The goal of management of ACM is to decrease the incidence of sudden cardiac death. This raises a clinical dilemma: How to prophylactically treat the asymptomatic patient who was diagnosed during family screening. A certain subgroup of individuals with ACM are considered at high risk for sudden cardiac death. Associated characteristics include: * Young age * Competitive sports activity * Malignant familial history * Extensive RV disease with decreased right ventricular ejection fraction. * Left ventricular involvement * Syncope * Episode of ventricular arrhythmia Management options include pharmacological, surgical, catheter ablation, and placement of an implantable cardioverter-defibrillator. Prior to the decision of the treatment option, programmed electrical stimulation in the electrophysiology laboratory may be performed for additional prognostic information. Goals of programmed stimulation include: * Assessment of the disease's<unk>  potential * Evaluate the hemodynamic consequences of sustained VT * Determine whether the VT can be interrupted via antitachycardia pacing. Regardless of the management option chosen, the individual is typically advised to undergo lifestyle modification, including avoidance of strenuous exercise, cardiac stimulants (i.e.: caffeine, nicotine, pseudoephedrine) and alcohol. Regarding physical activity and exercise, ARVC patients, as well as gene carriers of pathogenic ARVC-associated desmosomal mutations, should not participate in competitive sports. These patients should be advised to limit their exercise programmes to leisure-time activities and remain under clinical surveillance. Medications Pharmacologic management of ACM involves arrhythmia suppression and prevention of thrombus formation. Sotalol, a beta blocker and a class III antiarrhythmic agent, is the most effective antiarrhythmic agent in ACM. Other antiarrhythmic agents used include amiodarone and conventional beta blockers (i.e.: metoprolol). If antiarrhythmic agents are used, their efficacy should be guided by series ambulatory holter monitoring, to show a reduction in arrhythmic events. While angiotensin converting enzyme inhibitors (ACE Inhibitors) are well known for slowing progression in other cardiomyopathies, they have not been proven to be helpful in ACM. Individuals with decreased RV ejection fraction with dyskinetic portions of the right ventricle may benefit from long term anticoagulation with warfarin to prevent thrombus formation and subsequent pulmonary embolism. Catheter ablation Catheter ablation may be used to treat intractable ventricular tachycardia. It has a 60–90% success rate. Unfortunately, due to the progressive nature of the disease, recurrence is common (60% recurrence rate), with the creation of new<unk>  foci. Indications for catheter ablation include drug-refractory VT and frequent recurrence of VT after ICD placement, causing frequent discharges of the ICD. Implantable cardioverter-defibrillator An ICD is the most effective prevention against sudden cardiac death. Due to the prohibitive cost of ICDs, they are not routinely placed in all individuals with ACM. Indications for ICD placement in the setting of ACM include: * Cardiac arrest due to VT or VF * Symptomatic VT that is not inducible during programmed stimulation * Failed programmed stimulation-guided drug therapy * Severe RV involvement with poor tolerance of VT * Sudden death of immediate family member Since ICDs are typically placed via a transvenous approach into the right ventricle, there are complications associated with ICD placement and follow-up. Due to the extreme thinning of the RV free wall, it is possible to perforate the RV during implantation, potentially causing pericardial tamponade. Because of this, every attempt is made at placing the defibrillator lead on the ventricular septum. After a successful implantation, the progressive nature of the disease may lead to fibro-fatty replacement of the myocardium at the site of lead placement. This may lead to undersensing of the individual's electrical activity (potentially causing inability to sense VT or VF), and inability to pace the ventricle. Heart transplant Heart transplant may be performed in ACM. It may be indicated if the arrhythmias associated with the disease are uncontrollable or if there is severe bi-ventricular heart failure that is not manageable with pharmacological therapy. Family screening All first degree family members of the affected individual should be screened for ACM. This is used to establish the pattern of inheritance. Screening should begin during the teenage years unless otherwise indicated. Screening tests include: * Echocardiogram * EKG * Signal averaged EKG * Holter monitoring * Cardiac MRI * Exercise stress test
Arrhythmogenic cardiomyopathy
Brugada syndrome
Ischemic cardiomyopathy
Long QT syndrome
Noncompaction cardiomyopathy
Alcoholic cardiomyopathy
Aicardi syndrome
Amyloid cardiomyopathy