To make a diagnosis of ACM requires either 2 major criteria or 1 major and 2 minor criteria or 4 minor criteria.Major criteria

Right ventricular dysfunction
Severe dilatation and reduction of RV ejection fraction with little or no LV impairment
Localized RV aneurysms
Severe segmental dilatation of the RV
Tissue characterization
Fibrofatty replacement of myocardium on endomyocardial biopsy
Electrocardiographical abnormalities
Epsilon waves in V1 – V3
Localized prolongation (>110 ms) of QRS in V1 – V3
Inverted T waves in V1 -V3 in an individual over 12 years old, in the absence of a right bundle branch block (RBBB)
Ventricular tachycardia with a left bundle branch block (LBBB) morphology, with superior axis
Family history
Familial disease confirmed either clinically or on autopsy or surgeryMinor criteria

Right ventricular dysfunction
Mild global RV dilatation and/or reduced ejection fraction with normal LV. Mild segmental dilatation of the RV
Regional RV hypokinesis
Tissue characterization
Electrocardiographical abnormalities
Late potentials on signal averaged EKG. Ventricular tachycardia with a left bundle branch block (LBBB) morphology, with inferior or unknown axis
Frequent PVCs (> 500 PVCs / 24 hours)

Management
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 arrhythmiaManagement 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 diseases arrhythmogenic potential
Evaluate the hemodynamic consequences of sustained VT
Determine whether the VT can be interrupted via antitachycardia pacing (ATP).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.