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Start free with EleplanPulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis
ORPHA:431353Category
Also called Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
What it is
A disorder that constitutes a rare subgroup of rare pulmonary hypertension characterized by obliterative fibrosis of the small pulmonary veins and venules and/or capillary infiltration of the pulmonary interstitium leading to increased pulmonary vascular resistance and right ventricular dysfunction.
Key facts
- Prevalence
- <1 / 1 000 000 (annual incidence, France)
- Age of onset
- All ages
- Classified as
- Category
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Genes reported in subtypes
Orphanet records these genes on 2 more specific entries under this disorder, not on this entry itself:
A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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