IJCS | Volume 32, Nº4, July/August 2019

430 Figure 3 - Top: TTE with dilated right chambers (A: parasternal view; B: right chambers parasternal view; C: short axis view showing RV outflow tract), hypertrabecular RV (D: four-chamber view) and biventricular systolic dysfunction. Bottom: CMR revealed LV end-diastolic volume in the upper limit of normality with mild systolic dysfunction, severe RV dilatation, dyskinesia in outflow tract and severe RV systolic dysfunction (E). Late gadolinium enhancement was visible in the RV outflow tract (transmural) and in the LV inferolateral wall (subepicardial) (F and G). TTE: transthoracic echocardiography; CMR: cardiac magnetic resonance; RV: right ventricle; LV: left ventricle. Braga et al. Sudden cardiac arrest in athletes Int J Cardiovasc Sci. 2019;32(4):428-432 Case Report end-diastolic volume in the upper limit of normality (104 ml/m 2 ) and mild global LV systolic dysfunction (LV ejection fraction 41%); RV was severely dilated (148 ml/m 2 ) with dyskinesia in the outflow tract and severe systolic dysfunction (RV ejection fraction 28%). Late gadolinium enhancement was found in the RV outflow tract (transmural) and in the LV inferolateral wall (subepicardial). These findings suggested diagnosis of ARVC with biventricular involvement (Figure 3E- G). Genetic test revealed desmoglein 2 gene variant of uncertain significance (c.874C > T). Due to frequent PVC and runs of nonsustained ventricular tachycardia (NSVT) with inferior axis and left block morphology non-responsive to beta- blockers, sotalol was started with success. Implantable cardioverter defibrillator (ICD) was implanted for secondary prevention. The patient was discharged home on sotalol 160 mg twice a day and lisinopril 5 mg daily. He was advised not to engage in competitive and/or endurance sports. Discussion ARVC is aheritableheartmuscledisorder characterized by VA, heart failure (HF) and SCD. 4 ARVC is usually diagnosed in adolescence-young adulthood and is more frequent in male patients. Known disease-causing genes mostly encode desmosomal proteins, although the true prevalence of these genes has yet to be determined. Therefore, a negative genetic test does not rule out the ARVC diagnosis. 5 The histopathological hallmark of ARVC is the replacement of myocardium by fibrofatty tissue, predominantly in the RV wall. It occurs mostly in the RV inflowtract, outflowtract andapex (“triangleof dysplasia”), from the epicardium to the endocardium. These changes lead to wall thinning and aneurysm formation. More infrequently, though not rare, there is LV involvement, as in our case. 6 Physical exercise may aggravate mechanical uncoupling of myocytes; therefore, exertion is a trigger of disease onset and progression, as well as VA. 4