In search of lost time – a case of myocardial perforation in a patient with arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is particularly hard to diagnose and manage. We present the case of a 49-year-old, a former professional football player, with a history of cardiac arrest in 2011 by ventricular tachycardia (VT) (normal coronary arteries). Between 2011 and 2019 the patient didn't present for reevaluation and resumed endurance sports activity. In 2019 he was directed to our clinic for a syncope followed by constrictive anterior chest pain and palpitations, the clinical expression of a VT for which cardioversion was required. Upon admission, the patient was at sinus rhythm with negative T waves in V1-V2. Echocardiography showed significant dilatation and dysfunction of the RV (TAPSE 16 mm, FAC 20%, S' 8.6 cm/s). To confirm the diagnosis of ARVC, cardiac MRI was performed, confirming fat infiltration in the RV free wall with biventricular involvement. Given the high arrhythmic risk, a two-chamber ICD was implanted. In the second postprocedural day, the patient presented important epigastric pain, with ECG signs of sensing and pacing malfunction and ventricular probe displacement on the radioscopy. Emergency surgery was performed, with successful extraction of the electrode. Postoperative progression was favorable under treatment with beta-blocker and amiodarone. In conclusion, this case is a particular one since we’ve documented two distinct phases in the evolution of the ARVC (electrical phase – 2011 and structural phase - 2019). Moreover, by resuming endurance sports activity and in concordance with the literature data, we can only assume that the progression of the disease was accelerated, with a greater arrhythmic risk.