title: Pulmonary veno-occlusive disease: diagnostic and therapeutic alternatives.
abstract: 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.
authors.affiliation: Department of the University of São Paulo School of Medicine, São Paulo, Brazil.
authors.forename: Carlos Eduardo Galvão
authors.initials: CE
authors.lastname: Barboza
authors.forename: Carlos Viana Poyares
authors.initials: CV
authors.lastname: Jardim
authors.forename: André Luís Dressler
authors.initials: AL
authors.lastname: Hovnanian
authors.forename: Bruno Arantes
authors.initials: BA
authors.lastname: Dias
authors.forename: Rogério
authors.initials: R
authors.lastname: Souza
mesh_terms: D001706:Biopsy; D018893:Bronchoalveolar Lavage; D001999:Bronchoscopy; D065128:Endothelin Receptor Antagonists; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008168:Lung / Q000473:pathology*; D008875:Middle Aged; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D017466:Receptors, Endothelin / Q000627:therapeutic use
chemical_list: D065128:Endothelin Receptor Antagonists; D017466:Receptors, Endothelin