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Pulmonary atresia with an intact ventricular septum (PA/IVS) is a rare congenital heart disease. Pulmonary regurgitation is a major late postsurgical complication and pulmonary valve replacement is the mainstream treatment.We report the case of a 15-year-old boy with PA/IVS who developed severe pulmonary regurgitation after prior surgical repair. Intraoperative angiography revealed the absence of bilateral femoral venous trunks, making transcatheter pulmonary valve replacement (TPVR) not feasible. Transthoracic pulmonary valve replacement using a self-expanding Venus P-Valve was successfully performed. Postoperative recovery was uneventful, and follow-up showed good valve function and improved right ventricular performance.Transthoracic implantation of a self-expanding pulmonary valve may serve as a safe and feasible alternative for patients with severe pulmonary regurgitation who are unsuitable for TPVR due to unfavorable vascular anatomy.