Key result
Applying modified pediatric criteria (>30% muscle replacement) successfully identified all 16 genetically confirmed ARVC/D cases, whereas adult criteria (>40%) missed 7 cases.
Why the study?
Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia is challenging due to the absence of a unique presentation and lack of specific reproducible diagnostic criteria.
Does the use of modified pediatric criteria (>30% muscle replacement) improve the diagnostic categorization of ARVC/D in pediatric patients compared to adult criteria?
Observational (n=16)
Does the use of modified pediatric criteria (>30% muscle replacement) improve the diagnostic categorization of ARVC/D in pediatric patients compared to adult criteria?
Absolute Event Rate: 100% vs 56.25%
Using a modified pediatric criterion of >30% muscle replacement improves the detection of ARVC/D in pediatric patients compared to the adult criterion of >40%.
Modified >30% criteria may enhance pediatric ARVC/D detection; leaves open validation before any practice change.
Background: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia is an inherited cardiomyopathy, characterized by replacement of the RV muscle wall with fibrofatty tissue. The diagnosis is challenging, due to the absence of a unique presentation and a lack of specific reproducible diagnostic criteria. Materials and methods: Slides and additional clinical information including follow up from 16 cases were reviewed. Pediatric criteria of >30% of muscle replacement was used, instead of >40% as used in adults. Results: All 16 cases were confirmed by genetic testing and show ARVC/D. Applying the adult criteria, 7 cases would not have been categorized as ARVC/D. Conclusion: The modified pediatric criteria for ARVC/D should be used for pediatric patients. Better detection will aid in genetic counseling in order to identify those additional family members susceptible to sudden cardiac deaths so they can be followed optimally.
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Chang et al. (2021) conducted an observational in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) (n=16). Modified pediatric criteria (>30% muscle replacement) vs. Adult criteria (>40% muscle replacement) was evaluated on Categorization as ARVC/D. Applying modified pediatric criteria (>30% muscle replacement) successfully identified all 16 genetically confirmed ARVC/D cases, whereas adult criteria (>40%) missed 7 cases.
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