Arrhythmogenic right ventricular cardiomyopathy with sinus node dysfunction

Introduction: The 2023 European Society of Cardiology Guidelines for the management of Cardiomyopathies recommends a phenotype-based, multimodal, multidisciplinary, and multiparametric approach to the diagnosis of cardiomyopathies. Case report: We present the case of a 70-year-old man, who underwent a routine preoperative examination before dermatological surgery. Initially, on the electrocardiogram (ECG), non-sustained ventricular tachycardia (nsVT) was seen, whereas a dilated right ventricle was observed on transthoracic echocardiography (TTE). After further cardiological examination, ECG criteria for right ventricular arrhythmogenic cardiomyopathy (ARVC) were fulfilled (negative T waves in V1-V3, presence of an epsilon wave), in addition to significant bradycardia with sinus node dysfunction, and recurrent nsVTs were detected during monitoring. Cardiac magnetic resonance (CMR) scan was performed to assess right ventricular function and morphology, which confirmed a dilated right ventricle (end-diastolic volume normalized to body surface area 155 mL/m2), however, no wall motion abnormality was detected. Transesophageal echocardiography ruled out atrial septal defects as a potential cause for right ventricular dilatation. Overall, a diagnosis of ARVC was established based on the 3 major criteria fulfilled by the 2010 ARVC Task Force Criteria. Subsequently, in view of the sinus node dysfunction and ARVC diagnosis with high risk features, we opted for primary preventive DDD-ICD implantation, followed by long-term drug therapy with beta-blocker therapy. Conclusion: ARVC is a rare structural myocardial disease, which is often challenging to diagnose, and the search for clinical clues and "red flags" (functional and morphological abnormalities, electrophysiological features) is of strategic importance.