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Peds > 15. TGA- Exam 4 > Flashcards

15. TGA- Exam 4 Flashcards

1
Q

TRANSPOSITION OF THE GREAT ARTERIES (TGA/TGV)=

A

–Discordant ventricular-arterial relationship
–Malformation in which the two great arteries carrying blood away from the heart are transposed or reversed.
The LV–> PA
The RV–> Aorta

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2
Q

an acyanotic defect with increased pulmonary BF could be what defects?

A

ASD
VSD
PDA
AVSD

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3
Q

an acyanotic defect with obstruction to BF from the ventricles could be what defects?

A

Coarctation of the aorta
Aortic stenosis
Pulmonary stenosis

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4
Q

an cyanotic defect with decreased pulmonary BF could be what defects?

A

TOF

Tricuspid Atresia

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5
Q

an cyanotic defect with mixed BF could be what defects?

A

TGA
TAPVR
Trunctus arteriosus
HLHS

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6
Q

TGA is incompatible with life unless what happens?

A

unless some communication exists between the two separate circulatory systems. (ASD or VSD)

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7
Q

with a TGA, what are the 2 parallel circulation exists

A
  1. Body—-RA—-RV—-AO—-Body

2. Lungs—LA—-LV—-PA—-Lungs

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8
Q

with a TGA, what do the 2 circulations cause

A 
Poor mixing
Hypoxia & Acidemia
Hyperventilation
Increased pulmonary flow
CHF
Myocardial depression
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9
Q

when is TGA the most common cyanotic congenital heart lesion

A

when presenting in the neonate

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10
Q

TGA % occurrence of congenital heart diseases

A

5%

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11
Q

is TGA more common in males or females? whats the ratio?

A

More common in males, with a ratio of about 3:1

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12
Q

what Maternal factors associated with an increased risk of TGA

A

rubella or other viral illness during pregnancy, alcoholism, maternal age over 40 and diabetes.

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13
Q

describe the embryology of TGA

A
  • Bulbus cordis defect
  • AFTER outflow tract septation development begins then:
  • -Improper spiraling of the aorticopulmonary septum
  • -Leads to congenital disruption in pulmonary and systemic circulations
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14
Q

Truncus Arteriosus becomes the…

A

aorta

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15
Q

Conus Cordis becomes the…

A

Pulmonary Artery

-Created by a septum that forms in the outflow tract from these swellings

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16
Q

Outflow tract septation occurs on what day

A

29

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17
Q

As is with TAPVR, without intervention infants with TGA will die when?

A

within their first year of life

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18
Q

dextro-transposition of the great arteries [d-TGA] =

A

aorta is anterior and to the right of the pulmonary artery

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19
Q

what is d- TGA % occurrence

A

60%

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20
Q

levo-transposition of the great arteries [L-TGA] =

A

aorta may be anterior and to the left of the pulmonary artery

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21
Q

what is the ONLY distinguishing characteristic that defines TGA

A

Discordant ventriculo-arterial connection

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22
Q

TGA w/IVS=

A

Transposition of the great arteries with intact ventricular septum

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23
Q

TGA w/VSD=

A

Transposition of the great arteries with ventricular septal defect

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24
Q

TGA W/VSD, LVOT obstruction=

A

Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction

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25
Q

TGA w/VSD, PVOD

A

Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease

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26
Q

Congenitally corrected TGA=

A

Aorta and PA are normal- LV and RV are switched

-Frequently an autopsy finding

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27
Q

how long ago was TGA first described

A

over 200 years

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28
Q

what where the first treatments to become available

A

-development of surgical atrial septectomy in the 1950s
-balloon atrial septostomy in the 1960s
–These palliative therapies were followed by:
Physiological procedures (atrial switch operation) Anatomic repair (arterial switch operation)

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29
Q

Today, the survival rate for infants with TGA is greater than __%.

A

90%

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30
Q

what is the initial treatment of TGA

A

maintaining ductal patency with continuous IV prostaglandin E1 infusion (PGE1)

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31
Q

what is the purpose of giving PGE1

A

↑ pulmonary blood flow
↑ increase left atrial pressure
promote L→R shunting at the atrial level ( ↓cyanosis).

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32
Q

giving PGE1 is especially important for what condition

A

severe LVOT obstruction

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33
Q

when is PGE1 not useful

A

the PDA will not help when the defect has intact septum’s- and separate circulations

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34
Q

surgical repair for a TGA: first operation=

A

arterial switch

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35
Q

alternative procedure for TGA with intact septum=

A

Atrial switch

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36
Q

alternative procedure for TGA with septal defect=

A

Rastelli

Rev Nikaidoh

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37
Q

In TGA w/IVS and LVOT obstruction. An arterial switch operation may not be feasible due to what?

