부산대학교 도서관

A 68–year–old woman affected by dyslipidemia had a clinical history notable for T wave inversion (TWI) in the lateral and precordial leads from 2008, with a normal echocardiogram (TTE) and a normal coronary angiogram in 2009. No family history of cardiomyopathy or sudden cardiac death. Given the persistence of these alterations (TWI extending from V1–V6 and in lateral leads), in 2019 TTE was repeated, but did not show any significant findings. Consecutive Holter ECG were performed and no significant arrhythmias or ventricular ectopics were recorded. Therefore, a cardiac magnetic resonance (CMR) was performed elsewhere in 2019 and reported the presence of a dyskinetic segment close to right ventricular (RV) apex, epicardial lipomatosis extending from the right ventricle to the left ventricular (LV). No wall motion abnormalities nor late gadolinium enhancement (LGE) area were noted. This MRi findings together with ECG repolarization abnormalities raised the suspiscion of arrhythmogenic right ventricular cardiomyopathy (ARVC). Therefore, the patient was referred to our Center. ECG was repeated and showed TWI in the precordial and lateral leads (Fig.1). TTE showed normal left and right ventricular function/ mass and normal RV dimensions. The patient underwent Coronary angiography that confirmed the absence of CAD. Cardiac MRI was repeated and showed massive epicardial lipomatosis surrounding right and left ventricle (Fig 2a, red arrows). In this T1–weighted DIR image there is hyperintense tissue that surrounds the left and right ventricle, which is more evident when compared with normal myocardium. The adipose nature of this finding was further confirmed with T1–mapping sequences (Fig 2b, yellow arrow). T1 mapping showed low T1 values (300–500 ms) near the right ventricle, compatible with adipose tissue. What seemed to be a dyskinetic apical segment was re–interpreted as “butterfly apex” (Fig 3, red arrow), a little–known anatomic variant that could be mistaken for a RV wall abnormality. Moreover, this segment is not usually involved in isolation in ARVC. Genetic testing for ARVC resulted negative.The final diagnosis was that of epicardial lipomatosis and butterfly apex presenting with T wave inversion in the precordial leads. No further criteria suggesting ARVC was present. We chose this case because it is emblematic of many potential pitfalls that might occur in the diagnostic pathway of ARVC.