What is the natural history of cardiac disease in Danon?

Danon disease causes severe HCM (wall thickness often >30 mm) in males presenting in adolescence; rapid progression to heart failure by age 20-25 in males; females present later (3rd-5th decade) with DCM.

Why is WPW common in Danon disease?

Glycogen accumulation in the atrioventricular node and His-Purkinje system disrupts normal conduction, causing delta waves (WPW pattern) and risk of life-threatening arrhythmia; ICD implantation is recommended.

What retinal disease occurs in Danon?

Pigmentary retinopathy with retinal atrophy in the posterior pole occurs in virtually all males; progressive visual loss; males should have annual ophthalmological assessment from childhood.

When is cardiac transplantation performed in Danon disease?

Cardiac transplant is the only effective intervention for end-stage Danon heart disease; given the young age at presentation, transplant evaluation should begin with first signs of cardiac compromise.

Is Danon disease purely cardiac?

Skeletal muscle weakness (proximal myopathy), intellectual disability in males, and retinopathy are major extracardiac features; females may have milder myopathy and often preserve cognition.

Danon Disease (LAMP2 Deficiency) Cardiac Severity Assessment

Assesses Danon disease severity using hypertrophic cardiomyopathy degree (wall thickness), left ventricular function, arrhythmia burden (WPW), and retinal disease to guide ICD implantation and transplant listing.

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Frequently Asked Questions

What is the natural history of cardiac disease in Danon?

Why is WPW common in Danon disease?

What retinal disease occurs in Danon?

When is cardiac transplantation performed in Danon disease?

Is Danon disease purely cardiac?