Brazilian guidelines for the pharmacological treatment of pulmonary hypertension. Official document of the Brazilian Thoracic Society based on the Grading of Recommendations Assessment, Development, and Evaluation methodology.

Pulmonary hypertension (PH) is a complex and heterogeneous clinical condition associated with high morbidity and mortality and can occur in the context of various diseases. It is characterized by increased pulmonary arterial pressure and progressive right ventricular overload. The current classification of the different forms of PH presentation comprises five groups, based on pathophysiological similarities and available therapeutic options. In recent decades, significant advances in pathophysiological understanding and the development of specific therapies have significantly modified the prognosis of patients with PH. This guideline aims to define updated Brazilian recommendations for the treatment of PH, with an emphasis on pulmonary arterial hypertension, considering the available scientific evidence and the particularities of the Brazilian health system. Four structured questions in the PICO format (patients, intervention, comparator group, and outcome) were developed by a multidisciplinary panel of Brazilian experts. Systematic literature reviews were conducted for each question, based on randomized clinical trials. Meta-analyses were conducted where methodologically feasible. The quality of evidence and the strength of recommendations were defined according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. The guideline was complemented by a comprehensive narrative review of the literature on clinical management of patients with PH, especially patients with pulmonary arterial hypertension, addressing general measures, specific therapeutic strategies, risk stratification, and treatment algorithm, always based on the best available scientific evidence and with the aim of contributing to the improvement of care for patients with PH in Brazil.