Preventing Arrhythmic Death in Patients With Tetralogy of Fallot: JACC Review Topic of the Week

Mitchell I. Cohen, Paul Khairy, Katja Zeppenfeld, George F. Van Hare, Dhanunjaya R. Lakkireddy, John K. Triedman

Research output: Contribution to journalReview articlepeer-review

23 Scopus citations


Patients with tetralogy of Fallot are at risk for ventricular arrhythmias and sudden cardiac death. These abnormalities are associated with pulmonary regurgitation, right ventricular enlargement, and a substrate of discrete, slowly-conducting isthmuses. Although these arrhythmic events are rare, their prediction is challenging. This review will address contemporary risk assessment and prevention strategies. Numerous variables have been proposed to predict who would benefit from an implantable cardioverter-defibrillator. Current risk stratification models combine independently associated factors into risk scores. Cardiac magnetic resonance imaging, QRS fragmentation assessment, and electrophysiology testing in selected patients may refine some of these models. Interaction between right and left ventricular function is emerging as a critical factor in our understanding of disease progression and risk assessment. Multicenter studies evaluating risk factors and risk mitigating strategies such as pulmonary valve replacement, ablative strategies, and use of implantable cardiac-defibrillators are needed moving forward.

Original languageEnglish
Pages (from-to)761-771
Number of pages11
JournalJournal of the American College of Cardiology
Issue number6
StatePublished - Feb 16 2021


  • arrhythmias
  • implantable cardioverter-defibrillator
  • sudden cardiac death
  • tetralogy of Fallot


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