Topic 13: TAPVR PAPVR

Question 1

Anomalous congenital connections of the pulmonary venous system represent a spectrum of conditions in which the pulmonary veins are partially or entirely connected to what?

Answer 1

the right atrium

Directly or via the systemic venous return

Question 2

2 Basic Classifications:

Answer 2

---Total Anomalous  (TAPVC / TAPVR)
Serious physiologic abnormalities
---Partial Anomalous  (PAPVC / PAPVR)
Mild physiologic abnormality
Can be asymptomatic

Question 3

TAPVR blood flow ?

Answer 3

Oxygenated blood returns from the lungs back to the right atrium or a vein flowing into the right atrium and NOT to the left side of heart.

In other words, blood simply circles to and
from the lungs and never gets out to the body
(2 separate circulations)

Question 4

Symptoms of Anomalous Pulmonary Venous Return?

Answer 4

The symptoms of anomalous pulmonary venous connections vary for each child, but commonly include:
•Cyanosis (a persistent blue or gray tone to the
skin, lips, or nails)
•Pale, cool or clammy skin
•Difficult/rapid breathing
•Tachycardia
•Poor appetite and insufficient weight gain (failure to thrive)
•Unusual tiredness or irritability

Question 5

With a TAPVR what is absolutely required for life?

Answer 5

ASD

If the infant is to live, a large ASD or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and rest of the body.
This L →R Shunt must have a R →L shunt for survival, that shunt is an ASD

Question 6

TAPVR - how are pulmonary veins shunted?

oxygenation?

Answer 6

All pulmonary veins shunted L→R (Lungs →RA)
Must have R→L shunt for survival (ASD)
All are cyanotic
Identical oxygenation in 4 chambers (w/ASD)

Question 7

TAPVR with an unrestricted ASD - pressures and saturations in heart?

Answer 7

RA - 7 (87%)
RV 45/6 (87%)
LA 7 (87%)
LV 90/4 (87%)

Question 8

Embryology of TAPVR

Answer 8

Total anomalous pulmonary venous return
(TAPVR) is a congenital (present at birth) heart defect.
Due to abnormal development during the first 8 weeks of pregnancy, the pulmonary veins are improperly connected

Question 9

TAPVR classifications?

Answer 9

1. Supracardiac (52%)
2. Cardiac (30%)
3. Infracardiac (12%)
4. Mixed (6%)

Question 10

Supracardiac TAPVR characteristics

Answer 10

Pulmonary Veins from Lungs
SVC
Bridging Veins 
Pulmonary Veins drain:
Vertical vein →Lt Brachiocephalic→SVC

Question 11

intracardiac TAPVR characteristics?

Answer 11

2nd most common
Pulm Veins Drains into coronary sinus or RA
Increased pulmonary vasculature
RV overload
Only 20% of I and II TAPVR survive to adults
(the rest die in 1st year)

Question 12

Supracardiac – what youll find on xray?

Answer 12

Dilated SVC + Lt vertical vein (snowman heart)
↑ Vasculature
↑ RV volume