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Differentiation of Uhl anomaly from arrhythmogenic right ventricular cardiomyopathy (ARVC)

Differentiation of Uhl anomaly from arrhythmogenic right ventricular cardiomyopathy (ARVC)
  Uhl anomaly ARVC
Family history No Yes (some patients)
Gender, ratio M:F 1.3:1 2.9:1
Age at presentation Infancy + childhood Adolescence + older
Usual mode of presentation Heart failure Arrhythmia, syncope, or sudden death
Exercise-induced deaths Rare Uncommon
Pathology Areas of complete absence of myocardium of parietal wall of RV: endocardial and epicardial layers are directly opposed Localized patchy replacement of the parietal wall of RV by fibrous and fatty tissue
ARVC: arrhythmogenic right ventricular cardiomyopathy.
Graphic 66378 Version 5.0

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