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
  • Supracardiac
    — Drain into left innominate vein via left vertical vein
    — CXR: shunt vascularity + Snowman sign – upper half is dilated superior vena cava on right + left vertical vein on left, body of snowman is enlarged right atrium
    — CT: pulmonary vein stenosis
    — Clinical: most common – 55%
  • Cardiac
    — Drain into coronary sinus or right atrium
    — CXR: shunt vascularity + enlarged right atrium
    — Clinical: second more common – 30%
  • Infracardiac
    — Drains into portal vein, hepatic vein, inferior vena cava through narrow channel
    — CXR: shunt vascularity + normal size heart
    — Clinical: most rare – 12%

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.