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Pathophys II > Advanced Pathophysiology Congenital Heart Defects > Flashcards

Advanced Pathophysiology Congenital Heart Defects Flashcards

1
Q

The word congenital** refers to

A

existing at birth

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2
Q

Congenital heart defects may develop from

A

chromosome abnormalities
single-gene abnormalities
conditions during pregnancy that affect the baby
combination of genetic and environmental problems
unknown causes

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3
Q

Of children with a heart defect, 30% have other

A

physical, developmental & cognitive disorders

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4
Q

Diagnosis of cardiac anomalies may be done

A

in utero, found on newborn physical, ECHO, EKG, Chest x-ray, cardiac cath, CMRI, CT, TEE, Holter recording

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5
Q

Preop eval for cardiac anomalies focuses on

A

heart murmur on preop evaluation
functional status, growth & development
reviews most recent echo/labs/tests
-children with a history of CHF, cyanosis, pHTN, and young age are at a potentially higher risk

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6
Q

There is _______ secondary to fluid filled lungs and a hypoxic environment.

A

high PVR

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7
Q

There is _______ secondary to large surface area of the low resistance utero-placental bed

A

low SVR

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8
Q

The most oxygenated blood from the _____ perfuses the _________ by shunting across the liver via the ductus venosus and shunting across the heart via the foramen ovale

A

umbilical vein; brain & heart

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9
Q

___________ precipitates the transition from fetal to adult circulation

A

clamping of the umbilical cord and inflation of the lungs

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10
Q

Lug inflation increases

A

PaO2
lowers PVR, increasing pulmonary blood flow and increased return to LA
increased LA pressure above RA pressure causes a functional closure of the PFO

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11
Q

The ductus arteriosus remains patent in utero due to

A

hypoxia, mild acidosis, and placental prostaglandins***

-removal of these factors at birth causes functional closure of DA

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12
Q

A PDA often occurs in

A

premature infants with lung disease

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13
Q

______ can be used to try & close a PDA

A

Indomethacin***** (an anti-prostaglandin)

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14
Q

Certain _______ can cause the newborn to revert to fetal circulation

A

physiologic stresses

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15
Q

The neonatal myocardium is characterized by general

A

immaturity & decreased number of myofibrils

-decreased contractility & decreased relaxation

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16
Q

The neonatal myocardium includes:

A

RV & LV are equal in size

  • parasympathetic is well developed
  • sympathetic innervation is poorly developed
  • immature SR results in poor release & reuptake of intracellular calcium
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17
Q

Classifications of CHD classified as

A

L-R shunts- “pink lesions”
R-L shunts- “blue lesions”
obstructive
“mixed” or cyanotic****

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18
Q

Describe left to right shunts & provide examples.

A

connects arterial and venous circulation resulting in increased pulmonary blood flow “pink lesions”
E.g. PDA, ASD, VSD

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19
Q

Describe right to left shunts & provide examples

A

venous blood is ejected systemically; there is decreased pulmonary blood flow & patients are cyanotic “blue lesions”
- e.g. ASD or VSD with pulmonary HTN, TOF during Tet spell

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20
Q

Describe obstructive CHD & provide examples.

A

prevent ventricular flow from either side of the heart, decrease cardiac output
-e.g. coarctation of the aorta, aortic stenosis

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21
Q

Describe “mixed” or cyanotic CHD & provide examples.

A

mixing of venous & arterial blood

-e.g. hypoplastic left heart syndrome

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22
Q

Large left to right shunts result in pulmonary over circulation. ______ is increased as a large component of LV output bypasses the systemic circulation, enters the lungs and rapidly returns to the left side of the heart.

A

RV preload

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23
Q

Describe Eisenmenger’s syndrome.

A

when large VSDS are uncorrected, the resulting pulmonary hypertension can reverse the shunting of blood across the defect. The previously “left to right shunt becomes right to left

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24
Q

Mixing lesions occur when a

A

functional single ventricle ejects the mixed systemic and pulmonary venous return.

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25
Q

Patients with mixing lesions are often

A

cyanotic and are often dependent on the PDA at birth

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26
Q

An atrial septal defect is often

A

asymptomatic and discovered incidentally (murmur)

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27
Q

Large atrial septal defects that are left untreated can cause

A

right sided volume overload (usual Qp/Qs>2) with RA & RV dilation & increased pulmonary blood flow

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28
Q

Repair for an atrial septal defect can be

A

a closure device in cath lab or surgery

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29
Q

The most common congenital defect in children is

A

ventricular septal defect

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30
Q

A ventricular septal defect leads to

A

pulmonary over circulation due to left to right shunting in an isolated lesion
if defect is large pressure equalizes in both ventricles & pulmonary blood flow will be greater leading to symptoms of CHF & irreversible damage to pulmonary vascular bed

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31
Q

_______ syndrome may occur in ventricular septal defect and describes the shunt reversing direction when the PVR is high enough.

