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 |
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