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Total Anomalous Pulmonary Venous Return


Type 1 - Supracardiac Type

 

  • Must have ASD for survival
  • All have anatomical L to R shunt at atrial level
  • All have functional R to L shunt of oxygenated blood to right side of heart

Two Types

  • Partial (PAPVR)
    • Mild physiologic abnormality
    • Usually asymptomatic
  • Total (TAPVR)
    • Serious physiologic abnormalities

Partial Anomalous Pulmonary Venous Drainage (PAPVR)

General

  • One of the four pulmonary veins may drain into right atrium
  • Mild or no physiologic consequence
  • Associated with ASD
    • Sinus venosus or ostium secundum types

TAPVR

  • All have shunt through lungs to R side of heart
  • All must also have R to L shunt for survival
  • Obligatory ASD to return blood to the systemic side
  • All are cyanotic
  • Identical oxygenation in all four chambers
  • Types
    • Supracardiac
    • Cardiac
    • Infracardiac
    • Mixed
  • Supracardiac Type—Type I
    • Most common (52%)
    • Pulmonary veins drain into vertical vein (behind left pulmonary artery) to left brachiocephalic vein to SVC
    • DDx: VSD with large thymus
    • Supracardiac Type 1—Imaging Findings
      • Snowman heart = dilated SVC and left vertical vein
      • Shunt vasculature 2° increased return to right heart
      • Enlargement of right heart 2° volume overload
  • Cardiac Type—Type II
    • Second most common: 30%
    • Drains into coronary sinus or RA
      • Coronary sinus more common
    • Increased pulmonary vasculature
    • Overload of RV leads to CHF after birth
    • 20% of I’s and II’s survive to adulthood
      • Remainder expire in first year
  • Infracardiac Type—Type III
    • Percent of total: 12%
    • Long pulmonary veins course down along esophagus
    • Empty into IVC or portal vein (more common)
    • Vein constricted by diaphragm as it passes through esophageal hiatus
    • Severe CHF (90%) 2° obstruction to venous return
    • Cyanotic 2° right to left shunt through ASD
    • Associated with asplenia (80%), or polysplenia
    • Prognosis = death within a few days
  • Mixed Type—Type IV
    • Percent of total: 6%
    • Mixtures of types I – III

 

Supracardiac-Type I-TAPVR. The heart has a "snowman shape." The pulmonary veins drain into an enlarged
vertical vein (blue arrow) which in turn drains into the brachiocephalic vein
and then to the superior vena cava (red arrow). The vasculature is shunt vasculature.
For the same photo without the arrows, click here