ORPHA:31837
Pulmonary venoocclusive disease
Publications
3,091
Trials
2
Interventional, condition-specific
Researchers
1,295
Distinct authors in sample
Gene link
—
Readiness
3/6
Stages with a signal
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0009937
- MeSH:D011668
- UMLS:C0034091
- NCIT:C85039
Additional Mondo synonyms (3)
PVOD · pulmonary capillary hemangiomatosis · pulmonary veno-occlusive disease
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
3/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
3,091 matched papers (1,595 in last 10 years) Source
- Phenotype characterisedPresent
40 HPO annotations (e.g. Elevated jugular venous pressure; Abnormally loud pulmonic component of the second heart sound; Pulmonary venous occlusion) Source
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- Interventional trialPresent
2 matched on ClinicalTrials.gov
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
40
Associated phenotypes · MONDO:0009937
- Elevated jugular venous pressure
- Abnormally loud pulmonic component of the second heart sound
- Pulmonary venous occlusion
- Dyspnea
- Centrilobular ground-glass opacification on pulmonary HRCT
Showing 5 of 40 — open Monarch for the full list.
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
Monarch fetch 2026-07-27
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
6 associated chemicals · 17 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.
- Carmustine · marker/mechanism
- COPP protocol · marker/mechanism
- Dactinomycin · marker/mechanism
- FAM protocol · marker/mechanism
- Mitomycin · marker/mechanism
- MOPP protocol · marker/mechanism
Pathways: Cytokine-cytokine receptor interaction; Autophagy - animal; Protein processing in endoplasmic reticulum; TGF-beta signaling pathway; Axon guidance; Hippo signaling pathway; Signaling pathways regulating pluripotency of stem cells; Hepatitis C
Literature
Is anyone studying this?
3,091
3,091 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.
3,091 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
1,595 in the last 10 years · low confidence
Phrase hits: 3,091 · MeSH hits: 54
Who's working on it?
1,295
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Montani D13 papers · 2026
Université Paris-Saclay, Faculté de Médecine, Le Kremlin-Bicêtre, France.
Papers in Europe PMC - 02Humbert M8 papers · 2026
Université Paris-Saclay, Faculté de Médecine, Le Kremlin-Bicêtre, France.
Papers in Europe PMC - 03Dorfmüller P7 papers · 2026
Department of Pathology, University of Giessen and Marburg Lung Center, Justus-Liebig University Giessen, Giessen, Germany.
Papers in Europe PMC - 04Hata A7 papers · 2026
Cardiovascular Research Institute, UCSF, San Francisco, California, USA.
Papers in Europe PMC - 05Prabhakar A7 papers · 2026
Cardiovascular Research Institute, UCSF, San Francisco, California, USA.
Papers in Europe PMC - 06Ghatpande P6 papers · 2026
Cardiovascular Research Institute, UCSF, San Francisco, California, USA.
Papers in Europe PMC - 07Grynblat J6 papers · 2026
School of Medicine, Université Paris- Saclay, Paris, France.
Papers in Europe PMC - 08Lagna G6 papers · 2026
Cardiovascular Research Institute, UCSF, San Francisco, California, USA.
Papers in Europe PMC - 09Escribano-Subias P5 papers · 2026
Pulmonary Hypertension Unit, Cardiology Department, Hospital Universitario 12 de Octubre, Madrid, Spain; Instituto de Investigación Hospital 12 de Octubre (i+12), Madrid, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III (ISCIII), Madrid, Spain; ERN-LUNG (European Reference Network on Rare Respiratory Diseases), Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain. Electronic address: pilar.escribano.subias@gmail.com.
Papers in Europe PMC - 10Graham BB5 papers · 2025
Lung Biology Center, Pulmonary and Critical Care Medicine, Zuckerberg San Francisco General Hospital, California, USA.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
2
interventional trials for this specific condition
2 interventional trials matched this specific condition name; none in our sample are currently recruiting.
Data as of 9 September 2026
2 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 84.5th percentile).
low confidence · 84.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
2 interventional trials matched after quoted-phrase search and title/condition post-filter.
No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 4 · after dedupe 4 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · dropped 4 · fetched 2026-07-27
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Pulmonary venoocclusive disease — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Pulmonary venoocclusive disease" OR "pulmonary capillary hemangiomatosis" OR "pulmonary veno-occlusive disease"
MeSH descriptor terms unioned into the query: Pulmonary Veno-Occlusive Disease
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Pulmonary venoocclusive disease" OR "pulmonary capillary hemangiomatosis" OR "pulmonary veno-occlusive disease"
Interventional trials matched via: both (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 2 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: PVOD
Confidence reasoning
- Preferred label is multi-word and distinctive
- 1 synonym(s) dropped by stoplist (may under-count)
- "pulmonary capillary hemangiomatosis" also appears on ORPHA:199241
- Publication count (3091) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity
Ingested 2026-07-26T02:14:44.497Z