Withdrawal of prostacyclin causes rebound pulmonary hypertension that can lead to sudden death. Despite these limitations, prostacyclin has provided significant benefit to many patients unresponsive to oral vasodilators. Patients have had sustained improvements for longer than eight years on prostacyclin, improving functional capacity and quality of life. Patients with advanced disease who fail to improve with prostacyclin or are unable to use it are often candidates for lung transplantation. Usually just the lungs are transplanted, but some patients receive both a new heart and lungs if their heart has been damaged or is dysfunctional.
Lung transplantation leads to a complete cure.