Electocardiographic features in Ebstein's disease

Tunis Med. 2021;99(6):614-622.


Introduction: 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.

Aim: To describe the electrocardiographic features observed in Ebstein's disease.

Methods: We conducted a retrospective descriptive study that enrolled 26 patients followed for ED.

Results: The mean age of discovery of the ME was 103.5±99 months [0-31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42%). Eight patients (30%) had wide QRS≥ 120 ms. Seven of these 8 patients (27%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84%), it was a complete block in 7 cases (27%). A preexcitation was found in 6 patients (23%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34%). It was a junctional tachycardia in 3 patients (11%), atrial flutter in 4 patients (15%) and atrial fibrillation in 2 patients (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.

Conclusion: Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.

MeSH terms

  • Atrial Fibrillation*
  • Ebstein Anomaly* / complications
  • Ebstein Anomaly* / diagnosis
  • Ebstein Anomaly* / epidemiology
  • Humans
  • Retrospective Studies
  • Tricuspid Valve / abnormalities
  • Wolff-Parkinson-White Syndrome* / surgery