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Home › Conditions › Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension

Part of Hypertension

Subtypes of Pulmonary Arterial Hypertension

Connective tissue disease-associated pulmonary arterial hypertension 1 Sjögren’s syndrome-associated pulmonary arterial hypertension 1 Systemic lupus erythematosus-associated pulmonary arterial hypertension 1 Systemic sclerosis-associated pulmonary arterial hypertension 1

8 published articles · Updated continuously

Clinical Trial Landscape

Clinical Trials for Pulmonary Arterial Hypertension

102 trials tracked for Pulmonary Arterial Hypertension: 53 in phase 3 or 4 and 22 with published results. The most-cited published study has 729 citations.

102Trials tracked
53Phase 3 & 4
0Recruiting
22With published results
Phase distribution
Phase 4 19 Phase 3 34 Phase 2 33 Phase 1 5 Other / NA 11
  1. Phase 3 A Study of Sotatercept for the Treatment of Pulmonary Arterial Hypertension (MK-7962-003/A011-11)(STELLAR) Completed · 729 cited
  2. Phase 4 A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis Completed · 172 cited
  3. Phase 3 The Efficacy and Safety of Initial Triple Versus Initial Dual Oral Combination Therapy in Patients With Newly Diagnosed Pulmonary Arterial Hypertension Completed · 171 cited
  4. Phase 3 A Study of Sotatercept in Participants With PAH WHO FC III or FC IV at High Risk of Mortality (MK-7962-006/ZENITH) Completed · 167 cited
  5. Phase 4 Riociguat rEplacing PDE-5i Therapy evaLuated Against Continued PDE-5i thErapy Completed · 159 cited
  6. Phase 3 Phase III Clinical Worsening Study of UT-15C in Subjects With PAH Receiving Background Oral Monotherapy Completed · 156 cited
Show 44 more trials
  1. Phase 3 Clinical Study to Evaluate the Effects of Macitentan on Exercise Capacity in Subjects With Eisenmenger Syndrome Completed · 130 cited
  2. Phase 3 Clinical Study of Macitentan in Patients With Pulmonary Arterial Hypertension to Psychometrically Validate the PAH-SYMPACT Instrument Completed · 65 cited
  3. Phase 4 Management of Acute Pulmonary Hypertensive Crisis in Children With Known Pulmonary Arterial Hypertension Completed · 60 cited
  4. Phase 4 REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension Completed · 58 cited
  5. Phase 3 Bosentan in Children With Pulmonary Arterial Hypertension Extension Study Completed · 42 cited
  6. Phase 4 Effect of Selexipag on Daily Life Physical Activity of Patients With Pulmonary Arterial Hypertension. Completed · 37 cited
  7. Phase 3 ACT-293987 in Pulmonary Arterial Hypertension Completed · 37 cited
  8. Phase 3 Study to Assess the Tolerability and the Safety of the Transition From Inhaled Treprostinil to Oral Selexipag in Patients With Pulmonary Arterial Hypertension Completed · 33 cited
  9. Phase 3 A Study of the Efficacy and Safety of Frespaciguat (MK-5475) in Participants With Pulmonary Arterial Hypertension (INSIGNIA-PAH: Phase 2/3 Study of an Inhaled sGC Stimulator in PAH) (MK-5475-007) Completed · 20 cited
  10. Phase 3 Clinical Study to Assess the Long-term Safety and Tolerability of ACT 064992 in Patients With Symptomatic Pulmonary Arterial Hypertension Completed · 18 cited
  11. Phase 4 EXPEDITE: A Study of Remodulin Induction Followed by Orenitram Optimization to Treat Pulmonary Arterial Hypertension Completed · 8 cited
  12. Phase 3 Safety Study of the Switch From Oral Selexipag to Intravenous Selexipag in Subjects With Stable Pulmonary Arterial Hypertension Completed · 7 cited
