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Pulmonary valve stenosis (PS) is a common congenital heart disease, its early diagnosis and treatment can improve growth and prognosis in children with pulmonary valve stenosis. With the development of the diagnostics and treatment, percutaneous balloon pulmonary valvuloplasty (PBPV) has replaced surgery as the first choice for isolated pulmonary valve stenosis. The article reviews the recent progress in diagnosis and treatment of pulmonary valve stenosis.