Disease · MONDO_0015924
pulmonary arterial hypertension
6 sourcesdiseases-2026-09-18-0.1.0
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ConductScience Library. pulmonary arterial hypertension. Diseases, record MONDO_0015924. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0015924
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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.
- Other names
Ayerza Arrilaga Syndrome, Ayerza Syndrome, Ayerza's Syndrome, Ayerzas Syndrome, HYPERTENSION PULM, Hypertension, Pulmonary, PAH, PHT - Pulmonary hypertension, PPH1, PULM HYPERTENSION, Pulmonary hypertension, NOS, Syndrome, Ayerza, Syndrome, Ayerza-Arrilaga, pulmonary hypertension, pulmonary hypertension (disorder), pulmonary hypertension, primary, 1
- Open Targets
- Highest stage of its drugs
- Approval, for 14 of its 118 drugs, from DailyMed, EMA Human Drugs, FDA, PMDA and TTD
- Open Targets
Drugs118
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 |
|---|---|---|---|---|---|---|---|---|
| TREPROSTINIL | CHEMBL1237119 | Small molecule | Approval | DailyMed, PMDA and TTD | 48 | 41 | 39 | Drugs and targets |
| BOSENTAN | CHEMBL957 | Small molecule | Approval | DailyMed, PMDA and TTD | 34 | 30 | 30 | Drugs and targets |
| AMBRISENTAN | CHEMBL1111 | Small molecule | Approval | DailyMed, PMDA and TTD | 33 | 29 | 29 | Drugs and targets |
| ILOPROST | CHEMBL494 | Small molecule | Approval | DailyMed, PMDA and TTD | 28 | 25 | 25 | Drugs and targets |
| MACITENTAN | CHEMBL2103873 | Small molecule | Approval | EMA Human Drugs and PMDA | 26 | 23 | 23 | Drugs and targets |
| TADALAFIL | CHEMBL779 | Small molecule | Approval | DailyMed, EMA Human Drugs and PMDA | 22 | 18 | 18 | Drugs and targets |
| RIOCIGUAT | CHEMBL2107834 | Small molecule | Approval | DailyMed and TTD | 20 | 18 | 18 | Drugs and targets |
| SELEXIPAG | CHEMBL238804 | Small molecule | Approval | DailyMed, PMDA and TTD | 20 | 17 | 17 | Drugs and targets |
| SOTATERCEPT | CHEMBL1743073 | Protein | Approval | FDA | 17 | 16 | 16 | Drugs and targets |
| SILDENAFIL CITRATE | CHEMBL1737 | Small molecule | Approval | DailyMed and PMDA | 15 | 11 | 11 | Drugs and targets |
1 to 10 of 118
See all 118 drugsTargets145
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 |
|---|---|---|---|---|---|---|
| PTGIR | Prostacyclin receptor | 9 | Prostanoid IP receptor agonist | Approval | https://conductscience.org/library/genes/5739 | Drugs and targets |
| EDNRA | Endothelin receptor, ET-A/ET-B | 8 | Endothelin receptor ET-A antagonist; Endothelin receptor, ET-A/ET-B antagonist | Approval | https://conductscience.org/library/genes/1909 | Drugs and targets |
| EDNRB | Endothelin receptor type B | 7 | Endothelin receptor ET-B antagonist; Endothelin receptor, ET-A/ET-B antagonist | Approval | https://conductscience.org/library/genes/1910 | Drugs and targets |
| PDE5A | cGMP-specific 3',5'-cyclic phosphodiesterase | 6 | Phosphodiesterase 5A inhibitor | Approval | https://conductscience.org/library/genes/8654 | Drugs and targets |
| KIT | Mast/stem cell growth factor receptor Kit | 4 | Stem cell growth factor receptor inhibitor | Phase 3 | https://conductscience.org/library/genes/3815 | Drugs and targets |
| PDGFRB | Platelet-derived growth factor receptor | 4 | Platelet-derived growth factor receptor beta inhibitor; Platelet-derived growth factor receptor inhibitor | Phase 3 | https://conductscience.org/library/genes/5159 | Drugs and targets |
| ABL1 | Bcr/Abl fusion protein | 3 | Bcr/Abl fusion protein inhibitor; Tyrosine-protein kinase ABL inhibitor | Phase 3 | https://conductscience.org/library/genes/25 | Drugs and targets |
| BCR | Bcr/Abl fusion protein | 3 | Bcr/Abl fusion protein inhibitor | Phase 3 | https://conductscience.org/library/genes/613 | Drugs and targets |
| GUCY1A1 | Soluble guanylate cyclase | 3 | Soluble guanylate cyclase activator; Soluble guanylate cyclase positive allosteric modulator | Approval | https://conductscience.org/library/genes/2982 | Drugs and targets |
| GUCY1A2 | Soluble guanylate cyclase | 3 | Soluble guanylate cyclase activator; Soluble guanylate cyclase positive allosteric modulator | Approval | https://conductscience.org/library/genes/2977 | Drugs and targets |
1 to 10 of 145
See all 145 targetsTrials365
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. 4 more are not shown, since the checks do not confirm them.
