5th July 2026

Management

General Management 

Patients diagnosed with ACM are advised against intense exercise or competitive sports to avoid triggering fatal arrhythmias and decelerate disease progression. Genetic counselling and testing may be offered to patients and their families to identify genetic mutations associated with ARVC and assess the risk of transmission to offspring. Annual ECG monitoring may aid in diagnosis, management and risk stratification. 

Medical Management

• Beta-blockers are the recommended first-line pharmacological therapy in patients with ACM to reduce arrhythmia burden. 
• Antiarrhythmics such as sotalol (non-selective beta-blocker with class III antiarrhythmic properties) and mexiletine (class Ib antiarrhythmic) can be used to manage arrhythmia-related symptoms not controlled by conventional beta-blocker therapy alone. 
• Caution should be exercised when prescribing amiodarone to young patients with ACM due to its long-term adverse effects.
• For patients with ARVC with phenotypic LV dysfunction, HFrEF RCTs have suggested inclusion of ACEi/angiotensin receptor neprilysin inhibitors (ARNIs), beta-blockers, mineralocorticoid receptor antagonists (MRA), and sodium–glucose co-transporter 2 inhibitors (SGLT2i).

Surgical Management

• Catheter ablation can be considered in refractory cases or when appropriate ICD interventions for ventricular arrhythmia is recurrent despite pharmacological therapy.
• Implantable cardiac defibrillator (ICD) is implicated in the secondary prevention of sudden cardiac death in patients with ACM who have survived a cardiac arrest or recovered from a ventricular arrhythmia resulting in haemodynamic instability. High-risk features, including unexplained syncope, ventricular dysfunction and non-sustained VT, should be considered to inform individualised decision-making for primary prevention ICD implantation. 
• Left Ventricular Assist Devices (LVADs) may be considered in advanced cases of ARVC with biventricular dysfunction, advanced heart failure, or life-threatening arrhythmias, while awaiting heart transplantation or as destination therapy.
• Heart Transplantation is considered in end-stage ARVC patients with refractory heart failure, recurrent ventricular arrhythmias, or poor prognosis despite optimal medical and surgical management.