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Background: This study was designed to investigate the effect of captopril on systolic pulmonary arterial pressure (PAP) and circulating endothelin-1 (ET-1) following surgical repair of tetralogy of Fallot (TOF). Methods and Results: Seventy-six consecutive patients were divided into captopril and control groups following successful repair of TOF. The captopril group was treated with oral captopril (0.2 mg/kg/day) for 12 months. Venous blood was taken before and after the surgery for the analysis of ET-1. Systolic PAP and ventricular function were assessed by Doppler echocardiography. In the control group, there was an increase in ET-1 and systolic PAP following the surgery (p < 0.05). In the captopril group, there was no significant increase in the systolic PAP and ET-1 during the 12-month follow-up. The PAP and ET-1 values in the captopril group were lower than those in the control group following the surgery (p < 0.05). Conclusions: Systolic PAP is elevated following the complete surgical repair of TOF. Increased circulating ET-1 may be responsible for the elevation of PAP. Captopril treatment prevents postsurgical increases in systolic PAP and circulating ET-1. [ABSTRACT FROM AUTHOR]