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Total Anomalous Pulmonary Venous Return
TAPVR and PAPVR
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 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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