Objective To evaluate the therapeutic effect of spironolactone on schistosomal pulmonary arterial hypertension(SPAH). Methods A total of 62 patients suffered from hepatosplenic schistosomiasis with pulmonary arterial hypertension were divided into the spironolactone group(n=31) and control group (n=31). All the patients underwent serial echocardiography and the clinical effect before and after the treatment was evaluated by assessing the mean pulmonary arterial pressure (mPAP) and pulmonary arterial diameter (PAD). At the same time, the varieties of the clinical symptoms, signs and the distance of the 6-minute walking test (6-MWT) were investigated. Results In spironolactone group, mPAP(-x±s) decreased from (31.8±7.1) mmHg to (21.2±2.1) mmHg, PAD(-x±s) decreased from (28.0±5.0) mm to (20.0±3.5) mm before and after the treatment respectively(P<0.01). There were significant differences in mPAP, PAD, the distance of 6-MWT and the heart function before and after the treatment in the spironolactone group. However, the data did not show the significant difference in the control group. Conclusion The therapeutic effect of spironolactone in the treatment of SPAH is satisfactory.