Pulmonary regurgitation(PR) and right ventricular dysfunction are the major complications in a longterm period after the repair of congenital heart diseases including tetralogy of Fallot(TOF). The purpose of this study is to investigate the effectiveness of pulmonary valve replacement (PVR) after the congenital heart repair. Twenty-five patients undergoing PVR for PR with clinical symptoms in a longterm period between March 2008 and December 2016 were retrospectively reviewed. The average age at the TOF repair was 2.6±2.6 years. The average age at the PVR was 21.6±11.2 years. The mean follow-up after the PVR was 52.8±27.5 months. There was neither hospital death nor late death. All the patients underwent PVR with a bio-prosthetic valve. Nine patients had concomitant tricuspid valve repair. PVR significantly reduced right ventricular end-diastolic volume( RVEDVI)[ 123±25 vs 101± 13% of normal, p=0.002], cardio-thoracic ratio (CTR) [56.0±6.9 vs 52.6±13.0%, p=0.03], and brain natriuretic peptide (BNP) [57.6±57.0 vs 38.7±31.3 pg/dl, p=0.03]. The relatively early PVR significantly improved RVEDVI, CTR and cardiac function.