Introduction: Hepatosplenic schistosomiasis mansoni, associated with pulmonary arterial hypertension (PAH), causes cardiac chamber remodeling. Little is known about its long-term evolution. Objective: To evaluate the alterations caused by PAH in patients with schistosomiasis and analyze the clinical and echocardiographic evolution over a period of 10 years. Methods: The study included 60 patients with PAH due to schistosomiasis and 50 healthy control patients. Clinical and echocardiographic data were evaluated, including dimensions, parietal thickness, function of the right and left chambers, and parameters of myocardial strain. Patients in the group with PAH due to schistosomiasis were followed for 10 years. Data were compared using Student's t test for unpaired samples with significance level of < 5%. Results: Patients with PAH had smaller left ventricular dimension without alteration in the ejection fraction, but with decreased left ventricular global longitudinal strain and left atrial reservoir strain. The right ventricular dimensions and parietal thickness were increased, and the parameters of systolic function (tricuspid annular plane systolic excursion, fractional area change, tricuspid s' wave, and the right ventricular global longitudinal strain) were significantly reduced. During the follow-up period, 18 patients (32%) died, and death was associated with higher functional class, decreased right ventricular longitudinal strain, larger right ventricular size, decreased fractional area change, and decreased tricuspid annular s' wave velocity. Conclusion: PAH related to schistosomiasis causes remodeling of the right chambers, with decreased parameters of systolic function and myocardial strain. The long-term evolution, with elevated mortality, presents with larger right ventricular dimensions, lower fractional area change, lower tricuspid annular systolic velocity, lower right ventricular global longitudinal strain, and more advanced functional class.