Abstract Rationale Schistosomiasis is a major cause of pulmonary arterial hypertension (PAH) worldwide. Schistosomiasis-associated hepatosplenic disease (SchHSD) is a form of severe schistosomiasis with liver fibrosis and portal hypertension, thought to be a precursor condition for development of schistosomiasis-PAH (SchPAH). We sought to characterize risk factors for developing PAH by comparing SchPAH with SchHSD subjects. Methods We are prospectively recruiting SchPAH and SchHSD cross-sectional cohorts at 4 clinical centers in Brazil. SchPAH subjects have PAH confirmed by right heart catheterization, whereas SchHSD subjects are screened via echocardiogram. Results To date, 40 SchPAH and 131 SchHSD subjects have been enrolled, with mean ages of 54 and 56 years respectively, of whom 58% and 47% are women. SchPAH subjects have an median mPAP of 51 mmHg, CI of 2.1 L/min/m2, and PVR of 890 dynes-sec-cm-5. Among 75 SchHSD subjects with echo data available so far, 11 (15%) had an estimated right ventricular systolic pressure above 35 mmHg. Conclusions We have prospectively recruited SchPAH and SchHSD cohorts in multicenter study based in Brazil. A higher female prevalence in SchPAH suggests shared biologic mechanisms with other PAH etiologies that increase disease risk in women. About 15% of SchHSD subjects could have pulmonary hypertension, as suggested by echo. Further analysis will identify clinical and laboratory parameters that correlate with PAH diagnosis. This abstract is funded by: NIH - NHLBI
Graham et al. (Fri,) studied this question.