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Peds > 13. TAPVR/PAPVR- Exam 4 > Flashcards

13. TAPVR/PAPVR- Exam 4 Flashcards

1
Q

Anomalous congenital connections of the
pulmonary venous system represent a
spectrum of conditions in which the…

A

pulmonary veins are partially or entirely
connected to the right atrium.
–Directly or via the systemic venous return

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2
Q

what better describes the anatomical situation rather than “drainage” or “return”?

A

Anomalous connection

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3
Q

which one (TAPVR or PAPVR) has Serious physiologic abnormalities

A

Total Anomalous (TAPVC / TAPVR)

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4
Q

which one (TAPVR or PAPVR) has Mild physiologic abnormality and Can be asymptomatic

A

Partial Anomalous (PAPVC / PAPVR)

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5
Q

Total Anomalous (TAPVC / TAPVR)=

A

Oxygenated blood returns from the lungs back to the RA or a vein flowing into the RA 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)

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6
Q

Total Anomalous (TAPVC / TAPVR) symptoms

A
  • Cyanosis
  • Pale, cool or clammy skin
  • Difficult/rapid breathing
  • Tachycardia
  • Failure to thrive
  • Unusual tiredness or irritability
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7
Q

what other defect is present with a TAPVR

A

ASD or PFO

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8
Q

what 4 things must be present with a TAPVR

A
  • 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)
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9
Q

describe the embryology of TAPVR

A

Due to abnormal development during the first 8 weeks of pregnancy, the pulmonary veins are improperly connected

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10
Q

what are the 4 classifications of TAPVR

A

Supracardiac
Cardiac
Infracardiac
Mixed

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11
Q

supracardiac TAPVR % occurance

A

52% –most common

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12
Q

intracardiac TAPVR % occurance

A

30% –second most common

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13
Q

infracardiac TAPVR % occurance

A

12%

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14
Q

mixed TAPVR % occurance

A

6%

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15
Q

with a supracardiac TAPVR, how do the Pulmonary Veins drain

A

Vertical vein → Lt Brachiocephalic→ SVC

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16
Q

with a supracardiac TAPVR, what 3 things will you see on the X-Ray

A

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

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17
Q

with a intracardiac TAPVR, how does it drain and what does this cause

A

Drains into coronary sinus or RA

  • -Increased pulmonary vasculature
  • -RV overload
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18
Q

what of % Type I and II TAPVR survive to adults (the rest die in 1st year)

A

only 20%

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19
Q

with a infracardiac TAPVR, how does it drain and what does this cause

A
  • 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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20
Q

describe a mixed TAPVR

A
  • 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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21
Q

The severity of TAPVR depends on what?

A

whether the pulmonary veins are obstructed

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22
Q

describe obstructed TAVPR

A
  • the pulmonary veins run into the abdomen, passing through the diaphragm.
  • This squeezes the 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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23
Q

Does this describe obstructed or non-obstructed TAPVR? Pulmonary venous HTN & secondary PA & RV
HTN

A

Obstructed TAPVR

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24
Q

Does this describe obstructed or non-obstructed TAPVR? Similar hemodynamics to a large ASD

A

NON-Obstructed TAPVR

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25
Q

Does this describe obstructed or non-obstructed TAPVR? L → R shunt magnitude is determined by RV compliance & ASD size

A

NON-Obstructed TAPVR

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26
Q

Does this describe obstructed or non-obstructed TAPVR? Less RV & PA volume overload

A

Obstructed TAPVR

27
Q

Does this describe obstructed or non-obstructed TAPVR? Rt heart & pulmonary volume overload

A

NON-Obstructed TAPVR

28
Q

Does this describe obstructed or non-obstructed TAPVR? Pulmonary venous edema

A

Obstructed TAPVR

29
Q

Does this describe obstructed or non-obstructed TAPVR? Complete mixing at RA level

A

NON-Obstructed TAPVR

30
Q

Does this describe obstructed or non-obstructed TAPVR? Minimal cyanosis due to large PBF

A

NON-Obstructed TAPVR

31
Q

Does this describe obstructed or non-obstructed TAPVR? More cyanosis & respiratory distress

A

Obstructed TAPVR

32
Q

Does this describe obstructed or non-obstructed TAPVR? Slight PA pressure elevation

A

NON-Obstructed TAPVR

33
Q

Does this describe obstructed or non-obstructed TAPVR? Complete mixing

A

Obstructed TAPVR

34
Q

Does this describe obstructed or non-obstructed TAPVR? Complete mixing

A

Obstructed TAPVR

35
Q

Partial Anomalous (PAPVC / PAPVR)=

A

-oxygenated blood returns from the lungs via the pulmonary veins back to the RA ans LA.
-In other words, the pulmonary return blood is divided
between the LA and RA in the return to the heart.

