Pulmonary veno-occlusive disease Report of case and revision of the literature
Enfermedad pulmonar veno-oclusiva Reporte de un caso y revisión de la literatura
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The real incidence of veno-occulusive pulmonary disease (VOPD) is unknown because it is largely underdiagnosed or is not classified as primary pulmonary hypertension, being in fact a variant of the primary disease, primarily affecting post-capillary pulmonary vasculature. It is also known as isolated pulmonary venous sclerosis, obstructive disease of the pulmonary veins or primary pulmonary hypertension of the venous type.
VOPD is the result of multiple aggressors associated to a great variety of risk factors. The diagnosis of the disease requires clinical, radiological and pathological features.
Vasodilators, immunosuppressants, anticoagulation and oxygen have been proposed as treatments with a poor prognosis, and with reported survival limited by the scarce reporting of cases.
We report a case of veno-occlusive pulmonary disease which was diagnosed in this institution.
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