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

Location:

• 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

Treatment

• 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. 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