Management of Inherited Arrhythmia Syndromes: A HiRO Consensus Handbook on Process of Care

Area of Science:

• Cardiology
• Genetics
• Public Health

Background:

• Inherited arrhythmia syndromes are rare genetic disorders predisposing individuals to sudden cardiac arrest and death.
• The Hearts in Rhythm Organization is a Canadian network dedicated to improving care for inherited cardiac conditions.
• The field is rapidly advancing with new research discoveries.

Purpose of the Study:

• To provide a practical, streamlined guide for diagnosing and managing pediatric and adult patients with inherited cardiac conditions.
• To improve health system utilization, clinical management, and research in this area.
• To offer consensus care pathways for common inherited arrhythmia syndromes.

Main Methods:

• Review of current research and clinical practices.
• Development of consensus care pathways for 7 specific inherited cardiac conditions.
• Inclusion of scenarios to guide investigation, diagnosis, risk stratification, and management.
• Provision of an approach for sudden cardiac arrest, sudden unexpected death, and family member management.

Main Results:

• Consensus care pathways are provided for 4 common inherited cardiac conditions predisposing to arrhythmia: Brugada syndrome, long QT syndrome, arrhythmogenic right ventricular cardiomyopathy, and catecholaminergic polymorphic ventricular tachycardia.
• A framework is offered for investigating and managing sudden cardiac arrest, sudden unexpected death, and first-degree relatives.
• Referral to specialized cardiogenetic clinics is recommended in most cases.

Conclusions:

• This review provides a practical framework for standardized, high-quality, multidisciplinary care for inherited cardiac conditions.
• The guide is intended for clinicians, researchers, patients, families, and allied health professionals.
• Implementation of these pathways can enhance care and research for individuals affected by inherited arrhythmia syndromes.