A

pulmonary (left ventricular outflow tract) stenosis or atresia

38
Q

If the ventricular septal defect is nonrestrictive and not too remote from the aorta, what procedure is possible

A

Rastelli intracardiac repair

39
Q

Rastelli procedure=

A

conduit from the RV to the PA , delaying repair

40
Q

what procedure may be necessary to establish adequate pulmonary blood flow while waiting for the full repair

A

placing an aorto-pulmonary shunt during the newborn period (BT or Central)

41
Q

what are the 2 types of Atrial Switches

A

Mustard

Senning

42
Q

what are the 2 types of Arterial Switches

A

Jatene

Le Compte

43
Q

MUSTARD PROCEDURE –ATRIAL BAFFLE=

A

restores the circulation, but reverses the direction of the blood flow in the heart

  • -Blood is pumped to the lungs via the LV and disseminated throughout the body via the RV.
  • -But the RV is not the optimal shape to support the high pressure work performed in a normal heart by the LV
44
Q

SENNING PROCEDURE (ATRIAL TISSUE BAFFLE)=

A
  • -A baffle is created within the atria that redirects the deoxygenated caval blood to the mitral valve and the oxygenated pulmonary venous blood to the tricuspid valve.
  • -The anatomic LV continues to act as the pulmonary pump and the anatomic RV acts as the systemic pump
45
Q

The Mustard procedure and Senning procedure are identical except for what?

A

the baffle is constructed from atrial tissue in the Senning and from pericardium in the Mustard

46
Q

when did complications from the Senning and Mustard procedure become known

A

1980’s

47
Q

after the complications from the Senning and Mustard procedures where discovers, what procedure did they start to use instead

A

neonatal arterial switch procedure, which is now standard therapy for transposition.

48
Q

The Mustard procedure was replaced in the late 1970s by the ____________

A

Jatene procedure (arterial switch)

49
Q

Jatene procedure (arterial switch) =

A

Native arteries are switched back to normal flow, so that the RV would be connected to the pulmonary artery and the LV would be connected to the aorta

50
Q

why was the Jatene procedure not possible prior to 1975

A

because of difficulty with re-implanting coronary arteries which perfuse myocardium

51
Q

what is the ideal procedure for TGA repair

A

ASO procedure [arterial switch]

52
Q

why is an ASO procedure ideal for TGA reapir

A

It represents an anatomic repair and establishes ventriculo-arterial concordance

53
Q

when should an ASO be performed? why?

A

This procedure should be performed when the infant is younger than 4 weeks, (the LV may not be able to handle systemic pressure in the pulmonary system)

54
Q

LE COMPTE MANEUVER=

A

Ascending aorta and pulmonary trunk are transected and switched. Coronary arteries are transected and re implanted into neo-aortic root. Straddles the neo-aorta

55
Q

CPB Considerations for TGA: Arterial+Venous Cannula

A

Arterial: Aortic
Venous: Single Atrial (bicaval if 4+ kg)

56
Q

CPB Considerations for TGA: Temp

A

DHCA/Low flow w/HCA

57
Q

CPB Considerations for TGA: Cardioplegia

A

Antegrade, Retrograde and Ostial (multiple dosing)

58
Q

CPB case notes for TGA: what is the typical weight of these children

A

These children are larger weight (around 3 kg)

59
Q

CPB case notes for TGA: be very careful while delivering what?

A

Be very careful with cardioplegia (flow/pressure)

60
Q

CPB case notes for TGA: is this a complete repair or just a palliation?

A

Most often, this is a complete correction not a palliation

61
Q

CPB case notes for TGA: what usually happens after the case? what should you try to do?

A

Open chest - post procedure (silastic patch)

– try to Reduce myocardial edema

62
Q

CPB case notes for TGA: whats the length and difficulty of the producure

A

Longer procedure (technically difficult)

63
Q

CPB case notes for TGA: whay might happen after the case despite a good prognosis

A

ECMO may be in the cards w/good prognosis

64
Q

Truncus Arteriosus=

A

a rare type of congenital heart disease in which a single blood vessel (truncus arteriosus) comes out of the right and left ventricles, instead of the normal two (pulmonary artery and aorta).

65
Q

If Truncus Arteriosus goes tntreated, what Two Problems occur

A

I. Too much pulmonary blood flow

II. The blood vessels to the lungs become permanently damaged. Pulmonary hypertension develops.

66
Q

what is The major problem in Truncus Arteriosus

A

the lungs are flooded with blood and the heart muscle is overloaded

67
Q

Truncus Arteriosus symptoms (8)

A 
Bluish skin (cyanosis)
Delayed growth or growth failure
Fatigue
Lethargy
Poor feeding
Rapid breathing (tachypnea)
Shortness of breath (dyspnea)
Widening of the finger tips (clubbing)
68
Q

what is the Trunctus Arteriosus thought to result from

A

failed septation of the embryonic truncus arteriosus

69
Q

Aortopulmonary and interventricular defects are believed to represent an abnormality of what?

