Congenital heart diseaseCohort study

Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Patients With Ebstein Anomaly

JACC Clin ElectrophysiolPublished

  • retrospective observational study
  • large tertiary referral center

Authors' conclusion

The lifetime burden of arrhythmic events in patients with EA is considerable, with more than half developing atrial arrhythmias by the age of 60 years. Intra-atrial re-entrant tachycardia/focal atrial tachycardia is the predominant arrhythmia, although the cumulative incidence of atrial fibrillation is also substantial. The significantly higher incidence of atrial arrhythmia observed in patients who underwent cardiac surgery is driven by a more advanced disease severity.

Study context

Population
Patients diagnosed with Ebstein anomaly (EA) between 2001 and 2024patients with EA (55.5% women, mean age at the last follow-up: 40.4 ± 17.8 years)
Participants
200 patients
Exposure
patients who underwent at least 1 cardiac surgery vs nonoperated patients

Main finding

As reported in the abstract

At least 1 episode of atrial arrhythmia

Any atrial arrhythmia

79 (39.5%)

Focal atrial tachycardia/intra-atrial re-entrant tachycardia

54 (27.0%)

Atrial fibrillation

37 (18.5%)

Both arrhythmias

12 (6.0%)

Observational data: associations, not effects.

Further findings

Outcomepatients who underwent at least 1 cardiac surgerynonoperated patientsEstimateP value
Risk factors independently associated with atrial arrhythmias — Cardiac surgery vs nonoperated patients · Time-dependent Cox regression modelsHR 3.10 (95% CI 1.80–5.32)
Risk factors independently associated with atrial arrhythmias — moderate-to-severe tricuspid regurgitation vs nonoperated patients · Time-dependent Cox regression modelsHR 2.44 (95% CI 1.47–4.06)
Risk factors independently associated with atrial arrhythmias — severe right ventricular dysfunction vs nonoperated patients · Time-dependent Cox regression modelsHR 2.40 (95% CI 1.21–4.78)
Risk factors independently associated with atrial arrhythmias — age vs nonoperated patients · Time-dependent Cox regression modelsHR 1.18 by 5-year increment (95% CI 1.09–1.28)
Atrial arrhythmia by cardiac surgery46.2%21.8%HR 3.10 (95% CI 1.80–5.32)P < 0.001

Estimated lifetime risk of atrial arrhythmia · ages 20, 40, and 60 years

Age 20 years
5.7% (2.3%-9.0%)
Age 40 years
25.0% (18.1%-31.9%)
Age 60 years
54.2% (44.1%-64.3%)

Original publication

Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Patients With Ebstein Anomaly. JACC Clin Electrophysiol. Published 30 July 2026. doi:10.1016/j.jacep.2026.06.020

Summarised from the published abstract; every number is checked against it