A

Eisenmenger’s

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32
Q

Describe restrictive VSD.

A

small size & limited pulmonary over circulation

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33
Q

Describe unrestrictive VSD.

A

large flow across the septum with balance between SVR & PVR

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34
Q

Isolated large VSDs are managed via

A

diuretics for the first few months of life

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35
Q

Indications for surgery for VSD include

A

poor feeding, reduced weight gain, & increases in incidence of respiratory infections

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36
Q

The _________ is at risk during VSD repair.

A

conduction system that runs along the ventricular septum

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37
Q

As PVR falls in the first months of life, the flow across the VSD can

A

increase greatly (as high as Qp/Qs >3)

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38
Q

The ductus arteriosus is a leftover fetal artery connection between the

A

aorta & the pulmonary artery

when this artery remains open after birth it is called a patent ductus arteriosus

39
Q

Unrestricted PDA will have significant

A

L to R shunting

40
Q

Significant______ into the pulmonary circulation lowers the systemic diastolic BP compromising distal perfusion (i.e. mesenteric/renal & coronary perfusion)

A

diastolic run off*****

41
Q

Patent ductus arteriosus is commonly closed with

A

cardiac catheterization (coil or device)

42
Q

Surgical closure for a patent ductus arteriosus is a

A

left thoracic approach and the ductus is closed by a suture tie or metal clip

43
Q

Whenever there is an abnormal connection between the let and right sides of the heart, there must be vigilance in

A

de-airing all intravenous fluids

smaller the child, the more significant air can be

44
Q

Even if the flow is predominantly left-to right, the direction can change at any time & if air enters the left side of the heart, it can

A

travel to the brain and cause ischemia

45
Q

Complete atrio-ventricular canal is a

A

free communication between all four chambers of the heart
- it is located where the atrial septums join the ventricular septums. involves atrias, ventricles, tricuspid, & mitral valves. Results in the formation of a single large valve

46
Q

The common AV valve is often

A

regurgitant

47
Q

_______ make the greatest percentage of patients with CAVC

A

down syndrome children

48
Q

CAVC requires _____ repair

A

surgical repair with septum patch & new valves

usually repaired <6 months before pulmonary vascular changes develop

49
Q

Problems with CAVC repair nclude

A

residual septal defects, AV valve regurgitation, post-op pulmonary reactivity, & conduction system damage

50
Q

Coarctation is a

A

narrowing in the aorta commonly occurring immediately distal to the origin of the left subclavian artery

51
Q

The coarctation is most often located near the

A

ductus arteriosus; if narrowing is proximal to the ductus it is ‘pre-ductal’; if it is distal to the ductus it is ‘post-ductal’

52
Q

Coarctation is frequently associated with

A

bicuspid aortic valve

53
Q

Critical coarctations will present with

A

circulatory collapse, shock, & acidosis due to poor distal perfusion
-PGE1 is started to reopen the ductus and distal perfusion remains ductal dependent until surgery

54
Q

Coarctation presents with

A

upper extremity hypertension, decreased lower extremity pulses, and LVH

55
Q

The surgical approach for repair of coarctation is

A

left thoracotomy and subclavian flap angioplasty sacrifices the left subclavian artery
the left arm will then need to be perfused by collateralization

56
Q

With surgical repair of coarctation, blood pressure must be measured in

A

the right arm

-the aortic cross clamp will be proximal to the left subclavian artery

57
Q

_________ can be done in the cardiac cath lab for coarctation.

A

Balloon dilation

58
Q

Pulmonary valve stenosis is a

A

narrowing that causes the RV to work harder to pump blood past the blockage
-usually it is part of other complex lesions

59
Q

Symptoms of pulmonary valve stenosis

A

depend on the severity of obstruction

60
Q

Pulmonary valve stenosis is often treated with

A

balloon dilation

61
Q

Aortic valve stenosis is a

A

narrowing that causes the LV to work harder to move blood past the blockage

62
Q

Severe aortic stenosis in utero may impair

A

LV development

63
Q

_______is a treatment option for severe aortic stenosis.

A

balloon dilation

64
Q

Valve replacement at young ages requires

A

may revisions over time

65
Q

The ross procedure is performed on patients diagnosed with

A

aortic stenosis and is an alternative to prosthetic valve replacement

66
Q

In the Ross procedure, the diseased

A

aortic root is resected the patient’s own pulmonary valve root is excised and implanted into the aortic position