A

conotruncal septation.

70
Q

with Trunctus Arteriosus, why is a PDA is not required to support the fetal circulation.

A

Because the common trunk originates from both the LV and RV, and PA’s arise directly from the common trunk

71
Q

what are the truncus arteriosus and bulbus cordis are divided by

A

aortico-pulmonary septum

72
Q

The truncus arteriosus gives rise to what

A

the ascending aorta and the pulmonary trunk.

73
Q

The bulbus cordis gives rise to what?

A

the smooth parts (outflow tract) of the LV and RV

74
Q

Truncus Arteriosis type 1=

A

truncus -> one PA -> two lateral pulmonary arteries
–characterized by origin of a single pulmonary trunk from the left lateral aspect of the common trunk, with branching of the left and right pulmonary arteries from the pulmonary trunk.

75
Q

Truncus Arteriosis type 2=

A

truncus -> two posterior/ posteriolateral pulmonary arteries
–characterized by separate but proximate origins of the left and right pulmonary arterial branches from the posterolateral aspect of the common arterial trunk

76
Q

Truncus Arteriosis type 3=

A

truncus -> two lateral pulmonary arteries

  • -Branch pulmonary arteries originate independently from the common arterial trunk or aortic arch, (most often from the left and right lateral aspects of the trunk).
  • -This occasionally occurs with origin of one pulmonary artery from the underside of the aortic arch, usually from a ductus arteriosus
77
Q

Since the truncal valve is above the VSD, how does the blood flow

A

blood is pumped from both the RV and LV to the lungs and to the body, (mixing is occurring)

78
Q

, PVR is lower than SVR… what does this cause

A

there is usually increased blood flow to the lungs. This increased PBF can lead to CHF

79
Q

with Tructus Arteriosis…Because the lung arteries are connected to the high pressure pumping chambers (LV/RV) there is pulmonary HTN. If the lungs are exposed to both high pressure and extra blood flow for an extended time (months to years), what happens

A

irreversible pulmonary hypertension can occur.

80
Q

PA Banding (palliative repair for trunctus arteriosis)=

A

involves banding the pulmonary arteries coming off the truncus (reduces pulmonary blood flow)

81
Q

where is the incision site for PA Banding and is this a CPB case

A

The left anterior thoracotomy approach through the second or third intercostal space gives excellent exposure for isolated pulmonary artery banding.
–Extracardiac procedure – no CPB

82
Q

Rastelli procedure (complete repair for trunctus arteriosis)=

A

connects the RV-PA. This tube is usually a homograft, (made from human cadaver tissue).
–During the Rastelli procedure, the VSD is closed with a Gore-Tex patch so that the aorta arises solely from the LV

83
Q

what is the ABCD steps for Surgical Repair of Truncus Arteriosus

A
  • A Origin of truncus arteriosus PA’s are excised and the truncus defect closed with direct suture. An RV ventriculotomy is made.
  • B VSD is closed with a prosthetic patch.
  • C Placement of a valved conduit into the pulmonary arteries.
  • D Proximal end of conduit is anastomosed to the RV.
84
Q

CPB Considerations for Truncus Arteriosus: incision

A

Median sternotomy

85
Q

CPB Considerations for Truncus Arteriosus: arterial + venous cannula

A

Arterial: Aortic
Venous: Single atrial cannula

86
Q

CPB Considerations for Truncus Arteriosus: temp

A

Mild to Moderate period DHCA

87
Q

CPB Considerations for Truncus Arteriosus: cardioplegia

A

Antegrade, possibly retrograde

88
Q

CPB Considerations for Truncus Arteriosus: aortic Xc time

A

moderate length

89
Q

Case notes for Truncus Arteriosus: what is is often required following repair of truncus arteriosus.

A

Delayed sternal closure

90
Q

Case notes for Truncus Arteriosus: what has Marked improvement post-op vs. pre-op

A

blood gases

91
Q

Case notes for Truncus Arteriosus: although antegreade cpg is standard, what should you be prepared for

A

retrograde CP (monitor CVP should be 30- 40 mmhg)

92
Q

Case notes for Truncus Arteriosus: will you see this patient for a redo? why?

A

At some point changing of the RV-PA Conduit would be done as a redo Rastelli ( Maybe twice ? )

Peds (17 decks)

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