Sudden cardiac death in congenital heart disease

Paul Khairy, Michael J. Silka, Jeremy P. Moore, James A. Dinardo, Jim T. Vehmeijer, Mary N. Sheppard, Alexander Van De Bruaene, Marie A. Chaix, Margarita Brida, Benjamin M. Moore, Maully J. Shah, Blandine Mondesert, Seshadri Balaji, Michael A. Gatzoulis, Magalie Ladouceur

Research output: Contribution to journalReview articlepeer-review

7 Scopus citations


Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Notwithstanding these constraints, identified higher-risk substrates include tetralogy of Fallot, transposition of the great arteries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation. Without autopsies, it is often impossible to distinguish SCD from non-cardiac sudden deaths. Asystole and pulseless electrical activity account for a high proportion of SCDs, particularly in patients with heart failure. High-quality cardiopulmonary resuscitation is essential to improve outcomes. Pulmonary hypertension and CHD complexity are associated with lower likelihood of successful resuscitation. Risk stratification for primary prevention implantable cardioverter-defibrillators (ICDs) should consider the probability of SCD due to a shockable rhythm, competing causes of mortality, complications of ICD therapy, and associated costs. Risk scores to better estimate probabilities of SCD and CHD-specific guidelines and consensus-based recommendations have been proposed. The subcutaneous ICD has emerged as an attractive alternative to transvenous systems in those with vascular access limitations, prior device infections, intra-cardiac shunts, or a Fontan circulation. Further improving SCD-related outcomes will require a multidimensional approach to research that addresses disease processes and triggers, taxonomy to better reflect underlying pathophysiology, high-risk features, early warning signs, access to high-quality cardiopulmonary resuscitation and specialized care, and preventive therapies tailored to underlying mechanisms.

Original languageEnglish (US)
Pages (from-to)2103-2115
Number of pages13
JournalEuropean heart journal
Issue number22
StatePublished - Jun 7 2022


  • Cardiac arrest
  • Congenital heart disease
  • Implantable cardioverter-defibrillator
  • Risk stratification
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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