Treatment of Pulmonary Arterial Hypertension: An Update

The actual guidelines on the treatment of pulmonary arterial hypertension (PAH) advocates, besides the prescription of nonspecific measures such as oxygen and diuretics, the sequential use of PAH-specific drugs according to predefined clinical targets. The PAH drugs licensed for this indication amount to 9 different compounds divided into three main therapeutic classes. Although most clinical studies, except for the most recent ones, were of short duration with questionable primary endpoints, there is some evidence that these specific therapies have improved the morbidity and the mortality of the disease. In addition to pharmacological treatment, pulmonary rehabilitation has also recently been shown to be beneficial in these patients. Despite all this encouraging progress, PAH remains an incurable disease, and further therapeutic modalities are much awaited.