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Familial dilated cardiomyopathy with conduction defect due to LMNA mutation

ORPHA:300751· ICD-10 I42.0

Definition

A rare familial cardiomyopathy characterized by left ventricular enlargement and/or reduced systolic function preceded or accompanied by significant conduction system disease and/or arrhythmias including bradyarrhythmias, supraventricular or ventricular arrhythmias. Disease onset is usually in early to mid-adulthood. Sudden cardiac death may occur and may be the presenting symptom. In some cases, it is associated with skeletal myopathy.

Prevalence
Unknown
Inheritance
Autosomal dominant
Age of onset
Adult