Abstract

Abstract Background Arrhythmogenic cardiomyopathy is still considered a rare and difficult–to–diagnose condition, yet a well–founded clinical–instrumental suspicion and proper diagnostic procedure can unveil this condition early from common instrumental findings. Clinical Case In September, a 34–year–old man presented to our center for the presence on the electrocardiogram of ventricular repolarization changes (negative T waves in D1, inferior site and in all precordial leads with mild ST elevation in V1– V2). He denied family history of cardiological events. In 2018, following a suspected preoperative EKG, he performed an echocardiogram and cardiac magnetic resonance imaging (CMR); these examinations showed mild left ventricular hypertrophy with preserved systolic function. Clinically, the patient reported rare episodes of palpitation presented on ambulatory EKG with the same repolarization changes as mentioned above. In consideration of the clinic–instrumental findings, a diagnostic workup with echocardiogram and CMR was started for suspicion of hypertrophic cardiomyopathy in the first hypothesis. On echocardiogram, an non dilated left ventricle was found with left ventricular ejection fraction, parietal thicknesses, and segmental kinetics within normal limits, in the absence of significant valvulopathy; however, frequent ventricular extrasystoles were reported during the examination. Given the EKG–graphic alterations, although there weren‘t any echocardiographic changes, a CMR was performed, showing extensive irregularity of the epicardial profile of the lateral and infero–lateral mid–apical wall, site of adipose infiltration, in which evidence of intramyocardial late gadolinium enhancement (LGE) was also present; the right ventricle showed extensive microbulging of the mid–basal free wall in the absence of adipose replacement or LGE. In view of the clinical–instrumental pattern, the diagnosis of "left dominant" arrhythmogenic cardiomyopathy with initial involvement of the right ventricle was made. Conclusions EKG–graphic interpretation may in some cases be the only instrumental data underlying clinical suspicion of arrhythmogenic cardiomyopathy, allowing early diagnosis of a condition at high risk of sudden cardiac death.

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