A Case of Congenital Absence of the Left-Sided Pericardium
A 40-year-old woman with no known past medical history presented to her primary care provider with 3 months of intermittent atypical chest pain, exertional dyspnea, and a dry cough.
The patient had no history of fever, weight loss, or recent contacts with tuberculosis or other infectious diseases. Her physical exam was unremarkable and she was hemodynamically stable. A differential diagnosis of atypical infection was made at that time. The patient was discharged on analgesia for the chest pain and referred to a pulmonologist for further investigation. The patient's electrocardiogram (ECG), complete blood count, and pulmonary function tests were unremarkable. Her chest X-ray showed left-sided and posterior deviation of the heart associated with elongation of the left border (Snoopy sign). The right heart border was obscured due to thoracic spine superimposition.
A computerized tomography (CT) scan was ordered to further characterize these findings. The CT scan showed complete absence of pericardium on the left side. A small pericardial layer on the right anterolateral portion of the heart over the right atrioventricular (AV) groove was visible. There was displacement of the heart to the left side (levoposition and levorotation). A tongue/pine-like extension of lung parenchyma was seen between the pulmonary artery and aorta. There was interposition of the lung tissue between the left heart base and the diaphragm as a band-like lucent band. A diagnosis of congenital absence of the left pericardium was made and the patient required no further management.
Five years after the CT scan, cardiac magnetic resonance imaging (CMRI) was performed, showing stable absence of the left pericardium. No significant changes from the CT scan were observed in the CMRI.
We present a case of congenital absence of the left pericardium, a rare cardiac manifestation. Imaging modalities played a pivotal role in its diagnosis and exclusion of other diseases and complications.
What is the most likely complication of an isolated absence of the left pericardium?