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Incremental value of the signal-averaged ECG for diagnosing arrhythmogenic cardiomyopathy
Charles Michael Pearman1, David Lee2, Brianna Davies3
1Center for Cardiovascular Innovation, Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia; Unit of Cardiac Physiology, Division of Cardiovascular Sciences, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.
Insights
The signal-averaged electrocardiogram (SAECG) is a surrogate for imaging abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Caution is advised when interpreting SAECG results without other ARVC evidence.
Area of Science:
- Cardiology
- Medical Diagnostics
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis relies on clinical, imaging, electrocardiographic, and genetic factors.
- The signal-averaged electrocardiogram (SAECG) is a minor criterion in ARVC diagnosis, but its clinical utility is debated.
Purpose of the Study:
- To evaluate the diagnostic utility of SAECG in ARVC.
- To assess SAECG's association with ARVC severity and other phenotypic features.
Main Methods:
- Utilized the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry, including probands and relatives.
- Compared SAECG parameters between ARVC patients with varying disease severity and healthy controls.
Main Results:
- SAECG abnormalities were present in 40% of controls and increased with ARVC severity (51% mild, 74% severe).
- SAECG strongly correlated with imaging abnormalities but not other phenotypic aspects.
- Patients with pathogenic variants and minimal ARVC phenotype showed SAECG results similar to healthy controls.
Conclusions:
- SAECG may serve as a surrogate marker for imaging-detected structural abnormalities in ARVC.
- Interpretation of SAECG findings requires caution, especially in individuals lacking other ARVC evidence.
Background:
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is currently diagnosed using a combination of clinical features, imaging, electrocardiography, and genetic investigations. An abnormal signal-averaged electrocardiogram (SAECG) is defined as a minor diagnostic criterion by the 2010 Task Force Criteria, but doubts remain about the value of this investigation.
Objective:
We evaluated the utility of the SAECG in diagnosing ARVC using the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry, a population representative registry of probands with ARVC and relatives, less influenced by referral bias.
Methods:
Probands with ARVC and family members from the Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry underwent phenotype review. SAECG parameters were compared individually and in combination between those with varying degrees of ARVC severity and healthy controls (family members of probands with ARVC and unexplained sudden death, free of evidence of cardiac disease).
Results:
A total of 196 patients with ARVC and 205 controls were included (mean age 44 ± 15 years; 186 of 401 men [46%]). SAECG abnormalities were seen in 83 of 205 controls (40%), 33 of 68 patients with ARVC and mild disease (51%), and 31 of 42 with severe disease (74%). The SAECG associated strongly with imaging abnormalities (major: odds ratio 3.0, 95% confidence interval 1.3-6.9; minor: odds ratio 3.5, 95% confidence interval 0.7-16.5) but not with other aspects of phenotype. Patients carrying pathogenic variants but with minimal phenotype had similar SAECGs to healthy controls (filtered QRS duration 111.2 ± 11.2 ms vs 111 ± 7.6 ms, P = .93; duration of low amplitude signals < 40 μV 32.3 ± 8.9 ms vs 34.2 ± 7.2 ms, P = .32; root mean square of the terminal 40 ms of the filtered QRS complex 43.1 ± 25.2 ms vs 38.2 ± 20.2 ms, P = .38).
Conclusion:
The SAECG appears to be a surrogate marker for structural abnormalities seen on imaging in those with ARVC. Great caution is required in interpreting SAECG findings in those without other corroborating evidence of an ARVC phenotype.
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