Congenital Pericardial Agenesis: An Innocent Finding or Clinically Significant Condition? A Case Series and Literature Review

Figures

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  Figure 1. ECG of Clinical Case 1: Sinus rhythm (heart rate 75 bpm) with right axis deviation (+120°). Low QRS voltage in the precordial leads with leftward displacement of the R/S transition zone. Incomplete right bundle branch block pattern. Right ventricular hypertrophy cannot be excluded.

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  Figure 2. (a) TTE of Clinical Case 1: The parasternal long-axis view is suboptimal due to marked cardiac levoposition with interposition of aerated lung tissue, precluding adequate visualization and reliable assessment of cardiac structures and function from this window; Ao—aortic; LA—left atrium; LV—left ventricle; RV—right ventricle. (b) TTE of Clinical Case 1: An apical five-chamber view was the only view obtainable from the apical window and was of suboptimal quality despite maximal left lateral positioning of the transducer. Standard apical four-, two-, and three-chamber views could not be acquired. Ao—aortic; LA—left atrium; LV—left ventricle; RA—right atrium; RV—right ventricle. (c) TTE of Clinical Case 1: The subcostal view provided satisfactory image quality and most closely approximated standard imaging planes; LA—left atrium; LV—left ventricle; RV—right ventricle.

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  Figure 3. Cardiac MRI in the coronal plane with a large field of view, demonstrating abnormal leftward displacement of the cardiac silhouette within the left hemithorax (yellow arrow).

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  Figure 4. Cardiac magnetic resonance imaging (MRI) in the four-chamber cine view, confirming abnormal cardiac position with pronounced levorotation (yellow arrow). The inferior pulmonary vein is compressed between the descending aorta and the left atrium, likely as a consequence of the altered cardiac orientation (white arrow).

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  Figure 5. CT of the chest in the coronal plane using a lung window, demonstrating interposition of lung parenchyma between the left hemidiaphragm and the cardiac base (yellow arrow).

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  Figure 6. Contrast-enhanced CT of the heart in the coronal plane, demonstrating interposition of lung parenchyma between the aorta and the pulmonary artery (yellow arrow). A prominent left atrial appendage is also noted (white arrow).

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  Figure 7. Cardiac MRI in the four-chamber cine view, demonstrating the pericardium as a thin linear structure anterior to the right ventricle, interposed between epicardial and mediastinal fat tissue (yellow arrow). The pericardium is not visualized posterior to the left-sided cardiac chambers (white arrow).

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  Figure 8. Cardiac MRI in the axial plane, demonstrating leftward displacement of the heart with rotation of the cardiac apex (yellow arrow).

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  Figure 9. Non-contrast CT of the chest, demonstrating leftward displacement of the heart with associated rotation (yellow arrow).

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  Figure 10. ECG-gated CT in the axial plane, demonstrating partial visualization of the pericardium anterior to the right-sided cardiac chambers (yellow arrow), with absence of the pericardium along the left side (white arrow).