title: Pulmonary veno-occlusive disease: advances in clinical management and treatments.
abstract: 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.affiliation: Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, F-94276, France.
authors.forename: Alice
authors.initials: A
authors.lastname: Huertas
authors.forename: Barbara
authors.initials: B
authors.lastname: Girerd
authors.forename: Peter
authors.initials: P
authors.lastname: Dorfmuller
authors.forename: Dermot
authors.initials: D
authors.lastname: O'Callaghan
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: David
authors.initials: D
authors.lastname: Montani
mesh_terms: D006801:Humans; D011237:Predictive Value of Tests; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D012307:Risk Factors; D016896:Treatment Outcome