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A case of incomplete endocardial cushion defect associated with mirror-image dextrocardia, IVC defect and azygos connection is reported. Intracardiac defect was corrected under moderate hypothermia with cardiopulmonary bypass. Three venous drainage cannulas were necessary to be indwelled into SVC, hepatic vein and right common iliac vein to maintain adequate venous drainage for extra-corporeal circulation. Thus, anomaly of venous system, which is commonly associated with mirror-image dextrocardia, must be recognized correctly and prepared before intracardiac correction.

Successful surgical repair of a 1-year-old girl of complete endocardial cushion defect associated with single atrium, persistent left superior vena cava draining into the left atrium, hemiazygos connection and double orifice mitral valve was reported. We repaired the endocardial cushion defect by using an endocardial cushion prosthesis and a new atrial septum was created to allow the left superior vena cava draining into the right atrium. We made no treatment for the accessory mitral valve orifice because of no regurgitation.

An adult case of partial anomalous pulmonary venous drainage of left pulmonary veins with dextrocardia, situs inversus and azygos continuation of inferior vena cava is reported. Surgical repair was accomplished by constructing a baffle to direct the blood flow from left pulmonary veins to the left atrium through the created atrial septal defect. This case is a rare type of PAPVD.