Risk stratification for ARVC:
Cardiac arrest due to VF can occur at any time during the disease course. Several risk factors, such as previous cardiac arrest, syncope, participation in competitive sports, young age, VT, severe right ventricular dysfunction, left ventricular involvement, and QRS dispersion of 40 ms or more, have been proposed.
In the North American ARVC/D Registry, the only risk factors for ventricular arrhythmias were spontaneous ventricular arrhythmias before enrolment and a younger age at ICD implantation.
Therapy of ARVC:
Asymptomatic patients or healthy gene carriers do not require prophylactic treatment.
They should undergo cardiac follow-up, including medical history, 12-lead ECG, 24-hour Holter monitoring, exercise testing, and echocardiography, on a regular basis.
Amiodarone has been shown to prevent ventricular arrhythmias more effectively than beta blockers in ARVC/D patients treated with ICD.
Sotalol at high doses (320–480 mg daily) has also been found partially effective, and beta blockers should, theoretically at least, be efficacious.
Treatment with ICD is indicated in patients with cardiac arrest, syncope, or haemodynamically poorly tolerated VT, or one or more risk factors for SCD.
The majority of VT in ARVC/D are monomorphic, and antitachycardia pacing is highly successful in terminating VT independently of its cycle length. Thus, all ICDs should be programmed for antitachycardia pacing.
A potential problem with ICD in ARVC/D is that the disease is often progressive, leading to loss of myocardium and reduced ventricular R wave sensing over time.
Physical activity is restricted to mild cardiovascular sports.