The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy

Figure 1. Electrocardiographic Findings in Patients With Arrhythmogenic Left Ventricular Cardiomyopathy (A) Electrocardiogram of patient #56 (19-year-old man, pathogenic variant in desmoglein-2 c.271G>T, p.Gly91Ter) shows left posterior fascicular block, a fragmented QRS in V1 to V3 and T-wave inversion in V3 to V5. (B) Electrocardiogram of patient #83 (71-year-old man, likely pathogenic variant in desmoglein-2 p.Val295Serfs∗6) displays global low QRS voltage, anterior T-wave inversion, and R/S ratio ≥0.5 in V1 (red box). (C) Electrocardiogram of patient #19 (35-year-old man with pathogenic variant in desmoplakin c.5210delG p.Gly1737AspfsTer16) displays low QRS voltage in limb leads and pathological inferior Q waves (blue boxes). (D) Patient #86 (14-year-old man, pathogenic variant in plakophilin-2, c.1378+1G>C), had an aborted sudden cardiac death at presentation; his electrocardiogram shows left posterior fascicular block and T-wave inversion in V1 to V3. A sum of the R-wave in I to II ≤8 mm and SV1 + RV6 ≤12 mm, a R/S ratio ≥0.5 in V1 are also present. All the electrocardiograms presented were performed at 25 mm/s with 1 mm/mV.

Figure 2. Electrocardiographic Changes During Follow-Up (A and B) Patient #38 is a man with a pathogenic variant in desmoplakin (deletion of the entire desmoplakin gene, 6p25.1-p24.3). Electrocardiogram at age 28 shows T-wave inversion in lateral leads. After 6 years, electrocardiogram shows the appearance of left posterior fascicular block (violet box) and T-wave inversion in leads V2 to V3 (asterisks). (C and D) Electrocardiograms of patient #45 (48-year-old man, likely pathogenic variant in desmoplakin c.860A>G, p.Asn287Ser) display the presence of low QRS voltages in lateral leads and the appearance over time of left posterior fascicular block and pathological Q waves in inferior leads (violet box). (E and F) Electrocardiogram of patient #108 (18-year-old man, pathogenic variant in desmoplakin c.2821C>T, P.Arg941∗) shows low QRS voltages in the lateral leads. After 4 years, the electrocardiogram shows the appearance of T-wave inversion in inferior leads and in V3 to V6 (asterisks), polymorphic premature ventricular beats.

Central Illustration. The Diagnostic and Prognostic Role of the Electrocardiogram in Arrhythmogenic Left Ventricular Cardiomyopathy ALVC = arrhythmogenic left ventricular cardiomyopathy; DSP = desmoplakin; ECG = electrocardiogram; LGE = late gadolinium enhancement; LPFB = left posterior fascicular block; LQRSV = low QRS voltages; MAE = major arrhythmic events; RVEF = right ventricular ejection fraction; TWI = T-wave inversion.