Disease · MONDO_0001999
idiopathic pulmonary arterial hypertension
6 sourcesdiseases-2026-09-18-0.1.0
Cite this record
ConductScience Library. idiopathic pulmonary arterial hypertension. Diseases, record MONDO_0001999. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0001999
On this page
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
- IPAH, primary pulmonary arterial hypertension, primary pulmonary hypertension
- Open Targets
Drugs12
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 |
|---|---|---|---|---|---|---|---|---|
| EPOPROSTENOL SODIUM | CHEMBL962 | Small molecule | Approval | PMDA | 1 | 0 | 0 | Drugs and targets |
| SELEXIPAG | CHEMBL238804 | Small molecule | Approval | DailyMed | 1 | 0 | 0 | Drugs and targets |
| ILOPROST | CHEMBL494 | Small molecule | Phase 3 | ClinicalTrials.gov | 3 | 3 | 3 | Drugs and targets |
| TREPROSTINIL | CHEMBL1237119 | Small molecule | Phase 3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| TERBOGREL | CHEMBL281398 | Small molecule | Phase 2 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| ALBUTEROL | CHEMBL714 | Small molecule | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| ASPIRIN | CHEMBL25 | Small molecule | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| CLOPIDOGREL | CHEMBL1771 | Small molecule | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| EMPAGLIFLOZIN | CHEMBL2107830 | Small molecule | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| FERRIC CARBOXYMALTOSE | CHEMBL2108597 | Phase 2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
1 to 10 of 12
See all 12 drugsTargets11
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 | 4 | Prostanoid IP receptor agonist | Approval | https://conductscience.org/library/genes/5739 | Drugs and targets |
| ADRB2 | Beta-2 adrenergic receptor | 1 | Beta-2 adrenergic receptor agonist | Phase 2 | https://conductscience.org/library/genes/154 | Drugs and targets |
| GUCY1A1 | Soluble guanylate cyclase | 1 | Soluble guanylate cyclase activator | Phase 2 | https://conductscience.org/library/genes/2982 | Drugs and targets |
| GUCY1A2 | Soluble guanylate cyclase | 1 | Soluble guanylate cyclase activator | Phase 2 | https://conductscience.org/library/genes/2977 | Drugs and targets |
| GUCY1B1 | Soluble guanylate cyclase | 1 | Soluble guanylate cyclase activator | Phase 2 | https://conductscience.org/library/genes/2983 | Drugs and targets |
| P2RY12 | P2Y purinoceptor 12 | 1 | Purinergic receptor P2Y12 antagonist | Phase 2 | https://conductscience.org/library/genes/64805 | Drugs and targets |
| PTGS1 | Cyclooxygenase | 1 | Cyclooxygenase inhibitor | Phase 2 | https://conductscience.org/library/genes/5742 | Drugs and targets |
| PTGS2 | Cyclooxygenase | 1 | Cyclooxygenase inhibitor | Phase 2 | https://conductscience.org/library/genes/5743 | Drugs and targets |
| SLC5A2 | Sodium/glucose cotransporter 2 | 1 | Sodium/glucose cotransporter 2 inhibitor | Phase 2 | https://conductscience.org/library/genes/6524 | Drugs and targets |
| TBXA2R | Thromboxane A2 receptor | 1 | Thromboxane A2 receptor antagonist | Phase 2 | https://conductscience.org/library/genes/6915 | Drugs and targets |
1 to 10 of 11
See all 11 targetsTrials11
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 |
|---|---|---|---|---|---|---|---|---|
| NCT00709956 | Iloprost Power 15 in Pulmonary Arterial Hypertension | Phase 3 | 64 | 2012-10-26 | Pulmonary Arterial Hypertension | ILOPROST | Its record names the disease | ClinicalTrials.gov |
| NCT01447628 | IV Iron Replacement for Iron Deficiency in Idiopathic Pulmonary Arterial Hypertension (IPAH) Patients | Phase 2 | 56 | 2022-03-07 | Pulmonary Arterial Hypertension, Iron Deficiency | FERRIC CARBOXYMALTOSE, IRON DEXTRAN | Its record names the disease | ClinicalTrials.gov |
| NCT00250640 | Observation of Patients With Primary Pulmonary Hypertension Receiving Prescribed Ventavis Inhalation Therapy Regarding Safety and Efficacy for up to 4 Years | 106 | Hypertension, Pulmonary | ILOPROST | Its record names the disease | ClinicalTrials.gov | ||
| NCT02223481 | Safety and Efficacy of Terbogrel in Patients With Primary Pulmonary Hypertension | Phase 2 | 71 | Hypertension, Pulmonary | TERBOGREL | Its record names the disease | ClinicalTrials.gov | |
| NCT00882947 | Post-Marketing Surveillance of Ventavis® in Chinese Patients With Primary Pulmonary Hypertension (PPH) | 38 | Primary Hypertension | ILOPROST | Its record names the disease | ClinicalTrials.gov | ||
| NCT02223494 | Safety of Terbogrel in Patients With Primary Pulmonary Hypertension | Phase 2 | 33 | Hypertension, Pulmonary | TERBOGREL | Its record names the disease | ClinicalTrials.gov | |
| NCT00105209 | A Study of Aspirin and Clopidogrel in Patients With Idiopathic Pulmonary Arterial Hypertension | Phase 2 | 20 | Hypertension, Pulmonary | ASPIRIN, CLOPIDOGREL | Its record names the disease | ClinicalTrials.gov | |
| NCT05493371 | Empagliflozin in Pulmonary Arterial Hypertension | Phase 2 | 8 | Idiopathic Pulmonary Arterial Hypertension | EMPAGLIFLOZIN | Its record names the disease | ClinicalTrials.gov | |
| NCT00041574 | Hospital-Based Program for Treatment of Severe Cardiopulmonary Disease With Inhaled Nitric Oxide | Phase 2 | 7 | Pulmonary Hypertension, Lung Disease, Sickle Cell Disease, Cardiac Transplant, Lung Transplant | NITRIC OXIDE | Its record names the disease | ClinicalTrials.gov | |
| NCT02108743 | Albuterol and Dynamic Hyperinflation in Idiopathic Pulmonary Arterial Hypertension | Phase 2 | 0 | Idiopathic Pulmonary Arterial Hypertension. | ALBUTEROL | Its record names the disease | ClinicalTrials.gov |
1 to 10 of 11
See all 11 trialsComparisons in its trials1
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 |
|---|---|---|---|---|
| Iloprost (5µg) / Placebo vs Placebo / Iloprost (5 µg): serious adverse events | 0 of 31 | 1 of 33 | 64 | NCT00709956 |
Source Trial comparisons
Papers from its trials2
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_0001999
GET https://conductscience.org/api/diseases/v1/MONDO_0001999/drugs