Pericardial absence—is symptomatic partial defect an enigmatic cardiovascular risk?

A fit 27-year-old man presented with paroxysmal chest pain. These symptoms had commenced several months previously and had resulted in an elective echocardiogram and coronary angiogram performed which were both normal. During a separate admission, he was also treated for presumed pericarditis with a course of oral steroids. No significant family histories were noted. He was on no medications. On examination, he was haemodynamically stable and cardiovascular examination was normal. Routine bloods including C-reactive protein, troponin and D-dimers were within normal limits. Electrocardiogram (ECG) was normal. Chest X-ray demonstrated a left-ward rotation of the heart and mediastinal shift (Figure 1A). Cardiac magnetic resonance imaging (CMR) demonstrated pericardium over lateral aspect of the heart and the pericardial defect as well as evidence of lung tissue interposed between the aorta and pulmonary artery (Figure 1B). Global left ventricular function was normal with no evidence of infarction or fibrosis. Symptomatic partial pericardial absence (PAb) without cardiac organ damage (CoD) was diagnosed, and it was decided to proceed conservatively with simple analgesia and ongoing surveillance for increased symptom frequency and/or evidence of cardiac dysfunction.