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Atrial fibrillation in Ebstein anomaly: weathering the storm
  1. Victor Waldmann1,
  2. Paul Khairy2
  1. 1 Unité médico-chirurgicale de Cardiologie Congénitale Adulte, Hôpital Europeen Georges Pompidou, Paris, France
  2. 2 Electrophysiology Service and Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, Montreal, Québec, Canada
  1. Correspondence to Dr Victor Waldmann, Unité médico-chirurgicale de Cardiologie Congénitale Adulte, Hôpital Europeen Georges Pompidou, Paris, France; victor.waldmann{at}

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Ebstein anomaly (EA), a form of congenital heart disease (CHD) that occurs in 1 to 20 000 live births, consists of a distinctive malformation of the right ventricle and tricuspid valve with adherence of septal and posterior leaflets to the underlying myocardium due to failure of embryological delamination. The consequent right atrial enlargement combined with the high prevalence of accessory pathways along the tricuspid annulus provide the substrate for various re-entrant tachyarrhythmias. While atrial flutter/tachycardias and atrioventricular re-entrant tachycardias have been well-described in these patients, data on the burden and outcomes associated with atrial fibrillation (AF) remain sparse.

In their Heart paper, Martin de Miguel and colleagues address this knowledge gap by reporting important observations on a retrospective cohort of 682 adult patients with EA from the Mayo Clinic, MN.1 The prevalence of AF at first assessment was 18%. After a median follow-up of 156 (81-240) months, 28% of the overall population had documented AF. The age at first AF episode was young (43±17 years) compared with patients without CHD but comparable to mixed cohorts of patients with CHD. For example, in a cohort of 482 patients with CHD and atrial arrhythmias from 12 North American centres, the presenting age of patients with AF was 41.0±17.2 years.2 Factors independently associated with AF in this multicentre cohort were older age, hypertension, number of cardiac surgeries and smoking history. In Martin de Miguel et al’s study, patients with EA who developed AF were older, more often male, had a higher prevalence of associated comorbidities (eg, hypertension, dyslipidaemia), had larger right and left atria and ventricles and lower metrics of systolic right and left ventricular function.

In addition to the high incidence of AF observed, an important finding that emerged …

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  • Contributors VW wrote the manuscript and PK made revisions.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

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