RecruitingPhase 3NCT07481981
Respiratory
Hereditary Pulmonary Arterial Hypertension
Also known as heritable PAH, BMPR2-related PAH, familial pulmonary arterial hypertension
Hereditary pulmonary arterial hypertension is a rare, life-threatening condition caused by autosomal dominant mutations, most commonly in BMPR2, leading to progressive obliterative remodelling of small pulmonary arteries and right heart fai
ORPHA:422 ↗Gene BMPR2Gene ACVRL1Gene ENGPrevalence Approximately 1–2 per million; BMPR2 mutations in ~70–80% of heritable casesOnset Variable; childhood to adult
113
studies recruiting now
as of 7 Sept 2026
1,001
studies registered in total
as of 7 Sept 2026
16
countries with a recruiting site
as of 7 Sept 2026
29 May 2026
most recent study posted
among recruiting studies
Recruiting trials
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An Adaptive Program of IKT-001 in Pulmonary Arterial Hypertension (PAH)
RecruitingNot applicableNCT04194632
Right Ventricular Pacing in Pulmonary Arterial Hypertension
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Natural History of Sickle Cell Disease
Showing the 5 most recently updated recruiting studies, as recorded 7 Sept 2026. Live status on each study page.
See all 113 recruiting studiesWhere recruiting studies are running
Countries with at least one recruiting site among the studies above, 7 Sept 2026. Tap a country to search trials there.
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Registry: PHAR Registry (Pulmonary Hypertension Association Registry) · Join ↗. Registries connect patients to researchers and often hear about trials first.
About Hereditary Pulmonary Arterial Hypertension
Hereditary pulmonary arterial hypertension is a rare, life-threatening condition caused by autosomal dominant mutations, most commonly in BMPR2, leading to progressive obliterative remodelling of small pulmonary arteries and right heart failure. Penetrance is incomplete (approximately 20%), and the disease shows variable expressivity, making genetic family screening essential. Despite advances in targeted therapies addressing the prostacyclin, endothelin, and nitric oxide pathways, there is no cure and prognosis remains guarded without transplantation.
Common clinical features
Progressive dyspnoea on exertionFatigueSyncope or pre-syncopeRight heart failure (peripheral oedema, ascites)Chest painHaemoptysisElevated mean pulmonary arterial pressure on right heart catheterisation
From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.
Before you apply
Things trial teams commonly ask about for Hereditary Pulmonary Arterial Hypertension. Not eligibility rules; those are set by each study.
- Right heart catheterisation confirming mPAP ≥25 mmHg at rest with PAWP ≤15 mmHg is the diagnostic gold standard required for most PAH trials; ensure this is on file.
- Genetic mutation status (particularly BMPR2) is increasingly used as a stratification variable; obtain formal genetic testing before applying.
- Baseline 6-minute walk distance and WHO functional class are near-universal eligibility and stratification criteria; obtain these measurements close to the trial screening date.
Related conditions
Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).