Clinical Case 1

A young 21-year-old athlete with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) was admitted to our institution for ventricular tachycardia (VT) recurrence. He was implanted with a subcutaneous implantable cardioverter-defibrillator (S-ICD) three weeks earlier because of an episode of syncope with fast palpitations during exercise and documented spontaneous VT with left bundle branch block morphology and inferior axis during ECG monitoring (figure). He had family history of ARVC/D with two affected uncles. Genetic testing was negative in the family for desmosomal gene mutations. Cardiac MRI showed only mildly dilated right and left ventricles compatible with history of competitive sport (cycling). Shortly after S-ICD implantation, the patient experienced appropriate shocks because of monomorphic VT recurrence despite beta-blocker therapy (nadolol 80 mg) and flecainide (200 mg).

Clinical Case 2

A 36-year-old patient with ARVC/D diagnosed in 2008 was admitted in 2018 for electrical storm in the setting of amiodarone-induced hyperthyroidism. He had been implanted with transvenous ICD in 2012 because of advanced disease with biventricular dysfunction. He experienced several ATP therapies (about 50) for well-tolerated monomorphic sustained VT (figure). He displayed severe biventricular dysfunction with LVEF at 35% and RVEF at 18%. He was first managed medically with bisoprolol titrated to 10 mg/day and, after endocrinologist approval, reintroduction of amiodarone. As VT reoccurred despite pharmacological therapy, plasmapheresis was decided to lower circulating thyroid hormones level. Despite normalized T3 level, the patient experienced several episodes of VT.