Extract

A 58-year-old man was referred to a regional clinic for moderate dyspnoea on exertion. A diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) was made based on two major Revised Task Force criteria: T-wave inversion in the right precordium (Panel A, upper part) and right ventricular (RV) dilatation with inferior akinesia on magnetic resonance imaging (MRI). A dual-chamber cardioverter-defibrillator (ICD) was implanted for primary prevention. The patient was transferred to our institution for further investigation. Posterior electrocardiogram (ECG) leads facing the left ventricle revealed the normal precordial pattern with positive QRS complexes (Panel A, lower part). Chest X-ray showed abnormal retrosternal presence of lung parenchyma and marked posterior heart displacement (Panel B). Cardiac MRI cine showed RV dilatation with inferior akinesia, contrasting with anterior wall hyperkinesia, and turbulent right pulmonary vein to left atrium inflow (Supplementary data online, Video S1). Right atrium and tricuspid annulus were squeezed from above by a horizontal ascending aorta, but the left atrium and mitral annulus were relatively respected (Panel C, Supplementary data online, Videos S2 and S3). The two atrioventricular annuli were unusually positioned orthogonal to one another (Supplementary data online, Video S4). Cardiac computed tomography showed leftward and posterior tilt of the heart around the vertebral body and the descending aorta with right pulmonary vessel elongation and excessive angulation (Panel D). Inferior and apical segments of both ventricles were in contact with the posterior chest wall and diaphragm. Complete absence of any pericardial component (zoom, white arrow), including the transverse sinus (Panel E, red star), was noted. RV angiography quantified the anterior wall hyperkinesia (Panel F). The ICD was extracted, and the patient was stable after 3 years of follow-up.

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