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Block 9 Week 1 > Fung > congenital heart disease > Flashcards

Fung > congenital heart disease Flashcards

1
Q

what is “congenital heart disease”?

A

general term used to describe abnormalities of the heart & great vessels that are present from birth

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2
Q

when do congenital heart defects occur?

A

during week 3 & 8 of gestation (faulty embryogenesis)

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3
Q

how many births (%) have a congenital CV defect?

A

1%

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4
Q

what is the most common form of CV disease among children?

A

congenital CV defects

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5
Q

what are the 2 classes of causes of congenital heart disease?

A

sporadic genetic abnormalities

AND

environmental factors

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6
Q

what are the 3 types of sporadic genetic abnormalities that cause CHD?

A

single gene mutation

small chromosomal deletions

tri/monosomies

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7
Q

what are the tri & monosomies that can cause CHD?

A

turner syndrome (XO) trisomy 13 trisomy 18 (edward) trisomy 21 (down)

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8
Q

what are the environmental factors that cause CHD?

A

congenital rubella

gestational diabetes

teratogens

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9
Q

what is a shunt?

A

abnormal communication btwn chambers or BVs

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10
Q

what is an atresia?

A

a complete obstruction btwn chambers or BVs

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11
Q

what are the 3 primary categories of CHD?

A

L to R shunt

R to L shunt

obstruction

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12
Q

what happens to blood in R>L shunts?

A

poorly oxygenated venous blood mixes w/ systemic arterial blood

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13
Q

how do pts w/ R>L shunt present?

A

hypoxemia & cyanosis (cyanotic congenital heart disease)

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14
Q

what are 2 clinical features of R>L shunts?

A

clubbing of fingers & toes (hypertrophic osteoarthropathy)

AND

polycythemia

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15
Q

what are the clinical situations assoc w/ R>L shunt?

A

tetralogy of fallot

transposition of great vessels

persistent truncus arteriosus

tricuspid atresia

total anomalous pulmonary venous connection

paradoxical embolism

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16
Q

what are the 4 cardinal features of tetralogy of fallot?

A

ventricular septal defect

subpulmonary stenosis

overriding aorta

R ventricular hypertrophy

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17
Q

T/F: in tetralogy of fallot, the ventricular septal defect is small

A

FALSE

normally large

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18
Q

what is subpulmonary stenosis?

A

obstruction of the R ventricular outflow tract

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19
Q

what is overriding aorta?

A

aorta forms the superior border of the VSD & therefore overrides the defect of both ventricles

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20
Q

what does tetralogy of fallot result from?

A

anterosuperior displacement of the infundibular septum

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21
Q

T/F: pts always die of tetralogy of fallot

A

false

can survive into adulthood even untreated

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22
Q

what are the clinical consequences of tetralogy of fallot dependent on?

A

degree of subpulmonic stenosis

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23
Q

what does mild subpulmonic stenosis resemble?

A

isolated VSD

acts more like a L>R shunt w/o cyanosis

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24
Q

what characterizes severe tetralogy of fallot?

A

greater resistance to RV outflow & R>L shunt

hypoplastic pulmonary arteries & aortic dilation

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25
Q

what is classic tetralogy of fallot?

A

severe

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26
Q

what is pink tetralogy of fallot?

A

mild

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27
Q

how do pts w/ tetralogy of fallot present at birth?

A

cyanotic

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28
Q

can you repair tetralogy of fallot?

A

yes complete surgical repair is possible

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29
Q

in transposition of the great vessels, where does the aorta come from?

A

R ventricle

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30
Q

in transposition of the great vessels, where does the pulmonary artery come from?

A

L ventricle

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31
Q

T/F: in transposition of the great vessels, the aorta is in front of the pulmonary artery

A

TRUE

this is the opposite of normal

(whatever vessel comes from the R ventricle goes in front, so normally that’s the pulmonary artery, but in transposition, it’s the aorta)

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32
Q

why does transposition of the great vessels occur?

A

abnormal formation of the truncal & aortopulmonary septa

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33
Q

what does the outcome of transposition depend on?

A

degree of blood mixing (via shunt)

magnitude of tissue hypoxia

ability of RV to maintain systemic circulation

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34
Q

what are the possible shunts in transposition?

A

VSD

patent foramen ovale

ductus arteriosus

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35
Q

which shunts are unstable in transposition?

A

patent foramen ovale & ductus arteriosus

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36
Q

which shunts are stable in transposition?

A

VSD

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37
Q

which shunts happen more in transposition?

A

PFO & ductus arteriosus: 65%

VSD: 35%

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38
Q

what do you do w/ pts that have an unstable shunt in transposition?

A

balloon atrial septostomy

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39
Q

what are the clinical features of transposition?

A

R ventricular hypertrophy

atrophic L ventricle

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40
Q

T/F: w/o surgery, most transposition pts live to 5 yo

A

FALSE

w/o surgery, most die w/i first months of life

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41
Q

how long can pts w/ transposition live if they get surgery?

A

into adulthood

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42
Q

why does persistent truncus arteriosus happen?

