Sudden Cardiac Death in Congenital Heart Disease

Mar 25, 2022
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Authors:
Khairy P, Silka MJ, Moore JP, et al.
Citation:
Sudden Cardiac Death in Congenital Heart Disease. Eur Heart J 2022;Mar 18:[Epub ahead of print].
Summary By:
Martin LaPage, MD, FACC

Quick Takes

  • The rate of sudden cardiac death (SCD) in congenital heart disease (CHD) is 20-30 times the general population and accounts for up to 25% of deaths in this population.
  • The etiology of SCD in CHD is multifactorial and may be amenable to ICD therapy in a minority of cases.
  • A broad and practical collection of teaching points is presented throughout the review.

Study Questions:

What is the current state of knowledge regarding sudden cardiac death (SCD) in the congenital heart disease (CHD) population?

Methods:

This is a broad review of multiple aspects of SCD in patients with CHD including epidemiology, pathophysiology, risk factors, resuscitation, and use of implantable cardioverter-defibrillators.

Results:

SCD accounts for up to 25% of deaths in CHD. Risk varies widely by type of CHD and associated comorbidities. Distinct understanding of CHD anatomical variations is important for all aspects of SCD management, from prevention to acute resuscitation to post-arrest management. Importantly, a minority of cases appear due to a shockable ventricular rhythm; indicating the importance of thoughtful consideration in the application of ICD therapy in this population at increased risk for complications. Knowledge gaps and suggested resolutory research directions are presented.

Conclusions:

SCD in CHD is impacted by multiple and sometimes poorly defined risk parameters. Ongoing research is imperative to define the best management practices to prevent and treat SCD in this population.

Perspective:

Renowned adult CHD expert Paul Khairy, along with a prominent group of international experts, produce a broad yet substantive review of SCD in CHD. While recognition of significant knowledge gaps is a prominent feature, the review is sprinkled with a wide variety of specific, practical, and clinically applicable teaching points from which any provider likely to encounter this population of patients would benefit.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Prevention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Quality Improvement

Keywords: Arrhythmias, Cardiac, Cardiopulmonary Resuscitation, Defibrillators, Implantable, Death, Sudden, Cardiac, Heart Arrest, Heart Defects, Congenital, Resuscitation, Risk Factors, Secondary Prevention, Shock


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