  13. Phase 3 An Open-Label, Long-Term Study of Oral Treprostinil in Subjects With Pulmonary Arterial Hypertension Completed · 5 cited
  14. Phase 4 Transition From Injectable Prostacyclin Medication to Inhaled Prostacyclin Medication Completed · 4 cited
  15. Phase 3 A Study of Sotatercept in Japanese Pulmonary Arterial Hypertension (PAH) Participants (MK-7962-020) Completed · 3 cited
  16. Phase 3 A Study to Assess Whether Macitentan Delays Disease Progression in Children With Pulmonary Arterial Hypertension (PAH) Completed
  17. Phase 4 BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed
  18. Phase 4 Safety And Efficacy Of Sildenafil In Children With Pulmonary Arterial Hypertension Completed
  19. Phase 4 TRUST-2: Safety and Efficacy of Intravenous Remodulin® in Patients in India With Pulmonary Arterial Hypertension (PAH) Completed
  20. Phase 4 Safety and Tolerability of Rapid Dose Titration of Subcutaneous Remodulin® Therapy in PAH Subjects (RAPID) Completed
  21. Phase 4 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed
  22. Phase 4 Safety Evaluation of Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis in Pulmonary Arterial Hypertension (PAH) Subjects Completed
  23. Phase 4 Epoprostenol for Injection in Pulmonary Arterial Hypertension - Extension of AC-066A401 Completed
  24. Phase 4 Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH) Completed
  25. Phase 4 Combination Therapy in Pulmonary Arterial Hypertension Completed
  26. Phase 4 Epoprostenol for Injection in Pulmonary Arterial Hypertension Completed
  27. Phase 4 Assess the Efficacy and Safety of Sildenafil When Added to Bosentan in the Treatment of Pulmonary Arterial Hypertension Completed
  28. Phase 4 Study of Add-on Ambrisentan Therapy to Background Phosphodiesterase Type-5 Inhibitor (PDE5i) Therapy in Pulmonary Arterial Hypertension (ATHENA-1) Completed
  29. Phase 3 A Pharmacokinetic Substudy of the TDE-PH-304 Protocol Completed
  30. Phase 3 Epoprostenol for Injection in Patients With Pulmonary Arterial Hypertension Completed
  31. Phase 3 Effects of Two Dosing Regimens of Bosentan in Children With Pulmonary Arterial Hypertension Completed
  32. Phase 3 FUTURE 3 Study Extension Completed
  33. Phase 3 Beraprost-314d Added-on to Tyvaso® (BEAT) Completed
  34. Phase 3 An Open-Label Extension Trial of UT-15C Sustained-release (SR) in Subjects With Pulmonary Arterial Hypertension Completed
  35. Phase 3 Pharmacokinetic Effects of QTI571 on Sildenafil and Bosentan in Pulmonary Arterial Hypertension Participants Completed
  36. Phase 3 A Study of Macitentan in Japanese Pediatric Participants With Pulmonary Arterial Hypertension Completed
  37. Phase 3 Epoprostenol for Injection (EFI/ACT-385781A) - Pulmonary Arterial Hypertension Completed
  38. Phase 3 A Long Term Extension Study Evaluating Safety Of Sildenafil Citrate When Used To Treat Pulmonary Arterial Hypertension (PAH) In Children Completed
  39. Phase 3 A Long Term Study of Ambrisentan in Pulmonary Arterial Hypertension Subjects Having Completed AMB-320 (NCT00423748) or AMB-321 (NCT00423202) Completed
  40. Phase 3 Selexipag (ACT-293987) in Pulmonary Arterial Hypertension Completed
  41. Phase 3 Safety and Efficacy of Imatinib Mesylate in Patients With Pulmonary Arterial Hypertension Completed
  42. Phase 3 Ranolazine and Pulmonary Hypertension Completed
  43. Phase 3 Iloprost Power 15 in Pulmonary Arterial Hypertension Completed
  44. Phase 3 Imatinib (QTI571) in Pulmonary Arterial Hypertension Completed

