Arrhythmogenic right ventricular dysplasia differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

The differential diagnosis of ARVC from other diseases can be challenging. It must be differentiated form RV outflow tract (RVOT) tachycardia or ectopy, Brugada syndrome, sarcoidosis, myocarditis, congenital abnormalities, pulmonary hypertension, RV infarct, and DCM, Athlete heart, Uhl's anomaly. At autopsy, one has to differentiate between normal fat deposition in the right ventricle and the presence of scars in the subepicardium due to ischemia

Differential Diagnosis

The differential diagnosis for the ventricular tachycardia due to ARVD include:

• Congenital heart disease
  • Repaired tetralogy of Fallot
  • Ebstein's anomaly
  • Uhl anomaly
  • Atrial septal defect
  • Partial anomalous pulmonary venous return
• Acquired heart disease
  • Tricuspid valve disease
  • Pulmonary hypertension
  • Right ventricular infarction
  • Bundle branch reentrant ventricular tachycardia
• Miscellaneous
  • Pre-excited AV re-entry tachycardia
  • Idiopathic RVOT ventricular tachycardia

Differentiating ARVD/C From RVOT VT

Ventricular tachycardia associated with ARVD/C may be difficult to differentiate from RVOT VT. Electrocardiographic clues that support the diagnosis of ARVD/C include multifocal and/or polymorphic ventricular tachycardia (VT) originating from the right ventricle, T wave inversions in the right precordial leads, slurred upstroke (≥55 ms) of the S wave in V₁ to V₃ leads, and the presence of an epsilon wave.

Differentiating ARVD/C From Dilated Cardiomyopathy

ARVD is distinguished from dilated cardiomyopathy by the greater degree of arrhythmogenicity. In dilated cardiomyopathy, although ventricular arrhythmia commonly occurs, it is rare in the absence of significant ventricular dysfunction. In contrast, ARVD is significantly associated with ventricular arrhythmia even in the absence of ventricular dysfunction. Furthermore, sudden cardiac death is the first clinical manifestation of the disease in more than 50% of probands with ARVD. Additionally, regional ventricular involvement and the presence of ventricular aneurysm, which are hallmarks of ARVD/C, argue against the diagnosis of dilated cardiomyopathy.