Learning Radiology xray montage

Congenital Defect of The Pericardium

General Considerations

  • Rare absence of a part or all of the pericardium
  • Due to failure of pericardial development secondary to premature atrophy of the left duct of Cuvier (cardinal vein) which then fails to nourish the left pleuropericardial membrane
  • Male:female ratio of 3:1
  • May be detected at any age but most commonly in low 20’s


  • Foraminal defect on left side               35%
  • Complete absence of left side             35% —levoposition of heart
  • Diaphragmatic surface                         17%
  • Total bilateral absence                         9%
  • Right sided                                           4%

Clinical findings

  • Mostly asymptomatic
  • May have:
    • Tachycardia
    • Palpitations
    • Right bundle block
    • Positional discomfort lying on left side
    • Chest pain

Imaging Findings

  • Focal bulge in area of main pulmonary artery or left atrium in partial defects
    • Sharply marginated
  • In complete form, heart rotates up and to the left
  • Lung may interpose between heart and left hemidiaphragm
  • Increased distance between sternum and heart 2° absence of sternopericardial ligament
  • Lung may interpose between aorta and main pulmonary artery on axial CT scans
  • Levoposition of heart
  • Pneumopericardium may occur following pneumothorax


  • Asymptomatic, complete absence of the pericardium and very small defects present no danger to the patient and require no intervention
  • Most authors advise leaving incidental defects untreated
  • Symptomatic foraminal defect may require surgery because of herniation and strangulation of left atrial appendage or herniation of LA/LV
    • Surgery can be enlargement of the defect to prevent strangulation or closure of the defect

Complications and associations

  • Associated congenital anomalies occur in about 30 per cent of the reported cases
  • Bronchogenic cysts
  • ASD, VSD, PDA, Tetralogy of Fallot, Mitral stenosis
  • Diaphragmatic hernia
  • Pulmonary sequestration 

Congenital Defect in the Pericardium

Congenital Defect in the Pericardium. Frontal and lateral chest radiographs demonstrate an unusually-shaped (yellow arrow) levopositioned (green arrow) heart. The heart is displaced upward from the left hemidiaphragm (white arrow) and there is a clear space between the sternum and the heart (blue arrow) on the lateral image.
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Congenital Defects of the Pericardium. Nigel E. Drury, Ravi J. De Silva, Roger M. O. Hall and Stephen R. Large.  Ann Thorac Surg 2007;83:1552–3