CTICasesLeft Ventricular Arrhythmogenic Cardiomyopathy with Desmoplakin Mutation
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Imaging findings
Cardiac MRI showed that the right ventricle appeared normal. However, the left ventricle demonstrated akinesis, particularly along the lateral wall, with reduced wall thickening and squeeze. Post-contrast images revealed delayed enhancement in the central myocardium and sub-epicardial surface of the left ventricle.
Key takeaways
This case highlights a left ventricular variant of arrhythmogenic cardiomyopathy (formerly ARVD/ARVC) caused by a desmoplakin mutation, identified in an asymptomatic patient with a family history of arrhythmias and sudden cardiac death. Unlike classic ARVC, which primarily affects the right ventricle, this mutation results in left ventricular involvement. Management involves monitoring for arrhythmias and considering defibrillator placement based on risk stratification. This demonstrates the expanding spectrum of genetic cardiomyopathies beyond traditional presentations.
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