Left atrial approach to close the atrial septal defect type II in case of right lung agenesis with extreme dextroversion

This paper presents a case of a rare combination of right lung agenesis and atrial septal defect type II (ASD II). Successful surgical repair of ASD II was performed at the age of six years with total circulatory arrest (TCA) and deep hypothermia, because of difficult approach. Computed tomography and intraoperative findings are described. From patho-embryological consideration, defects in the primitive respiratory apparatus in embryo stages were presumably responsible for agenesis of the lung. The patient was found to have a very short stump of the right pulmonary artery, but this malformation seems to be secondary.