A case report of successful atrioventricular junction pacing in Ebstein's anomaly.

We report a case of atrioventricular junction (AVJ) pacing in a patient with Ebstein's anomaly (EA). The patient was a 68-year-old man who suffered from pacemaker syndrome and complained of heart failure symptoms. He was initially diagnosed with EA in his thirties and received right ventricular (RV) apex pacing for safe during a surgery because of low heart rate atrial fibrillation (AF) 9 years ago. However, since the patient felt discomfort, the pacing rate was then programed down to 45-55 per/min. During recent years, he was often admitted for dyspnea, dizziness, or edema and was advised to undergo intracardiac repair, but he rejected this due to the high risk of the surgery. We believed that the patient's low heart rate and ventricular pacing burden (47.8%) might be important causes of the symptoms. Therefore, we suggested that the patient undergo an upgrade of the pacing mode. In consideration of possible abnormal cardiac coronary veins, we tried His bundle pacing (HBP) to upgrade pacing. However, the SelectSecure 3830 lead was fixed at the AVJ region and obtained steady pacing parameters. After the upgrading of the AVJ pacing mode. The patient's symptoms, exercise capacity and quality of life were all improved at the 2-year follow-up. Thus, we presented the first case of AVJ pacing in a patient with EA.