Schistosomiasis-associated pulmonary arterial hypertension: survival in endemic area in Brazil

•The PHC registry equation accurately predicted survival in Sch-PAH patients.•Sch-PAH survival cannot be safely predicted using only validated clinical variables only.•Survival studies in endemic regions of Schistosomiasis are needed to establish the real prognosis of Sch-PAH in the world. The survival of schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) patients in endemic areas is unknown, but can be estimated using predictive equations. We retrospectively analyzed all consecutive patients diagnosed with Sch-PAH referred to the Pronto SocorroCardiologico de Pernambuco between 2004 and 2010 using specific therapy and measured laboratory, diagnostic imaging, and baseline hemodynamic parameters. Observed and predicted survivals according to the National Institutes of Health (NIH) and Pulmonary Hypertension Connection (PHC) registry equations were compared by the Kaplan–Meier method, log-rank test and Cox proportional hazards model. Sixty-eight patients (47 [69.1%] women) observed for a mean of 3.1years (range, 7–72months), median survival was 74months, and 42 (61.7%) survived. The sex and age distributions were similar for functional class I/II and III/IV patients. Hemodynamic abnormalities were severe: mean right atrial pressure, 12.6±6.2mmHg; mean pulmonary artery pressure, 60.3±13.69mmHg; pulmonary vascular resistance, 14.62±7.04 Wood units; and cardiac index, 2.3±0.8L/min/m2. The usual idiopathic PAH predictors were not prognostic in Sch-PAH patients. The 1-, 3- and 5-year survival rates were 92.1%, 75.2%, and 50.8%, respectively, and those estimatedby the NIH and PHC registry equations were 68%, 45% and 32% (p=0.001), and 93%, 79% and 68% (p=0.340), respectively. Sch-PAH patients in endemic areas have severe hemodynamic profiles and reduced long-term survivaldespite treatment. The PHC registry equation may be a useful tool to estimate survival in Sch-PAH.