Pulmonary arterial hypertension is a feature of a spectrum of diseases that includes elevated pulmonary vascular resistance, induces right ventricular insufficiency and heart failure, and threatens the life. The aetiology and pathogenesis is diverse and associated with elevated morbidity and mortality. Treatment is frequently deficient and empirical. Fortunately, in recent years, randomized clinical trials have shown useful effects of various drugs on pulmonary arterial hypertension. This article reviews the pharmacological and not pharmacological therapeutic options to treat pulmonary arterial hypertension and attempts to provide a proposal of algorithm for its management based upon the evidence and available guidelines for clinical management.