Showing the 50 most-cited and recently-updated of 102 trials. Browse the full registry →

Trial data sourced from ClinicalTrials.gov. Counts describe the research landscape and are not a treatment recommendation. Informational only — not medical advice.

What the trials found For clinicians

Pulmonary Arterial Hypertension: what the trials found

Several therapies have demonstrated significant improvements in clinical markers for pulmonary arterial hypertension. Sotatercept showed a statistically significant increase in 6-minute walk distance (6MWD) and a significant reduction in pulmonary vascular resistance (PVR) at week 24 20. Treprostinil diolamine was associated with a significant reduction in NT-proBNP levels by week 24 22, while inhaled treprostinil significantly improved 6MWD and CAMPHOR quality of life scores 16.

Other interventions showed varying degrees of efficacy. Riociguat demonstrated a statistically significant improvement in the number of participants with satisfactory clinical response at week 24 2. Macitentan was evaluated for PVR, 6MWD, and NT-proBNP levels, though results did not reach statistical significance compared to the control 21. Bosentan trials showed mixed results; while one study reported a significant change in 6MWD 12, another trial showed no significant difference in 6MWD at week 12 14.

Additional findings include a reduction in Borg Dyspnea Scores with treprostinil 6 and remodulin 8, as well as improvements in pulmonary vascular resistance with macitentan 4. Sildenafil was associated with changes in WHO functional class, blood pressure, and heart rate over an extended period 10.

Ambrisentan was associated with a recorded mean change in 6MWD at 12 weeks 13 and various improvements in 6MWD, Dyspnea Index, and CAMPHOR scores across multiple time points 19.

For the clinician treating this condition

  • Sotatercept is associated with significant improvements in both functional capacity (6MWD) and hemodynamic markers (PVR) at 24 weeks.
  • Treprostinil (both diolamine and inhaled forms) shows consistent evidence for improving patient outcomes, including NT-proBNP levels and quality of life scores.
  • Clinical responses to Riociguart and Bosentan vary; while Riociguart showed significant clinical response at 24 weeks, Bosentan's impact on 6MWD was not consistently significant across different study designs.

AI synthesis of 13 cited trials, updated Jun 29, 2026. Informational only — not medical advice; trial data sourced from ClinicalTrials.gov. How we use AI.

HCP Mode — summaries include clinical detail, trial data, and statistical outcomes.
Patient Mode — summaries use plain language, avoiding clinical jargon.
Multidisciplinary management strategies improve outcomes for pregnant women with pulmonary arterial hypertension
Diabetes & Endocrinology Sys. Review
Multidisciplinary management strategies improve outcomes for pregnant women with pulmonary arterial hypertension New management strategies for pregnant women with pulmonary arterial hypertension
This narrative review synthesizes management strategies for the preconception, antenatal, and postpartum periods in patients with pulmonary …
Managing high blood pressure in the lungs during pregnancy requires a team approach to keep both mother and baby safe before, during, and af…
Frontiers Aug 10, 2026
Vascular smooth muscle cell immune interactions drive remodeling across cardiovascular diseases
Oncology Sys. Review
Vascular smooth muscle cell immune interactions drive remodeling across cardiovascular diseases How blood vessel cells drive heart disease and high blood pressure
This narrative review synthesizes mechanisms of VSMC-immune interactions in atherosclerosis, hypertension, aortic aneurysms, and arteritis. …
A new review reveals how blood vessel cells change in heart disease, high blood pressure, and more.
Frontiers Jun 7, 2026
Mechanical shear stress influences endothelial RNA methylation in atherosclerosis and pulmonary arterial hypertension
Genetics & Precision Medicine Sys. Review
Mechanical shear stress influences endothelial RNA methylation in atherosclerosis and pulmonary arterial hypertension Mechanical forces on blood vessel cells may change how they repair themselves
This narrative review examines the interplay between hemodynamic shear stress and epitranscriptomic mechanisms in endothelial cells. The aut…
New review suggests blood flow forces might alter how RNA works in cells lining our vessels, potentially affecting heart and lung health.
Frontiers Jun 5, 2026
Narrative review highlights limited data on connective tissue disease-associated PAH in Han Chinese populations
Rheumatology Sys. Review
Narrative review highlights limited data on connective tissue disease-associated PAH in Han Chinese populations New data reveals how lung pressure risks differ for Chinese patients with connective tissue disease
This narrative review examines connective tissue disease-associated pulmonary arterial hypertension, specifically systemic sclerosis-associa…
A new review shows that lung pressure risks for Chinese patients with connective tissue disease differ from Western populations and require …
Frontiers May 6, 2026
Systematic review of immunosuppression for SLE-associated intestinal pseudo-obstruction
Allergy & Immunology Meta-analysis
Systematic review of immunosuppression for SLE-associated intestinal pseudo-obstruction SLE Can Shut Down the Gut — But It Might Be Reversible
This review synthesized 43 cases of SLE-associated intestinal pseudo-obstruction.
Lupus can paralyze the gut without a blockage, but early treatment with immunosuppressives often reverses this dangerous complication.
Frontiers Apr 20, 2026
Inflammatory and metabolic markers differ across systemic sclerosis lung phenotypes in retrospective study
Rheumatology Cohort
Inflammatory and metabolic markers differ across systemic sclerosis lung phenotypes in retrospective study Scleroderma Lung Damage May Leave Hidden Clues in Blood
A retrospective cross-sectional study of 314 SSc inpatients found higher inflammatory markers (ESR, hs-CRP, CRP/albumin ratio, SII) and anti…
Routine blood tests may soon reveal hidden lung damage patterns in scleroderma patients, helping doctors spot dangerous complications before…
Frontiers Apr 16, 2026
FDA Approves Winrevair for Pulmonary Arterial Hypertension (PAH) in Adults
Pulmonology & Critical Care FDA Approval
FDA Approves Winrevair for Pulmonary Arterial Hypertension (PAH) in Adults FDA approves a new treatment for a serious lung condition called pulmonary arterial hypertension
The FDA has approved Winrevair for the treatment of adults with pulmonary arterial hypertension (PAH, WHO Group 1) to improve exercise capac…
A new FDA-approved injection helps adults with pulmonary arterial hypertension exercise longer, improve daily life, and lower the risk of ho…
FDA Mar 31, 2026
Parenteral Treprostinil Shows Efficacy in Pediatric PAH, Meta-Analysis Confirms
Pediatrics Meta-analysis
Parenteral Treprostinil Shows Efficacy in Pediatric PAH, Meta-Analysis Confirms Can parenteral treprostinil help kids with pulmonary arterial hypertension?
Meta-analysis of 143 patients shows parenteral treprostinil benefits in pediatric PAH. Data supports efficacy and safety.
Parenteral treprostinil shows clear treatment benefits and safety for children with pulmonary arterial hypertension, offering hope for famil…
Mar 28, 2026

