Pulmonary veno-occlusive disease is a rare and serious form of pulmonary hypertension requiring careful, individualised clinical management. The treatment approach must balance therapeutic benefit with the significant risk of pulmonary oedema that characterises this condition.
When vasodilator therapy is considered, current evidence supports initiating with a single pulmonary vasodilator agent. Therapy may be used as a palliative measure to slow disease progression in patients who are not transplant candidates, or as a bridge to lung transplantation. The complete agent selection, dosing strategy, monitoring parameters, and criteria for escalation are defined in the structured protocol.