Pulmonary veno-occlusive disease: diagnostic and therapeutic alternatives.
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.
Barboza
Carlos Eduardo Galvão
CE
Department of the University of São Paulo School of Medicine, São Paulo, Brazil.
Jardim
Carlos Viana Poyares
CV
Hovnanian
André Luís Dressler
AL
Dias
Bruno Arantes
BA
Souza
Rogério
R
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
D065128:Endothelin Receptor Antagonists; D017466:Receptors, Endothelin