Total Anomalous Pulmonary Venous Return

  • Etiology: Developmental failure of common pulmonary vein at left atrium which is classified according to where branch pulmonary veins hook up at – supracardiac or cardiac or infracardiac
  • Imaging: Admixture lesion leads to cyanosis and increased pulmonary blood flow
  • Imaging Type I – Supracardiac (50%):
    — Drain into left innominate vein via left vertical vein or superior vena cava or azygous vein
    — 50% obstructed
    — Imaging CXR: Shunt vascularity and Snowman sign – upper half of snowman is dilated superior vena cava on right and left vertical vein on left while lower half of snowman is enlarged right atrium
    — Imaging CT: Pulmonary vein stenosis
  • Imaging Type II – Intracardiac (30%):
    — Drain into right atrium or coronary sinus
    — 20% obstructed
    — Imaging CXR: Shunt vascularity and enlarged right atrium
  • Imaging Type III – Infracardiac:
    — Drain into inferior vena cava or portal veins or hepatic veins or ductus venous
    — 90% obstructed
    — Imaging CXR: Shunt vascularity and normal size heart
  • Imaging Type IV – Mixed multilevel drainage (5%):
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:
    — Often present with congestive heart failure or cyanosis
    — Right to left shunt is critical and is often due to patent foramen ovale or atrial septal defect
    — 14% have associated cardiac lesions

Radiology Cases of Total Anomalous Pulmonary Venous Return

CXR of totally anomalous pulmonary venous return
CXR AP shows increased pulmonary vascularity and a normal sized heart with an upturned cardiac apex.

Radiology Cases of Supracardiac Total Anomalous Pulmonary Venous Return

CXR and angiogram of total anomalous pulmonary venous return
CXR AP (above) shows increased pulmonary blood flow and a snowman-shaped heart, AP angiogram venous phase (below) shows dilated superior vena cava on right and left vertical vein on left.

Radiology Cases of Infracardiac Total Anomalous Pulmonary Venous Return

CXR of infradiaphragmatic total anomalous pulmonary venous
CXR AP (above) shows mild cardiomegaly and pulmonary vascular congenstion. CXR AP (below) from 1 week later shows increasing cardiomegaly and pulmonary vascular congestion.
CXR and CT of infracardiac total anomalous pulmonary venous return
CXR AP (above) shows mild cardiomegaly with marked pulmonary vascular congestion. Coronal CT MIP with contrast of the chest (below left) shows the pulmonary veins from both lungs draining into the vertical vein which crosses the diaphragm and then narrows as it connects to the dilated ductus venosus which then drains into the inferior vena cava. This is better demonstrated on the 3D CT (below right).

Gross Pathology Cases of Infradiaphragmatic Total Anomalous Pulmonary Venous Return

Gross pathological image of infradiaphragmatic total anomalous pulmonary venous return
Gross pathological image shows the pulmonary veins joining into a common vein which descends through the diaphragm and which drains into the main portal vein within the liver.