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.