Background: A congenital pericardial defect is a rarely observed anomaly that usually accompanies other defects. In most cases the defect is discovered intraoperatively during cardio-thoracic procedures.
One third of patients with congenital pericardial defects have some additional anomalies,such as defects of the heart or other organs. They are exceptional and difficult to diagnose.Case Report: A 3-year-old boy with severe cyanosis admitted to our clinic. After echocardiographic examination tetralogy of Fallot was diagnosed. Neither echocardiography nor angiography showed a pericardial defect. Totally corrective surgery was planned. A large pericardial defect was detected intraoperatively, where no intervention regarding to pericardial defect was required.Conclusions: In this case the patient had adapted to the anomaly – this is why it did not require special intervention. Additionally, postoperative adhesions between the pericardial defect and the heart surface should prevent the displacement of the heart or the lung. In our opinion correction of large defects in lateral pericardial walls is not necessary. To our knowledge, tetralogy of Fallot associated with congenital pericardial defect has not been reported yet, and this is described in detail.

Keywords: left pericardial defect, Tetralogy of Fallot, surgical exposure