Congenital absence of pericardium

  • rare and usually asymptomatic
  • most common: complete absence of left pericardium
  • associated anomalies: ASD, ToF, PDA, bronchogenic cysts, pulmonary sequestration
  • interposition of lung between aorta and main pulmonary artery in the aortopulmonary windows is the most reliable sign
  • rotation of cardiac axis to the left side