An accordion not to be played

Riccardo Gorla, Ornella Rimoldi, Francesco De Cobelli, Alessandro Durante, Jacopo Pizzicannella, Paolo G. Camici

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)S81-S83
JournalJournal of Cardiovascular Medicine
Publication statusPublished - Jan 1 2015

ASJC Scopus subject areas

  • Medicine(all)


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