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Danon Disease Myocardial Fibrosis

Imaging findings

Cardiac MRI of a young male shows severe left ventricular hypertrophy and extensive mid-myocardial late gadolinium enhancement (LGE) in the inferolateral and anterolateral segments. Pathological testing confirmed Danon disease.

Key takeaways

Danon disease is a rare X-linked dominant lysosomal storage disorder affecting skeletal and cardiac muscle. It classically presents with severe hypertrophic cardiomyopathy, Wolff-Parkinson-White syndrome, and extensive myocardial fibrosis.

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