36
Q

what is a PAPVR associated with

A

ASD (sinus venosis or secundum)

37
Q

with a PAPVR, how many of the PV’s drain into the RA

A

One or more of the 4 PV’s drain to the RA

38
Q

what has been used with some success to decompress the venous circuit and improve cardiac output in cases of a restrictive inter-atrial communication.

A

Balloon atrial septostomy (BAS)

39
Q

Since an ASD is imperative to survival – it is better if it is restrictive or non-restrictive

A

non-restrictive

40
Q

what is the goal of surgical treatment

A

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).

41
Q

when is surgical repair performed emergently

A

in the newborn period for newborns with TAPVR and
obstructed pulmonary veins. Some of these children will actually require ECMO prior to surgery because of their marked hemodynamic instability

42
Q

what is one of the true pediatric emergencies

A

Obstructive TAPVR

43
Q

_____ is normally corrected without complications.

A

PAPVC

44
Q

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

A

TAPVC

45
Q

____% of patients undergoing repair of TAPVC require multiple interventions due to recurrent ____ after initial successful correction, (with an ______ poor outcome at each representation).

A

10-15%
stenosis
increasingly

46
Q

when do Children with TAPVR without obstruction to

the pulmonary veins typically undergo surgical repair electively

A

days or weeks after the diagnosis is made.
–although the surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months.

47
Q

In TAPVR surgical repair, the pulmonary veins frequently return to a common confluence behind the?

A

LA

  • -resulting in a normal connection of PV->LA
  • -All other abnormal routes for pulmonary venous drainage are tied off.
48
Q

what cases have the higher surgical mortality or death rate

A

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

49
Q

Critically ill newborns who do survive the surgery may require what?

A

a prolonged period of post-operative intensive care

50
Q

CPB: Arterial + Venous Cannulas

A

Arterial: aortic
Venous:
–PAPVR (larger child) Bicaval
–TAPVR (newborn) Single Atrial

51
Q

CPB: Temperatures

A
  • Hypothermia: Circulatory arrest will be utilized partially or completely
  • -DHCA or intermittent depending on exposure and visualization
52
Q

In the past, almost all infants with TAPVC were repaired using _____ and ______. Now it can be performed with ______ and _______ perfusion.

A

profound hypothermia and circulatory arrest

bicaval cannulation and low flow hypothermic

53
Q

what is the benefit of using circ arrest

A

allowing a bloodless field with excellent exposure of the pulmonary venous confluence without the need for unnecessary manipulation or clamping of the pulmonary veins

54
Q

what is the benefit of using circ arrest

A

allowing a bloodless field with excellent exposure of the pulmonary venous confluence without the need for unnecessary manipulation or clamping of the pulmonary veins

55
Q

On occasion, it is helpful to introduce brief periods of _______ during the most critical portions of the operation to optimize surgical exposure with a nearly bloodless field

A

circulatory arrest

56
Q

Similarly some centers have advocated the use of ____ during resuscitation. In few cases, it has been adopted after the repair to support neonates with residual _____.

A

ECMO

pulmonary hypertension

57
Q

Case Notes: Pre and Post ____ is a big possibility

A

ECMO

58
Q

Case Notes: Birth weights tend to be ____

A

small

59
Q

Case Notes: The pulmonary blood flow undergoes a BIG change and can _____ the body

A

shock

think Qp/Qs

60
Q

Case Notes: These cases tend to be _____ cases

A

“CALL IN”

61
Q

Case Notes: Even post septostomy, they can still _____

A

struggle

62
Q

Case Notes: Pulmonary HTN must ______ in order for complete restoration of normal circulation

A

decrease

63
Q

Case Notes: Look for small weight children in severe ____

A

distress

64
Q

Case Notes: Impressive _____ may develop

A

acidosis

Peds (17 decks)

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