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pulmonary veno-occlusive disease; pulmonary vein thrombosis (PVOD)
Thrombosis of the pulmonary veins & venules.
Etiology:
1) viral infection
2) Mycoplasma
3) toxic reaction to drugs & chemicals
4) frequently occurs in association with COPD
Epidemiology:
1) uncommon to rare
2) occurs in young adults & children [3]
3) male:female ratio is 6:1
Pathology:
- vascular changes originate in the small pulmonary veins & venules
- pathogenesis is unknown
Genetics:
- associated with defects in BMPR2
- links with primary pulmonary hypertension via BMPR2 & EIF2AK4
- associated with EIF2AK4 variants [3]
Clinical manifestations:
- pulmonary hypertension
Laboratory:
1) arterial blood gas (ABG)
a) hypoxemia
b) acidemia
c) hypercarbia
2) complete blood count (CBC)
- polycythemia
* also see ARUP consult [4]
Special laboratory:
-> cardiac catherization
a) mean pulmonary artery pressure 25-40 mm Hg
b) cardiac index is normal
Radiology:
- imaging suggestive but not diagnostic
- pleural effusion may be noted [3]
- chest radiograph: prominent interstitium
- CT of thorax: septal thickening
Differential diagnosis:
1) cor pulmonale
2) congestive heart failure
Management: oxygen
Related
cardiac catheterization
pulmonary artery pressure (PAP)
pulmonary hypertension
General
pulmonary vascular disease
veno-occlusive disease
Database Correlations
OMIM 265450
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 780
- OMIM :accession 265450
- Medical Knowledge Self Assessment Program (MKSAP) 17, 18.
American College of Physicians, Philadelphia 2015, 2018
- ARUP Consult:
EIF2AK4-Associated Disorders
https://arupconsult.com/ati/eif2ak4-associated-disorders