A 19-year-old Chinese female student was referred to our institution for abnormal T-wave inversions on the precordial leads of a screening electrocardiogram (ECG) (Figure 1), as well as a chest X-ray (CXR) showing cardiomegaly (Figure 2). These investigations were performed as part of routine preenrollment health screening for a local university course. She had no significant past medical history of note, nor any family history of sudden cardiac death or inheritable cardiac diseases. She had no cardiac symptoms and was physically active.
Physical examination was unremarkable. A repeat ECG showed symmetrical T-wave inversion in leads V1-V4. No epsilon waves were observed. An exercise stress echocardiogram was ordered, which returned negative for inducible myocardial ischemia at 95% maximum predicted heart rate after an exercise duration of 12 minutes (achieving 13.3 METs). However, a baseline echocardiography showed a visually and quantitatively impaired right ventricular function with tricuspid annular plane systolic excursion (TAPSE) of 1.4 cm and tricuspid annulus tissue Doppler S wave of 0.08 m/s. A focal segment of the distal right ventricular free wall appeared to be dyskinetic and outbulging, suggestive of right ventricular aneurysm. Left ventricular ejection fraction was normal at 69% with normal valves and atrial dimensions (Figures 3-6).
In view of the findings suggestive of possible arrhythmogenic right ventricular cardiomyopathy (ARVC), a cardiac magnetic resonance imaging (MRI) was performed. Cardiac MRI without contrast showed right-sided pericardium but no pericardium present over the left heart, with characteristic displacement of the heart into the left hemithorax (Figures 7-9). There was normal right ventricular volumes and function (RVEDVi 86 ml/m2, RVESVi 35 ml/m2, and RVEF 60%) with no regional wall abnormality, specifically no dyskinetic or aneurysmal segment of the right ventricle. There were no structural abnormalities or entrapment of any epicardial coronary vessel at the edges of the defect. The patient was informed of the diagnosis of congenital partial absence of pericardium and discharged from follow-up.