Abstract
Ebstein's anomaly and congenital hypoplasia of the right ventricle give rise to signs and symptoms of partial obstruction to outflow from the right atrium, and this may lead to right heart failure. Successful surgical correction or relief of these defects has not been previously reported. Anastomoses between the superior vena cava and the right pulmonary artery have been established in a 4-year-old girl with congenital hypoplasia of the right ventricle and in two patients, aged 8 and 14 years, with Ebstein's anomaly. Relief of the distention and of the elevated pressure in the right atrium was immediately apparent in these three patients. The 14-year-old girl died four days after operation, and autopsy revealed a thrombosis of the left main pulmonary artery, but the two patients aged 4 and 8 years continued to show marked clinical improvement. The desirability of eventual closure of the septal defect remains to be decided.
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