Total Anomalous Pulmonary Venous Return

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

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