Fig. 6

The cardiac abnormality of Naxos disease was not clinically manifest until the patient was ≈15 years of age, and onset was usually with palpitations or syncope. Two-dimensional echocardiogram of the heart showing typical features of ARVD: severe right ventricular (RV) dilatation and multiple saccular aneurysmal segments (arrows). There is also right atrial (RA) dilatation. In contrast, the left ventricle (LV) and left atrium (LA) are normal. Accompanying ECG shows abnormal T-wave inversion in leads V1 through V4, consistent with abnormal repolarization affecting the RV. Epsilon waves are also observed. Original materials from an open access article [23]