Exam 3 CHD 4-5 Flashcards

1
Q

Corrective procedure for Transposition of the Great Arteries (Vessels) (TGA)?

A

Arterial Switch

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2
Q

What are the 2 forms of TGA?

A

D-Transposition (Dextrotransposition)

L-Transposition (Levotransposition)

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3
Q

Which type of TGA is most common?

A

D-Transposition

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4
Q

Which type is where misdirected folding of embryonic heart tube- folding to the left side instead of right?

A

L-Transposition

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5
Q

Which type is where the Truncus Arteriosus fails to divide properly?

A

D-Transposition

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6
Q

What is the common origin of the aorta and pulmonary artery?

A

Truncus Arteriosus

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7
Q

Describe what happens with Dextrotransposition of the Great Arteries

A

The Truncus Arteriosus (common origin of the aorta and PA) fails to divide properly and creates 2 parallel circulations.

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8
Q

What 3 defects creates an exception to the parallel circulation that normally occurs with D-TGA?

A

PDA, ASD, VSD (Additional communications)

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9
Q

Trace the blood flow from the RA-Aorta with L-TGA

A

RA-MV-LV-PA-LA-TV-RV-Aorta

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10
Q

Which TGA is associated with switching the position of the RV and LV with NO AFFECT on the Great Vessels?

A

L-TGA

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11
Q

Describe what happens with Levotransposition of the Great Arteries

A

Misdirected folding of the embryonic heart tube occurs, it folds to the left side instead of the right. The RV and LV are switched and there is no affect on the great vessels.

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12
Q

TGA normally occurs with other anomalies, or in isolation?

A

Isolation

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13
Q

TGAs accounts for ____% of all CHDs.

A

6%

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14
Q

Sx of L-TGA at birth?

A

Asymptomatic at birth

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15
Q

Sx of R-TGA at birth w/o shunting lesions?

A

Profound Cyanosis

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16
Q

Sx of R-TGA at birth w/ shunting lesions?

A

Initially asymptomatic, progresses to tachypnea, tachycardia, heart failure, feeding problems, respiratory distress w/o cyanosis. LV volume overload and L to R shunting.

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17
Q

Auscultation, ECG and CXR associated with R-TGA

A

No murmur to Loud murmur (depends on shunt lesion)
RAD and RVH on ECG
Egg-shaped heart with narrow Stalk on CXR

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18
Q

Med/Surg Tx of D-TGA in neonate w/o sufficient shunting.

A

Prostaglandin Infusion- for patency of DA or stent placement
Balloon Septostomy- to create or increase ASD
O2
Tx of HF
Decrease PAP
Arterial Switch Operation- transecting PA and Aorta and reanastomosing to RV and LV

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19
Q

Anesthetic MGMT of TGA

Induction technique?

A

Inhalation or IV Induction
Ketamine 1-2mg/kg incrementally
Fentanyl 2-15mcg/kg incrementally
Rocuronium 1mg/kg

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20
Q

Anesthetic MGMT of TGA

Invasive lines?

A

Yes Arterial and CVP

Note- VSD closure needs bicaval cannulation, so use femoral venous line, not jugular

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21
Q

Anesthetic MGMT of TGA

Myocardial Ischemia concerns?

A

MI can occur after cross clamping is removed due to poor coronary anastomosis or air emboli in coronary artery- Increase CPP to flush out air. If no improvement, may need to go back on CPB to reassess anastomosis.

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22
Q

Anesthetic MGMT of TGA

____ to assess function of repair and presence of air

A

Echo

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23
Q

Anesthetic MGMT of TGA

Anticipate _______ HTN

A

Pulmonary HTN- can cause compression of Coronary Arteries and MI

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24
Q

Anesthetic MGMT of TGA

Inotropes?

A

More than likely

Use Dopamine, Epinephrine, and Milrinone

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25
Q

Anesthetic MGMT of TGA

LV will be _________, so be careful with ______

A

LV will be NONCOMPLIANT, be careful with FLUIDS- give slowly and in small amounts

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26
Q

Anesthetic MGMT of TGA

Coagulation is possible?

