Ebstein Anomaly Focusing on Pre-excited Atrial Fibrillation Management

Abstract

Introduction: Ebstein’s anomaly is a rare abnormality of the heart associated with atrialization right ventricle and apical (downward) displacement of the tricuspid valve functional annulus. Twenty percents of patients with Ebstein’s anomaly accompanied with accessory pathway. The dilatation of atrium and aging process may develop atrial fibrillation (AF).Case Description: A 35 years old patient with recurrency palpitation, accompanied with dizziness and epigastric discomfort. He had history of taking propafenone 3 x 150 mg for long time while the palpitation recurrent. He was hospitalization due to propafenone could not suppress the palpitation. During monitor in hospital revealed haemodynamic stable with heart rate 160-180 beats/minute irregularly irregular. The electrocardiography showed atrial fibrillation with pre-excitation WPW syndrome. We performed electrical cardioversion 100 joule. Then the atrial fibrillation was convert to sinus rhythm with WPW pattern. The propafenone 3 x 150mg was continued. The patient was performed catheter radiofrequency ablation of the accessory pathway. Electrophysiology showed AV fusion at right anteroseptal pathway and preexcited atrial fibrillation with shortest RR interval 220 ms that converted by cardioversion. The ablation was successfully performed. Discussion: The accessory pathway is a complication of ebstein anomaly. Digoxin, beta-blockers, diltiazem, verapamil, and amiodarone are potentially harmful in pre-excited atrial fibrillation. Propafenone reduces fast inward potential by sodium channels, reduces spontaneous automaticity and prolongs the effective refractory periode so could be used in this case. Catheter ablation of accessory pathway in Ebstein anomaly with WPW syndrome was class I recommendation. In our case, the accessory pathway was successfully ablated.