  • the coronary arteries are then re-implanted into the “neo aortic” root
  • An RV to PA connection and valve is made with cadaveric tissue or a conduit
  • the RV-PA connection may require revision over time but it provides better long term solution to the aortic valve
67
Q

Advantages to the Ross procedure include

A

freedom from long-term anticoagulation

-the valve grows as the patient grows

68
Q

Disadvantages to the Ross procedure include

A

single valve disease (aortic) is treated with 2 valve procedure

69
Q

A Blalock-Taussing-Thomas shunt is an operation to

A

create a type of systemic to pulmonary shunt

70
Q

The Classic BTS is when the

A

subclavian artery*** is divided and directly anastomosed to the ipsilateral pulmonary artery

71
Q

The modified BTS is when a

A

synthetic shunt** between the subclavian artery & PA

72
Q

With the classic BTS, it allows the

A

patient’s own subclavian artery to grow so no need for revision

  • the pulses in the ipsilateral arm will be decreased or non-palpable
  • prudent to expect a classic BTS in older adult survivors of CHDs
73
Q

With the modified BTS, the ipsilateral arm

A

reflects true pressures & is available for a-line placement

  • artificial material will not grow with the patient
  • hypotension leads to sluggish flow and possibly thrombosis which can be critical*****
74
Q

The four key features of the tetralogy of fallot are

A
  1. ventricular septal defect (VSD)
  2. right ventricular outflow tract obstruction (RVOT)
  3. overriding aorta (aorta lies directly over the VSD)
  4. Right ventricular hypertrophy
    * ****
75
Q

Repair for TOF is usually within

A

first 6 months of life

76
Q

Neonates with hypercyanotic spells who are too small for definitive repair may be palliated with a

A

BTT shunt

77
Q

In TOF, hypertrophy of the right ventricular myocardium is secondary to

A

pressure overload

78
Q

The limitation of pulmonary blood flow & the magnitude of ventricular level right to left shunting account for the degree of ______ in TOF

A

cyanosis

79
Q

On XR, TOF can be seen as a

A

Boot shaped heart due to RVH

80
Q

The most common cyanotic cardiac lesion is

A

TOF

81
Q

Hypercyanotic or “TET spells” are

A

acute dynamic increases in the pulmonary outflow tract obstruction (spasm) may result in an intensely cyanotic episode due to right to left shunting
-combination of RVOTO & VSD

82
Q

Causes of TET spells include

A

crying, feeding, acidosis, catecholamines, & surgical stimulation

83
Q

Treatment for TET spells includes,

A

increase SVR or relax the spasm

  • child will squat to increase afterload
  • this position increases the SVR, decreases the HR, and decreases the right to left shunt across the VSD
84
Q

Anesthetic treatment for TET spells includes

A

100% fiO2, sedation, fluid, beta blocker, or alpha agonist to increase afterload and slow down the heart rate

85
Q

Tetralogy of Fallot repair includes closure of

A

ventricular septal defect with a patch & relief of right ventricular outflow tract obstruction by removing some of the thickened muscle

86
Q

TOF repair eliminates intracardiac shunting at the

A

ventricular level (cyanosis) and addresses the right ventricular outflow tract obstruction (may also include enlarging the left and right pulmonary arteries)

87
Q

Anesthesia for TOF repair includes

A

avoid “Tet spell”
generous premedication
sufficient anesthesia & analgesia
avoid reductions in SVR
RV output is the limiting factor on overall CO
treat a Tet spell quickly with phenylephrine if necessary

88
Q

Treatment of a Tet spell intraoperatively includes

A

exacerbated right to left shunting (Cyanosis)***

  • 100% oxygen
  • knees to chest
  • fluid bolus
  • hyperventilation
  • sedation
  • esmolol 0.5 mg/kg IV or propranolol 0.1-0.3 mg/kg bolus
  • phenylephrine 1-10 mcg/kg IV
89
Q

After repair of the TOF, the hypertrophied RV has

A

poor compliance which is worsened in the immediate post op period due to right ventriculotomy
-maintain adequate filling volume

90
Q

A PFO or small ASD is created in TOF repair, that becomes a

A

“pop off valve” if right sided pressures increase

CO is maintained at the expense of modest systemic desaturation

91
Q

After TOF repair, over time the tricuspid valve may become

A

incompetent leading to RV overload & ventricular ectopy

92
Q

________ can be damaged in the TOF repair

A

The conduction system

93
Q

RV decompensation may occur over time in the TOF repair due to

A

free pulmonary insufficiency in trans-annular patch repair

Pathophys II (43 decks)

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