Rare diseases · Sign or symptom
Pulmonary arterial hypertension
Increased blood pressure in blood vessels of lungs
HP:0002092
What it means
Pulmonary hypertension is defined mean pulmonary artery pressure of 25mmHg or more and pulmonary capillary wedge pressure of 15mmHg or less when measured by right heart catheterisation at rest and in a supine position.
Pulmonary hypertension can be secondary to known cardiac, pulmonary, or hepatic disease. If no cause is identifiable, the term primary pulmonary hypertension is used.
In everyday care
Most people with this sign do not have a rare disease — it appears in common conditions far more often. These general reference pages cover the same topic:
Rare diseases that can present with this90
Very common80–99%
8Common30–79%
15- 17q23.1q23.2microdeletion syndrome
- Absence of the pulmonary artery
- COG1-CDG
- Congenital total pulmonary venous return anomaly
- Dysmorphism-short stature-deafness-difference of sex development syndrome
- Encephalocraniocutaneous lipomatosis
- Keutel syndrome
- Microcephaly-seizures-intellectual disability-heart disease syndrome
- Mucopolysaccharidosis-like syndrome with congenital heart defects and hematopoietic disorders
- Multiple mitochondrial dysfunctions syndrome type 1
- Neonatal acute respiratory distress syndrome due to SP-B deficiency
- POEMS syndrome
- Primary hyperaldosteronism-seizures-neurological abnormalities syndrome
- Takayasu arteritis
- Yunis-Varon syndrome
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Primary pulmonary hypertension · Pulmonary artery hypertension
Tracking symptoms like this for someone? Eleplan keeps symptoms, diagnoses, medications and every specialist in one plan.
Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.