Estrategia terapéutica en hipertensión arterial pulmonar

Research and the advances in treatment of pulmonary arterial hypertension can lead to a change in the natural history of this disease. The basis of the treatment is the use of specific pulmonary vasodilators, although other measures need to be taken into account such as physical activity, psychological support, anticoagulant and diuretic treatment, oxygen therapy, contraceptive use. Prior to any type of specific treatment, a study is required on the acute vaso-reactivity of the pulmonary vascular bed, since patients with a positive response could benefit from treatment with calcium antagonists. In the case of a negative response or on not being sustained responders, there are now drugs available for the three main metabolic pathways implicated in the development of the disease. These include prostanoids (epoprosterenol, treprostinil and iloprost, selexipag), endothelin receptor antagonists or ERA (bosentan, macitentan and ambrisentan), phosphodiesterase-5 inhibitors or PDE5 (sildenafil and tadalafil), and guanylate cyclase stimulators (riociguat). The choice of initial drug, whether single therapy or in combination, will depend on multiple factors such as functional class, comorbidities, interactions with other drugs, tolerability and safety, and finally the availability and preferences.