What are the current guidelines for the management and follow-up of patients diagnosed with arrhythmogenic cardiomyopathy?

Management and follow-up of patients diagnosed with arrhythmogenic cardiomyopathy (ACM) should involve specialist cardiology input due to the complexity and risk of arrhythmias and sudden cardiac death.

Although the provided UK guidelines primarily focus on atrial fibrillation and heart failure, principles applicable to ACM management include:

• Specialist referral: Patients with ACM require prompt cardiology referral for diagnosis confirmation, risk stratification, and management planning, including consideration of implantable cardioverter-defibrillator (ICD) therapy to prevent sudden cardiac death.
• Arrhythmia management: Antiarrhythmic drug therapy may be considered under specialist guidance, with beta-blockers often used first-line to control arrhythmias. Amiodarone may be used cautiously, especially in patients with ventricular impairment, but long-term use requires specialist oversight due to toxicity risks ¹,³.
• Device therapy: ICD implantation is a cornerstone in preventing sudden death in high-risk ACM patients, guided by specialist assessment.
• Lifestyle advice: Patients should be counselled on avoiding strenuous exercise and other triggers that may exacerbate arrhythmias.
• Regular follow-up: Ongoing specialist follow-up is essential to monitor arrhythmia burden, ventricular function, and device function if implanted. This includes periodic reassessment of risk and treatment efficacy.
• Family screening: Given the genetic nature of ACM, cascade screening of relatives is recommended under specialist genetics and cardiology services.

In primary care, the role includes supporting medication adherence, monitoring for side effects, and facilitating timely specialist review if symptoms worsen or new arrhythmias develop.

Note: Specific UK NICE or CKS guidelines dedicated solely to arrhythmogenic cardiomyopathy were not included in the provided context; however, the management principles align with those for complex arrhythmias and cardiomyopathies requiring specialist care as outlined in the atrial fibrillation and heart failure guidelines ¹,³.