Ebstein's Anomaly between Valve Damage and Cardiac Arrhythmia

Ebstein’s anomaly (E.A) is a congenital malformation that represents less than 1% of all congenital heart disease. They are predisposed to arrhythmias because of abnormal cardiac anatomy. We report the case of a 60-year-old female with a history of early infancy diagnosis of E.A, admitted for tachycardia with wide QRS complex. An electrophysiology study revealed an atrial rhythm and programmed atrial stimulation induced atrial tachycardia identical to the clinical tachycardia with multiple circuits. Radiofrequency ablation was not performed because of technical difficulties. At two months follow-up, the patient’s cardiac condition worsened and echocardiography revealed worsening tricuspid regurgitation. She underwent surgical repair. The postoperative course was uneventful. The diagnosis of this anomaly is mainly made by transthoracic echocardiogram, which constitutes the cornerstone of imaging examinations. Diagnosis is made when there is an apical displacement of the tricuspid septal leaflets past 8mm/m². Cardiac arrhythmias are frequent with Ebstein malformation; sudden death and accessory pathway fast-conducting AV with anterograde and retrograde conduction are commonly described in most patients. While, wide-QRS tachycardia, ventricular tachycardia (VT), or flutter through the para-septal pathway may occur. The management of tachyarrhythmias associated with E.A remains a real challenge. Even if it can be difficult given the atrial dilatation, electrophysiological exploration with possible radiofrequency ablation of symptomatic supraventricular tachyarrhythmias remains necessary. Otherwise, supraventricular tachyarrhythmia in E.A, can also be ablated surgically.