Fanconi aplastic anemia is a genetic marrow failure that is accompanied by some congenital system anomalies. It is one of the most common causes of congenital anemia in childhood. An association with congenital heart disease is rare. Hematologic problems can be challenging in pediatric patients undergoing open heart surgery and preoperative evaluation is important. In our case report, we aimed to present our preoperative evaluation and surgical approach in a seven-year-old patient with Fanconi aplastic anemia who underwent open heart surgery for a concomitant atrial septal defect

Keywords: Aplastic anemia, Atrial septal defect, Fanconi anemia, Cardiac surgery