idiopathic pulmonary arterial hypertension
Findings
No curated finding names idiopathic pulmonary arterial hypertension yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A sporadic form of pulmonary arterial hypertension (PAH) characterized by elevated pulmonary arterial resistance leading to right heart failure. IPAH is progressive and potentially fatal and not associated with an underlying condition or family history of PAH. The etiology is unknown.
Definition from the Mondo Disease Ontology (MONDO:0001999), read 2026-09-29. CC BY 4.0.
Features
16 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- DyspneaHPOHP:0002094
- Obligate (100% of cases)
- Abnormal jugular vein morphologyHPOHP:3000042
- Very frequent (80% to 99% of cases)
- Elevated pulmonary artery pressureHPOHP:0004890
- Very frequent (80% to 99% of cases)
- Increased pulmonary vascular resistanceHPOHP:0005317
- Very frequent (80% to 99% of cases)
- Pulmonary arterial hypertensionHPOHP:0002092
- Very frequent (80% to 99% of cases)
- Right ventricular hypertrophyHPOHP:0001667
- Very frequent (80% to 99% of cases)
- Ankle swellingHPOHP:0001785
- Frequent (30% to 79% of cases)
- Chest painHPOHP:0100749
- Frequent (30% to 79% of cases)
- Congestive heart failureHPOHP:0001635
- Frequent (30% to 79% of cases)
- Edema of the dorsum of feetHPOHP:0012098
- Frequent (30% to 79% of cases)
- Heart murmurHPOHP:0030148
- Frequent (30% to 79% of cases)
- SyncopeHPOHP:0001279
- Frequent (30% to 79% of cases)
Reported absent (2)
- Abnormality of connective tissueHPOHP:0003549
- Chronic hemolytic anemiaHPOHP:0004870
Show the remaining 4
- Tricuspid regurgitationHPOHP:0005180
- Frequent (30% to 79% of cases)
- HemoptysisHPOHP:0002105
- Occasional (5% to 29% of cases)
- PalpitationsHPOHP:0001962
- Occasional (5% to 29% of cases)
- Pedal edemaHPOHP:0010741
- Occasional (5% to 29% of cases)
Where it sits
Other names
3 names
Resolves to: idiopathic pulmonary arterial hypertension
- Also called
- IPAHprimary pulmonary arterial hypertensionprimary pulmonary hypertension