Total anomalous pulmonary venous connection (supracardiac type: report of 3 cases)

심장상부 환류형 총폐정맥환류이상증의 수술치험 3례 보고

TAPVC represents 1 to 4% of all congenital cardiac defects. Generally severe and refractory cardiac failure develops in the majority of patients in the early infancy. In a small minority of patient, they are relatively asymptomatic in infancy, but symptoms will develop of necessity In the later life. Our three cases had dyspnea on exertion and cyanosis and had a history of frequent respiratory infections. All 3 cases had the typical showman configuration on roentgenograms of the chest. Current surgical therapy has greatly altered the unfavorable course of these patients after institution of extra-corporeal circulation. We have experienced three cases from June, 1973 to May, 1983, the patients were undergone complete repair with extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery in the Yonsei University. The ages ranged from 12 years to 14 years, all cases were supracardiac type of TAPVC. Only one case died due to bleeding from anastomosis site between LA and pulmonary venous trunk. In the remaining two patients with complete corrections of TAPVC, there was excellent relief of symptoms such as dyspnea and cyanosis. Their postoperative course have been good during follow-up.