A 26-year-old man presents with sustained monomorphic ventricular tachycardia of left bundle branch block morphology with inferior axis. ECG in sinus rhythm shows an epsilon wave in V1 to V3 and T wave inversions beyond V1. MRI shows right ventricular dilation with regional akinesis. Family history includes a brother who died suddenly at 24 years. Which pathological finding underlies this disease?

• A Granulomatous inflammation with non-caseating giant cells
• B Amyloid deposition in the interstitium of both ventricles
• C Hypertrophied disorganized myocytes with myofibrillar whorls
• D Fibrofatty replacement of right ventricular myocardium ✓

Explanation

Arrhythmogenic right ventricular cardiomyopathy is caused by desmosomal gene mutations leading to fibrofatty replacement of RV myocardium, producing VT of LBBB morphology, epsilon waves, and sudden death in young people. Non-caseating granulomas suggest sarcoidosis, which also causes VT and heart block but is distinguished by extracardiac disease and patchy FDG uptake. Disorganized hypertrophied myocytes define HCM, and amyloid gives a restrictive picture with thick walls.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.