Pulmonary arterial hypertension (PAH) is a life-threatening disease characterised by an elevated pulmonary vascular resistance in the absence of left ventricular disease, and increasing pulmonary artery pressure, with consequent right ventricular failure and death [1]. Several aetiologies have been associated with PAH, including connective tissue diseases, congenital heart diseases and chronic infections, such as HIV. Due to its high prevalence in developing countries, one of the most relevant forms of PAH worldwide is the one associated to schistosomiasis [2]. Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is present in 5% of patients with the hepatosplenic form of the disease [3]. Due to its histological and haemodynamic similarities to idiopathic PAH (IPAH), it is classified within group 1 (pre-capillary pulmonary hypertension) of the current pulmonary hypertension classification [4]. However, despite the similarities, a previous cohort has shown that Sch-PAH has a distinct, more benign course than IPAH, even in the absence of specific therapy [5]. Data regarding the efficacy of specific PAH therapy in Sch-PAH are scarce. Improvements in functional class, cardiac output and 6-min walking test distance (6MWT), using phosphodiesterase-5 inhibitors (PDE5i) or endothelin-1 receptor antagonists (ERA) were demonstrated in a small cohort of 12 Sch-PAH patients [6]. The effect of PAH treatment on hard end-points, such as clinical worsening or survival, has not been evaluated in this population, until this date. The aim of this study was to compare the survival of newly diagnosed Sch-PAH patients treated with PAH targeted therapies against a group of untreated patients from a historical cohort. Data from all consecutive, newly diagnosed Sch-PAH patients referred to our centre in Brazil were analysed. Sch-PAH was characterised by the presence of mean pulmonary arterial pressure ⩾25 mmHg with pulmonary artery occlusion pressure ⩽15 mmHg, in the absence of significant lung parenchymal disease, left ventricular dysfunction or chronic thromboembolic disease, associated with the presence of periportal fibrosis and/or left liver lobe enlargement associated with at least one of the following features: positive epidemiology, previous treatment of schistosomiasis or identification of eggs in stool or rectal biopsy [7]. Since specific PAH therapy became widely available in Brazil after 2010, patients diagnosed before this date received only supportive therapy and comprised our historical cohort. After 2010, Sch-PAH patients were regularly treated with PDE5i, ERA or both, at discretion of the attending physician, in accordance to the available guidelines [8, 9]. Baseline clinical, demographical, including New York Heart Association (NYHA) functional class (FC) assessment, 6MWT, brain natriuretic peptide (BNP) and haemodynamic data were collected. Analysis was performed using the SPSS 21 statistical package (SPSS, Inc.). All continuous variables are expressed as mean±SD and compared using t-test. Categorical d...