Document Detail


Pulmonary veno-occlusive disease: advances in clinical management and treatments.
MedLine Citation:
PMID:  21510732     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Pulmonary veno-occlusive disease (PVOD) is a rare disorder that can be misdiagnosed as idiopathic pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered as idiopathic PAH. PVOD and idiopathic PAH share a similar clinical presentation, genetic background and hemodynamic profile. A definite diagnosis of PVOD necessitates a surgical biopsy, but since it represents a high-risk procedure in these patients, it is contraindicated. Therefore, a noninvasive diagnostic approach using chest high-resolution computed tomography, arterial blood gas analysis, pulmonary function tests and bronchoalveolar lavage is helpful to detect PVOD. PVOD is characterized by a poor prognosis and the possibility of developing severe pulmonary edema with specific PAH therapy. Lung transplantation remains the treatment of choice.
Authors:
Alice Huertas; Barbara Girerd; Peter Dorfmuller; Dermot O'Callaghan; Marc Humbert; David Montani
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Expert review of respiratory medicine     Volume:  5     ISSN:  1747-6356     ISO Abbreviation:  Expert Rev Respir Med     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-04-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101278196     Medline TA:  Expert Rev Respir Med     Country:  England    
Other Details:
Languages:  eng     Pagination:  217-31     Citation Subset:  IM    
Affiliation:
Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, F-94276, France. david.montani@abc.aphp.fr.
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