OBJECTIVES: To determine the effectiveness and safety of macitentan in the treatment of patients older than 12 years diagnosed with hereditary or idiopathic pulmonary hypertension or pulmonary arterial hypertension related to a) connective tissue disease, b) congenital systemic-to-pulmonary shunts c) infection HIV, d) the use of drugs or toxic exposure. These patients should would find in functional class II, III or IV from WHO. METHODS: Systematic review of the literature. It was included systematic reviews, Meta-analyzes and randomized clinical trials comparing placebo and macitentan in outcomes such as morbi-mortality, death or hospitalization for PAH, adverse events and serious adverse events. The quality of the evidence was evaluated by the GRADE tool and the risk of bias in clinical trials with the Cochrane methodology. RESULTS: A randomized, double-blind, with 492 participants (115 weeks follow-up period) was found and comparing macitentan vs. placebo, obtaining results such as morbidity and mortality: HR 0.55 (CI 97.5%; 0.39 to 0.76), absolute risk reduction of 16.65% (95% CI 7.86 - 25.07). Mortality or hospitalization: HR 0.50 (CI 97.5%, 0.34 -0.75; P <0.001) absolute risk reduction 15.23% (95% CI 6.67 - 23.5). Serious adverse events (at least 1 per patient): RR 0.82 (95% CI 0.68 to 0.98), absolute risk reduction 9.98% (95% CI 1.14 - 18.60). CONCLUSIONS: Patients treated for 115 weeks with macitentan had a 16.65% reduction in the occurrence of death outcomes, atrioseptostomy, lung transplantation, initiation of therapy with prostanoids or worsening of pulmonary arterial hypertension. Similarly, with the use of macitentan there is an absolute risk reduction of 15.23% for the outcome death or hospitalization for PAH. Serious adverse events are presented 10% less in patients treated with macitentan.