Research across Hypertension

Related studies from across the Hypertension family.

Nocturnal hypertension affects 42.5% of population samples, isolated nocturnal hypertension 17.3%
Cardiology Meta-analysis
Nocturnal hypertension affects 42.5% of population samples, isolated nocturnal hypertension 17.3% High blood pressure during sleep is common in many people
This meta-analysis estimates the prevalence of nocturnal hypertension and isolated nocturnal hypertension in population-based and community …
Nearly half of people with high blood pressure experience it specifically at night, a finding that helps doctors plan better screening tools…
Frontiers Oct 28, 2026
Study-defined hypertension is not a statistically significant predictor of recurrence in intracranial aneurysms
Neurology Meta-analysis
Study-defined hypertension is not a statistically significant predictor of recurrence in intracranial aneurysms High blood pressure may not predict aneurysm recurrence after treatment
This systematic review and meta-analysis of 2,442 observations from 8 studies found no statistically significant association between study-d…
New data suggests that a history of high blood pressure does not clearly predict if an aneurysm will return after surgery.
Frontiers Oct 26, 2026
Short-chain fatty acids and intestinal macrophages modulate metabolism in metabolic syndrome and obesity
Nutrition & Obesity Medicine Sys. Review
Short-chain fatty acids and intestinal macrophages modulate metabolism in metabolic syndrome and obesity Short Chain Fatty Acids May Help Manage Metabolic Syndrome
This narrative review explores how short-chain fatty acids (SCFAs) like butyrate, acetate, and propionate influence intestinal macrophage im…
Short-chain fatty acids like butyrate may influence how the body handles inflammation and metabolism in people with obesity and insulin resi…
Frontiers Oct 23, 2026
Gut microbiome dysbiosis may contribute to cognitive decline through neuroinflammation and hypertension-mediated cerebrovascular injury
Neurology Sys. Review
Gut microbiome dysbiosis may contribute to cognitive decline through neuroinflammation and hypertension-mediated cerebrovascular injury Gut health and blood pressure may impact brain health
This narrative review explores the gut-brain-vascular axis, proposing that gut microbiome dysbiosis contributes to cognitive decline via neu…
New research explores how an unbalanced gut and high blood pressure might work together to cause brain aging and cognitive decline.
Frontiers Oct 20, 2026
RAAS inhibitors modulate inflammatory responses and oxidative stress across multiple chronic conditions
Drug Pipeline Sys. Review
RAAS inhibitors modulate inflammatory responses and oxidative stress across multiple chronic conditions RAAS Inhibitors May Protect Organs Beyond Lowering Blood Pressure
This systematic review synthesizes the roles of ACEI, ARB, DRI, ARNI, and MRA in modulating inflammatory responses and oxidative stress. The…
New research suggests certain blood pressure medications may protect the heart, kidneys, and lungs by reducing inflammation and oxidative st…
Frontiers Oct 16, 2026
Aerobic Training Lowers Blood Pressure in Hypertension Across 26 Studies
Cardiology Meta-analysis
Aerobic Training Lowers Blood Pressure in Hypertension Across 26 Studies Aerobic training shows significant blood pressure drops for adults
This meta-analysis of 26 studies (1,576 adults with hypertension) found that aerobic training produced statistically significant and clinica…
Aerobic exercise provides meaningful reductions in both systolic and diastolic blood pressure for adults living with hypertension.
Frontiers Oct 14, 2026
KLF1 variants identified in neonate with severe hemolytic anemia and pulmonary hypertension
Pediatrics Sys. Review
KLF1 variants identified in neonate with severe hemolytic anemia and pulmonary hypertension Genetic testing helps identify a rare cause of severe anemia
