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Total Anomalous Pulmonary Venous Return (TAPVR)

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

Total Anomalous Pulmonary Venous Drainage (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—X-ray Findings

• Snowman heart = dilated SVC+ 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

WH/rev2002

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