClinicalTrials.govnone stated
- NLM's MeSHNLM's
- Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- PubMedNLM's terms
ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.
For software
Read this disease from software.
The record and each of its lists are JSON, twenty rows a page. No key.
GET https://conductscience.org/api/diseases/v1/EFO_0022954
GET https://conductscience.org/api/diseases/v1/EFO_0022954/papers?page=2