Ebstein's anomaly of the tricuspid valve is a rare and complex congenital heart defect. Its etiology is unknown and in the majority of cases it is multifactorial. Mortality in the neonatal period is high. The authors present a case of severe Ebstein's anomaly diagnosed antenatally. In the perinatal period a Blalock-Taussig shunt was performed and at the age of five months the infant underwent successful percutaneous pulmonary valvuloplasty. The clinical outcome has been favorable and we decided to postpone a Glenn procedure. At 18 months the child is asymptomatic.
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