| Trial | Title | Phase | Participants | Results first posted | Conditions | Drugs | Linked by | Source |
|---|---|---|---|---|---|---|---|---|
| NCT01266265 | Study of Incidence of Respiratory Tract AEs in Patients Treated With Tyvaso® Compared to Other FDA Approved PAH Therapies | 1,333 | 2016-02-17 | Pulmonary Arterial Hypertension | EPOPROSTENOL, TREPROSTINIL | Its record names the disease | ClinicalTrials.gov | |
| NCT00811018 | A Long-Term, Open-Label Study to Evaluate the Safety of Sitaxsentan Sodium Treatment in Patients With Pulmonary Arterial Hypertension | Phase 3 | 1,192 | 2012-04-24 | Pulmonary Arterial Hypertension | SITAXENTAN | Its record names the disease | ClinicalTrials.gov |
| NCT01106014 | Selexipag (ACT-293987) in Pulmonary Arterial Hypertension | Phase 3 | 1,156 | 2016-03-01 | Pulmonary Arterial Hypertension | SELEXIPAG | Its record names the disease | ClinicalTrials.gov |
| NCT01027949 | An Open-Label Extension Trial of UT-15C Sustained-release (SR) in Subjects With Pulmonary Arterial Hypertension | Phase 3 | 894 | 2021-05-10 | Pulmonary Arterial Hypertension | TREPROSTINIL | Its record names the disease | ClinicalTrials.gov |
| NCT00660179 | Study of Macitentan (ACT-064992) on Morbidity and Mortality in Patients With Symptomatic Pulmonary Arterial Hypertension | Phase 3 | 742 | 2014-05-05 | Pulmonary Arterial Hypertension | MACITENTAN | Its record names the disease | ClinicalTrials.gov |
| NCT01112306 | ACT-293987 in Pulmonary Arterial Hypertension | Phase 3 | 709 | 2022-09-19 | Pulmonary Arterial Hypertension | SELEXIPAG | Its record names the disease | ClinicalTrials.gov |
| NCT01560624 | Phase III Clinical Worsening Study of UT-15C in Subjects With PAH Receiving Background Oral Monotherapy | Phase 3 | 690 | 2020-02-13 | Pulmonary Arterial Hypertension | TREPROSTINIL DIOLAMINE | Its record names the disease | ClinicalTrials.gov |
| NCT01178073 | A Study of First-Line Ambrisentan and Tadalafil Combination Therapy in Subjects With Pulmonary Arterial Hypertension (PAH) | Phase 3 | 610 | 2015-04-28 | Hypertension, Pulmonary | AMBRISENTAN, TADALAFIL | Its record names the disease | ClinicalTrials.gov |
| NCT00667823 | Clinical Study to Assess the Long-term Safety and Tolerability of ACT 064992 in Patients With Symptomatic Pulmonary Arterial Hypertension | Phase 3 | 550 | 2022-02-10 | Pulmonary Arterial Hypertension | MACITENTAN | Its record names the disease | ClinicalTrials.gov |
| NCT01560637 | An Open-Label, Long-Term Study of Oral Treprostinil in Subjects With Pulmonary Arterial Hypertension | Phase 3 | 471 | 2022-06-02 | Pulmonary Arterial Hypertension | TREPROSTINIL DIOLAMINE | Its record names the disease | ClinicalTrials.gov |
1 to 10 of 365
See all 365 trialsComparisons in its trials101
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 |
|---|---|---|---|---|---|
| Tyvaso vs Control: serious adverse events | 96 of 666 | 94 of 667 | 1,333 | NCT01266265 | Trial comparisons |
| Selexipag vs Placebo: serious adverse events | 252 of 575 | 272 of 577 | 1,152 | NCT01106014 | Trial comparisons |
| UT-15C vs Placebo: deaths from any cause | 17 of 346 | 18 of 344 | 690 | NCT01560624 | Trial comparisons |
| UT-15C vs Placebo: serious adverse events | 116 of 346 | 110 of 344 | 690 | NCT01560624 | Trial comparisons |
| Active vs Placebo Arm: Clinical Worsening Assessment | 8 of 174 | 12 of 176 | 350 | NCT00325442 | Trial comparisons |
| Active vs Placebo Arm: serious adverse events | 32 of 174 | 33 of 176 | 350 | NCT00325442 | Trial comparisons |
| Bosentan vs Placebo: serious adverse events | 73 of 159 | 102 of 174 | 333 | NCT00303459 | Trial comparisons |
| Sotatercept Plus Background PAH Therapy (DBPC Period) vs Placebo Plus Background PAH Therapy (DBPC Period): Number of Participants Who Discontinued Study Treatment Due to an AE | 3 of 163 | 10 of 160 | 323 | NCT04576988 | Trial comparisons |
| Sotatercept Plus Background PAH Therapy (DBPC Period) vs Placebo Plus Background PAH Therapy (DBPC Period): Number of Participants Who Experienced an Adverse Event (AE) | 138 of 163 | 140 of 160 | 323 | NCT04576988 | Trial comparisons |
| UT-15C SR vs Placebo: serious adverse events | 23 of 157 | 23 of 153 | 310 | NCT00887978 | Trial comparisons |
1 to 10 of 101
See all 101 comparisons in its trialsPapers from its trials208
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/MONDO_0015924
GET https://conductscience.org/api/diseases/v1/MONDO_0015924/drugs?page=2