Modern pharmaceutical treatment of pulmonary arterial hypertension

The review is devoted to modern pharmaceutical treatment of pulmonary arterial hypertension (PAH). In 1970-80s, it was based on high-dose calcium antagonists, CA (e.g., nifedipine 240 mg/d), which were effective only in some patients with primary (idiopathic) PAH. CA were combined with digoxin, indirect anticoagulants and oxygen. In 1990s, PAH therapy included prostanoids, endothelin receptor (ER) blockers and phosphodiesterase (PDE) inhibitors, which are more selective pulmonary vasodilatators than CA, and therefore are safer for long-term treatment. Prospective studies have demonstrated that continuous intravenous infusion of epoprostenol and ET receptor blocker bosentan treatment improve survival of PAH patients receiving traditional therapy. Clinical effectiveness of epoprostenol is similar to that of subcutaneously administered trepostinil, and bosentan is similar to PDE inhibitor Type 5 sildenafil. Bosentan increases the effectiveness of intravenous epoprostenol and inhaled trepostinil