Doença veno-oclusiva pulmonar: alternativas diagnósticas e terapêuticas

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.

Targeted therapy in pulmonary veno-occlusive disease: time for a rethink?

“…Bosentan, an endothelin receptor antagonist, was reported to be effective in improving hemodynamics [20, 22, 27], however, pulmonary edema occurred in some cases and the outcomes varied [21–23]. Macitentan was first proved to be beneficial for PVOD in 2016, and brought a clear improvement when combined with sildenafil [25].…”

Pulmonary Veno-Occlusive Disease: An 80-Year-Old Mystery

“…This study suggested the possible application of BAL in the diagnosis of PVOD, which had a sensitivity of 88% and a specificity of 91% when the Golde score cutoff was set at 20. A successful application in a suspected case was reported afterwards [64]. …”

“…Bosentan, an endothelin receptor antagonist, has been reported to achieve improvement in very few cases [64,73]. However, it is also on the list of agents which produce pulmonary edema at a rate of 33% (2 out of 6 patients) [6].…”