Abstract
BACKGROUND: Ebstein's anomaly is a rare congenital heart disease characterized by apical displacement of the septal and posterior tricuspid valve leaflets. It is commonly associated with other defects such as patent foramen ovale or accessory atrioventricular pathways. CASE SUMMARY: We describe a case of an Ebstein anomaly diagnosed in an adult in his 50s in association with a septal defect between the left ventricle and right atrium (Gerbode defect). The diagnosis was confirmed on magnetic resonance imaging. A third anomaly was noted on coronary angiography, consisting of an aberrant origin of the right coronary artery from the left sinus of Valsalva. The patient was paucisymptomatic until he developed typical atrial flutter. Catheter ablation was employed after first arrhythmia recurrence and the patient is to date in good clinical condition. CONCLUSION: The association of Ebstein's anomaly-Gerbode defect is extremely rare, and to our knowledge, this is the first case that presents in addition an anomalous coronary artery. Both structural defects were without haemodynamic significance, and there was no proof of myocardial ischaemia. As the case illustrates, congenital disorders, even when in conjunction, can have a silent clinical course and multimodality imaging is sometimes necessary for a complete and final diagnosis.