ABSTRACT
Right sided aortic arch is an uncommon congenital anomaly. It can be classified into three types, depending on the left aortic arch's degenerating pattern and the branching pattern of the great vessels. It can be associated with major congenital heart disease, depending on the type of right sided aortic arch. We report a case of an 18-years-old female who has right sided aortic arch with atrial septal defect (ASD). In our case, the patient had a right sided aortic arch and aberrant left subclavian artery, also she had ASD (ostium secundum) and moderate tricuspid regurgitation with pulmonary hypertension. The patient was successfully performed patch closure of ASD and tricuspid valve annuloplasty via midline sternotomy. The patient had uneventful postoperative course.