pulmonary veno-occlusive disease; pulmonary vein thrombosis (PVOD)
Jump to navigation
Jump to search
Introduction
Thrombosis of the pulmonary veins & venules.
Etiology
- viral infection
- Mycoplasma
- toxic reaction to drugs & chemicals
- frequently occurs in association with COPD
Epidemiology
- uncommon to rare
- occurs in young adults & children[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
Laboratory
Diagnostic procedures
- cardiac catherization
- mean pulmonary artery pressure 25-40 mm Hg
- 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
Management
More general terms
Additional terms
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 780
- ↑ OMIM https://mirror.omim.org/entry/265450
- ↑ 3.0 3.1 3.2 3.3 Medical Knowledge Self Assessment Program (MKSAP) 17, 18. American College of Physicians, Philadelphia 2015, 2018
- ↑ 4.0 4.1 ARUP Consult: EIF2AK4-Associated Disorders https://arupconsult.com/ati/eif2ak4-associated-disorders