Total Anomalous Pulmonary Venous Return

  • Etiology: developmental failure of common pulmonary vein at left atrium
  • Branch pulmonary veins hook up at
    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.