Chest
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Comparative Study
Failure of medical therapy in pulmonary arterial hypertension. Is there an alternative diagnosis?
Despite the development of targeted therapies for pulmonary arterial hypertension (PAH), some patients fail to respond to medical therapy. There are a number of types of PAH, one of which is pulmonary venoocclusive disease (PVOD). Unlike other PAH types, the hallmark pathology of PVOD is fibrous occlusion of the small venules, potentially with arterial involvement. It was hypothesized that a lack of response to medical therapy in clinically diagnosed PAH can be explained by misdiagnosed PVOD. ⋯ PVOD is an underdiagnosed and commonly misdiagnosed cause of pulmonary hypertension, which may present with a failure to respond to medical therapy. Further studies on PVOD may help confirm whether a change in its classification as a type of PAH is necessary.