This case report and literature review describes a neonate with unexplained Coombs/DAT-negative severe hemolytic anemia, pulmonary hypertens…
A rare genetic variant in the KLF1 gene can cause severe anemia and lung issues in newborns, making early testing vital for accurate diagnos…
Frontiers Oct 13, 2026
AI prescreening tool identifies primary aldosteronism with AUROC 0.709 in hypertension
Diabetes & Endocrinology RCT
AI prescreening tool identifies primary aldosteronism with AUROC 0.709 in hypertension AI tool helps identify patients with high blood pressure condition
In a retrospective cohort of 22,264 adults with hypertension or PA testing, an AI prescreening tool using EHR data achieved an AUROC of 0.70…
A new AI tool uses electronic health records to help doctors find patients who need testing for a specific heart condition.
medRxiv Oct 9, 2026
Aquatic Exercise Therapy for Blood Pressure Management in Elderly Patients with Hypertension
Cardiology Meta-analysis
Aquatic Exercise Therapy for Blood Pressure Management in Elderly Patients with Hypertension Pool workouts may lower blood pressure in older adults
Aquatic exercise therapy significantly lowers systolic and diastolic blood pressure in elderly patients, offering a viable non-pharmacologic…
A new analysis finds aquatic exercise may cut systolic blood pressure by about 11 points in older adults with hypertension.
Frontiers Oct 9, 2026
Mineralocorticoid receptor antagonists exert cardioprotective effects by restoring coronary microvascular function
Cardiology Sys. Review
Mineralocorticoid receptor antagonists exert cardioprotective effects by restoring coronary microvascular function Mineralocorticoid Receptor Antagonists May Improve Heart Function in Heart Failure
This mini-review examines the mechanisms of mineralocorticoid receptor antagonists in patients with heart failure, hypertension, and chronic…
New research highlights how mineralocorticoid receptor antagonists may protect the heart by improving blood flow in small vessels for patien…
Frontiers Oct 7, 2026
One anastomosis gastric bypass provides durable weight loss and metabolic improvements over 15 years
Nutrition & Obesity Medicine Meta-analysis
One anastomosis gastric bypass provides durable weight loss and metabolic improvements over 15 years Gastric Bypass Shows Durable Weight Loss and Health Improvements
This meta-analysis of 1729 patients evaluates the long-term outcomes of one anastomosis gastric bypass (OAGB). The analysis indicates durabl…
Gastric bypass surgery can lead to lasting weight loss and improved conditions like diabetes and high blood pressure over 15 years.
Oct 6, 2026
Selective aldosterone synthase inhibitors reduce systolic blood pressure by 7.9 mmHg in patients with CKD
Nephrology Meta-analysis
Selective aldosterone synthase inhibitors reduce systolic blood pressure by 7.9 mmHg in patients with CKD Aldosterone Synthase Inhibitors May Lower Blood Pressure in CKD Patients
This meta-analysis of 1148 patients with CKD and uncontrolled hypertension found that selective aldosterone synthase inhibitors (ASIs) reduc…
New research suggests that aldosterone synthase inhibitors could help manage high blood pressure and kidney damage in patients with chronic …
Oct 3, 2026

Questions about Pulmonary Arterial Hypertension

Does the new drug Winrevair improve exercise capacity for people with Pulmonary Arterial Hypertension?

Yes, the drug Winrevair (sotatercept) improves exercise capacity in adults with pulmonary arterial hypertension by increasing distance walked and oxygen use.

Full answer →
Can parenteral treprostinil help children with pediatric Pulmonary Arterial Hypertension based on recent studies?

Yes, recent studies show parenteral treprostinil can improve outcomes in children with PAH, though evidence is from observational studies, not large trials.

Full answer →