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Congenital Absence of the Left Pericardium

Imaging findings

Chest radiograph shows displacement and rotation of the heart into the left hemithorax with a prominent, elongated left heart border. Chest CT confirms complete absence of the left hemicardium. The heart is shifted posteriorly and to the left, and lung tissue is seen herniating into the spaces between the aorta and pulmonary artery, as well as into the transverse and oblique sinuses.

Key takeaways

Congenital absence of the pericardium is a rare anomaly that typically affects the left side. It results from premature regression of the left duct of Cuvier during embryogenesis. Most patients are asymptomatic throughout life, but the characteristic CT features include leftward heart displacement and lung herniation into the transverse/oblique sinuses. Partial absence is more dangerous as it carries a risk of cardiac herniation and strangulation.

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