Complete atrioventricular canal defect accompanied by complete atrioventricular block is one of the rare congenital heart abnormalities. A 32-year-old mother was referred to our clinic as her fetal ultrasonography was suggestive of a congenital heart abnormality. Echocardiographic examination of the newborn showed dextrocardia, a large atrial septal defect, single atrioventricular valve, ventricular septal defect, and aortic insufficiency. The newborn was diagnosed as having complete atrioventricular canal defect, complete atrioventricular block, and situs inversus totalis, and implantation of an epicardial pacemaker was decided as the initial intervention. A VVI pacemaker generator was implanted in the subdiaphragmatic position and a pediatric epicardial lead was implanted in a region close to the right ventricular apex. The patient was extubated on the fifth postoperative day and was then discharged with improved general condition. A literature review showed no reported case of complete atrioventricular canal defect and complete atrioventricular block accompanied by situs inversus totalis.

Keywords : Heart block/congenital/therapy; infant; pacemaker, artificial; situs inversus