THE CARDIAC ANOMALY AND THERAPY OF ASPLENIA SYNDROME

Abstract

Seventeen cases of asplenia syndrome in Gunma children's medical center are reported. All of these patients had a cardiac anomaly such as common atrium, single ventricle, pulmonary stenosis, pulmonary atresia, total anomalous pulmonary venous return (TAPVR), common atrioventricular canal, and bilateral superior vena cava. Many patients had cyanosis and congestive heart failure.Prostaglandin E1 was administrated to patients with severe cyanosis due to pulmonary atresia or severe pulmonary stenosis. The hypoxic state of these patients improved, and no patient died in the neonatal period. Five patients were operated on by the Blalock-Taussig shunt procedure, and in all of them the hypoxia improved.Twelve patients died in infancy or childhood (3 mo-11 y 1 mo). all 10 patients who had TAPVR died of congestive heart failure and infection, and the other 2 died of infection.Only five of the patients are still alive (2 y 8 mo-5 y 9 mo), but if the operation for TAPVR is indicated and successful, the prognosis of asplenia syndrome seems to be improved.