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Disease

Familial Primary Pulmonary Hypertension

Late-stage therapeutic developmentEmerging research
MeSHFamilial Primary Pulmonary Hypertension
1
Publications
22
Clinical trials
2014
Latest publication
Latest activity
beta

Recent clinical, regulatory, research and industry developments relating to this disease.

What's happening now

An analyst briefing on current research, clinical, regulatory and industry activity surrounding this disease.

Executive briefingUpdating summary…Momentum: Moderate
Key developments
  • 4 clinical trials expected to report results, the earliest in Q2 2027.
Upcoming Milestones
Clinical Milestones10View all 10
Results expected 4
Other updates 5
+2 more in the activity timeline below
Activity timeline10

Clinical trials

13 sponsors · 4 new · 0 completed in the last 12 months (net +0)

The current development programme across all trial phases.

Clinical programme
22
All trials
5
Active
16
Late-stage
5
Completed
Late-stage studies

A Phase 3, Multicenter, Randomized, Double-Blinded, Placebo-Controlled Study to Evaluate the Safety and Efficacy of L606 (Treprostinil Liposome Inhalation Suspension) in Participants With Pulmonary Hypertension Associated With Interstitial Lung Disease (WHO Group 3)

Phase 3 · Recruiting · Liquidia Technologies, Inc.

A Phase 3, 2-Part, Open-Label Study to Evaluate the Safety and Tolerability of Liposomal Treprostinil Inhalation Suspension (L606) in Subjects With Pulmonary Arterial Hypertension or Pulmonary Hypertension Associated With Interstitial Lung Disease

Phase 3 · Active, not recruiting · Liquidia Technologies, Inc.

Effect of the Traditional Chinese Medicine Yufeng Ningxin in Patients With Hypertension: a Randomized, Double-blind, Placebo-controlled Trial

Phase 4 · Recruiting · Beijing Anzhen Hospital

An Adaptive, 2-Part, Randomized, Double-Blind, Placebo-Controlled Trial to Evaluate the Efficacy and Safety of IKT-001 in Pulmonary Arterial Hypertension (PAH)

Phase 3 · Recruiting · Inhibikase Therapeutics

Apixaban Plus Carvedilol to Prevent Portal Hypertension Complications in Cirrhosis: A Randomized Single-Blind Placebo-Controlled Trial at AIMS, Hyderabad, Pakistan

Phase 4 · Recruiting · Asian Institute Of Medical Sciences

A Randomized, Double-Blind, Phase IV Clinical Study to Evaluate the Antihypertensive Efficacy and Changes of Neurohormonal Markers of Fimasartan and Atenolol With Exaggerated Blood Pressure Response During Exercise in Essential Hypertensive Patients

Phase 4 · Completed · Boryung Pharmaceutical Co., Ltd
Recruiting

A Phase 3, Multicenter, Randomized, Double-Blinded, Placebo-Controlled Study to Evaluate the Safety and Efficacy of L606 (Treprostinil Liposome Inhalation Suspension) in Participants With Pulmonary Hypertension Associated With Interstitial Lung Disease (WHO Group 3)

Phase 3 · Recruiting · Liquidia Technologies, Inc.

Effect of the Traditional Chinese Medicine Yufeng Ningxin in Patients With Hypertension: a Randomized, Double-blind, Placebo-controlled Trial

Phase 4 · Recruiting · Beijing Anzhen Hospital

An Adaptive, 2-Part, Randomized, Double-Blind, Placebo-Controlled Trial to Evaluate the Efficacy and Safety of IKT-001 in Pulmonary Arterial Hypertension (PAH)

Phase 3 · Recruiting · Inhibikase Therapeutics

Apixaban Plus Carvedilol to Prevent Portal Hypertension Complications in Cirrhosis: A Randomized Single-Blind Placebo-Controlled Trial at AIMS, Hyderabad, Pakistan

Phase 4 · Recruiting · Asian Institute Of Medical Sciences
Recently completed

A Randomized, Double-Blind, Phase IV Clinical Study to Evaluate the Antihypertensive Efficacy and Changes of Neurohormonal Markers of Fimasartan and Atenolol With Exaggerated Blood Pressure Response During Exercise in Essential Hypertensive Patients

Phase 4 · Completed · Boryung Pharmaceutical Co., Ltd

A Randomized, Multicenter, Parallel Design Phase III Clinical Study to Evaluate the Efficacy and Safety of the Combination of Lercanidipine/Valsartan in Hypertensive Patients Who Are Not Adequately Controlled on Lercanidipine 10mg Monotherapy.

Phase 3 · Completed · LG Life Sciences

An Eight-week Randomised Double-blind Study to Compare the Efficacy and Safety of Telmisartan 80mg Plus Amlodipine 5 mg Fixed-dose Combination vs. Telmisartan 40 mg Plus Amlodipine 5 mg Fixed-dose Combination in Patients With Hypertension

Phase 3 · Completed · Boehringer Ingelheim

An Open-label Study to Evaluate the Antihypertensive Effects of the Fixed-dose Combination of Telmisartan 80 mg and Amlodipine 5 mg (T80/A5) Given Once Daily by 24 h ABPM in Patients With Moderate to Severe Hypertension

Phase 3 · Completed · Boehringer Ingelheim

An Open-label, Multicenter Study to Evaluate the Efficacy and Safety of a 4 Week Therapy With the Single Pill (SPC) Combination of Aliskiren 300 mg / Amlodipine 10 mg in Hypertensive Patients Not Adequately Respond to an Uptitrated 4 Week Therapy With the SPC of Olmesartan 40 mg / Amlodipine 10 mg, With a Potential Extension if Patients Still Not Adequately Respond With a 4 Week Therapy With the SPC Aliskiren 300 mg / Amlodipine 10 mg / HCTZ 12,5 mg

Phase 3 · Completed · Novartis

Evidence coverage

8 treatments

How much of this condition's readable clinical evidence the confidence engine has incorporated, across its most-studied treatments. This measures coverage of the evidence base — not whether any treatment works.

Substantial evidence coverage
61% · 59/97 eligible items incorporated
lowers systolic blood pressure in hypertension51%
lowers diastolic blood pressure in hypertension42%

Largest gap: no meaningful change (9, trial-readability).

Research activity

1 papers

Key research shaping understanding of this disease, combining the latest publications with the most influential evidence.

Most influential

Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.

Annals of the rheumatic diseases · 2014 · 523 cites
Recent publications

Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.

Annals of the rheumatic diseases · 2014 · 523 cites
Major themes1
  • Algorithms1
Leading journals1
  • Annals of the rheumatic diseases1
Leading researchers8
  • Bonderman D1
  • Chadha-Boreham H1
  • Coghlan JG1
  • Denton CP1
  • Distler O1
  • Doelberg M1
  • Grünig E1
  • Heinzl H1
Affiliations (unnormalised)6
  • Center for Medical Statistics1
  • Centre for Pulmonary Hypertension1
  • Centre for Rheumatology1
  • Clinical Development1
  • Department of Rheumatology and Clinical Immunology1
  • Medical University of Vienna1

Reference

Authoritative identity, definition & identifiers.

Defined in MeSH

Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.

Identifiers
MeSHFamilial Primary Pulmonary Hypertension
References & data sources
  • Disease identity & definition — NLM Medical Subject Headings (MeSH), public domain
  • Clinical trials — ClinicalTrials.gov (U.S. National Library of Medicine)
  • Research activity — Europe PMC (EMBL-EBI) + OpenAlex-derived paper links
  • Related entities are derived from literature co-mention (studied together) — associative, not causal.