Emerging treatments for pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a life-threatening disease characterised by a progressive pulmonary vasculopathy with ensuing right heart failure if left untreated. In the 1980s, prior to the current treatment era, idiopathic PAH carried a very poor prognosis, with a median survival of 2.8 years from the time of diagnosis. Since then, continuous intravenous epoprostenol has been used for the treatment of severe PAH with tremendous success, improving haemodynamics, quality of life, exercise capacity, functional class and even survival. In addition, in the past 5 years there have been several new advances in the treatment of PAH; however, there is still no cure. A better understanding of how the currently available agents work together is essential to optimise the long-term care of patients with PAH. Ultimately, additional agents that target the underlying pulmonary vasculopathy and endothelial abnormalities are necessary to cure this fatal disease. This comprehensive review of the currently available and emerging novel therapies provides insight into future management of PAH patients.