Risk stratification for ventricular arrhythmias and sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy: an update

ABSTRACT

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined disease associated with a significant risk of ventricular arrhythmias and sudden cardiac death (SCD). Implantable cardioverter-defibrillators (ICDs) are the only effective preventive measure. Over the past 30 years, much effort has been invested in determining predictors of adverse arrhythmic events in these patients.

Areas covered: This review summarizes available evidence on risk stratification for ARVC, with an emphasis on recent research findings. While efforts are ongoing to define risk predictors, several recent publications have synthetized and built on this knowledge base. A recently published meta-analysis has clarified the strongest predictors of ventricular arrhythmias in ARVC, which vary depending on the population included. Three management guidelines/expert consensus documents have integrated the previously described risk predictors into proposed ICD recommendations. Furthermore, a risk prediction model has allowed the integration of multiple risk factors to provide individualized risk prediction and to inform shared-decision making regarding ICD implantation.

Expert opinion: Over the past few years, knowledge of risk prediction in ARVC has been consolidated and refined. Further improvements may be made by the considering additional predictors such as exercise and by targeting more specific surrogate outcomes for SCD.

KEYWORDS:

• Arrhythmogenic right ventricular cardiomyopathy
• sudden cardiac death
• ventricular tachycardia
• implantable cardioverter defibrillator
• inherited heart disease

Article highlights box

• Sudden Cardiac Death (SCD) prevention is the cornerstone of arrhythmogenic right ventricular cardiomyopathy (ARVC) management; implantable cardioverters defibrillators (ICD) are the only effective therapy

• There is no uniformly accepted algorithm for ICD indications in ARVC patients without a prior history of sustained ventricular arrhythmias

• Many arrhythmic risk factors have been proposed but the literature has largely been hampered by small sample sizes and heterogenous populations

• The strongest arrhythmic risk factors identified in a recent meta-analysis include: young age, male sex, the presence of prior sustained or non-sustained ventricular tachycardia, syncope, the extent of T-wave inversion and right ventricular function

• An arrhythmic risk calculator integrating these predictors in addition to ventricular ectopy burden has recently been developed to provide individualized risk prediction in ARVC and facilitate decision making for ICD implantation

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer Disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.