PAPVR/TAPVR

Question 1

2 Basic Classifications OF anomolous pulmonary venous connections:

Answer 1

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

Question 2

TAPVR

Answer 2

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 3

The symptoms of anomalous pulmonary venous connections vary for each child, but commonly include:

Answer 3

 •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 4

TAPVR. what is required for survival?

Answer 4

ASD

Question 5

TAPVR. If the infant is to live,

Answer 5

a large atrial septal defect (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

 All pulmonary veins shunted

Answer 6

L→R (Lungs → RA)

Question 7

must have

Answer 7

R→L shunt for survival (ASD) All are cyanotic

Question 8

oxygenation in 4 chambers with ASD

Answer 8

Identical oxygenation

Question 9

Total anomalous pulmonary venous return (TAPVR) is what type of defect

Answer 9

a congenital (present at birth) heart defect.

Question 10

Due to abnormal development during the first 8 weeks of pregnancy,

Answer 10

the pulmonary veins are improperly connected.

Question 11

anomalous venous connections prevelance for TAPVR

Answer 11

1. Supracardiac(52%)

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

Question 12

anomalous venous connections prevelance for TAPVR

Answer 12

1. Supracardiac(52%)

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

Question 13

supracardiac TAPVR

Answer 13

 Most common  Pulmonary Veins drain:  Vertical vein → Lt Brachiocephalic→ SVC

Question 14

supracardiac TAPVR what you see in x ray

Answer 14

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

Question 15

intracardiac tapvr

Answer 15

 2nd most common  Drains into coronary sinus or RA  Increased pulmonary vasculature  RV overload

Question 16

type I and II TAPVR survival to adults

Answer 16

20%. rest die in first year

Question 17

infracardiac TAPVR

Answer 17

 Long pulmonary veins course down the esophagus
 Empty in portal or IVC  Veins constricted thru diaphragm (obstructive)  Severe CHF (obstructive)  Associated w/asplenia  Death in a few days

Question 18

Mixed TAPVR

Answer 18

 Usually a mix of types I,II and III
 Severity can vary significantly
 All encompassing mix of whatever does not fit in the other classes

Question 19

OBSTRUCTIVE TAPVR severity

Answer 19

The severity of this condition depends on whether the pulmonary veins are obstructed

 In obstructed TAVPR, the pulmonary veins run into the abdomen, passing through the diaphragm

Question 20

In obstructed TAVPR, the pulmonary veins run

Answer 20

into the abdomen, passing through the diaphragm

Question 21

obstructed TAVPR squeezes

Answer 21

veins and narrows them, causing the blood to back up into the lungs (RA, RV pressures increase).
 Causes symptoms early - deadly if not recognized and surgically corrected.

Question 22

obstructive tapvr consequences

Answer 22

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

Question 23

non obstructive tapvr consequences

Answer 23

 Similar hemodynamics to a large ASD
 L → R shunt magnitude is determined by RV compliance & ASD size
 Rt heart & pulmonary volume overload
 Complete mixing at RA level
 Minimal cyanosis due to large PBF
 Slight PA pressure elevation`

Question 24

In PAPVR, oxygenated blood returns from the

Answer 24

lungs via the pulmonary veins back to the right and also to the left atrium. In other words, the pulmonary return blood is divided between the LA and RA in the return to the heart.

Question 25

PAVR one or more

Answer 25

of the 4 PV’s drain to the RA  Mild or no physiological consequence  Associated w/ASD (sinus venosis or secundum)

Question 26

papvr mixing possiblities

Answer 26

Any mix is possible Of the 4 PV’s: 3:1 2:2

Question 27

surgical treatment BAS

Answer 27

Balloon atrial septostomy (BAS) has been used with some success to decompress the venous circuit and improve cardiac output in cases of a restrictive inter-atrial communication.  Since an ASD is imperative to survival – it is better if it is non-restrictive

Question 28

SURGICAL TREATMENT GOAL

Answer 28

The goal of surgical repair is to recreate an unobstructed venous inflow to the left side chambers and repair of the associated anomalies, such as closure of atrial septal defect (ASD).

Question 29

Surgical repair is performed emergently i

Answer 29

in the newborn period for newborns with Total Anomalous Pulmonary Venous Return and obstructed pulmonary veins. Some of these children will actually require extracorporeal life support (ECMO) prior to surgery because of their marked hemodynamic instability  Obstructive TAPVR is one of the true pediatric emergencies

Question 30

PAPVC is normally corrected

Answer 30

without complications.

Question 31

TAPVC still carries significant

Answer 31

morbidity and mortality in low volume centers, (due to the severe hemodynamic and metabolic compromise).

Question 32

10-15% of patients undergoing repair of TAPVC

Answer 32

require multiple interventions due to recurrent | stenosis after initial successful correction, (with an increasingly poor outcome at each representation).

Question 33

Children with TAPVR without obstruction to the pulmonary veins typically undergo

Answer 33

the pulmonary veins typically undergo surgical repair electively days or weeks after the diagnosis is made.  In these children, although the surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months.

Question 34

Children with TAPVR without obstruction to the pulmonary veins typically undergo

Answer 34

the pulmonary veins typically undergo surgical repair electively days or weeks after the diagnosis is made.  In these children, although the surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months.

Question 35

In Total Anomalous Pulmonary Venous Return, the pulmonary veins frequently return

Answer 35

to a common confluence behind the LA.

Question 36

The surgical repair takes advantage of this fact. The common PV confluence is

Answer 36

connected to the back of the LA, resulting in a normal connection of PV->LA

Question 37

All other abnormal routes for pulmonary venous drainage

Answer 37

(are tied off).

Question 38

As one might guess, the surgical mortality or death rate is higher when surgery is

Answer 38

performed emergently in critically ill newborns with obstructed pulmonary venous return.  This is because they are very sick going to surgery.

Question 39

Critically ill newborns who do survive the surgery may require

Answer 39

a prolonged period of post-operative intensive care.

Question 40

cannulation

Answer 40

Arterial: aortic  Venous:  PAPVR (larger child) Bicaval TAPVR (newborn)SingleAtrial

Question 41

hypothermia

Answer 41

Circulatory arrest will be utilized partially or completely:  DHCA or intermittent depending on exposure and visualization

Question 42

In the past, almost all infants with TAPVC were repaired using

Answer 42

profound hypothermia and circulatory arrest |  now it can be performed with bicaval cannulation and low flow hypothermic perfusion.

Question 43

Circulatory arrest has the advantage of allowing a bloodless field with excellent exposure of

Answer 43

the pulmonary venous confluence without the need for unnecessary manipulation or clamping of the pulmonary veins

Question 44

On occasion, it is helpful to introduce brief periods of circulatory arrest during the most critical portions of the operation to

Answer 44

optimize surgical exposure with a nearly bloodless field.

Question 45

Similarly some centers have advocated the use of extracorporeal membrane oxygenation (ECMO) during

Answer 45

resuscitation.

Question 46

In few cases, ECMO has been adopted after the repair to

Answer 46

support neonates with residual pulmonary hypertension.

Question 47

case notes

Answer 47

 These children are very sick  Pre and post ECMO are a BIG POSSIBILITY  Birth weights tend to be small  The pulmonary blood flow undergoes a BIG change and can shock the body (think Qp/Qs)

Question 48

these cases tend to be

Answer 48

call in

Question 49

Even post septostomy

Answer 49

they can still struggle  ECMO post op is “frequent”

Question 50

Pulmonary hypertension must decrease in order

Answer 50

for complete restoration of normal circulation |  Look for small weight children in severe distress. Impressive acidosis may develop