Pulmonary veno-occlusive disease: diagnostic and therapeutic alternatives

J Bras Pneumol. 2008 Sep;34(9):749-52. doi: 10.1590/s1806-37132008000900015.
[Article in English, Portuguese]


Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Surgical biopsy was usually required for diagnostic confirmation. However, the morbidity, mortality and limited benefit of this procedure have generated discussion regarding noninvasive diagnostic techniques. We present the case of a female patient with progressive dyspnea, hypoxemia and pulmonary hypertension, the last diagnosed via catheterization. Computed tomography revealed septal thickening and diffuse micronodules. Bronchoalveolar lavage revealed occult alveolar hemorrhage. Treatment with an endothelin antagonist was started, resulting in symptomatic and functional improvement. Occult alveolar hemorrhage differentiates PVOD from idiopathic pulmonary hypertension. We believe that this finding, in combination with characteristic tomographic findings, is sufficient to establish a diagnosis of PVOD.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • Bronchoalveolar Lavage
  • Bronchoscopy
  • Endothelin Receptor Antagonists
  • Female
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Lung / pathology*
  • Middle Aged
  • Pulmonary Veno-Occlusive Disease / complications
  • Pulmonary Veno-Occlusive Disease / drug therapy
  • Pulmonary Veno-Occlusive Disease / pathology*
  • Receptors, Endothelin / therapeutic use


  • Endothelin Receptor Antagonists
  • Receptors, Endothelin