Pulmonary veno-occlusive disease
Pulmonary veno-occlusive disease (PVOD) is considered an uncommon variant of primary pulmonary hypertension (PPH) that preferentially affects the postcapillary pulmonary vasculature.
It is thought to account for ~ 5 - 10% of cases initially considered to be idiopathic PAH 2.
It is rare but can potentially affects all age groups with no geographic predilection. The majority of affected patients are under 50 years of age 1. In the paediatric population, both sexes are equally affected. In the adult population, the ratio of male to female patients is approximately 2 : 1.
PVOD can be idiopathic or can complicate other conditions which include 2
- connective tissue disease
- HIV infection
- bone marrow transplantation 3
- pulmonary Langerhans cell histiocytosis
Its presentation can be similar to other forms of pulmonary aterial hypertension.
In general, imaging features can be non-specific and a definitive diagnosis often requires a lung biopsy 1.
Reported HRCT findings include1-2,4
- smooth interlobular septal thickening
- regions of ground-glass opacity : can be
- diffuse multifocal 1,4
- perihilar 4
- patchy 4
- centrilobular 2,4
- pleural effusions
- enlarged central pulmonary arteries
- normal calibre pulmonary veins
- mosaic pattern of lung attenuation
Treatment and prognosis
PVOD generally carries a poor prognosis (some report a 72% mortality at one year from diagnosis 7). Lung transplantation is the treatment of choice 2. It is omportant ot note that therapy commonly used to treat patients with primary pulmonary hypertension, such as calcium-channel blockers (CCBs) 1, epoprostenol (PGI2) 1 or prostacylin 6 can lead to disastrous outcomes in patients with PVOD.
It is thought to have been first described by Hora et al in 1934 1.
- 1. Holcomb BW, Loyd JE, Ely EW et-al. Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000;118 (6): 1671-9. doi:10.1378/chest.118.6.1671 - Pubmed citation
- 2. Montani D, Price LC, Dorfmuller P et-al. Pulmonary veno-occlusive disease. Eur. Respir. J. 2009;33 (1): 189-200. doi:10.1183/09031936.00090608 - Pubmed citation
- 3. Troussard X, Bernaudin JF, Cordonnier C et-al. Pulmonary veno-occlusive disease after bone marrow transplantation. Thorax. 1984;39 (12): 956-7. doi:10.1136/thx.39.12.956 - Free text at pubmed - Pubmed citation
- 4. Frazier AA, Franks TJ, Mohammed TL et-al. From the Archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 27 (3): 867-82. doi:10.1148/rg.273065194 - Pubmed citation
- 5. Swensen SJ, Tashjian JH, Myers JL et-al. Pulmonary venoocclusive disease: CT findings in eight patients. AJR Am J Roentgenol. 1996;167 (4): 937-40. AJR Am J Roentgenol (abstract) - Pubmed citation
- 6. Resten A, Maitre S, Humbert M et-al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 2004;183 (1): 65-70. AJR Am J Roentgenol (full text) - Pubmed citation
- 7. Holcomb BW, Loyd JE, Ely EW et-al. Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000;118 (6): 1671-9. doi:10.1378/chest.118.6.1671 - Pubmed citation
Synonyms & Alternative Spellings
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|Pulmonary veno occlusive disease||✗|
|Pulmonary veno occlusive disease (PVOD)||✗|
|Pulmonary veno-occlusive disease (PVOD)||✗|