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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

7 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 680 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 · 680 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 · 170 cited
  4. Phase 4 Riociguat rEplacing PDE-5i Therapy evaLuated Against Continued PDE-5i thErapy Completed · 161 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 · 131 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 · 127 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 · 56 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 · 36 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 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
  10. Phase 3 Clinical Study to Assess the Long-term Safety and Tolerability of ACT 064992 in Patients With Symptomatic Pulmonary Arterial Hypertension 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.
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.

Maternal hypertensive disorders of pregnancy are associated with increased offspring asthma risk
Psychiatry Meta-analysis
Maternal hypertensive disorders of pregnancy are associated with increased offspring asthma risk Maternal Pregnancy High Blood Pressure Linked to Higher Asthma Risk
This meta-analysis of over three million mother-infant pairs found a significant association between maternal hypertensive disorders of preg…
New data suggests that mothers with high blood pressure during pregnancy may have children at a higher risk for developing asthma.
Frontiers Jul 29, 2026
Inflammation and immune dysregulation drive chronic kidney disease progression and associated tissue remodeling
Nephrology Sys. Review
Inflammation and immune dysregulation drive chronic kidney disease progression and associated tissue remodeling Inflammation and Immune Issues Drive Chronic Kidney Disease Progression
This mini review synthesizes the role of inflammation and immune dysregulation in the initiation and progression of chronic kidney disease. …
Persistent inflammation and immune system issues are central drivers of chronic kidney disease, leading to tissue damage and potential compl…
Frontiers Jul 24, 2026
Neural-immune-cardiovascular axis emerges as therapeutic target in atherosclerosis, hypertension, heart failure
Allergy & Immunology Sys. Review
Neural-immune-cardiovascular axis emerges as therapeutic target in atherosclerosis, hypertension, heart failure New pathways link nerves and immunity in heart disease
This systematic review synthesizes evidence on the neural-immune-cardiovascular axis in cardiovascular homeostasis and pathogenesis. It exam…
Scientists are mapping how the connection between your nervous system and immune cells may drive conditions like high blood pressure and hea…
Frontiers Jul 22, 2026
Structured exercise may improve blood pressure and outcomes in hypertensive disorders of pregnancy
Cardiology Meta-analysis
Structured exercise may improve blood pressure and outcomes in hypertensive disorders of pregnancy Structured exercise may help manage high blood pressure during pregnancy
This scoping review examines structured exercise interventions, including aerobic training, resistance training, stretching, and yoga, for w…
New research suggests that structured exercises like yoga and resistance training could improve health for pregnant women with high blood pr…
Frontiers Jul 21, 2026
Unified cardiometabolic disease framework links T2DM, ASCVD, HFpEF, MASLD, hypertension, and CKD via shared pathophysiology
Cardiology Guideline
Unified cardiometabolic disease framework links T2DM, ASCVD, HFpEF, MASLD, hypertension, and CKD via shared pathophysiology New Framework Links Heart and Kidney Health in Diabetes
This guideline provides a narrative review of the unified cardiometabolic disease (UCD) continuum. It argues that conditions like T2DM, ASCV…
Experts suggest that diabetes, heart failure, and kidney disease may share a single underlying cause rather than being separate conditions.
Frontiers Jul 19, 2026
21% hypertension prevalence in transgender adults: meta-analysis of cardiovascular morbidities
Cardiology Meta-analysis
21% hypertension prevalence in transgender adults: meta-analysis of cardiovascular morbidities Transgender adults face high rates of heart disease
This meta-analysis of observational studies reports pooled prevalence of major cardiovascular morbidities among transgender adults aged ≥18 …
One in five transgender adults has high blood pressure, and many have heart disease, a new analysis finds.
Frontiers Jul 15, 2026
Ultra-processed food consumption is associated with higher risk of hypertension and cardiovascular disease
Cardiology Meta-analysis
Ultra-processed food consumption is associated with higher risk of hypertension and cardiovascular disease Ultra-processed foods linked to higher risk of heart disease
This systematic review and meta-analysis of 46 studies indicates that higher ultra-processed food (UPF) consumption is associated with a 9% …
Eating more ultra-processed foods is linked to a higher risk of high blood pressure and heart disease in new research.
medRxiv Jul 12, 2026
Triple antihypertensive therapy lowers systolic BP by 5.4 mmHg more than dual therapy
Cardiology Meta-analysis
Triple antihypertensive therapy lowers systolic BP by 5.4 mmHg more than dual therapy Triple drug therapy lowers blood pressure more than double therapy
This meta-analysis of randomized trials involving 13,461 adults with hypertension found that triple antihypertensive therapy reduced systoli…
Adding a third medication can significantly lower high blood pressure and improve control rates for adults compared to using only two drugs.
medRxiv Jul 9, 2026
Patients with cardiovascular diseases show measurable prevalence of active cancer across multiple conditions
Cardiology Sys. Review
Patients with cardiovascular diseases show measurable prevalence of active cancer across multiple conditions Heart disease patients show measurable risk of developing various cancers
This systematic review and meta-analysis analyzed 4,759,695 patients to determine the prevalence of active cancer in individuals with variou…
New data shows that people with heart conditions like stroke or heart failure have a measurable rate of active cancer cases.
medRxiv Jul 9, 2026
E-Coach WeChat model improves chronic disease self-management scores in hypertension patients
Cardiology RCT
E-Coach WeChat model improves chronic disease self-management scores in hypertension patients Digital Coaching Improves Management for Patients with Hypertension
This randomized controlled trial of 250 middle-aged and elderly hypertension patients found that the E-Coach platform integrated with WeChat…
A new digital coaching model using a mobile platform helped patients manage hypertension and improve their daily exercise habits.
Frontiers Jul 9, 2026
Genetic Architecture of Pulmonary Artery Diameter and Shared Systemic Vascular Biology
Genetics & Precision Medicine Meta-analysis
Genetic Architecture of Pulmonary Artery Diameter and Shared Systemic Vascular Biology Genetic markers linked to lung artery size in COPD patients
A large-scale meta-analysis identifies 44 independent genetic signals influencing pulmonary artery diameter, highlighting shared pathways wi…
New data links specific genetic markers to the size of pulmonary arteries, potentially helping us understand heart and lung health in COPD.
medRxiv Jul 8, 2026
Health literacy is moderately associated with improved self-care behaviors in patients with multiple chronic diseases
Primary Care & Family Medicine Meta-analysis
Health literacy is moderately associated with improved self-care behaviors in patients with multiple chronic diseases Better health literacy helps people manage chronic diseases more effectively
This meta-analysis of 138 studies found a moderate positive association (r = 0.29, 95% CI: 0.26-0.31) between health literacy and self-care …
Understanding medical information and using digital tools can help people with chronic conditions like diabetes or heart failure manage thei…
Jul 7, 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 →