Abstract

Our ultimate goal in treating patients is to improve their quality of life and to increase survival. The optimal treatment for primary pulmonary hypertension will continue to change as our understanding of its causes improves and as progress is made in lung transplantation. There is no one best treatment for all patients. Optimal medical and surgical treatment must be tailored to the individual with changes in therapeutic regimens based on serial evaluations. Quality of life and survival have improved with current treatments and the future should offer additional therapies-inhaled nitric oxide, endothelin receptor blockers, and other modulators of the pulmonary vascular bed-to improve further the treatment of this disease. In conclusion, although primary pulmonary hypertension, if untreated, is most often a rapidly progressive and fatal disease, recent advances in the treatment have significantly improved the outcome for patients. Although transplantation is often considered the only definitive treatment for patients with primary pulmonary hypertension, medical treatment seems to be an effective long term palliation to successful transplantation as well as a possible alternative treatment to transplantation in selected children and adults. Quality of life and cost analyses, as well as longer follow up studies are needed to determine the best treatment for patients with primary pulmonary hypertension.