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Background: Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension characterized by remodelling of the pulmonary venules. PVOD and pulmonary arterial hypertension share similar clinical presentation. It is important to differentiate between these two conditions as PVOD carries a worse prognosis and life-threatening pulmonary oedema may occur following the initiation of conventional therapy. Case presentation: We are reporting a case of pulmonary hypertension in a middle-aged lady who presented with hemoptysis and features of right heart failure. After extensive work up, no definite aetiology of pulmonary hypertension could be found out. Standard therapy did not cause any symptomatic improvement. After right heart catheterization, we got pre capillary pulmonary hypertension and along with typical findings in HRCT scan of thorax we established PVOD. We did not try lung biopsy as the procedure often lands up with complications. As we could not arrange for lung transplant we eventually lost the patient. Conclusions: High suspicion and thorough systematic evaluation helped us to diagnose PVOD in this case. Thus, early diagnosis should be the primary aim in suspected cases of PVOD so that we can prepare for lung transplant at the earliest.

Pulmonary veno-occlusive disease: a rare and enigmatic cause of pulmonary hypertension—a case report