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Arrhythmogenic RV dysplasia (ARVD) is an inherited disease that causes fatty infiltration and dilation of the right ventricle. The most common clinical manifestations of the disease are arrhythmias and sudden cardiac death (SCD). ARVD accounts for a sizable percentage of SCD in young people, including athletes. However, despite its deadly expression, it can be a very difficult disease to diagnose.
In an NIH-funded international registry, 108 locally diagnosed ARVD patients consented to undergo a systematic and standardized workup including electrocardiogram, signal-averaged ECG (SAECG), Holter monitor, electrophysiology study, echocardiography, magnetic resonance imaging, RV angiography, and RV biopsy. The findings were adjudicated at six co…