title: An accordion not to be played.
abstract: 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.
authors: aCardio-Thoracic and Vascular Department, San Raffaele Hospital and Vita-Salute University San Raffaele bIBFM CNR cRadiology Department, San Raffaele Hospital and Vita-Salute University San Raffaele, Milan, Italy.
authors: Riccardo
authors: R
authors: Gorla
authors: Ornella
authors: O
authors: Rimoldi
authors: Francesco
authors: F
authors: De Cobelli
authors: Alessandro
authors: A
authors: Durante
authors: Jacopo
authors: J
authors: Pizzicannella
authors: Paolo G
authors: PG
authors: Camici
mesh_terms: D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis*; D006352:Heart Ventricles / Q000000981:diagnostic imaging*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D014463:Ultrasonography