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A case of Ebstein's anomaly and partial atrioventricular canal associated with double orifice mitral valve is reported. Patch closure of the primum defect, plication of the atrialized ventricle, and tricuspid valve replacement with a St. Jude Medical valve were carried out successfully, and the patient is doing well 2 years and 9 months after the operation. To our knowledge, this combination of congenital heart defects has not been reported in the literature.
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