A

Yeap- often need antifibrinolytics

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27
Q

Anesthetic MGMT of TGA

Postoperatively, have high risk of:

A

dysrhythmias and conduction defects

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28
Q

Anesthetic MGMT of TGA

Maintenance- Keep PVR ____

A

Keep PVR DOWN

Deep GA will blunt reactive Pulm. HTN

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29
Q

Anesthetic MGMT of TGA

Avoid ____HR and ___ CO with limited myocardial reserve.

A

Avoid Decreased HR and Decreased CO

Control BP

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30
Q

Single vessel from the heart gives rise to both aorta and pulmonary artery.

A

What is Truncus Arteriosus?

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31
Q

Truncus Arteriosus is associated with ______Syndrome

A

DiGeorge

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32
Q

List the Clinical Features and Concerns of DiGeorge Syndrome (Table 15-4)

A

Absent of small thymus
T-Cell Abnormality w/ associated immunodeficiency
Hypoparathyroidism w/ associated hypocalcemia
Dysmorphic features, particularly a small mouth
Increased surgical morbidity and mortality
Irradiated blood products needed to prevent graft-vs-host disease

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33
Q

Mortality is high with Truncus Arteriosus, and surgery is performed _____

A

early in life

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34
Q

3 factors influencing mortality with TA.

A

Presence of:
Truncal Valve Stenosis
Coronary Abnormalities
Low Birth Weight

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35
Q

Describe the surgery for TA repair

A

Closure of VSD, disconnect pulmonary arteries, place graft between RV and PA to provide pulmonary blood flow.

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36
Q

Type 1 TA

A

Main PA and Aorta arises from BASE of truncus

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37
Q

Type 2 TA

A

R and L pulmonary arteries arise SEPARATELY from truncus, close to each other

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38
Q

Type 3 TA

A

Pulmonary arteries arise on OPPOSITE sides of truncus

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39
Q

Type 4 TA

A

PA branches are ABSENT, pulmonary blood flow from aortopulmonary collaterals.

40
Q

All forms of TA have mixing of _____ and ____ blood, with significant ____ to _____ shunting and ______overcirculation.

A

All forms of TA have mixing of oxygenated and deoxygenated blood, with significate L to R shunting and PULMONARY overcirculation

41
Q

TA leads to (4 things)

A

Cyanosis
Failure to Thrive
CHF
Pulmonary HTN

42
Q

Induction concerns with TA
Intubation may be _____ due to DiGeorge facial anomalies.
Do not _____ or ________ if not intubated

A

Intubations may be DIFFICULT

Do not PREOXYGENATE or HYPERVENTILATE if not intubated.

43
Q

Anesthetic MGMT of TA

IV induction agents:

A

Ketamine 1-2mg/kg
Fentanyl 2-4mcg/kg
Roc 1mg/kg

44
Q

Anesthetic MGMT of TA

Avoid _____ventilation and maintain O2 sat _____%

A

Avoid Hyperventilation

O2- 75-85%

45
Q

Anesthetic MGMT of TA

Keep DBP > ____ to perfuse coronary arteries

A

20mmHg

46
Q

Anesthetic MGMT of TA

Maintenance drugs

A

Fentanyl 20-50mcg/kg
Roc
Midazolam 0.1-0.2 mg/kg
+/- Volatile agent

47
Q

Anesthetic MGMT of TA

FiO2 -and avoid _________

A

Fio2 0.21

Avoid Hyperventilation

48
Q

Anesthetic MGMT of TA

Will probably require postop ____

A

ventilation

49
Q

Why do we avoid hyperventilation with TA?

A

Hyperventilation causes decreased PVR, leading to Increased Shunt and CHF

50
Q

What art the two types of Anomalous Pulmonary Venous Connections

A

Total and Partial

51
Q

Describe the difference between TAPVC and PAPVC.

