Mod VI: Congenital Heart Disease Flashcards

1
Q

Congenital Heart Disease

Congenital Heart Diseases are present in about what percentage of newborn infants?

A

1%

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2
Q

Congenital Heart Disease

What are the causes of Congenital Heart Disease?

A

Idiopathic

Genetic

Environmental

(rubella 1st trimester, lithium, FAS)

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3
Q

Congenital Heart Disease

What are risk factors for Congenital Heart Disease?

A

Parent with CHD

Prematurity

Multiple gestations

Noncardiac congenital anomalies (Down’s syndrome)

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4
Q

Congenital Heart Disease

Signs & Symptoms of Congenital Heart Disease in infants are:

A

Tachypnea

Failure to gain weight

Tachycardia (>200)

Heart murmur

Congestive heart failure

Hypoxemia

Cyanosis

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5
Q

Congenital Heart Disease

Signs & Symptoms of Congenital Heart Disease in children are:

A

Dyspnea

Failure to grow

Decreased exercise tolerance

Heart murmur

Congestive Heart Failure

Cyanosis

Clubbing of digits

Squatting (To increase SVR)

HTN

Chest pain

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6
Q

Congenital Heart Disease

T/F: Most Congenital Heart Diseases are diagnosed prior to birth

A

True

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7
Q

Congenital Heart Disease - Diagnosis

T/F: Congenital Heart Disease is apparent during first week of life in 50% of afflicted neonates and before 5yrs in all remaining

A

True

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8
Q

Congenital Heart Disease - Diagnosis

What’s the initial diagnostic test recommended for CHD?

A

US Echocardiography

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9
Q

Congenital Heart Disease - Diagnosis

Test that demonstrates valvular dysfunction and septal defects

A

Doppler US

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10
Q

Congenital Heart Disease - Diagnosis

Tests that demonstrate anomalies involving great vessels

A

CT scan - MRI

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11
Q

Congenital Heart Disease - Diagnosis

What’s the most definitive diagnostic technique for CHD?

A

Cardiac catherization

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12
Q

Congenital Heart Disease

Problems afflicting patients with Congenital Heart Disease include:

A

Pulmonary vascular disease & associated PHTN

Congestive heart failure

Infective endocarditis (VSD/PDA)

Requires prophylaxis antibiotics

Hypertension (Coarctation)

Polycythemia (HCT > 65%)

Physiologic response to chronic hypoxemia - Increases risk for thromboembolism

Coagulation defects

Deficiency in VT K clotting factors - Defective PLT aggregation

Brain abscess development

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13
Q

Problems Afflicting Patient with Congenital Heart Disease

Congenital Heart Disease a/w Infective endocarditis (VSD/PDA) Requires Prophylaxis with which drugs?

A

Antibiotics

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14
Q

Problems Afflicting Patient with Congenital Heart Disease

Polycythemia (HCT > 65%) a/w Congenital Heart Disease is a physiologic response to:

A

Chronic hypoxemia

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15
Q

Problems Afflicting Patient with Congenital Heart Disease

Polycythemia (HCT > 65%) a/w Congenital Heart Disease increase risk for:

A

Thromboembolism

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16
Q

Problems Afflicting Patient with Congenital Heart Disease

Coagulation defects a/w Congenital Heart Disease are a consequence of:

A

Deficiency in Vit K clotting factors

Defective PLT aggregation

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17
Q

Pathophysiology of Congenital Heart Disease

T/F: Management of anesthesia for patients with CHD requires a thorough knowledge of the pathophysiology of each cardiac defect

A

True

However, this is confusing due to complexity of lesions

Utilization of a structured approach that emphasizes ratio of pulmonary blood flow & systemic blood flow based on resistance in these vascular beds is helpful

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18
Q

Pathophysiology of Congenital Heart Disease

Important pathophysiologic questions w/ CHD include:

A

Is there on obstruction?

Is there a shunt?

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19
Q

Pathophysiology of Congenital Heart Disease

What are the effects of R side obstruction?

A

Blood unable to go from RV to lungs

↓ pulmonary blood flow => hypoxemia/cyanosis

Blood does not get oxygenated

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20
Q

Pathophysiology of Congenital Heart Disease

What are effects of L side obstruction?

