Long-term therapeutic outcomes in pulmonary arterial hypertension

Background: The prognosis of patients with untreated pulmonary arterial hypertension has historically been poor, but recent advances in our understanding of its pathobiology have led to the development of numerous therapeutic options over the last decade. Methods: Systematic literature review of all randomised studies performed in patients with pulmonary arterial hypertension including long-term extension observations (Medline 1990-2007). Findings: This article provides an overview of the currently available data on the short and long-term effects of emerging agents on exercise capacity, functional capacity, haemodynamics, quality of life, and survival in patients with pulmonary arterial hypertension. Prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have shown great promise in short-term randomised clinical trials, but the long-term effects of these classes of medication have not been well established to date. Conclusion: Several long-term cohort studies have recently suggested that these drugs can maintain their beneficial effects on important clinical outcomes, but further studies will be needed before definitive conclusions can be drawn regarding the ultimate utility and optimal role of each agent in the therapeutic repertoire.