A

TAPVC- All pulmonary veins insert into anomalous site

PAPVC- One or more pulmonary veins drain into either venous or right side of heart instead of the LA.

52
Q

What are the 3 types of TAPVC

A

Supracardiac
Cardiac
Infracardiac

53
Q

Describe Supracardiac TAPVC

A

Supracardiac- Pulmonary veins connect to SVC through an ASCENDING VERTICAL VEIN

54
Q

Describe Cardiac TAPVC

A

Cardiac- Pulmonary veins connect to RA through CORONARY SINUS

55
Q

Describe Infracardiac TAPVC

A

Infracardiac- Pulmonary veins connect to IVC through COMMON VEIN.

56
Q

S/Sx of TAPVC

A

CHF, Cyanosis, Respiratory Distress, and Tachypnea

57
Q

TAPVC
ECG
CXR
ECHO

A

TAPVC
ECG- RA and RV Enlargement
CXR- Cardiomegaly and Pulmonary Edema
ECHO- Will Identify Shunting Lesion, Cardiac Size and Ventricular Function

58
Q

Tx for TAPVC

A

Surgical Correction

Closing Shunting Lesions with a Patch

59
Q

Anesthesia MGMT of TAPVC

A

Induction Roc 1mg/kg, Fent 1-3mcg/kg IV
Volatile agents are rarely tolerated in obstructed TAPVC
Maintenance- Fent 20-50mcg in divided doses. Reduction of PVR will worsen pulmonary edema
Postop vent support. May need inotropes.

60
Q

Anatomic Features (4) of Hypoplastic Left Heart Syndrome HLHS

A

Hypoplastic LV
Mitral Stenosis or Atresia
Aortic Stenosis or Atresia
Hypoplastic Aortic Arch

61
Q

At birth, neonates with HLHS will present with (3)

A

Heart Failure
Shock
Cardiovascular Collapse

62
Q

ECG and CXR findings with HLHS

A

ECG- RAD, RVH

CXR- Cardiomegaly, prominent pulmonary vascular markings.

63
Q

Surgical Tx of HLHS

A

Convert to single-ventricle circulation where RV becomes single systemic ventricle and pulmonary blood flow moves passively from SVC and IVC (aka Fontan Circulation)

64
Q

Anesthetic management of HLHS

A
Balance PVR with SVR
Infuse Prostaglandin 
Use normal to high PaCO2 and Low FIO2
High dose Opioid technique
Venous Access through Femoral Vein
Hypothermia may be needed
Coagulation is possible, antifibrinolytics are used (TXA)
65
Q

Ventilation for HLHS

A

Maintain O2 Sat 75-85%
Avoid Hyperventilation- Increases pulmonary blood flow,
Sat >85% leads to hypoperfusion

66
Q

Maintenance drugs for HLHS

A

Fent 20-40mcg/kg
Rock 1mg/kg
Volatiles on CPB
Midazolam 0.1-0.2mg/kg

67
Q

HLHS Stage 2 correction occurs at _____ and Stage 3 correction at ______

A

6 months old

2-6 years old

68
Q

Long term outcome of HLHS

A

RV will fail and Pt will need transplant

69
Q

Describe Tricuspid Atresia (TA)

A

Absence or permanent closure of tricuspid valve

70
Q

Describe the different types of TA

A

I - Most common, normal relationship of great vessels to ventricles
II - D transposition of the Great Vessels
III- L transposition of the Great Vessels

71
Q

3 features of TA

A

RV is hypoplastic
ASD is present
Pulmonary Blood Flow is restricted d/t pulmonary stenosis or atresia

72
Q

TA- Will have ____to ____ shunting, with various degrees of cyanosis

A

R to L shunting

73
Q

Initial palliative procedure for TA

A

Blalock-Taussig Systemic to PA shunt

74
Q

Definitive procedure for TA

A

Bidirectional Glenn Shunt or Modified Fontan

75
Q

Sx of TA

50% of patients are symptomatic by _____ of life

A

24 hours

76
Q

Sx of TA

If Pt has decreased pulmonary blood flow:

