Pulmonary veno-occlusive disease 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 affect 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.
Its presentation can be similar to other forms of pulmonary arterial hypertension such as progressive dyspnea, cough, and also haemoptysis. Pulmonary capillary wedge pressure is usually normal due to patchy involvement.
It is characterised by extensive occlusion of pulmonary veins by fibrous tissue in a patchy distribution. This may be loose and oedematous or dense and sclerotic 7.
Pulmonary veno-occlusive disease can be idiopathic (in most cases, the causes is not known) or can complicate other conditions which include 2:
- connective tissue disease
- HIV infection
- bone marrow transplantation 3
- pulmonary Langerhans cell histiocytosis
In general, imaging features can be non-specific and a definitive diagnosis often requires a lung biopsy 1.
Reported HRCT chest 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: most cases 8
- pleural effusions
- enlarged central pulmonary arteries
- normal calibre pulmonary veins
- mosaic pattern of lung attenuation
Treatment and prognosis
Pulmonary veno-occlusive disease generally carries a poor prognosis (some report a 72% mortality at one year from diagnosis 1). Lung transplantation is the treatment of choice 2. It is important to note that vasodilators commonly used to treat patients with primary pulmonary hypertension, such as calcium-channel blockers (CCBs) 1, epoprostenol (PGI2) 1 or prostacyclin 6 can lead to disastrous outcomes (severe pulmonary oedema) in patients with pulmonary veno-occlusive disease.
History and etymology
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. Mandel J, Mark EJ, Hales CA. Pulmonary veno-occlusive disease. Am. J. Respir. Crit. Care Med. 2000;162 (5): 1964-73. doi:10.1164/ajrccm.162.5.9912045 - Pubmed citation
- 8. Matsushita K, Kanna M, Yazawa T et-al. Long-term survivor with pulmonary veno-occlusive disease. Circulation. 2012;125 (12): e503-6. Circulation (full text) - doi:10.1161/CIRCULATIONAHA.111.088450 - Pubmed citation