Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC) Differential Diagnoses

Updated: Dec 29, 2020
  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Jose M Dizon, MD  more...
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Diagnostic Considerations

Idiopathic right ventricular tachycardia

Idiopathic right ventricular tachycardia is a benign disorder that may mimic arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) owing to the occurrence of exercise-induced left bundle branch block (LBBB) morphology ventricular tachycardia with inferior axis. The heart is structurally normal, and there is no history of ARVD/ARVC or sudden cardiac death in the family.

Electrocardiography (ECG) in RV tachycardia shows upright T waves in V2 -V5, and epsilon waves are absent. [29, 30]

Uhl anomaly

This is a rare disorder characterized by total lack of RV myocardium and results in a very thin-walled RV (parchment RV). [31] In ARVD, the myocardium is not completely absent and is replaced by a variable degree of fibrosis.

Dilated cardiomyopathy

Biventricular dilatation and congestive heart failure may mimic advanced ARVD with LV involvement. Characteristic ECG and cardiac MRI (CMRI) abnormalities in ARVD help to distinguish the two entities.


Patients with pulmonary sarcoidosis may have RV dilatation. Sarcoidosis is more common in women and Black persons. ECG may show a heart block. Chest x-rays show hilar lymphadenopathy. The LV is more often involved, resulting in LV systolic dysfunction. Late gadolinium enhancement may be seen in the interventricular septum in a nonischemic pattern.


Typically, myocarditis has a more acute presentation, preceded by a prodrome such as fever, chills, myalgia, chest pain. Troponin may be elevated. CMRI reveals edema on T2-weighted images, and late gadolinium enhancement is seen in the subepicardium in a nonischemic distribution.

Brugada syndrome

The ECG in Brugada syndrome reveals right BBB and persistent ST segment elevation in the right precordial leads. However, no abnormalities are found on imaging studies, including echocardiography and late gadolinium enhancement MRI.

Differential Diagnoses