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Total Anomalous Pulmonary Venous Return

TAPVR and PAPVR
  

  • 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

l Partial (PAPVR)

l Mild physiologic abnormality

l Usually asymptomatic

l Total (TAPVR)

l Serious physiologic abnormalities

 Partial Anomalous Pulmonary Venous Drainage (PAPVR)

General

l One of the four pulmonary veins may drain into right atrium

l Mild or no physiologic consequence

l Associated with ASD

l Sinus venosus or ostium secundum types 

Total Anomalous Pulmonary Venous Drainage (TAPVR)

l All have shunt through lungs to R side of heart

l All must also have R to L shunt for survival

l Obligatory ASD to return blood to the systemic side

l All are cyanotic

l Identical oxygenation in all four chambers

l Types

l Supracardiac

l Cardiac

l Infracardiac

l Mixed

l Supracardiac Type—Type I

l Most common (52%)

l Pulmonary veins drain into vertical vein (behind left pulmonary artery) to left brachiocephalic vein to SVC

l DDx: VSD with large thymus

l Supracardiac Type 1—X-ray Findings

l Snowman heart = dilated SVC+ left vertical vein

l Shunt vasculature 2° increased return to right heart

l Enlargement of right heart 2° volume overload

l Cardiac Type—Type II

l Second most common: 30%

l Drains into coronary sinus or RA

l Coronary sinus more common

l Increased pulmonary vasculature

l Overload of RV leads to CHF after birth

l 20% of I’s and II’s survive to adulthood

l Remainder expire in first year

l Infracardiac Type—Type III

l Percent of total: 12%

l Long pulmonary veins course down along esophagus

l Empty into IVC or portal vein (more common)

l Vein constricted by diaphragm as it passes through esophageal hiatus

l Severe CHF (90%) 2° obstruction to venous return

l Cyanotic 2° right to left shunt through ASD

l Associated with asplenia (80%), or polysplenia

l Prognosis = death within a few days

l Mixed Type—Type IV

l Percent of total: 6%

l Mixtures of types I – III

 

WH/rev2002

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