Disease · MONDO_0005149

pulmonary hypertension

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. pulmonary hypertension. Diseases, record MONDO_0005149. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0005149

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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: pulmonary hypertension; Open Targets
Open Targets ID: MONDO_0005149; Open Targets
Disease Ontology: DOID:6432; Disease Ontology
MeSH: D006976; MeSH
Highest stage of its drugs: Approval, for 15 of its 105 drugs, from EMA Human Drugs, PMDA and TTD; Open Targets

Drugs105

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
SILDENAFIL	CHEMBL192	Small molecule	Approval	EMA Human Drugs	36	33	33	Drugs and targets
NITRIC OXIDE	CHEMBL1200689	Small molecule	Approval	EMA Human Drugs and PMDA	24	22	22	Drugs and targets
ILOPROST	CHEMBL494	Small molecule	Approval	EMA Human Drugs	22	21	21	Drugs and targets
TREPROSTINIL	CHEMBL1237119	Small molecule	Approval	EMA Human Drugs	21	19	18	Drugs and targets
BOSENTAN	CHEMBL957	Small molecule	Approval	EMA Human Drugs	12	10	10	Drugs and targets
TADALAFIL	CHEMBL779	Small molecule	Approval	EMA Human Drugs	12	10	10	Drugs and targets
RIOCIGUAT	CHEMBL2107834	Small molecule	Approval	EMA Human Drugs	11	10	10	Drugs and targets
MACITENTAN	CHEMBL2103873	Small molecule	Approval	EMA Human Drugs	9	6	6	Drugs and targets
AMBRISENTAN	CHEMBL1111	Small molecule	Approval	EMA Human Drugs	8	5	5	Drugs and targets
EPOPROSTENOL	CHEMBL1139	Small molecule	Approval	TTD	7	4	4	Drugs and targets

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Targets141

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	8	Prostanoid IP receptor agonist	Approval	https://conductscience.org/library/genes/5739	Drugs and targets
EDNRA	Endothelin receptor, ET-A/ET-B	6	Endothelin receptor ET-A antagonist; Endothelin receptor, ET-A/ET-B antagonist	Approval	https://conductscience.org/library/genes/1909	Drugs and targets
ADRA2A	Adrenergic receptor	5	Adrenergic receptor agonist; Adrenergic receptor alpha antagonist; Adrenergic receptor alpha-2 agonist	Approval	https://conductscience.org/library/genes/150	Drugs and targets
ADRA2B	Adrenergic receptor	5	Adrenergic receptor agonist; Adrenergic receptor alpha antagonist; Adrenergic receptor alpha-2 agonist	Approval	https://conductscience.org/library/genes/151	Drugs and targets
ADRA2C	Adrenergic receptor	5	Adrenergic receptor agonist; Adrenergic receptor alpha antagonist; Adrenergic receptor alpha-2 agonist	Approval	https://conductscience.org/library/genes/152	Drugs and targets
EDNRB	Endothelin receptor type B	5	Endothelin receptor ET-B antagonist; Endothelin receptor, ET-A/ET-B antagonist	Approval	https://conductscience.org/library/genes/1910	Drugs and targets
NR3C1	Glucocorticoid receptor	5	Glucocorticoid receptor agonist	Unknown	https://conductscience.org/library/genes/2908	Drugs and targets
PDE5A	cGMP-specific 3',5'-cyclic phosphodiesterase	5	Phosphodiesterase 5A inhibitor	Approval	https://conductscience.org/library/genes/8654	Drugs and targets
ADRA1A	Adrenergic receptor	4	Adrenergic receptor agonist; Adrenergic receptor alpha antagonist; Adrenergic receptor alpha-1 antagonist	Approval	https://conductscience.org/library/genes/148	Drugs and targets
ADRA1B	Adrenergic receptor	4	Adrenergic receptor agonist; Adrenergic receptor alpha antagonist; Adrenergic receptor alpha-1 antagonist	Approval	https://conductscience.org/library/genes/147	Drugs and targets

