Over the last decade, new medical and surgical treatments have become available for primary pulmonary hypertension that have influenced the natural history of the disease. Vasodilator therapy is aimed at overcoming pulmonary vasoconstriction with consequent decrease in afterload and improved right ventricular output. The endpoint of vasodilator therapy is therefore to reduce pulmonary vascular resistance, without causing significant systemic hypotension. Prostacyclin, a potent vasodilator and antiaggregating agent, has been successfully used by continuous infusion in the treatment of severe pulmonary hypertension. The marked improvement in quality of life observed with the use of prostacyclin in these patients, as well as the increased survival time on the waiting list for transplantation, has influenced us to adopt prostacyclin infusion as a fundamental element of our therapeutic strategy for the management of this rare but fatal condition.