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Canadian Journal of Cardiology

Implantable Cardioverter-Defibrillators in Adults With Congenital Heart Disease: Toward the More Global Assessment of Healthspan

  • Paul Khairy
    Correspondence
    Corresponding author: Dr Paul Khairy, Montréal Heart Institute, 5000 Belanger St E, Montréal, Québec H1T 1C8, Canada. Tel.: +1-514-376-3330 ext 3800; fax: +1-514-593-2581.
    Affiliations
    Electrophysiology Service and Adult Congenital Heart Disease Center, Montréal Heart Institute, Université de Montréal, Montréal, Canada
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Published:December 13, 2022DOI:https://doi.org/10.1016/j.cjca.2022.12.005
      Sudden cardiac death is thought to be the second most common cause of mortality in the growing population of adults with congenital heart disease (CHD), surpassed only by heart failure–related deaths.
      • Khairy P.
      • Silka M.J.
      • Moore J.P.
      • et al.
      Sudden cardiac death in congenital heart disease.
      Sudden cardiac deaths account for up to 25% of all causes of mortality in adults with CHD, with rates that vary widely across the broad spectrum of congenital heart malformations. Risk stratification for sudden cardiac death in view of selecting appropriate candidates for primary-prevention implantable cardioverter-defibrillators (ICDs) is an evolving and imperfect science. Several professional societies have issued evidence-based recommendations that are largely consistent and address diverse scenarios, including systemic left ventricular systolic dysfunction in patients with biventricular physiology, unexplained syncope, tetralogy of Fallot, and dysfunction of a univentricular heart or systemic right ventricle.
      • Khairy P.
      • van Hare G.F.
      • Balaji S.
      • et al.
      PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD).
      ,
      • Khairy P.
      Primary prevention implantable cardioverter-defibrillators in adults with congenital heart disease: recommendations from professional societies.
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