Unit 11 - Congenital Heart Defects Flashcards

1
Q

where does fetal gas exchange occur

A

placenta

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2
Q

Carries oxygenated blood from the mother to the fetus

A

umbilical vein

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3
Q

Carry deoxygenated blood from the fetus to the mother

A

umbilical artery (1)

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4
Q

Shunts blood from the umbilical vein to the IVC (bypasses liver)

A

ductus venousus

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5
Q

Shunts blood from the RA to the LA (bypasses lungs)

A

foramen ovale

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6
Q

Shunts blood from the pulmonary artery to the aorta (bypasses lungs)

A

ductus arteriosus

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7
Q

6 ways fetal circulation differs from adult circulation

A
  1. placenta is organ of respiration
  2. circulation arranged in parallel
  3. R-L shunting across foramen ovale and ductus arteriosus
  4. SVR is low
  5. PVR is high
  6. minimal pulmonary blood flow
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8
Q

fetal organ of respiration

A

placenta

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9
Q

purpose of foramen ovale

A

shunt blood from RA to LA

Oxygen-rich blood bypasses the lungs and is preferentially delivered to the heart and developing brain

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10
Q

purpose of foramen ovale

A

shunt blood from RA to LA

Oxygen-rich blood bypasses the lungs and is preferentially delivered to the heart and developing brain

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11
Q

purpose of ductus arteriosus

A

shunt blood from pulmonary trunk to aorta

Lower oxygen blood bypasses the lungs and is delivered to the lower body

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12
Q

purpose of ductus venosus

A

allows oxygen-rich blood from placenta to bypass liver

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13
Q

when does the foramen ovale close

A

functional closure: when LAP > RAP (cord clamping increases SVR)

anatomic closure: 3 days

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14
Q

when does the ductus arteriosus close

A

functonal closure: SVR > PVR (increased PaO2 & decreased prostaglandins from placenta)

Anatomic closure: several weeks

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15
Q

adult remnant of foramen ovale

A

fossa ovalis

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16
Q

adult remnant of ductus arteriosus

A

ligamentum arteriosum

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17
Q

when does the ductus venosus close

A

anatomic closure when umbilical cord is clamped

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18
Q

adult remnant of ductus venosus

A

Ligamentum venosum

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19
Q

% of adult population with PFO

A

30%

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20
Q

complication of PFO

A

Increases risk of paradoxical air embolism (embolus travels to brain instead of lungs)

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21
Q

meds that can open or close PDA

A

opens: prostaglandin E1 (PGE1)
closes: indomethacin (prostaglandin synthesis inhibitor)

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22
Q

plays a key role in trauma, where a rapid deceleration tears the ligament & results in partial or complete aortic dissection

A

Ligamentum arteriosum

adult remnant of ductus arteriosus

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23
Q

plays a key role in trauma, where a rapid deceleration tears the ligament & results in partial or complete aortic dissection

A

Ligamentum arteriosum

adult remnant of ductus arteriosus

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24
Q

size and direction of shunt depends on what 3 factors

A
  1. Ratio of PVR to SVR
  2. Pressure gradients between cardiac chambers or arteries involved
  3. Compliances of cardac chambers
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25
Q

how does ratio of PVR to SVR affect direction of shunt

A

R - L shunt occurs when PVR is > SVR
L - R shunt occurs when SVR is > PVR

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26
Q

causes of increased PVR

A
  • hypercarbia
  • hypoxemia
  • acidosis
  • collapsed alveoli
  • Trendelenburg
  • hypothermia
  • vasoconstrictors
  • increased SNS tone
  • light anesthesia
  • pain
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27
Q

causes of decreased PVR

A
  • hypocarbia
  • adequate oxygenation
  • alkalosis
  • hemodilution
  • vasodilators
  • nitric oxide
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28
Q

causes of increased SVR

A
  • vasoconstrictors
  • fluid bolus
  • increased SNS tone
  • pain
  • anxiety
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29
Q

causes of decreased SVR

A
  • volatiles
  • propofol
  • decreased SNS tone
  • hemodilution
  • sepsis
  • anaphylaxis (histamine release, vasodilation, capillary leak)
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30
Q

patho of cyanotic shunts

A

↓ pulmonary blood flow = hypoxemia, LV volume overload, LV dysfunction

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31
Q

5 examples of R-L cardiac shunts (5 T’s)

A
  1. Tetralogy of Fallot (most common)
  2. Transposition of the great arteries
  3. Tricuspid valve abnormality (Ebstein’s anomaly)
  4. Truncus arteriosus
  5. Total anomalous pulmonary venous connection
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32
Q

how do cyanotic shunts affect inhalation induction

A
  • shunted blood doesn’t pass through lungs to pick up volatile
  • shunted blood dilutes volatile in L heart
  • decreased FA/FI rise
  • slower inhalation induction
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33
Q

how does volatile solubility affect inhalation induction in cyanotic shunts

A

slower inhalation induction
* most profound with less soluble agents (N2O and Desflurane)
* less of an issue with more soluble agents (isoflurane)

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34
Q

how is IV induction affected by cyanotic shunts

A

R - L shunt allows IV medication to bypass lungs and directly enter systemic circulation.

The drug reaches the vessel-rich organs faster, resulting in a faster onset

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35
Q

HD goals with cyanotic shunts

A

maintain SVR
decrease PVR

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36
Q

patho of acyanotic shunt

A

L-R shunt
* oxygenated pulmonary venous blood recirculates through R heart and lungs
* decreased systemic flow
* increased pulmonary flow

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37
Q

examples of acyanotic CHD

A
  • VSD (most common)
  • ASD
  • PDA
  • Coarctation of aorta
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38
Q

how do cyanotic shunts affect anesthesia induction

A

R  L shunt allows IV medication to bypass lungs and directly enter systemic circulation. The drug reaches the vessel-rich organs faster, resulting in a faster onset

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39
Q

HD goals with acyanotic shunt

A
  • avoid increased SVR
  • avoid decreased PVR by avoiding alkalosis, hypocapnia, high FiO2, and vasodilation
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40
Q

complications of increased pulm blood that occurs in acyanotic shunts

A
  • Volume overload of both ventricles -› biventricular failure
  • Ventricular hypertrophy
  • Decreased lung compliance + increased airway resistance
  • Pulmonary hypertension
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41
Q

what is Eisenmenger’s syndrome

A

a patient with a left-to-right shunt develops pulmonary hypertension

Increased right heart pressures cause a flow reversal through the cardiac defect, ultimately leading to a right-to-left shunt, hypoxemia, and cyanosis

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42
Q

most common cyanotic CHD

A

tetralogy of fallot

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43
Q

4 assoc defects in ToF

A
  1. Right ventricular outflow tract obstruction
  2. Right ventricular hypertrophy
  3. Ventricular septal defect
  4. Overriding aorta
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44
Q

strongly correlates with amount of shunt in ToF

A

degree of RVOT obstruction

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45
Q

which is assoc with pHTN - cyanotic or acyanotic shunts

A

acyanotic (L-R shunt)

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46
Q

effect of increased RVOT obstruction in ToF

A

more deoxygenated blood is shunted through the VSD and out into the aorta

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47
Q

how does the body compensate for RVOTO in ToF

A

erythropoiesis

leads to polycythemia and increases the risk of thromboembolism and stroke

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48
Q

what precipitates a Tet spell

A

increased sympathetic activity (crying, agitation, pain, defecation, fright, or trauma)

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49
Q

how does a Tet spell cause hypoxemia

A
  • ↑ SNS activity = ↑ contractility and ↑ RVOTO
  • ↑ resistance at level of RVOT favors flow through VSD
  • net effect: ↑ R-L shunting, hypoxemia
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50
Q

treatment of peri-op Tet spell

A
  • FiO2 100%
  • IVF
  • Increase SVR with phenylephrine
  • Reduce SNS stimulation (deepen anesthesia, beta-blockade with a short-acting agent - esmolol)
  • Avoid inotropes
  • Avoid excessive airway pressure
  • knee-chest position to
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51
Q

how does a child experiencing a Tet spell respond

A
  • hyperventilation with onset of hypoxemia
  • assumes squatting position
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52
Q

why does a child experiencing a Tet spell squat

A

increased IAP compresses abdominal arteries
* increased RV preload
* increased SVR
* increased blood flow through RVOT

restores pulmonary blood flow, reduces the magnitude of the right-to-left shunt, and improves oxygenation.

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53
Q

medications to avoid in patients with unrepaired ToF

A

ephedrine
dobutamine
epinephrine
morphine
meperidine
atracurium

avoid inotropes and histamine-releasing drugs

54
Q

HD goals in pts with ToF

A

increase SVR
decrease PVR
maintain contractility & HR
increase preload

55
Q

best induction agent for pt with unrepaired ToF

A

Ketamine (1 - 2 mg/kg IV or 3 - 4 mg/kg IM)

increases SVR and reduces shunting

increased contractility is minor compared to benefit of increasing SVR

56
Q

best induction agent for pt with unrepaired ToF

A

Ketamine (1 - 2 mg/kg IV or 3 - 4 mg/kg IM)

increases SVR and reduces shunting

increased contractility is minor compared to benefit of increasing SVR

57
Q

CXR findings in pt with ToF

A

The heart may take on a “boot-shaped” appearance

58
Q

EKG changes in ToF

A

RVH can cause RAD

59
Q

best induction agent for a pt with ToF

A

ketamine

60
Q

why are some pts with ToF polycythemic

A

chronic hypoxemia stimulates RBC production

61
Q

failure of the fossa ovalis to close results in what type of atrial septal defect

A

secundum

62
Q

most common congenital cardiac anomaly in children

A

VSD

63
Q

the most common congenital cardiac defect in adults

A

bicuspid aortic valve.

64
Q

most common site of ASD

A

fossa ovalis

also called an ostium secundum ASD

65
Q

a significant number of VSDs close by what age

A

2 yrs old

66
Q

early s/s of ASD

A

poor exercise tolerance

67
Q

later s/s of ASD

A

include atrial flutter, atrial fibrillation, and CHF

68
Q

antibiotic prophylaxis for acyanotic CHD

A

only indicated within 6 months of valve repair

69
Q

common closure of ASD

A

percutaneous transcatheter device

70
Q

how are VSDs usually closed

A

open approach

71
Q

most common site of VSD

A

middle of the ventricular septum, just below the septal leaflet of the tricuspid valve

This is also called a perimembranous VSD

71
Q

most common site of VSD

A

middle of the ventricular septum, just below the septal leaflet of the tricuspid valve

This is also called a perimembranous VSD

72
Q

conditions assoc with VSD

A
  • trisomy 13, 18, 21
  • VACTERL
  • CHARGE
73
Q

The physiologic consequence of the VSD is a function of ?

A

the pressure gradients between the RV and LV, and in turn, these are dependent on PVR and SVR

74
Q

pulmonary blood flow in large vs. small VSD

A
  • small: marginal increase (tolerate GA well)
  • large: RV/LV pressures equalize, PVR and SVR determine direction of blood flow
75
Q

syndrome strongly assoc with CoA

A

turner syndrome

76
Q

where does CoA typically occur

A

just before or after the ductus arteriosus

in rare instances, it occurs proximal to the left subclavian artery

77
Q

where does CoA typically occur

A

just before or after the ductus arteriosus

in rare instances, it occurs proximal to the left subclavian artery

78
Q

what is CoA

A

narrowing of the thoracic aortic lumen

79
Q

presentation of preductal vs. postductal CoA

A
  • Preductal coarctation is less common and usually presents in the neonate
  • Postductal coarctation is more common and usually presents in the adult
80
Q

SBP in upper vs lower extremities in CoA

A

SBP is elevated in the upper extremities

SBP is reduced in the lower extremities

81
Q

CXR changes in CoA

A

Rib notching may be visible (due to increased vessel diameter)

82
Q

required for lower body perfusion in severe CoA

A

PDA

83
Q

what is differential cyanosis

A

pink, well-perfused upper body + blue, poorly-perfused lower body

may be seen in severe CoA

84
Q

what is differential cyanosis

A

pink, well-perfused upper body + blue, poorly-perfused lower body

may be seen in severe CoA

85
Q

administered to maintain ductus arteriosus patency in severe CoA

A

PGE1

temporary until surgery

86
Q

administered to maintain ductus arteriosus patency in severe CoA

A

PGE1

temporary until surgery

87
Q

surgical repair of CoA

A
  • often through left thoracotomy + end-to-end anastomosis
  • aortic cross clamp used - can cause paraplegia
88
Q

why do some surgeons cool the child 34-35 deg C during CoA repair

A

aortic-cross clamp used during an open procedure can cause paraplegia

89
Q

indications for CoA surgical repair

A
  • exercise intolerance
  • chest pain
  • headaches
  • lower extremity claudication
90
Q

presentation of CoA in adulthood

A

secondary HTN

91
Q

characterized by a downward (apical) displacement of the tricuspid valve

A

Ebstein’s anomaly

92
Q

what causes RA enlargement in Ebstein’s anomaly

A

part of the right ventricle becomes part of the right atrium (atrialization), and this causes right atrial enlargement

93
Q

septal defects often assoc with ebstein’s anomaly

A

ASD or PFO

94
Q

Most common congenital defect of the tricuspid valve

A

ebstein’s anomaly

95
Q

how does ebstein’s anomaly affect onset of IV drugs

A

onset of IV drugs may be prolonged due to the pooling of drugs in the enlarged RA

96
Q

arrythmia commonly assoc with ebstein’s anomaly

A

SVT

97
Q

common postop complication assoc with ebstein’s anomaly

A

RV failure

98
Q

critical to management of a pt with ebstein’s anomaly

A

Maintenance of RV function is critical (risk of congestive heart failure)

99
Q

what is transposition of the great arteries

A

each great vessel arises from the wrong ventricle
* RV gives rise to the aorta
* LV gives rise to the pulmonary artery

100
Q

RV circuit in TGA

A
  • Systemic venous (desaturated) blood → RV → aorta → repeat
  • This blood circulates through the systemic circulation but not the pulmonary circulation
101
Q

LV circuit in TGA

A
  • Pulmonary venous blood (well oxygenated) → LV → lungs → repeat
  • This blood circulates through the pulmonary circulation but not the systemic circulation
102
Q

required for extrauterine survival with TGA

A

depends on the mixing of blood through an ASD (best case scenario), VSD, or PFO

103
Q

management of TGA after birth

A

The PDA can be kept open with a prostaglandin infusion (this is a temporary fix)

104
Q

management of TGA after birth

A

The PDA can be kept open with a prostaglandin infusion (this is a temporary fix)

105
Q

TGA repair options

A
  • Rashkind procedure creates an interarterial pathway to allow some oxygenated blood to reach the systemic circulation
  • Definitive surgical correction includes intraatrial baffle and arterial switch procedures
106
Q

goal of surgical HLHS correction

A

separating the pulmonary and systemic circulations

107
Q

goal of surgical HLHS correction

A

separating the pulmonary and systemic circulations

108
Q

4 anatomic features of HLHS

A
  • Hypoplastic LV
  • Hypoplastic aortic arch
  • Mitral and aortic stenosis or atresia
  • Ductal-dependent circulation
109
Q

why should the RUE be used to monitor BP in CoA

A

rarely, obstruction can be proximal to L subclavian a. and reduce perfusion to LUE

110
Q

2 cardiac signs of CoA

A
  1. upper extremity SBP > lower
  2. differential cyanosis
111
Q

surgical goal of Norwood stage 1

A

Aortic reconstruction - aortic arch now arises from the pulmonary trunk

pulmonary arteries are disconnected from the pulmonary trunk and are used to create a shunt from the subclavian artery or the right ventricle

112
Q

surgical goal of Norwood stage 1

A

Aortic reconstruction - aortic arch now arises from the pulmonary trunk

pulmonary arteries are disconnected from the pulmonary trunk and are used to create a shunt from the subclavian artery or the right ventricle

113
Q

when is Norwood stage 1 completed in HLHS

A

neonatal period

114
Q

when is Norwood stage 2 completed

A

3-6 months old

115
Q

surgical goals of Norwood stage 2

A

The shunt from the first procedure is taken down and a new connection is made between the SVC and the pulmonary arteries.

116
Q

surgical goals of Fontan procedure

A

Conversion to Fontan circulation - The IVC is connected to the PA with a conduit

117
Q

when is Fontan surgery completed

A

2-4 yrs old

118
Q

circulation after Fontan completion

A
  • single ventricle pumps blood systemically
  • pulmonary blood flow is passive from SVC/IVC to PA
119
Q

what does pulmonary blood flow depen on after Fontan completion

A

completely dependent on negative intrathoracic pressure during spontaneous breathing

Increased PVR is detrimental to pulmonary blood flow

120
Q

what does pulmonary blood flow depend on after Fontan completion

A

completely dependent on negative intrathoracic pressure during spontaneous breathing

Increased PVR is detrimental to pulmonary blood flow

121
Q

ventilation after Fontan completion

A

Positive-pressure ventilation reduces pulmonary blood flow and should be avoided/ minimized. Spontaneous ventilation is preferred

122
Q

what is truncus arteriosus

A

single artery that gives rise to the pulmonary, systemic, and coronary circulations

no specific pathway for blood to enter the pulmonary circulation before being pumped into the systemic circulation

123
Q

CHD commonly seen with truncus arteriosus

A

VSD

124
Q

consequence of decreased PVR in truncus arteriosus

A

steals blood from the systemic and coronary circulations

125
Q

result of surgical correction of truncus arteriosus

A

can restore a two ventricle arrangement by separating the pulmonary from the systemic circulation with closure of the VSD

126
Q

CHDs assoc with prolonged inhalation induction

A

R-L shunts
* ToF
* ebstein’s anomaly

127
Q

hydration in HLHS patients

A

do not let them get dry - preload dependent

128
Q

shunt lesions with outflow tract obstruction

A
  • Tetralogy of Fallot
  • Ebstein’s anomaly
  • Pulmonary stenosis with atrial or ventricular septal defect
  • Eisenmenger’s syndrome
129
Q

fetal shunt unable to reopen after birth

A

ductus venosus

130
Q

inhaled anesthetic to avoid with ASD

A

N2O