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Trials217

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. 2 more are not shown, since the checks do not confirm them.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT00768300	(ARTEMIS-IPF) Randomized, Placebo-Controlled Study to Evaluate Safety and Effectiveness of Ambrisentan in IPF	Phase 3	494	2014-04-08	Idiopathic Pulmonary Fibrosis	AMBRISENTAN	Its record names the disease	ClinicalTrials.gov
NCT02630316	Safety and Efficacy of Inhaled Treprostinil in Adult PH With ILD Including CPFE	Phase 2/3	326	2021-07-13	Pulmonary Hypertension, Interstitial Lung Disease, Combined Pulmonary Fibrosis and Emphysema	TREPROSTINIL	Its record names the disease	ClinicalTrials.gov
NCT03068130	Extended Access Program to Assess Long-term Safety of Bardoxolone Methyl in Patients With Pulmonary Hypertension RANGER	Phase 3	261	2023-10-12	Pulmonary Hypertension	BARDOXOLONE METHYL	Its record names the disease	ClinicalTrials.gov
NCT02633293	An Open Label Extension Study to Evaluate Inhaled Treprostinil in Adult PH With ILD Including CPFE	Phase 2/3	243	2022-12-02	Pulmonary Hypertension, Interstitial Lung Disease, Combined Pulmonary Fibrosis and Emphysema	TREPROSTINIL	Its record names the disease	ClinicalTrials.gov
NCT00380068	Safety and Efficacy Study of Ambrisentan in Subjects With Pulmonary Hypertension	Phase 3	224	2010-11-19	Pulmonary Hypertension	AMBRISENTAN	Its record names the disease	ClinicalTrials.gov
NCT01065454	A Study to Test the Effects of Riociguat in Patients With Pulmonary Hypertension Associated With Left Ventricular Systolic Dysfunction	Phase 2	202	2013-12-25	Hypertension, Pulmonary, Ventricular Dysfunction, Left	RIOCIGUAT	Its record names the disease	ClinicalTrials.gov
NCT03496623	A Phase 3 Adaptive Study to Evaluate the Safety and Efficacy of Inhaled Treprostinil in Participants With Pulmonary Hypertension (PH) Due to Chronic Obstructive Pulmonary Disease (COPD)	Phase 3	188	2023-11-24	Pulmonary Hypertension, Chronic Obstructive Pulmonary Disease	TREPROSTINIL	Its record names the disease	ClinicalTrials.gov
NCT00517933	Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis	Phase 3	180	2013-08-23	Pulmonary Fibrosis, Hypertension, Pulmonary	SILDENAFIL	Its record names the disease	ClinicalTrials.gov
NCT02951429	Efficacy, Safety, and Tolerability Study of Pirfenidone in Combination With Sildenafil in Participants With Advanced Idiopathic Pulmonary Fibrosis (IPF) and Intermediate or High Probability of Group 3 Pulmonary Hypertension	Phase 2	177	2020-11-12	Idiopathic Pulmonary Fibrosis	PIRFENIDONE, SILDENAFIL	Its record names the disease	ClinicalTrials.gov
NCT02036970	Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT	Phase 2	166	2021-07-23	Pulmonary Arterial Hypertension, Pulmonary Hypertension, Interstitial Lung Disease, Idiopathic Interstitial Pneumonia, Idiopathic Pulmonary Fibrosis, Sarcoidosis, Respiratory Bronchiolitis Associated Interstitial Lung Disease, Desquamative Interstitial Pneumonia, Cryptogenic Organizing Pneumonia, Acute Interstitial Pneumonitis, Idiopathic Lymphoid Interstitial Pneumonia, Idiopathic Pleuroparenchymal Fibroelastosis	BARDOXOLONE METHYL	Its record names the disease	ClinicalTrials.gov

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Comparisons in its trials64

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
Ambrisentan vs Placebo: serious adverse events	73 of 329	25 of 163	492	NCT00768300	Trial comparisons
Inhaled Treprostinil vs Placebo: Incidence of Clinical Worsening	37 of 163	54 of 163	326	NCT02630316	Trial comparisons
Active Inhaled Treprostinil vs Placebo: deaths from any cause	10 of 163	12 of 163	326	NCT02630316	Trial comparisons
Active Inhaled Treprostinil vs Placebo: serious adverse events	38 of 163	42 of 163	326	NCT02630316	Trial comparisons
Sildenafil vs Placebo: Change in 6-minute Walk Distance From Enrollment to Week 12 (≥ 20% Improvement)	9 of 89	6 of 91	180	NCT00517933	Trial comparisons
Sildenafil vs Placebo: serious adverse events	13 of 89	15 of 91	180	NCT00517933	Trial comparisons
Pirfenidone+Sildenafil vs Pirfenidone+Placebo: deaths from any cause	28 of 88	36 of 89	177	NCT02951429	Trial comparisons
Pirfenidone+Sildenafil vs Pirfenidone+Placebo: serious adverse events	54 of 88	55 of 89	177	NCT02951429	Trial comparisons
Riociguat up to 2.5 mg Tid vs Placebo: Number of Serious Adverse Events During Safety Follow-up Phase	18 of 73	14 of 74	147	NCT02138825	Trial comparisons
Inhaled Nitric Oxide (iNO) vs Placebo (Nitrogen): Necrotizing Enterocolitis (NEC)	5 of 59	10 of 65	124	NCT00955487	Trial comparisons

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Papers from its trials114

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
41904795	RESULT	1	PubMed
39046727	RESULT	1	PubMed
38803412	RESULT	1	PubMed
36614843	RESULT	1	PubMed
36027570	RESULT	1	PubMed
34790128	RESULT	1	PubMed
34449189	RESULT	1	PubMed
27812547	RESULT	1	PubMed
25918120	RESULT	1	PubMed
24431095	RESULT	1	PubMed

1 to 10 of 114

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Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

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_0005149

GET https://conductscience.org/api/diseases/v1/MONDO_0005149/drugs?page=2

API reference For AI assistants