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Abstract:
Hypoplasia of the right ventricle occurring as an isolated abnormality is an uncommon cause of cyanotic congenital heart disease. Its presentation in the newborn may mimic the ductus dependent and less stable right ventricular hypoplasias such as tricuspid atresia and pulmonary atresia. However, the natural history of this abnormality contrasts with those ductus dependent lesions by the rather benign course it presents. A patient with only marked reduction in right ventricular size and commensurate reduction in size of the tricuspid and pulmonary valves is described. Although cyanotic this child has shown none of the critical problems frequently encountered with the more severe forms of the right ventricular hypoplasias.