A

Blood unable to flow from LV to systemic circulation

Tissues organs do not get perfused

↓ systemic blood flow => hypoperfusion/acidosis/shock

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21
Q

Pathophysiology of Congenital Heart Disease

How can shunt be defined?

A

Mixing of pulmonary/systemic circulations

(or mixing of oxygenated and de-0xygenated blood)

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22
Q

Pathophysiology of Congenital Heart Disease

What determines the direction of of shunt?

A

Ratio of pulmonary blood flow (Qp) / systemic blood flow (Qs)

Qp:Qs

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23
Q

Pathophysiology of Congenital Heart Disease

Qp:Qs < 1 means:

A

Pulmonary blood flow < Systemic blood

Instead of flowing to the lungs, blood is flowing to the left side

[R to L shunt]

Blood flowing directly to the left fails to be oxygenated

This leads to hypoxemia and cyanosis

Ineffective pulmonary blood & mixing systemic/pulmonary circulations => hypoxemia/cyanosis

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24
Q

Pathophysiology of Congenital Heart Disease

Qp:Qs > 1 means:

A

Pulmonary blood flow > Systemic blood

[L to R shunt]

Volume/pressure overload of R ventricle => CHF

Pulmonary overcirculation => Pulmonary HTN/ ↑ PVR

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25
Q

Pathophysiology of Congenital Heart Disease

Qp:Qs = 1 means

A

No shunt

Balanced flow

Bi-directional shunt of equal magnitude

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26
Q

Pathophysiology of Congenital Heart Disease

Shunt flow dependent on balance between PVR & SVR. ↑ PVR relative to SVR would lead to what shunt direction?

A

R to L shunt

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27
Q

Pathophysiology of Congenital Heart Disease

Shunt flow dependent on balance between PVR & SVR. ↑ SVR relative to PVR would lead to a shunt in which direction?

A

L to R shunt

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28
Q

Pathophysiology of Congenital Heart Disease

Factors that would increase PVR, cause a R-to-L shunt, and affect Qp:Qs Ratio include:

A

Hypoxia

Hypercapnia

Acidosis

High PIP

PEEP

Hypothermia

Polycythemia

Decreased LV output

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29
Q

Pathophysiology of Congenital Heart Disease

Factors that would decrease PVR, cause a L-to-R shunt, and affect Qp:Qs Ratio include:

A

High FiO2

Hypocapnia

Alkalosis

Improved LV output

Anemia

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30
Q

Classification of Congenital Heart Defects

Lesions causing left-to-right shunting (volume overload of the left ventricle or left atrium resulting in increased pulmonary blood flow) include:

A

Atrial Septal Defect

Ventricular Septal Defect

Patent ductus arteriosus

Atrioventricular Septal Defect

(Common complete atrioventricular canal)

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31
Q

Classification of Congenital Heart Defects​

Lesions causing outflow obstruction (resulting in pressure overload on the left ventricle, and increased myocardial work) inlude:

A

Aortic Stenosis

Coarctation of the aorta*

(Narrowing of the aorta distal to the aortic valve)

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32
Q

Classification of Congenital Heart Defects​

Lesions causing right-to-left shunting (cyanosis resulting from obstruction/decreased pulmonary blood flow) include:

A

Tetralogy of Fallot

Tricuspid atresia

Pulmonary atresia

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33
Q

Classification of Congenital Heart Defects​

Lesions causing right-to-left shunting (cyanosis due to mixing of the pulmonary and systemic circulations/increase pulmonary blood flow) include:

A

Hypoplastic left heart syndrome

Truncus arteriosus

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34
Q

Classification of Congenital Heart Defects​

Lesions causing separation of the pulmonary & systemic circulations

A

Transposition of the great vessels

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35
Q

Classification of Congenital Heart Defects​

Acyanotic (L→R) shunt lesions include:

