Arrhythmogenic Right Ventricular Cardiomyopathy Diagnostic Criteria Explained

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is currently diagnosed using the widely accepted Task Force Criteria. These criteria are designed to evaluate the presence of the disease by analyzing six key areas of clinical manifestation and characteristics. In total, there are nine diagnostic indicators—five major and four minor. A patient can be diagnosed with ARVC if they meet two major criteria, one major and two minor criteria, or four different minor criteria.

The Six Diagnostic Categories

These six categories cover various aspects of the disease and help doctors determine whether structural, electrical, or familial signs point to ARVC:

1. Regional or Global Structural and Functional Abnormalities of the Right Ventricle

Major criteria in this category involve significant dilation of the right ventricle (RV), reduced right ventricular ejection fraction, or the presence of regional dilation or aneurysms. These findings are typically assessed using imaging techniques such as echocardiography, cardiac MRI, or angiography.

2. Histological Evidence of Myocardial Tissue Replacement

One of the hallmark signs of ARVC is the replacement of normal myocardial tissue with fibrofatty tissue. This change can be confirmed through endomyocardial biopsy or post-mortem examination.

3. Repolarization Abnormalities

Electrocardiographic (ECG) changes such as inverted T waves in leads V2 and V3 are considered major indicators of repolarization issues. These findings suggest underlying structural heart changes associated with ARVC.

4. Depolarization and Conduction Delays

Minor criteria include widened QRS complexes in leads V1 to V3 and the presence of Epsilon waves—small deflections that occur between the QRS complex and the T wave on an ECG. These findings suggest abnormal electrical activity in the right ventricle.

5. Arrhythmias

Recurring episodes of left bundle branch block (LBBB) morphology ventricular tachycardia or frequent premature ventricular contractions (PVCs) are considered significant arrhythmic manifestations of ARVC.

6. Family History and Genetic Factors

A confirmed family history of ARVC, based on either clinical diagnosis or post-mortem findings in a first-degree relative, is a major diagnostic factor. Minor criteria include unexplained sudden death in a family member under 35 years of age or the identification of ARVC in a family member during the evaluation of another individual.

Comprehensive Diagnosis is Essential

It's important to emphasize that ARVC cannot be diagnosed based on a single abnormality or test result. The diagnostic process must be comprehensive, incorporating multiple criteria across the six defined categories. This multidisciplinary approach ensures accuracy and reduces the risk of misdiagnosis. Integrating clinical findings, imaging, ECG changes, and genetic or familial history allows for a more reliable assessment of the disease.