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Flashcards in PAPVR/TAPVR Deck (50):
1

2 Basic Classifications OF anomolous pulmonary venous connections:

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

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TAPVR

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)

3

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

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TAPVR. what is required for survival?

ASD

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TAPVR. If the infant is to live,

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

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 All pulmonary veins shunted

L→R (Lungs → RA)

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must have

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

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oxygenation in 4 chambers with ASD

Identical oxygenation

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

a congenital (present at birth) heart defect.

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

the pulmonary veins are improperly connected.

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anomalous venous connections prevelance for TAPVR

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

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anomalous venous connections prevelance for TAPVR

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

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supracardiac TAPVR

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

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supracardiac TAPVR what you see in x ray

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

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intracardiac tapvr

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

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type I and II TAPVR survival to adults

20%. rest die in first year

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infracardiac TAPVR

 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

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Mixed TAPVR

 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

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OBSTRUCTIVE TAPVR severity

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

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In obstructed TAVPR, the pulmonary veins run

into the abdomen, passing through the diaphragm

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obstructed TAVPR squeezes

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.

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obstructive tapvr consequences

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

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non obstructive tapvr consequences

 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`

24

In PAPVR, oxygenated blood returns from the

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.

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PAVR one or more

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

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papvr mixing possiblities

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

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surgical treatment BAS

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

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SURGICAL TREATMENT GOAL

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).

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Surgical repair is performed emergently i

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

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PAPVC is normally corrected

without complications.

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TAPVC still carries significant

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

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10-15% of patients undergoing repair of TAPVC

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

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Children with TAPVR without obstruction to the pulmonary veins typically undergo

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.

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Children with TAPVR without obstruction to the pulmonary veins typically undergo

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.

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

to a common confluence behind the LA.

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

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

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All other abnormal routes for pulmonary venous drainage

(are tied off).

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

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

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Critically ill newborns who do survive the surgery may require

a prolonged period of post-operative intensive care.

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cannulation

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

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hypothermia

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

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

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

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

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

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

optimize surgical exposure with a nearly bloodless field.

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

resuscitation.

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

support neonates with residual pulmonary hypertension.

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case notes

 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)

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these cases tend to be

call in

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Even post septostomy

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

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Pulmonary hypertension must decrease in order

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