Topic 13: TAPVR, PAPVR

Question 1

4 classifications and percent occurrences of TAPVR

Answer 1

Supracardiac: 52%
Cardiac: 30%
Infracardiac: 12%
Mixed: 6%

Question 2

BF for supra cardiac TAPVR

Answer 2

Pulmonary vein–> vertical vein–> L. Brachiocephalic–> SVC–>RA

You see dilated SVC and vertical vein, increased vasculature and increased RV volume (snowman heart)

Question 3

BF for Intracardiac TAPVR

Answer 3

Pulm vein–>coronary sinus or RA

See increased pulm vasculature and RV volume

Question 4

BF in Infracardiac TAPVR

Answer 4

Long pulmonary veins course down esophagus–>IVC or portal vein

Veins get constricted in diaphragm
Poor VR
Associated with aspelnia
Severe CHF

Question 5

Obstructive TAPVR

Answer 5

Pulmonary veins run into abdomen, passing through the diaphragm
Veins get squeezed and narrowed so blood backs up into lungs and increases right heart pressures

Question 6

Symptoms of obstructed TAPVR

Answer 6

Pulmonary venous hypertension and secondary PA and RV hypertension 
Less RV and PA volume overload
Pulmonary venous edema
More cyanosis and respiratory distress 
Complete mixing

Question 7

Symptoms of non-obstructive TAPVR

Answer 7

Similar hemodynamics to large ASD
L->R shunt magnitude determined by RV compliance and ASD size
Right heart and pulmonary volume overload
Complete mixing at RA level
Minimal cyanosis due to large pulmonary BF
Slight PA pressure elevation

Question 8

Symptoms of anomalous pulmonary connections

Answer 8

Cyanosis
Pale, cool, or clammy skin
Difficult/rapid breathing
Tachycardia
Poor appetite and insufficient weight gain
Unusual tiredness and irritability

Question 9

Treatment for TAPVR and PAPVR

Answer 9

1.Balloon atrial septostomy
-Goal is to recreate unobstrictes venous inflow to left heart then close ASD
2.