2022 HRS Expert Consensus Statement on Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

April 29, 2022—The Heart Rhythm Society (HRS) developed the 2022 HRS Expert Consensus Statement on Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders to provide comprehensive guidance for clinicians caring for patients with neuromuscular disorders (NMDs), who are at increased risk for cardiac complications including arrhythmias, heart failure, and sudden cardiac death.

Because NMDs—such as muscular dystrophies and mitochondrial myopathies—are rare and complex, patient care often requires coordination across multiple specialties. This document emphasizes a multidisciplinary approach, bringing together expertise from electrophysiology, cardiology (adult and pediatric), heart failure, neurology, rehabilitation medicine, and anesthesiology.

The consensus statement offers practical, actionable recommendations across key areas of care, including:

• Cardiac evaluation and risk stratification
• Use of cardiovascular implantable electronic devices (CIEDs)
• Management of arrhythmias and conduction disease
• Considerations for anticoagulation and other therapies

Dedicated sections address specific neuromuscular conditions, including Duchenne and Becker muscular dystrophy, myotonic dystrophy types 1 and 2, Emery-Dreifuss muscular dystrophy, facioscapulohumeral muscular dystrophy, and mitochondrial myopathies such as Friedreich ataxia and Kearns-Sayre syndrome.

Throughout the document, there is a strong emphasis on shared decision-making, recognizing the importance of aligning treatment strategies with patient values, family involvement, and the complexities of these progressive conditions.