Treatment of pulmonary arterial hypertension: A review of drugs available for advanced therapy

Pulmonary hypertension (PH) has traditionally been considered a rare disease with a uniformly poor prognosis. However, this was prior to the introduction of advanced therapies for this condition, and more recent registries in the treatment era have shown 5-year survival rates of up to 65%. Prior to 2000, there was only one licensed therapy for pulmonary arterial hypertension (PAH); less than 20 years later, the US Food and Drug Administration has approved 14 different medications for PAH. This review aims to summarise for the general pulmonologist the evidence for the current internationally available advanced therapies for PAH (World Health Organization Group I disease), which is characterised haemodynamically by the presence of precapillary PH in the absence of another cause. The benefit of these agents, either alone or in combinations, is now undisputed and their use is advocated in all current international guidelines for PAH. The improvement in survival of patients with PAH over the concurrent timeline emphasises the importance both of the availability and usage of effective therapies and of patients being seen in specialist centres, where physicians are familiar with using these therapies.