Ebstein's disease, pre-excitation and atrial fibrillation

Patient

28-year-old man with Ebstein's disease with pre-excitation; at the age of 11, the patient underwent a first procedure for the removal of a right posterolateral accessory pathway responsible for palpitations; current emergency hospitalization for sudden palpitations with unstable hemodynamics; injection of 2 ampules of intravenous amiodarone allowed a reduction of the arrhythmia with clinical stabilization;

Trace

ECG recorded on arrival; irregular and rapid ventricular rhythm (shortest RR measured at 230 ms); accordion-like pattern characteristic of atrial fibrillation due to ventricular pre-excitation; when the QRS complexes are maximally pre-excited (broadest QRS), we observe a left axis, a QS pattern in V1 and a transition between V1 and V2; the electrocardiographic pattern is characteristic of an atrial fibrillation due to accessory pathway;

Trace

This patient underwent a successful ablation procedure of a right posterolateral accessory pathway; characteristic pattern of Ebstein's disease: sinus rhythm, right atrial enlargement, prolonged PR interval (first degree atrioventricular block in relation to a prolonged right atrial depolarization time); incomplete right bundle branch block;

Exergue

Patients with Ebstein's disease are at increased risk of sudden death due to the presence of one or more accessory pathways but also with an increased incidence of atrial arrhythmias.