A

R-to-L shunting and Cyanosis
Tachypnea
Prominent A Waves
Failure to Thrive

77
Q

Sx of TA

If Pt has increased pulmonary blood flow:

A

minimal cyanosis
but will still have
tachypnea, tachycardia, hepatomegaly, prominent A waves, feeding difficulties and CHF

78
Q

TA
Auscultation
ECG
ECHO

A

TA
Auscultation- Holosystolic murmur of a VSD or continuous murmur of PDA
ECG- LAD, LVH, RA enlargment
ECHO- absent of closed tricuspid valve, enlarged chambers (except RV), RVOT obstruction, PAP, flow moving across VSD.

79
Q

Development of Severe Pulmonary HTN due to L-to-R shunt, where the increased PVR eventually causes shunt reversal to R-to-L shunt

A

What is Eisenmenger Syndrome

80
Q

Sx of Eisenmenger Syndrome

A

Increased Hypoxia with Decreased Exercise Tolerance
Enlarged RA and RV with Arrhythmias
Hyperviscosity due to Hypoxia

81
Q

Eisenmenger Syndrome
ECG
CXR

A

ECG - RVH

CXR- Prominent pulmonary vessels

82
Q

Anesthetic MGMT of Eisenmenger Syndrome

A

Similar to management of other forms of pulm. HTN
Avoid Insufflation- Worsens R-to-L shunt
PVR is fixed and does not respond to SVR changes
Keep SVR at preop levels

83
Q

Overall CHD anesthetic implications

Is air bubbles good?

A

Nope

84
Q

Overall CHD anesthetic implications

Qp:Qs > 1.5:1 limit _____ blood flow to prevent ___failure due to volume overload

A

pulmonary

RV

85
Q

L to R Shunts

Little affect on onset of IV and inhalational agents as long as ________ is maintained

A

CO

86
Q

L to R shunts

Pts with elevated pulmonary blood flow- Maintain or slightly increase ______

A

PAP

87
Q

L to R shunts

Minimize agents that ___SVR or ____PVR

A

Increase SVR or Decrease PVR

88
Q

R to L shunts
These favor R-to-L shunting
__PVR, ___RVOTO (infundibula spasm), ___SVR

A

Increased PVR
Increased RVOTO
Decreased SVR
all favor R to L shunting

89
Q

With RVOTO, changes in _____ does not alter Qp:Qs ratio, while changes in _____ does.

A

PVR does not alter

SVR does

90
Q
Anaphylactic/neurogenic shock
Anemia
Cirrhosis
Vasodilators 
Anesthetic Agents
Histamine
Alpha Blockers
Ganglionic Blockers
All Do What?
A

Decrease SVR

91
Q
Increase or Decrease PVR
Syndromes of low CO
Hypovolemia
Cardiogenic Shock
Hypothermia
Vasoconstrictors
All Do What?
A

Increase SVR

92
Q
Increase or Decrease PVR
100% O2
Hypocarbia
Normothermia
Low mean AW pressures or SV
A

Decrease

93
Q
Increase or Decrease PVR
Hypothermia
A1 agonists
Sympathetic Stimulation
Increased SVR
A

Decrease

94
Q
Increase or Decrease PVR
Acidosis
Hypoxemia
Hypervarbia
High mean AW pressure
Catecholamine release
A

Increase

95
Q
Increase or Decrease PVR
Avoidance of catecholamine release
Low mean AW pressures or SV
Hypothermia
Increased SVR
Hypocarbia
A

Decrease

96
Q
Meds like neo, ketamine, N2O
Hypothermia
B2 agonist
All A1 agonists
Deep GA
NA
Decreased SVR
A

Increase

and yes, this table lists hypothermia and A1 agonist as both increasing and decreasing PVR. What a fuck!

97
Q

Who can go fuck right off?

A

You guessed it!!!