P169 LEFT–DOMINANT ARRHYTHMOGENIC CARDIOMYOPATHY (LDAC)

Abstract

Abstract Arrhythmogenic cardiomyopathy (ACM) is characterised by myocardial structural alterations with fibro–adipose replacement/infiltration, most frequently affecting the right ventricle. Malignant arrhythmias represent the most dangerous clinical manifestation. We describe the case of a 38–year–old man who came to the Emergency Room of our hospital due to polytrauma. Following the unexpected ECG finding of signs of necrosis in the infero–lateral area and negative T waves in the antero–lateral area, a cardiological examination was performed. The patient, a smoker, had non–contributory family and personal history of cardiovascular diseases and sudden cardiac death. However, he reported several unspecified episodes of “absence seizures”. The transthoracic echocardiogram showed mild reduction in global left ventricular systolic function (EF 45–50%) due to regional changes in motion extending beyond the territory distribution of one single coronary vessel. Holter–ECG showed a Sustained Ventricular Tachycardia (SVT) symptomatic for syncope. A coronary angiography was therefore performed, which excluded any significant coronary stenosis and a cardiac MRI revealed the presence of fibro–adipose replacement of the left ventricle and, to a lesser extent, of the right side of the interventricular septum. Medical therapy with Bisoprolol, Ramipril, Spironolactone was initiated and an ICD was implanted as secondary prevention. No endomyocardial biopsy was carried out due to the high risk of the procedure; genetic testing proved to be negative. A diagnosis of borderline arrhythmogenic cardiomyopathy was therefore made. This case is of special interest as ACM with predominant left ventricular involvement (LDAC) is a rare cardiomyopathy. Diagnosis is made difficult by the presence of phenocopies and by the fact that the diagnostic criteria in use mostly focus on the forms with predominantly right–sided involvement. In the case of suspicious ECG changes, SVT or resuscitated cardiac death with imaging compatible with cardiomyopathy, it is important to raise the diagnostic suspicion of LDAC.