(FIT)-Module 1, Workshop 1: Electrocardiographic/Electrophysiologic Correlation-London Case 5 Answer

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Video Summary

The young woman likely has arrhythmogenic cardiomyopathy (ACM), traditionally known as arrhythmogenic right ventricular cardiomyopathy. This condition often involves both ventricles over time. Her diagnosis is supported by history, transthoracic echo findings, and electrocardiogram results. Most ACM cases are due to desmosomal gene mutations, like a four-base deletion in PKP2. Exercise restriction is advised to prevent arrhythmias and the rapid progression of ACM. Diagnosis is based on criteria including right ventricular issues, histology findings, ECG abnormalities, arrhythmias, familial history, and genetic confirmation. High-level aerobic activity should be avoided, but some exercise remains beneficial.

Keywords

arrhythmogenic cardiomyopathy

desmosomal gene mutations

PKP2 gene deletion

exercise restriction

diagnostic criteria