Diagnosis
The differential diagnosis for the ventricular tachycardia due to ACM include:

Congenital heart disease
Repaired tetralogy of Fallot
Ebsteins anomaly
Uhls anomaly
Atrial septal defect
Partial anomalous venous return
Acquired heart disease
Tricuspid valve disease
Pulmonary hypertension
Right ventricular infarction
Bundle-branch re-entrant tachycardia
Miscellaneous
Pre-excited AV re-entry tachycardia
Idiopathic RVOT tachycardia
SarcoidosisIn order to make the diagnosis of ACM, a number of clinical tests are employed, including the electrocardiogram (EKG), echocardiography, right ventricular angiography, cardiac MRI, and genetic testing. Electrocardiogram
90% of individuals with ARVD have some EKG abnormality. The most common EKG abnormality seen in ACM is T wave inversion in leads V1 to V3. However, this is a non-specific finding, and may be considered a normal variant in right bundle branch block (RBBB), women, and children under 12 years old. RBBB itself is seen frequently in individuals with ACM. This may be due to delayed activation of the right ventricle, rather than any intrinsic abnormality in the right bundle branch. The epsilon wave is found in about 50% of those with ACM. This is described as a terminal notch in the QRS complex. It is due to slowed intraventricular conduction. The epsilon wave may be seen on a surface EKG; however, it is more commonly seen on signal averaged EKGs. Ventricular ectopy seen on a surface EKG in the setting of ACM is typically of left bundle branch block (LBBB) morphology, with a QRS axis of −90 to +110 degrees.
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.