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Pulmonary veno-occlusive disease: advances in clinical management and treatments Huertas A; Girerd B; Dorfmuller P; O'Callaghan D; Humbert M; Montani DExpert Rev Respir Med 2011[Apr]; 5 (2): 217-29; quiz 230-1Pulmonary 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.|Humans[MESH]|Predictive Value of Tests[MESH]|Pulmonary Veno-Occlusive Disease/diagnosis/epidemiology/*therapy[MESH]|Risk Factors[MESH]|Treatment Outcome[MESH] |