A

Ventricular Septal Defect

Atrial Septal Defect

Patent Ductus Arteriosus

Atrioventricular Septal Defects

Common Complete Atrioventricular Canal

Aortic Stenosis

Coarctation of the Aorta

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36
Q

Classification of Congenital Heart Defects​

Cyanotic (R→L) shunt lesions include:

A

Tetralogy of Fallot

Transposition of the Great Arteries

Hypoplastic Left Heart Syndrome (HLHS)

Tricuspid valve abnormalities (Ebstein’s anomaly)

Truncus arteriosus

Total anomalous pulmonary venous connection

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37
Q

Acyanotic or Predominantly Left-to-Right Lesions

Pathophysiologic changes that accompany Left-to-Right shunt lesions include:

A

Decreased systemic blood flow

Low Cardiac output - Hypotension

Increased pulmonary blood flow

Pulm HTN - RVH

38
Q

Acyanotic or Predominantly Left-to-Right Lesions

Hemodynamic goals for Left-to-Right shunt lesions include:

A

Avoid increased SVR

Avoid decreased PVR

How? => Decrease FiO2 - Hypoventilation

(High FiO2 and Hypocapnia/hyperventiation will decrease PVR)

Any of the above will worsen the shunt!!!

39
Q

Acyanotic or Predominantly Left-to-Right Lesions

Pathophysiology of Left-to-Right Lesions:

A

Simple shunts with isolated abnormal communications between the L & R side of the heart

Pressure L side heart > Pressure R side heart => L-to-right shunt

Blood flow to R side heart & lungs increases

Shunt flow depends on balance between PVR & SVR

PVR < SVR => L-to-R shunting

40
Q

Acyanotic or Predominantly Left-to-Right Lesions

Clinical manifestations:

A

Pulmonary vascular congestion

Decreased lung compliance

Increased work of breathing

Chronic increases PBF

Irreversible ↑ PVR

RVH → Cor Pulmonale

41
Q

Acyanotic or Predominantly Left-to-Right Lesions

What’s the most common acyanotic or predominantly Left-to-Right Lesion?

A

Ventricular Septal Defect (VSD)

Most common (25%)

Mostly “isolated” VSD = VSD w/o any other type of anomalies

42
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

Clinical symptoms related to size of shunt - what are Clinical symptoms of Small shunt?

A

No clinical symptoms

43
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

Clinical symptoms related to size of shunt - what are Clinical symptoms of Large shunt?

A

Growth failure

CHF

Recurrent pulmonary infections

Eisenmenger’s syndrome (untreated)

44
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

Condition that arises when untreated Ventricular Septal Defect (VSD) leads to Pulmonary vascular disease => ↑ PVR, and Shunt reverses and flows R-to-L (cyanosis)

A

Eisenmenger’s syndrome

Deadly during delivery or shortly after birth d/t severe cyanosis, b/c the body is unable to compensate for the acute change from L to R to R to L shunt flow

45
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

Shunt flow is determine by PVR & SVR. How does ↓ PVR & ↑ SVR affect an existing L-to-R shunt?

A

↑ L-to-R shunting

(Makes it worse)

46
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

Shunt flow is determine by PVR & SVR - How does ↑ PVR & ↓ SVR affect an existing L-to-R shunt?

A

↓ L-to-R shunting

47
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

T/F: Most small Acyanotic (L→R) defects close w/o intervention

A

True

(40% by 3yrs and 75% by 10yrs)

48
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

How are Large Acyanotic (L→R) defects treated?

A

Require surgical closure before ↑ PVR irreversible

49
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

Anesthetic considerations similar to which other congenital heart defect?

A

ASD

50
Q

Acyanotic (L→R) - Ventricular Septal Defect (VSD)

Blood flow in VSD

A

See picture

51
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)

Prevalence of ASD

A

Accounts for 7.5% of CHD

52
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)

T/F: Most small Atrial Septal Defect (ASD) are asymptomatic, with spontaneous closure occurring in the 1st yr. of life

A

True

53
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

Pathophysiology of large ASD

A

Acyanotic L to R shunt (pulmonary overflow)

54
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

Symptoms of Atrial Septal Defect (ASD)​

A

DOE - SVT - CHF

Pulmonary HTN

Recurrent pulmonary infections

55
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

Treatment of large Atrial Septal Defect (ASD)​

A

Requires surgical repair or

Placement of a device via catheterization

56
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

Blood flow in Atrial Septal Defect (ASD)​

A

See picture

57
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

Anesthetic considerations for ASD and VSD will focus on avoiding which changes to which two hemodynamic variables?

