An accordion not to be played

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We present the case of a 52-year-old man with normal coronary arteries admitted to our department after being resuscitated from ventricular fibrillation. Transthoracic echocardiography raised suspicion of left-ventricular non-compaction. Cardiac magnetic resonance excluded this, but showed several systolic bulgings of the right ventricle and a characteristic focal ‘crinkling’ of the right-ventricular outflow tract known as the ‘accordion’ sign, a specific marker of desmosomal gene mutations, thus suggesting the presence of an arrhythmogenic cardiomyopathy. Extensive mid-myocardial late gadolinium enhancement and fatty infiltration, predominantly of the left ventricle, finally confirmed the diagnosis of left dominant arrhythmogenic cardiomyopathy, a rare variant of arrhythmogenic right-ventricular cardiomyopathy.

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