The management of pulmonary arterial hypertension (PAH) has changed dramatically over the last decade. Where once the physician had only limited tools to combat this devastating condition, recent randomised controlled trials have shown that there are now treatments that both prolong the rate of progression and improve survival. The ‘gold standard’ of treatment, due to its beneficial effect on survival, is epoprostenol, a prostacyclin analogue. However, there are a number of problems with the prostacyclin analogues, mainly centred on their administration and cost, which led to their use only in severely ill patients. A better understanding of the pathophysiology of PAH has led to a number of other pharmacological targets, namely antagonism of endothelin (ET) receptors and increasing local levels of nitric oxide (NO) via inhibition of phosphodiesterase 5.
The successful treatment of PAH means that there is now a growing population of patients on disease-modifying agents, so it is essential that physicians are aware of their use, benefits and side effects.
For UK healthcare professionals only