Incomplete Pentalogy of Cantrell: A Case Report

Adult congenital heart defect operations still maintain their importance in cardiovascular surgery practice. Atrial septal defect (ASD) is the most commonly seen congenital heart defect in this group, and it may be associated with various anomalies. This report presents the case of a patient with ASD who was also diagnosed with pentalogy of Cantrell, a rare multiple congenital malformation syndrome characterized by a combination of features: a midline supraumbilical abdominal wall defect, a defect of the lower sternum, a defect of the diaphragmatic pericardium, deficiency of the anterior diaphragm, and congenital cardiac anomalies. In the absence of concomitant severe anomalies in patients with pentalogy of Cantrell, preoperative transthoracic echocardiography (TTE) may not be adequate to detect the congenital defects. Therefore, further imaging modalities are vital for the evaluation of these patients.