UCSF

Arrhythmogenic right ventricular cardiomyopathy is an inherited disorder characterised by progressive replacement of ventricular myocardium by fibrofatty tissue that predisposes patients to ventricular arrhythmias, heart failure and sudden death. Treatment focuses on slowing disease progression, decreasing the burden of arrhythmias and preventing sudden cardiac death through placement of implantable cardioverter-defibrillators (ICDs), catheter ablation and the use of antiarrhythmic medication. Although only ICDs have been demonstrated to affect patient mortality, antiarrhythmic medications are important adjuncts in reducing patient morbidity and inappropriate ICD therapy. Of the individual antiarrhythmic agents available, sotalol, beta-blockers and amiodarone appear to be most effective in arrhythmia suppression. Calcium-channel blockers may be effective in selected patients. For patients who are refractory to single agent therapy, combination therapy may be considered with the most effective combinations being sotalol + flecainide and amiodarone + beta-blockers.