Electrocardiographic features in Ebstein's disease

Abstract

Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease. The purpose of our work is to describe the electrocardiographic features observed in ED. This was a retrospective descriptive study that enrolled 26 patients followed for ED. The mean age of discovery of the ED was 103.51 ± 99 months [0–31 years]. The diagnosis of ED is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42.3%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42.3%). Eight patients (30.7%) had wide QRS ≥ 120 ms. Seven of these 8 patients (26.9%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84.6%), it was a complete block in 7 cases (26.9%). A preexcitation was found in 6 patients (23.1%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34.6%). It was a junctional tachycardia in 3 patients (11.5%), atrial flutter in 4 patients (15.4%) and atrial fibrillation in 2 patients (7.7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome. Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.