Heart failure & cardiomyopathies

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

JACC AdvPublished Review of data of consecutive patients

Authors' conclusion

In ALVC, ECG is a valuable diagnostic tool and may have a relevant prognostic role, since LFPB is a strong and independent predictor of MAE.

Study context

Population
Patients with arrhythmogenic left ventricular cardiomyopathy (ALVC)consecutive patients with ALVC (81.5% desmoplakin pathogenic/likely pathogenic variants)
Participants
125 patients
Comparison
ECG, cardiac magnetic resonance and clinical predictors of major arrhythmic events (MAEs)
Outcome
ECG signs in ALVC
Methodological detailslogistic regression, Cox regression
  • Logistic regression
  • Cox regression

Main finding

As reported in the abstract

Left posterior fascicular block (LPFB)
13.6%
Pathological Q waves
26.4%
R/S ratio in V1 ≥0.5
26.4%
SV1 + RV6 ≤12 mm and RI + RII ≤8 mm
44%
Normal ECG
Fifteen (12%)

Further findings

Independent predictors of MAE (multivariable logistic regression)

OR (95% CI)
LPFB4.7 (1.2–18.3)
Syncope84.95 (14–496)
Transmural late gadolinium enhancement9.95 (2.3–36)
Right ventricular ejection fraction0.92 (0.87–0.97)

Independent predictors of MAE in primary prevention (Cox regression)

HR (95% CI)
LPFBPrimary prevention scenario3.98 (1.3–12.0)
SyncopePrimary prevention scenario19.13 (5.8–63.0)
Transmural late gadolinium enhancementPrimary prevention scenario10.57 (2.9–38.0)

MAE (sudden cardiac death, aborted sudden cardiac death, appropriate ICD shock)

MAE occurred
35 patients (28%)

Predictive capability of model including the 4 variables

Area under the curve
0.9

Original publication

The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy. JACC Adv. Published 12 May 2025. doi:10.1016/j.jacadv.2025.101766

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