A

Avoid ↑ SVR

(worsen L-R shunting)

Avoid ↓ PVR

(by avoiding high FiO2, and avoiding low ETCO2)

58
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

T/F: Anesthetic considerations for ASD and VSD include Strict avoidance of air emboli

A

True

59
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

How does the L-to-R shunting affect the rate of inhalation induction? why?

A

More rapid with inhalation induction

D/t _rapid decrease in the arterial to venous difference of agen_t

This is all theoretical - No real differences seen clinically

60
Q

Acyanotic (L→R) - Atrial Septal Defect (ASD)​

How does the L-to-R shunting affect the rate of IV induction? why?

A

Slower rate of IV inductions

D/t Diluted arterial blood concentration

This is all theoretical - No real differences seen clinically

61
Q

Acyanotic (L→R)

Failure of ductus arteriosus to close after birth (review fetal circulation and transition at birth!) results in a condition called:

A

Patent Ductus Arteriosus (PDA)

62
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

What’s the prevalence of PDA? What age group is most affected?

A

PDA accounts for 7.5% CHD

Most common in the premature infants

63
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

What are the causes of Patent Ductus Arteriosus (PDA)?

A

Hypoxemia

Hypercarbia/acidosis

Persistent pulmonary HTN in the newborn

64
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

Which hemodynamic variables determine shunt flow in Patent Ductus Arteriosus (PDA)?

A

SVR & PVR

PDA is Nonrestrictive when SVR > PVR

Which results in L-to-R shunt

(blood flow from aorta back into pulmonary artery

65
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

Blood flow in PDA

A

See image

66
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

Clinical presentation of PDA:

A

Pulmonary congestion

CHF

(widened pulse pressure, continuous systolic/diastolic murmur)

67
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

How long after birth should PDA be normally closed?

A

2-3 days after birth

68
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

Medical Treatment of Patent Ductus Arteriosus (PDA)

A

Indomethacin (↓ PGE1 levels)

Remember that it is the reduction in prostaglandins production after removal of the placenta that results in the closure of the ductus arteriosus

Indomethacin reduces levels of prostaglandins (PGE1)

69
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

When is Surgical ligation (NICU/Cath lab) of Patent Ductus Arteriosus (PDA) indicated?

A

Decreased renal or platelet function

(contraindication to indomethacin use)

Indomethacin unsuccessful

Decreased systemic oxygenation due to shunting

70
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

What surgical approch is used for ligation (NICU/Cath lab) of Patent Ductus Arteriosus (PDA)?

A

Left thoracotomy approach

Video-assisted ligation (minimally traumatizing)

71
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

What’s a contraindication to indomethacin use?

A

Decreased renal or platelet function

72
Q

Acyanotic (L→R) - Patent Ductus Arteriosus (PDA)

Anesthetic considerations for PDA are similar to Anesthetic considerations for which other Acyanotic (L→R) lesions?

A

ASD/VSD

Avoid increasing SVR => this will worsen the shunt

Avoid decreasing PVR => by avoiding high FiO2 and hyperventilation

73
Q

Acyanotic (L→R)

Which anatomical defects are present in Atrioventricular Septal Defects?

A

ASD

VSD

Single atrioventricular valve

Lack of separation of the mitral and tricuspid valves

74
Q

Acyanotic (L→R) - Atrioventricular Septal Defects

Atrioventricular Septal Defects are common in children with which genetic condition?

A

Trisomy 21

(Down syndrome)

75
Q

Acyanotic (L→R) - Atrioventricular Septal Defects

What are the characteristics of Shunt flow w/ AVSD in the initial neonatal period? why is that and what does that results in?

A

Bidirectional

D/t ↑ PVR

Results in mild hypoxemia

76
Q

Acyanotic (L→R) - Atrioventricular Septal Defects

How is Shunt flow in AVSD with ↓ PVR

A

Predominantly L to R

77
Q

Acyanotic (L→R) - Atrioventricular Septal Defects

What are the Symptoms of AVSD?

A

CHF

Tachypnea/dyspnea

Poor feeding

Pulmonary HTN

(with <u>pulmonary vascular disease</u> developing overtime)

78
Q

Acyanotic (L→R) - Atrioventricular Septal Defects

What’s the Treatment for AVSD?

A

Surgical repair required within the 1st year of life

79
Q

Obstructing Lesions

What’s the prevalence of Congenital Aortic Stenosis?

A

Accounts for 5% of all CHD

80
Q

Obstructing Lesions - Congenital Aortic Stenosis

Anatomic and physiologic changes a/w congenital Aortic Stenosis include:

A

Unicuspid or bicuspid stenotic valve

↑ LVEDP & ↑ LAP => Pulmonary edema

L to R shunt at atrial level

Concentric LVH with_↑ Myocardial O2 requirements_

81
Q

Obstructing Lesions - Congenital Aortic Stenosis

T/F: Symptoms of Congenital Aortic Stenosis are related to severity of stenosis and ventricular function

A

True

82
Q

Obstructing Lesions - Congenital Aortic Stenosis

Systemic blood flow in neonate with critical Congenital Aortic stenosis is dependent on:

A

Maintaining a Patent Ductus Arteriosus

Ductal-dependent systemic blood flow (R-L shunting)

Closure of PDA after birth => cardiogenic shock

83
Q

Obstructing Lesions - Congenital Aortic Stenosis

What’s the treatment for critical Congenital Aortic stenosis in Neonate?

A

Prostaglandin E1 to maintain open ductus arteriosus until definitive surgery can be performed

84
Q

Obstructing Lesions - Congenital Aortic Stenosis

Congenital Aortic Stenosis is most commonly diagnosed in older children. What s/s is it a/w?

A

Angina pectoris w/o CAD (LVH)

CHF

Syncope

85
Q

Obstructing Lesions - Congenital Aortic Stenosis

In Critical AS, the aortic valve opens, but cannot supply enough blood to the body. Some part of the blood supply to the body must be supplied by

A

A Patent Ductus Arteriosus

86
Q

Obstructing Lesions - Congenital Aortic Stenosis

Surgical intervention for Congenital Aortic Stenosis include

A

Valvuloplasty/Valvotomy

Valve replacement

87
Q

Obstructing Lesions - Congenital Aortic Stenosis

Why do Surgical intervention for Congenital Aortic Stenosis include require prophylactic antibiotics?

A

These pts are predisposed to infective endocarditis

88
Q

Obstructing Lesions - Congenital Aortic Stenosis

Anesthetic management of peds with Congenital Aortic Stenosis is the same as for the adult patient with aortic stenosis. What does it entail?

A

Avoid sudden↓ SVR

Maintain NSR

Avoid bradycardia (↓ C.O.)

Avoid tachycardia (impairs ventricular filling)

Optimize volume to maintain venous return & LV filling pressures

Predisposed to infective endocarditis => prophylactic antibiotics

Avoid hyperoxygenation

↑PBF thus reducing systematic blood flow

Accept SaO2 > 75%

Reduce FiO2 SaO2 > 85%

Maintain Controlled hypoventilation (↑ PVR)

If there is excessive PBF (SaO2 > 85%) & reduced SBF (↓ MAP)

89
Q

Obstructing Lesions - Congenital Aortic Stenosis

In the Anesthetic management of peds with Congenital Aortic Stenosis, why must hyperoxygenation be avoided?

A

Decreases PVR => ↑ pulm blood flow (PBF)

=> reduced systematic blood flow

Accept SaO2 > 75%

Reduce FiO2 SaO2 > 85%

90
Q

Obstructing Lesions - Congenital Aortic Stenosis

In the Anesthetic management of peds with Congenital Aortic Stenosis, which intervention is appropriate if there is excessive PBF (SaO2 > 85%) & reduced SBF (↓ MAP)?

A

Controlled hypoventilation (to ↑ PVR)