Absent Pulmonary Valve Syndrome

  • Etiology: Absence of the pulmonary valve
  • Imaging: Absent pulmonary valve with aneurysmal dilation of main pulmonary artery with or without dilation of its branches
  • DDX:
  • Complications: Poor prognosis
  • Treatment: Surgical
  • Clinical: May be associated with many other types of congenital heart disease

Radiology Cases of Absent Pulmonary Valve Syndrome

CXR and CT of main pulmonary arterial aneurysm due to absent pulmonary valve syndrome
CXR AP (above) shows an abnormal and prominent contour to the left superior mediastinum. The right superior mediastinum contour represents the thymus. Coronal CT with contrast of the chest (below) shows aneurysmal dilation of the main pulmonary artery. The pulmonary valve was absent.