|
 |
|
|||||||
|
|||||||||
Copyright ©ERS Journals Ltd 2009
Pulmonary veno-occlusive disease
1 Centre National de Référence de l'Hypertension Artérielle Pulmonaire, Dept of Pneumology and Intensive Care, and 2 Radiology Dept, Université Paris-Sud 11, Hôpital Antoine-Béclère, Assistance Publique – Hôpitaux de Paris, Clamart, France.
CORRESPONDENCE: M. Humbert, Centre National de Référence de l'Hypertension Artérielle Pulmonaire, Service de Pneumologie, Hôpital Antoine Béclère, Assistance Publique – Hôpitaux de Paris, Université Paris-Sud, 157 rue de la Porte de Trivaux, 92140 Clamart, France. Fax: 33 146303824. E-mail: marc.humbert{at}abc.aphp.fr
Keywords: Alveolar haemorrhage, BMPR2, computed tomography, diffusing capacity of the lung for carbon monoxide, pulmonary arterial hypertension, pulmonary veno-occlusive disease
Received: June 15, 2008
Accepted July 4, 2008
Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5–10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.
This article has been cited by other articles:
|
|
 |
|
  D. Montani, X. Jais, P. Dorfmuller, G. Simonneau, O. Sitbon, and M. Humbert Goal-oriented therapy in pulmonary veno-occlusive disease: a word of caution Eur. Respir. J., November 1, 2009; 34(5): 1204 - 1206. [Full Text] [PDF]
|
|
|
|
 |
|
 Authors/Task Force Members, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J.-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT) Eur. Heart J., October 2, 2009; 30(20): 2493 - 2537. [Full Text] [PDF]
|
|
|
|
 |
|
 D. Montani, L. Achouh, O. Sitbon, G. Simonneau, and M. Humbert Pulmonary Venoocclusive Disease and Failure of Specific Therapy Chest, October 1, 2009; 136(4): 1181 - 1181. [Full Text] [PDF]
|
|
|
|
 |
|
 M. Humbert and L. J. Rubin Combining evidence and experience in pulmonary hypertension Eur. Respir. Rev., September 1, 2009; 18(113): 135 - 136. [Full Text] [PDF]
|
|
|
|
|
|
M. Palazzini and A. Manes Pulmonary veno-occlusive disease misdiagnosed as idiopathic pulmonary arterial hypertension Eur. Respir. Rev., September 1, 2009; 18(113): 177 - 180. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. M. Jones, A. Dawson, G. H. Jenkins, A. G. Nicholson, D. M. Hansell, and N. K. Harrison Sarcoidosis-related pulmonary veno-occlusive disease presenting with recurrent haemoptysis Eur. Respir. J., August 1, 2009; 34(2): 517 - 520. [Full Text] [PDF]
|
|
|
|
 |
|
 G. Simonneau, I. M. Robbins, M. Beghetti, R. N. Channick, M. Delcroix, C. P. Denton, C. G. Elliott, S. P. Gaine, M. T. Gladwin, Z.-C. Jing, et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol., June 30, 2009; 54(1 Suppl): S43 - S54. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Humbert Update in Pulmonary Hypertension 2008 Am. J. Respir. Crit. Care Med., April 15, 2009; 179(8): 650 - 656. [Full Text] [PDF]
|
|
|
|
|
|
M. Humbert More pressure on pulmonary hypertension Eur. Respir. Rev., March 1, 2009; 18(111): 1 - 3. [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |