4. Congenital Heart Disease Flashcards

1
Q

atresia

A

opening in the body that has been narrowed or closed of valves

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2
Q

coarctation

A

narrowing

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3
Q

cor pulmonale

A

right ventricular hypertrophy (RVH)

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4
Q

infundibulum

A

muscle below the pulmonic valve

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5
Q

what can infundibular spasm cause?

A

worsens pulmonic stenosis

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6
Q

the infundibulum develops abnormally in what condition

A

tetralogy of fallot

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7
Q

palliative surgery

A

operation intended to decrease severity of symptoms until pt can tolerate operation to fix condition

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8
Q

when is palliative surgery particularly common?

A

pediatric heart operations

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9
Q

palliative operation examples

A

BT shunt in order to keep child alive until they can tolerate open heart surgery for TOF correction

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10
Q

BT shunt

A

blaylock-taussig shunt

artificial connection between aortic arch and pulmonary artery (artificial ductus arteriosus)

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11
Q

infective endocarditis

A

infection of the heart chambers or valves

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12
Q

what is a BT shunt

A

artificial connection between aortic arch and pulm artery

artificial patent ductus arteriosus

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13
Q

what is necessary for pts with infective endocarditis?

A

prophylactic antibiotics have been recommended for patients who need to undergo operations
(unrepaired, palliated or corrected they all need it)

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14
Q

common risk of CHD pts

A

infective enxocarditis

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15
Q

compensating polycythemia

A

abnormally high Hct

-pts with congenital heart disease suffer from hypoxia and they make more red blood cells to compensate

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16
Q

what are pts with compensating polycythemia more at risk for?

A

thrombosis

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17
Q

pts with CHD suffer from

A

hypoxia

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18
Q

pts with CHD compensate for hypoxia by

A

incr RBC
== incr Hct

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19
Q

treatment for polycythemia

A

fluid replacement
brings Hct down towards normal

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20
Q

paradoxical embolism

A

embolism that travels to the left side of the heart via ASD or VSD and is in arterial circulation (stroke!!)

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21
Q

where does a normal embolism travel

A

travel to the lungs and stay on the right side of the heart/pulm artery

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22
Q

what do anesthetists need to be very vigilant about when there is a risk for paradoxical embolism?

A

air bubbles in IV line

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23
Q

cardiac shunt

A

abnormal blood flow pathways from one side of the heart to another

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24
Q

which pts have an increased risk of paradoxical embolism with IV air injection?

A

pts w/ Right to Left shunt across ASD/VSD

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25
Q

what are the 4 possible cardiac shunts?

A

patent foramen ovale (PFO)
patent ductus arteriosus (PDA)
Atrial septal defect (ASD)
ventricular septal defect (VSD)

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26
Q

affect of increase SVR on cardiac shunt

A

more left to right shunt

increase pulmonary blood flow

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27
Q

affect of decrease in SVR on cardiac shunt

A

more right to left shunt

worsen hypoxemia

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28
Q

affect of increase PVR on cardiac shunt

A

more right to left shunt

worsen hypoxemia

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29
Q

affect of decrease PVR on cardiac shunt

A

more left to right shunt

increase pulmonary blood flow

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30
Q

are patients with right to left or left to right shunts more prone to have compensating polycythemia?

A

pts with right to left shunt because they have more hypoxia

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31
Q

problems with R-L shunt

A
  1. decr pulm BF
  2. hypoxia
  3. paradoxic embolism
  4. compensating polycythemia
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32
Q

R-L shunt anesthetic induction effects

A

inhalational slower
IV faster

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33
Q

R-L shunt worsened by

A

incr PVR
decr SVR

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34
Q

R-L shunt management

A
  1. decr PVR
  2. mx/incr SVR
  3. prevent IV air bubbles
  4. saline epidural LOR
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35
Q

what increases PVR?

A

hypoventilation

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36
Q

what can we do to decrease PVR?

A

higher FiO2

modest hyperventilation

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37
Q

what decreases SVR?

A

anesthetic agents (regional and general) titrate slowly

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38
Q

what induction agent can be used to maintain SVR?

A

ketamine

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39
Q

can you do single shot spinal anesthetic with right to left cardiac shunt?

A

no it is contraindicated

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40
Q

in a right to left cardiac shunt how will the inhalational induction speed change?

A

slower
blood from R will enter L without picking up agent
dilutes vapor in arterial blood

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41
Q

in a right to left cardiac shunt how will the intravenous induction speed change?

A

faster

drugs get to the L side of the heart faster

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42
Q

in a patient with right to left cadiac shunt what is the alteration to epidural catheter placement?

A

MUST use saline for loss of resistance

for risk of air in vein getting into systemic circulation

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43
Q

what does the left to right cardiac shunt do to pulmonary blood flow?

A

promotes excessive pulmonary blood flow

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44
Q

what can excessive pulmonary blood flow lead to?

A

increase PVR
dyspnea
incr P on right side of heart
right ventricular hypertrophy
right heart failure

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45
Q

what can the L-to-R shunt/pulm congestion be worsened by?

A

decreases in PVR
increases in SVR

increases in SVR

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46
Q

How is the anesthetic inhalation induction effected in the L-to-R shunt?

A

minimally (if any)

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47
Q

how is the anesthetic intravascular induction affected in the L-to-R shunt?

A

slightly prolonged

it is slightly diluted

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48
Q

how should we change preload in a L-to-R shunt?

A

preload maintained to limit the amount of left to right shunting

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49
Q

L-R cardiac shunt management

A
  1. mx/incr preload
  2. mx/incr PVR
    • low FiO2
    • slight hypoventilation (PaCO2 40-50 mmHg)
  3. decr SVR
  4. prevent IV air bubbles
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50
Q

eisenmengers syndrome

A

eventually the pressure in the right heart becomes large enough that the shunt converts to a right to left shunt.

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51
Q

is eisenmengers syndrome more likely with high or low PAP?

A

higher PAP

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52
Q

which do you need to be extremely vigilant in preventing intravenous air bubbles? R-to-L shunt or L-to-R shunt?

A

BOTH

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53
Q

when managing a congenital heart disease patient what should you ask?

A

does this pt have too much pulm blood flow OR not enough pulm blood flow?
what can I do to correct that?

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54
Q

if the patient has too much pulm blood flow how can we decrease left to right shunting?

A

increase PVR
hypoventilate
lower fiO2
decrease SVR

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55
Q

if the patient doesnt have enough pulm blood flow how can we improve left to right shunting?

A

increase SVR
decrease PVR
hyperventilate
increase Fio2

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56
Q

patent ductus arteriosus

A

ductus arteriosus never closed after birth

**some congenital heart disease having a PDA is necessary for survival

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57
Q

before birth how does the blood flow through the ductus arteriosus?

A

right to left shunt

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58
Q

after birth how does the blood flow through the ductus arteriosus?

A

left to right shunt

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59
Q

if no other defects are present how should blood flow through the PDA?

A

left to right
(from aorta to pulm artery)
increase pulmonary BF/congestion

increasing pulm blood flow

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60
Q

right to left shunt acorss PDA

A
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61
Q

what is different about a patient with a PDAs blood pressure?

A

low diastolic blood pressure

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62
Q

anesthetic management of pt with PDA 3

A

1 measures to decrease left to right shunt and limit pulmonary congestion
2 up to 3 doses of indomethacin to close PDA
3 invasive monitoring is not essential in uncomplicated PDA

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63
Q

what should the fiO2 and PaCO2 be for a patient with a PDA?

A

low FiO2

PaCO2 40-50mmHg

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64
Q

preductal circulation

A

blood flow to areas of the body proximal to the ductus arteriosus

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65
Q

postductal circulation

A

blood flow to areas of the body distal to the ductus arteriosus

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66
Q

if the ductal connection is distal to the subclavian then the preductal circulation includes 3

A
head (left common carotid)
right arm (brachiocephalic)
left arm (left subclavian)
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67
Q

if the ductal connection is distal to the subclavian then the postductal circulation includes

A

lower extremities and abdomen

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68
Q

if the ductal connection is proximal to the subclavian then the preductal circulation includes 2

A

head
R arm

right arm

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69
Q

if the ductal connection is proximal to the subclavian then the postductal circulation includes

A

left arm
lower extremities/abdomen

lower extremities and abdomen

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70
Q

is paradoxic air embolism more or less likely with ductus arteriosus porximal to subclavian

A

more risk ofr air embolism

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71
Q

where should you take a preductal blood sample from?

A

RIGHT radial artery

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72
Q

where should you take a postductal blood sample from?

A

artery in leg: femoral, dorsalis pedis, or posterior tibial artery

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73
Q

if preductal oxygen saturation is significantly different from postductal oxygen saturation what does that suggest?

A

a heart defect with right to left shunting across a PDA

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74
Q

aortopulmonary window

A

hole between aorta and pulm artery

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75
Q

aortopulmonary window shunt

A

Left to Right

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76
Q

aortopulmonary window symptoms

A

pulmonary congestion

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77
Q

Patient foramen ovale

A

foramen ovale never closed after birth
(10-25%)

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78
Q

how is blood expected to shunt in a PFO?

A

left to right

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79
Q

anesthetic management for patient with PFO 2

A

1 decrease left to right shunting to limit pulmonary congestion
2 should not allow any air in IV

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80
Q

atrial septal defect

A

ASD is hole between right and left atria

LARGER PFO

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81
Q

anesthetic management of pt with ASD

A

higher preload
elevated PVR
lower SVR

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82
Q

ventricular septal defect

A

hole between left and right ventricles

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83
Q

anesthetic management of pt with VSD

A

higher preload
elevated PVR
lower SVR

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84
Q

Ebsteins Anomaly

A

malformation of tricuspid valve that results in tricuspid regurg= high right atrial pressure and right atrial enlargement

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85
Q

what other defect is more likely to occur when the pt has ebsteins anomaly

A

ASD (perforated foramen ovale)

Right to left shunt

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86
Q

how does blood shunt with ebsteins anomaly and ASD?

A

right to left

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87
Q

anesthetic management of ebsteins anomaly

A
decrease PVR
  -hyperventilate
  - incr FiO2
increase SVR
avoid air bubbles in IV line
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88
Q

what will the patients SpO2 be for ebsteins anomaly with ASD?

A

lower b/c it is mixed venous/arterial blood

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89
Q

what would the hypoxemia in ebsteins anomaly cause?

A

compensating polycythemia

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90
Q

eisenmengers syndrome

A

left to right shunt reverses into a right to left shunt

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91
Q

why does flow reverse in eisenmengers syndrome?

A

pressure on the right side of the heart increases due to RV dilation/strengthening

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92
Q

clinical implication of eisenmengers syndrome

A

once it develops cyanosis ensures with varying degrees of heart failure and pulm congestion

HIGH RISK for surgery

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93
Q

anesthetic management for left to right shunts that have possible eisenmengers physiology 3

A

1 maintain SVR
2 maintain PVR
3 fine balance managing oxygenation

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94
Q

high FiO2 eisenmengers

A

leads to RHF

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95
Q

low FiO2 eisenmengers

A

leads to cyanosis

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96
Q

interrupted aortic arch

A

aorta isnt fully developed and there is a gap between ascending and descending aorta

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97
Q

in order for a pt with interrupted aortic arch to survive what two other defects must they have?

A

PDA
VSD

ASD or VSD

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98
Q

what direction do you want blood flow through VSD in interrupted aortic arch?

A

L to R shunting through VSD

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99
Q

where does the oxygenated blood from the left ventricle perfuse? interrupted aortic arch pt

A

right upper extremity and part of the head

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100
Q

where does blood flow to the lower extremities come from in a pt with interrupted aortic arch?

A

right ventricle through PDA

mixed venous/arterial blood

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101
Q

how do the blood pressure, pulse and spO2 differ from right arm to left arm

A

normally higher in the right because the interruption is usually proximal to the left subclavian

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102
Q

anesthetic management for interrupted aortic arch

A
keep ductus arteriosus patent
maintain preload
maintain SVR
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103
Q

what are the 4 defects of tetralogy of fallot?

A

1 pulmonic stenosis (right ventricular outflow tract obstruction RVOT)
2 right ventricular hypertrophy
3 VSD
4 overriding aorta

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104
Q

in tetralogy of fallot how does the blood shunt?

A

right to left across the VSD

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105
Q

what type of blood is perfusing the body through the overriding aorta in TOF patient

A

dexoy blood from RV
oxy blood from LV

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106
Q

SaO2 in pt with overriding aorta

A

Lower SaO2 due to mixed venous/arterial perfusion

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107
Q

what are the two ways for blood to get to the lungs in patients with TOF?

A
  1. stenotic pulmonary valve
  2. L - R shunting across PDA

BF through both is limited

PDA (left to right shunt)

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108
Q

is a PDA necessary for life sustaining pulm blood flow in a patient with TOF?

A

YES

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109
Q

problems w/TOF

A
  1. pulm BF is limited
  2. R - L shunting causing hypoxia
  3. PDA is necessary
110
Q

indundibular spasm ______ hypoxia

A

worsens hypoxia symptoms

111
Q

tet spell

A

sudden onset life threatening hypoxic spell in pts with TOF

112
Q

what are tet spells caused by

A

infundibular spasm which triggers R - L shunting

subsequent increase in right to left shunt

113
Q

pt that is experiencing a tet spell can do what at home to help?

A

squat or use valsalva maneuver

114
Q

causes of infundibular spasm 2

A
  1. sympathetic stimulation (crying)
    • tachycardia
    • incr contractility
    • hyperventilation
  2. hypovolemia
115
Q

why does spontaneous hyperventilation cause an infundibular spasm?

A

decreases intrathoracic pressure with increases venous return and increases right to left shunt

116
Q

how does hypovolemia cause a infundibular spasm?

A

underfilled heart decreases diameter of RVOT

117
Q

treatment for tet spell 7

A

1- 100% O2
2 - mod hyperventilation to decr PVR
3 - ketamine incr SVR
4 - phenylephrine incr SVR
5 - fluid bolus to incr preload
6- knee to chest
7 - beta blocker
8 - avoid beta agonists

118
Q

why does knee to chest position help during tet spell?

A

incr SVR which forces blood into the pulmonary system

119
Q

why does a beta blocker help during tet spell?

A

decr HR improves diastolic filling/preload
incr heart size
incr RVOT

120
Q

why should you avoid beta agonists during tet spell?

A

incr contractility which worsens infundibular spasm

121
Q

anesthetic managment for tetralogy of fallot 7

A

1- PDA kept open
2- R-L shunt managment
3 - avoid hypotension on induction
4 - consider phenylephrine/ketamine to treat hypotension
5 - minimize sympathetic stimulation
6 - mx or incr preload

122
Q

what medicine is given to keep the PDA open?

A

PGE1 (prostaglandins)

123
Q

how should you induce TOF pts?

A

IV induction with ketamine

124
Q

why do we mx or incr preload in TOF pts?

A

to help keep the RVOT open

125
Q

TOF surgical repair: initial surgery

A

palliative BT shunt to improve pulm BF and allow for PDA to close

126
Q

TOF surgical repair: later surgery

A

close VSD
repair/replace pulmonic valve

127
Q

Transposition of Great Arteries (TGA)

A

RV pumps into aorta
LV pumps into pulmonary artery

128
Q

without shunts where is the deoxy and oxy blood in TGA

A

oxy blood in pulmonary system
dexoy blood in systemic systm

129
Q

most common type of TGA

A

D-TGA

130
Q

D-TGA

A

aorta positioned to the right and front of the pulmonary artery

131
Q

L-TGA

A

aorta to the left and front of pulmonary artery

132
Q

how many shunts must be present in TGA

A

at least 2

133
Q

which shunts are present in TGA

A

R - L
L - R

134
Q

TGA R-L shunt

A

shunt blood towards the lungs
(anatomy is reversed)

135
Q

TGA L-R shunt

A

shunt blood away from lungs
(anatomy is reversed)

136
Q

problems with TGA

A
  1. decr BF to lungs
  2. low SpO2
  3. possible compensating polycythemia
  4. air bubbles are catastrophic
137
Q

TGA inhalational induction

A

slower
delayed

138
Q

TGA IV induction

A

faster
more potent

139
Q

TGA managment

A
  1. keep PDA open w/PGE1
  2. incr preload
  3. mx/incr SVR
  4. decr PVR
  5. IV induction w/ketamine
  6. TIVA mx
  7. avoid air bubbles
140
Q

which shunt do we want to favor in TGA

A

R - L shunting
(shunting blood towards the pulmonary system on the L)

141
Q

which induction drug for TGA

A

ketamine to incr SVR

142
Q

why is TIVA preferred for TGA

A

VA are cardiac depressants
inhaled anesthetics dont reach the brain as well in TGA

143
Q

TGA surgical repair

A

baffle (atrial switch)

144
Q

baffle

A

divers venous blood to L heart to be pumped out of LV to lungs

145
Q

atrial switch

A

LA functions as normal RA

146
Q

2 types of baffle/atrial switch procedures

A

mustard baffle
senning baffle

147
Q

mustard baffle uses

A

synthetic material

148
Q

senning baffle uses

A

pts own tissue

149
Q

most common repair for TGA

A

arterial switch

150
Q

arterial swtich

A

aorta and pulmonary artery are disconnected and reconnected to the proper ventricles

151
Q

TAPVR

A

pulmonary veins empty into RA
(all blood returns to RH)

152
Q

TAPVR pts have

A

ASD for R-L shunting

153
Q

TAPVR problems

A
  1. pulmonary congestion
  2. R - L shunting
  3. postop CO compromised
154
Q

TAPVR has ________ pulmonary BF

A

increased pulmonary BF
(even though it has R-L shunting)

155
Q

TAPVR R-L shunt does not cause

A

hypoxia
mixing of venous/arterial blood

156
Q

why does TAPVR R-L shunt not incr hypoxia?

A

blood is mixed before the blood is shunted

157
Q

what should you avoid in TAPVR?

A

air bubbles

158
Q

what should you give post-op in TAPVR pts?

A

inotropes to prevent low CO and pulm HTN

159
Q

pulmonary atresia

A

pulmonary valve is blocked

160
Q

pulmonary atresia pts need

A
  1. R-L shunt through ASD/VSD
  2. PDA
161
Q

pulm atresia problems

A
  1. pulmonary BF limited
  2. R-L shunting
  3. ventricles become overloaded – CHF symptoms
162
Q

pulm atresia management

A
  1. Keep PDA open w/PGE1
  2. R-L shunt managment
  3. Give inotropes (if CHF symtoms)
163
Q

truncus arteriosus

A

aorta and pulmonary artery arise from same trunk
trunk acts like overriding aorta

164
Q

trunk in truncus arteriosus is supplied by which ventricles

A

both RV and LV

165
Q

truncus arteriosus shunts

A

L - R (LV -> pulmonary artery)
R-L (RV -> aorta)

166
Q

in truncus arteriosus what is the body perfused with

A

mixed venous/arterial blood

167
Q

which system receives the majority of the blood flow in truncus arterious

A

lungs
(excessive pulmonary blood flow)

168
Q

truncus arteriosus management

A

limit pulm BF by mx an appropriate SVR:PVR ratio

169
Q

incr flow to pulmonary system in truncus arteriosis will do what?

A

decr flow to systemic circulation

170
Q

Atrioventricular canal defects

A
  1. ASD
  2. VSD
  3. one atrioventricular valve
171
Q

Atrioventricular canal defect AKA

A

endocardial cushion defect
common atrioventricular canal (CAVC)
septal defect

172
Q

Atrioventricular canal shunts

A

R-L
L-R

173
Q

which shunt is more prominent in atrioventricular canal defect?

A

L-R shunting

174
Q

pulm BF in AV canal defect

A

excessive pulm BF

175
Q

what blood is pt perfused with in AV canal defect?

A

mixed venous/arterial blood
- low SpO2
- compensating polycythemia

176
Q

AV canal defect managment

A
  1. limit pulm BF by incr PVR
  2. avoid air bubbles
  3. give inotropes for CHF symptoms
177
Q

hypoplastic left heart syndrome (HLHS)

A

severely underdeveloped LH structures incapable of pumping blood to body

178
Q

HLHS defects

A
  1. underdeveloped LV
  2. small/closed mitral/aortic valves
  3. stenotic ascending aorta
179
Q

what is required in HLHS?

A

ASD (L - R shunt)
PDA (RV - aorta)

180
Q

if pulmonary BF increases in HLHS

A

CO decreases
higher % of blood goes to lungs instead of aorta

181
Q

if pulmonary BF decreases in HLHS

A

CO increases
higher % blood through PDA to aorta

182
Q

how do you incr CO in HLHS?

A

incr PVR

183
Q

4 main problems in HLHS?

A
  1. overworked RV (volume overload)
  2. too much pulm BF
  3. reduced CO
  4. perfusion w/mixed venous/arterial blood (low Spo2) through PDA
184
Q

management of HLHS

A
  1. keep PDA open w/PGE1
  2. limit pulm BF
185
Q

what is controversial and contraindicated in pts with HLHS? Why?

A

supplemental O2
because it will decr CO and incr pulm congestion

186
Q

what do HLHS pts need to stay alive?

A

immediate palliative surgery while waiting for heart transplant

187
Q

norwood procedure treats

A

HLHS

188
Q

norwood procedure

A

RV is attached to aorta
BT shunt placed

189
Q

what does attaching the RV to the aorta do?

A

incr CO
decr BF to lungs (0 pulm BF)

190
Q

what does a BT shunt do in norwood procedure?

A

allow pt to receive pulmonary BF

191
Q

BT shunt blood flow in norwood

A

L - R from brachiocephalic artery to pulmonary artery

192
Q

norwood procedure fixes what problems?

A

excessive pulm BF
CO

193
Q

norwood procedure does not fix?

A

hypoxia
overworked RV

194
Q

when does the norwood procedure take place?

A

during 1st week of life

195
Q

sano shunt

A

modified norwood
connects RV and pulm artery

196
Q

sano shunt benefits

A

possible improved survival
better coronary diastolic perfusion

197
Q

BT shunt cons

A

lungs steal some blood from coronaries during diastole

198
Q

Hemi-Fontan (Bidirectional Glenn) procedure for HLHS

A
  1. SVC attached to pulm artery
  2. BT shunt removed
199
Q

hemi-fontan benefits

A

allows BF to lungs w/o BT shunt
reduced RV strain

200
Q

problems after hemi-fontan

A

still have incr RV strain
still have hypoxia due to mixed perfusion

201
Q

fontan procedure

A

IVC connected to pulm artery
(bypass RA)

202
Q

blood flow after fontan

A

venous return bypass right heart
- no RV strain
blood flow to lungs is passive

203
Q

what is the RV pumping after fontan

A

RV pumps arterial blood to body

204
Q

management for pts w/fontan physiology

A
  1. mx preload
  2. slight hypoventilation
  3. SV is preferred
  4. arterial and central venous monitoring
205
Q

why do you need to mx preload in fontan?

A

pulmonary BF is passive so its harder to get blood into lungs with hypovolemia

206
Q

why is hypoventilation preferred in fontan?

A

incr pulm BF
incr CO

hypoventilation
= hypercarbia
= decr cerebral vascular resistance
= incr cerebral BF
= incr cerebral venous drainage
= incr pulm BF

207
Q

which pts are impacted by protein losing enteropathy?

A

fontan pts

208
Q

PLE

A

excessive loss of proteins across the intestinal mucosa

209
Q

PLE etiology

A

unkown

210
Q

PLE develops

A

5 years after fontan in 30% pts

211
Q

PLE symptoms

A

peripheral edema
ascites
pleural
pericardial effusions

212
Q

PLE treatment

A

digoxin
diuretics
parenteral albumin
SVR reduction
cardiac transplant

213
Q

tricuspid atresia

A

blocked tricuspid valve

214
Q

tricuspid atresia requires ____ for blood to get to lungs

A

ASD
and
VSD

215
Q

tricuspid atresia problems

A
  1. underdeveloped RV
  2. overworked LV
  3. hypoxia due to mixed perfusion
  4. too much or too little pulm BF
216
Q

which ventricle is doing all the work in tricuspid atresia

A

LV

217
Q

what can pts die of if tricuspid atresia is not corrected?

A

LHF

218
Q

when would tricuspid atresia have too much pulm BF?

A

large VSD
no pulm stenosis

219
Q

when would tricuspid atresia have too little pulm BF?

A

small VSD
and/or
pulm stenosis

220
Q

incr PVR in pulm atresia will

A

decr pulm BF
incr CO

221
Q

decr PVR in pulm atresia will

A

incr pulm BF
decr CO

222
Q

management of tricuspid atresia with reduced pulm BF

A

decr PVR

223
Q

management of tricuspid atresia with incr pulm BF

A

incr PVR

224
Q

tricuspid atresia surgical option with reduced pulm BF

A

palliative BT shunt

225
Q

tricuspid atresia surgical option with incr pulm BF

A

palliative pulmonary artery banding

226
Q

final surgical repair option for tricuspid atresia

A

fontan procedure

227
Q

fontan procedure results in tricuspid atresia

A
  1. decr LV strain
  2. oxygenated perfusion
  3. incr reliability of BF to lungs
228
Q

double outlet right venticle

A

right ventricle supplies both the pulmonary artery and aorta

229
Q

double outlet right ventricle needs

A

VSD to allow oxy blood to get to RH

230
Q

problems with double outlet RV

A
  1. RV overworked
  2. hypoxia due to mixed perfusion
  3. possible pulm stenosis
231
Q

double outlet RV BF w/pulm stenosis

A

no excessive pulmonary BF

232
Q

double outlet RV BF no pulm stenosis

A

excessive pulm BF

233
Q

double outlet RV w/pulm stenosis managment

A

decr PVR

234
Q

double outlet RV no pulm stenosis

A

incr PVR

235
Q

decr SVR in double outlet RV will

A

decr pulm BF

236
Q

coarctation of aorta

A

narrowing of aorta distal to left subclavian artery

237
Q

problems with coarctation of aorta

A
  1. decr CO
  2. aortic dissection risk
  3. BP different between lower and upper extremities
238
Q

decr CO in coarctation of aorat causes

A

poor peripheral perfusion
metabolic acidosis
high afterload
CHF
aortic regurge

239
Q

BP in coarctation of aorta in extremiteis

A

lower extremities will have lower BP compared to upper extremities

240
Q

management of coarctation of aorta

A
  1. mx preload (forward flow)
  2. low-normal contractility
  3. low-normal HR
  4. avoid bradycardia
  5. mx SVR
241
Q

how is CO increased in coarcation of aorta

A

HR-dependent

242
Q

why is hypotension bad in coarcation of aorta

A

perfusion will be seriously compromised

243
Q

Shone’s syndrome

A

Left-heart defects
1. coarctation of aorta
2. sub-aortic obstruction
3. supravalvular mitral ring (mitral stenosis)
4. parachute mitral valve (regurge)

244
Q

Shone’s syndrome problems

A
  1. smaller LV
  2. decr LV function
  3. pulm HTN
245
Q

Shone’s syndrome surgical intervention

A
  1. aortic arch repair
  2. mitral valve replacement
  3. aortic valve replacement (Ross procedure)
246
Q

vascular rings

A

abnormal development of aortic arch that encircles trachea and/or esophagus

247
Q

vascular rings leads to

A

tracheal compression
dyspnea
dysphagia

248
Q

vascular rings AKA

A

double aortic arch

249
Q

vascular ring surgery is typically

A

thoracotomy
(sometimes sternotomy w/bypass)

250
Q

what size and type ETT w/vascular rings

A

smaller
armored (avoid compression)

251
Q

can you extubate w/vascular rings?

A

if tracheomalacia and stenosis are not anticipated

252
Q

vascular rings induction

A

inhalational w/spontaneous ventilation

253
Q

what is avoided in vascular rings pts?

A

NMB agents
- only given when airway and PPV have been assessed

254
Q

dextrocardia

A

heart is on right side of body

255
Q

2 types of dextrocardia

A

situs inversus (most common)
situs solitis

256
Q

situs inversus

A

reversal of normal positions of abdominal organs and heart

less comorbidities

257
Q

situs solutus

A

normal abdonminal organs
reverse heart

more comorbidities

258
Q

what should you change for dextrocardia pts?

A

ECG lead and defib paddles are reversed

259
Q

Ross procedure indication

A

children w/diseased aortic valve

260
Q

why are prosthetic valves bad in children

A
  1. artificial valves not small enough
  2. artificial valves dont grow w/child
  3. incr risk of aortic stenosis w/aging
  4. requires anticoags for life
261
Q

Ross Procedure

A

aortic valve is replaced w/pts pulmonic valve

pulmonic valve is replaced with homograph from cadaver

262
Q

Ross advantages

A

near-zero embolic risk
valve grows with child

263
Q

Ross disadvantages

A

pulmonary homograft will develop regurge or stenosis in 15-20 years requiring another surgery

264
Q

considerations in pts with repair CHD

A

tailor anesthetic to current heart function of pt as determined by diagnostic tests

265
Q

ductal dependent systemic BF

A

PDA required for adequate systemic BF

266
Q

ductal dependent systemic BF examples

A

interrupted aortic arch
HLHS

267
Q

pts w/ductal dependent systemic BF typicall have

A

LVOT

268
Q

ductal dependent pulmonary BF

A

PDA required for adequate pulm BF

269
Q

ductal dependent pulm BF examples

A

TOF
pulmonary atresia

270
Q

ductal dependedn pulm BF pts typically have

A

RVOT