The several randomized controlled trials (RCTs) conducted in the last 25 years in the field of pulmonary arterial hyper- tension (PAH) have led to the approval of over 10 drugs, which seek to restore the right balance between vasoconstrictor and vasodilator factors, and to the consequent increase of the median survival. However, no medical treatment is currently curative. Ongoing research is focusing on how to preserve right ventricle (RV) function, curbing the inexorable progres- sion of the disease towards right heart failure and death. Below, we provide a comprehensive review of the current available PAH specific therapies and briefly report the therapeutic strategies adopted by PH specialists in clinical practice.
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