Abstract
A third case of congenital marked hypoplasia of the right ventricular myocardium (Uhl's anomaly) associated with pulmonary valvular atresia and absent tricuspid leaflets is reported. The anatomic differences between this disease, hypoplasia of the right ventricle and Ebstein's anomaly are discussed. During the neonatal period the clinical diagnosis is difficult. Malformations like tricuspid atresia, pulmonary atresia with intact ventricular septum and Ebstein's anomaly can all give almost the same clinical picture. The prognosis of the complete form of Uhl's anomaly remains poor.
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