Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension with dominant involvement of
venous system – venules and septal veins. The diagnosis is complex, based on noninvasive investigations (echocardiography,
blood gas analysis, functional lung tests, HRCT) and right heart catheterization. Both in PVOD and pulmonary
arterial hypertension (PAH) severe precapillary pulmonary hypertension is present, but it is important to differentiate
these two clinical conditions as patients with PVOD have a worse prognosis and the risk of fatal pulmonary edemas following
the initiation of specific PAH therapy. Currently, there is no specific therapy for PVOD, the only therapeutic option
is lung transplantation.