ISOLATED HYPOPLASIA OF THE RIGHT VENTRICLE AND TRICUSPID VALVE IN SIBLINGS

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Abstract
In two sibling infants, isolated hypoplasia of the right ventricle and tricuspid valve (an anomaly not described elsewhere) gave the clinical appearance of a right-to-left interatrial shunt with evidence of diminished right ventricular function. Other malformations that may produce this picture are tricuspid atresia, pulmonary atresia with an intact ventricular septum and a small right ventricular chamber, Ebstein''s malformation, and congenital tricuspid stenosis. Correct differential diagnosis is important because of the possibility of definitive surgery in some cases of pulmonary atresia and congenital tricuspid stenosis. Electrocardiography and angiocardiography appear to be the principal methods for achieving such diagnosis.