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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 713 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 · 713 cited
  2. Phase 4 A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis Completed · 173 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 4 Riociguat rEplacing PDE-5i Therapy evaLuated Against Continued PDE-5i thErapy Completed · 162 cited
  5. Phase 3 Phase III Clinical Worsening Study of UT-15C in Subjects With PAH Receiving Background Oral Monotherapy Completed · 155 cited
  6. 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 · 150 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 · 61 cited
  4. Phase 4 REPAIR: Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension Completed · 57 cited
  5. Phase 3 Bosentan in Children With Pulmonary Arterial Hypertension Extension Study Completed · 45 cited
  6. Phase 4 Effect of Selexipag on Daily Life Physical Activity of Patients With Pulmonary Arterial Hypertension. Completed · 38 cited
  7. Phase 3 ACT-293987 in Pulmonary Arterial Hypertension Completed · 34 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 Clinical Study to Assess the Long-term Safety and Tolerability of ACT 064992 in Patients With Symptomatic Pulmonary Arterial Hypertension Completed · 18 cited
  10. 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 · 17 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 · 2 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.

Research across Hypertension

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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 →