The diagnostic value of electrocardiogram in the left variants of desmosomal arrhythmogenic cardiomyopathy

Figures

• 

  Figure 1. Depolarization abnormalities in patients with left variant of desmosmal arrhythmogenic cardiomyopathy. (A) The electrocardiogram performed in a 29-year-old male with a pathogenic mutation in desmoplakin gene shows low QRS voltage in limb leads. (B) A 38-year-old female with desmoplakin mutation displays on electrocardiogram pathological Q-waves in inferior leads (leads III–aVF) and the sum of the R-wave in leads I and II ≤ 8 mm and the sum of the S-wave in lead V1 and R-wave in lead V6 ≤ 12 mm. Repolarization abnormalities (T-wave inversion) in anterolateral leads (V3–6) are also present. (C) A case of a 48-year-old man affected by arrhythmogenic left ventricular cardiomyopathy with a pathogenic mutation on DSG2 gene. His electrocardiogram shows pathological Q-waves in inferior leads (boxes) and a prominent R-wave in V1 with an R/S ratio ≥ 0.5. ALVC, arrhythmogenic left ventricular cardiomyopathy; DSG2, desmoglein-2; DSP, desmoplakin; LQRSV, low QRS voltages.

• 

  Figure 2. Conduction disturbances, electrocardiogram changes, and ventricular arrhythmias in patients with left variant of desmosmal arrhythmogenic cardiomyopathy. (A) The electrocardiogram performed in a 36-year-old male with arrhythmogenic left ventricular cardiomyopathy due to a desmoplakin pathogenic variant displays a left anterior fascicular block. (B) A 42-year-old male with desmosmal arrhythmogenic cardiomyopathy (pathogenic desmoplakin variant) shows on electrocardiogram a left posterior fascicular block (AQRS ≈ +110°). His cardiac magnetic resonance (C–D) reveals the presence of subepicardial late gadolinium enhancement involving the left ventricular inferior and inferoseptal wall (arrows). The patient experienced an aborted cardiac arrest few months after the diagnosis. (E–G) A case of a 30-year-old-male with diagnosis of dACM after an out-of hospital aborted cardiac arrest. The 12-lead surface electrocardiogram recorded during recovery showed an extreme axis deviation in the frontal plane (AQRS ≈ >120°). His electrocardiogram at the age of 16 did not show significant electrocardiogram abnormalities (E). Beyond the clinical onset, the scar on cardiac magnetic resonance was…