Continuous therapy with intravenous prostacyclin for primary pulmonary hypertension: a bridge to heart-lung transplantation

Primary pulmonary hypertension (PPH) continues to be a major problem. Vasodilator therapy has been of variable success in selected patients. Recently, with the introduction of prostacyclin (Prostaglandin I2, epoprostenon) as a potent vasodilator, several reports have shown a positive effect in primary pulmonary hypertension. We report here two patients with PPH who responded favorably to continuous intravenous therapy with prostacyclin. Both patients responded with a reduction in pulmonary vascular resistance, increase in cardiac output and dramatic improvement of right heart failure. Although therapy with prostacyclin is extremely expensive it may be used as a bridge to heart-lung transplantation in patients with end-stage PPH who do not respond adequately to alternative therapy.