The Heart Rhythm Society Releases Expert Consensus Statement on Management of Arrhythmias in Patients with Neuromuscular Disorders

SAN FRANCISCO, CA, April 29, 2022 – Today, the Heart Rhythm Society (HRS) announced a consensus statement on the diagnosis and management of arrhythmias to help improve the care of patients with neuromuscular disorders (NMD). The statement is a result of international collaboration between recognized experts and organizations across the cardiac electrophysiology, adult cardiology, pediatric cardiology, heart failure, neurology, physical medicine and rehabilitation, and anesthesiology fields. The statement was released today as part of Heart Rhythm 2022.

Neuromuscular disorders, like muscular dystrophy and mitochondrial myopathies, impact the function of muscles due to problems with the nerves and muscles in the body. Patients with NMDs are at high risk of life-limiting comorbidities like cardiac arrhythmias, heart failure, or sudden cardiac death.

Because these are rare disorders, treatment for NMDs requires a multidisciplinary approach to care. There is an unmet need to establish clinical recommendations that can be utilized by the collective spectrum of practitioners and specialists involved in patient care. The consensus statement was created with the goal of providing a guide for electrophysiologists, cardiologists, and other clinicians and health care professionals who care for patients with arrhythmic complications of NMDs.

"When it comes to treating rare, neuromuscular disorders, it truly takes a village of experts. As clinicians, it is our imperative to provide proactive treatment and referrals for patients at high risk of cardiac events," said Deepak Bhakta, MD, MBA, FHRS, FACC, FAHA, FACP, CCDS, Indiana University Health and Vice-Chair of the consensus statement. "Because the path forward is not always clear, our hope is that these guidelines will serve as a practical resource to help reduce the burden of arrhythmia complications on this critical patient population and improve their overall care."

The expert consensus statement was led by HRS in collaboration with 13 other leading international medical organizations and provides actionable tools for clinicians around cardiac testing, cardiovascular implantable electronic devices, oral anticoagulation, and other treatment considerations with dedicated sections on the following disorders:

• Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2
• Myotonic dystrophy type 1 and type 2
• Emery-Dreifuss muscular dystrophy and limb girdle muscular dystrophy type 1B
• Facioscapulohumeral muscular dystrophy
• Mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome

The writing committee, comprised of internationally recognized experts from 12 countries, places a strong importance throughout the recommendations on shared decision-making among patients, their families, and clinicians.

The consensus paper will be published in Heart Rhythm Journal and can be found at www.heartrhythmjournal.com.

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