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Palazzini M et al. – A 27–yr–old female with a 6–month diagnosis of idiopathic pulmonary arterial hypertension (PAH) confirmed elsewhere was referred to authors' centre with worsening dyspnoea. This case emphasises that PVOD is an under–recognised and often misdiagnosed form of pulmonary hypertension. Therefore, accurate diagnosis of PVOD requires comprehensive clinical and diagnostic work–up. While lung transplantation remains the treatment of choice for patients with PVOD, targeted therapies for PAH in addition to high doses of diuretics merit evaluation.


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