Key result
Desmoplakin cardiomyopathy drives arrhythmias, HF, and SCD, typically presenting with ring-like subepicardial LGE on MRI.
Desmoplakin cardiomyopathy is an inherited condition characterized by arrhythmias, heart failure, and sudden cardiac death, with a distinct ring-like subepicardial late gadolinium enhancement on cardiac MRI.
Recognizing ring-like subepicardial LGE may avert misdiagnosis as myocarditis; extends DSP cardiomyopathy phenotype but leaves open specificity.
Desmoplakin (DSP) cardiomyopathy is an inherited condition caused by desmosome gene DSP mutation, resulting in arrhythmias, heart failure, and sudden cardiac death. Cardiac MRI typically presents with ring-like subepicardial late gadolinium enhancement which can be mistaken for myocarditis or fatty replacement.
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Han et al. (2026) studied this question. Desmoplakin cardiomyopathy causes arrhythmias, heart failure, and sudden cardiac death, typically presenting on cardiac MRI with ring-like subepicardial late gadolinium enhancement.
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