291 BIVENTRICULAR ARRHYTMOGENIC CARDIOMYOPATHY: COULD ELECTROCARDIOGRAPHY SEE BEYOND THE CARDIAC MRI?


 We described the case of a 34 year-old young athlete who was referred to our Department because of palpitations and fatigue, arising after hard effort but also present at rest. Family history was unremarkable for sudden cardiac death or cardiomyopathies. Electrocardiography (ECG) evidenced multifocal premature ventricular contractions (PVCs): left bundle branch block (LBBB) morphology and superior/inferior axis; right bundle branch block (RBBB) morphology and undetermined axis. Cardiac MRI showed extensive mid wall and subepicardial late gadolinium enhancement in the postero-lateral left ventricular wall. We decided to perform electroanatomic mapping (EAM) because we didn't explain the origin of the PVCs with LBBB configuration. During EAM we found an area extending from the right ventricular outflow tract to the subtricuspidal lateral inferior wall which it had not described by cardiac MRI. In the low voltage areas, the electrograms inscribed by the catheter's electrodes was fractionated with different waveforms, a finding that corresponds to a deficiency of healthy myocardial tissue. We performed an endomyocardial biopsy in the interventricular septum close to the ventricular out flow tract. The histological examination of the biopsy samples showed fibro-fatty replacement. Therefore genetic testing revealed a pathogenic desmoplakin mutation. According to “Padua Criteria”, we made a diagnosis of biventricular arrhythmogenic cardiomyopathy. We finally implanted a transvenous cardioverter defibrillator.
 
 
 Diagnosis of arrhythmogenic cardiomyopathy (ACM) is complex and requires the combination of multiple clinical, histological and genetic parameters. This clinical case demonstrated that ECG represent an essential tool in diagnostic work-up of the disease, and electroanatomic mapping is useful in selected cases for the documentation of the diagnosis of arrhytmogenic cardiomyopathy.
