Rare diseases · Sign or symptom
Pulmonary artery stenosis
Narrowing of lung artery
HP:0004415
What it means
An abnormal narrowing or constriction of the pulmonary artery, in the main pulmonary artery and/or in the left or right pulmonary artery branches.
Stenosis, or narrowing, of the branches of the pulmonary artery is a type of congenital heart disease that, if left untreated, may lead to significant complications. Ideally, interventions to treat stenosis occur before significant complications or long-term sequelae take place, often within the first 2 years of life.
Rare diseases that can present with this21
Common30–79%
6Sometimes5–29%
10- Autosomal recessive malignant osteopetrosis
- Cardiac diverticulum
- Common arterial trunk
- Congenital total pulmonary venous return anomaly
- Kagami-Ogata syndrome due to paternal uniparental disomy of chromosome 14
- Kleefstra syndrome
- Ogden syndrome
- Pancreatic hypoplasia-diabetes-congenital heart disease syndrome
and 2 more in this range
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
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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.