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The aim of this study was to analyze the midterm results following the Ross-Konno procedure in children. Between 1999 and 2008, 29 patients with complex left ventricular outflow tract obstruction underwent the Ross-Konno procedure. There were 12 (41%) infants (group A) and 17 (59%) older patients (group B). The median age at operation was 3.3 years (range, 6 days to 16 years). At 7 years of follow-up, survival was 96% (1 late death), with no differences between groups A and B. Freedom from aortic regurgitation ≥ mild was 81%, with no differences between groups A and B. No residual gradient was noted in the left ventricular outflow tract in either group. Freedom from mitral regurgitation ≥ mild was 100% in group B and 41% in group A (P = .0029). The mitral regurgitation was associated with morphological abnormalities of the mitral valve and with development of endocardial fibroelastosis after failed intervention during the newborn period. Freedom from reoperation was 73% in group B and 24% in group A (P = .0093). All patients are now in sinus rhythm, and 43% are without medication. With the technical aspects of this procedure well accomplished, mortality is low, and the functional outcome is encouraging. At midterm follow-up, there was no residual or recurrent outflow tract obstruction and an excellent function of the neoaortic valve. The higher incidence of mitral regurgitation in infants, which was associated with morphological abnormalities of mitral valve and development of endocardial fibroelastosis, is worrisome.