Pulmonary veno-occlusive disease: advances in clinical management and treatments

Expert Rev Respir Med. 2011 Apr;5(2):217-29; quiz 230-1. doi: 10.1586/ers.11.15.


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.

Publication types

  • Review

MeSH terms

  • Humans
  • Predictive Value of Tests
  • Pulmonary Veno-Occlusive Disease / diagnosis
  • Pulmonary Veno-Occlusive Disease / epidemiology
  • Pulmonary Veno-Occlusive Disease / therapy*
  • Risk Factors
  • Treatment Outcome