A

failure of separation of the embryologic truncus arteriosis into the aorta & pulmonary artery

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43
Q

what is persistent truncus arteriosus?

A

single great vessel receives blood from both ventricles & coronary circulation

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44
Q

why is there a danger of irreversible pulmonary HTN w/ persistent truncus arteriosus?

A

associated VSD that produces systemic cyanosis & increased pulmonary blood flow

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45
Q

what is tricuspid atresia?

A

complete obstruction of the tricuspid valve orifice

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46
Q

why does tricuspid atresia happen?

A

unequal division of the AV canal

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47
Q

what does tricuspid atresia result in?

A

enlarged mitral valve

underdeveloped R ventricle

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48
Q

how do the L ventricle & pulmonary artery communicate in tricuspid atresia?

A

R>L shunt thru atria (ASD, PFO) & a VSD arises from hypoplastic R ventricle

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49
Q

how do babies w/ tricuspid atresia look?

A

cyanosis

high mortality rate

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50
Q

what happens in total anomalous pulmonary venous circulation (TAPVC)?

A

pulmonary veins fail to join the L atrium

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51
Q

why do you get TAPVC?

A

failure of development (atresia) of common pulmonary vein

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52
Q

during fetal life, where do the primitive systemic venous channels drain from & to?

A

from the lung

into left innominate vein or coronary sinus

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53
Q

how does venous blood enter the atrium in TAPVC?

A

PFO or ASD

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54
Q

what are the clinical features of TAPVC?

A

volume & pressure overload >>> hypertrophy & dilation of R heart

dilation of pulmonary trunk

hypoplastic L atrium

possible cyanosis d/t R>L shunt of ASD

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55
Q

what do L>R shunts increase?

A

pulmonary blood flow & volume

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56
Q

T/F: pulmonary circuit is usu low flow, high resistance

A

FALSE

low flow, low resistance

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57
Q

what is the first response to increased blood flow in a L>R shunt?

A

medial hypertrophy vasoconstriction to maintain distal pressures & prevent pulmonary edema

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58
Q

what does prolonged increased pulmonary vasoconstriction in L>R shunt induce?

A

obstructive intimal lesions (hyaline & hyperplastic arteriosclerosis)

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59
Q

what happens w/ pulmonary vascular resistance in a L>R shunt?

A

it reaches systemic levels & converts the L>R shunt to a R>L shunt

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60
Q

what is the L>R to R>L reversal called?

A

late cyanotic CHD or Eisenmenger syndrome

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61
Q

after the irreversible pulmonary HTN develops, can you still repair the structural defects of CHD?

A

NOPE

62
Q

what are 3 examples of L>R shunts?

A

ASD

VSD

PDA

63
Q

what is ASD?

A

abnormal fixed opening in the atrial septum

64
Q

what causes ASD?

A

incomplete tissue formation that allows communication of blood btwn L & R atria

65
Q

are PFO & ASD the same thing?

A

nope

66
Q

what are the 3 types of ASD?

A

secundum

primum

sinus venosus

67
Q

what is the most common type of ASD?

A

secundum: 90%
primum: 5%

sinus venosus: 5%

68
Q

what is ASD secundum?

A

results from deficient or fenestrated oval fossa near center of atrial septum

69
Q

where does ASD primum occur?

A

adjacent to AV valves

70
Q

where does ASD sinus venosus occur?

A

near the entrance of the SVC

71
Q

what is ASD sinus venosus assoc w/?

A

anomalous pulmonary venous return to the R atrium

72
Q

what are the clinical features of ASD?

A

L>R shunt d/t lower pulmonary vascular resistance & greater R ventricle distensibility

MURMUR d/t inc pulmonary blood flow

no sx before 30 yo

73
Q

T/F: irreversible pulmonary HTN is common w/ ASD

A

FALSE

it’s unusual

74
Q

how do you prevent complications w/ ASD?

A

surgical or catheter-based repair

75
Q

what are 3 complications of ASD?

A

heart failure

paradoxical embolization

irreversible pulmonary vascular disease

76
Q

what is the foramen ovale?

A

it’s a small hole in the atrial septum at the oval fossa that allows oxygenated blood from the placenta to bypass the lungs in the fetus

77
Q

what happens to the foramen ovale after birth?

A

it is forced shut d/t increased BP on the L heart in 80% of ppl

78
Q

in 20% of people where the foramen ovale doesn’t close, what happens in times of increased R sided pressure?

A

the flap opens & can create a R>L shunt

79
Q

what can cause increased R sided pressure?

A

bowel movement

coughing

sneezing

80
Q

T/F: there is a small possibility of paradoxical embolism w/ PFO

A

TRUE

81
Q

what is the most common form of congenital cardiac anomaly?

A

VSD

82
Q

how many VSDs occur in isolation?

A

20-30%

most are assoc w/ another congenital anomaly

83
Q

how do you classify VSD?

A

size & location

84
Q

what type of VSD is most common?

A

membranous (90%)

85
Q

what are VSDs below the pulmonary valve called?

A

infundibular VSD

86
Q

what is a swiss cheese septum?

A

sometimes multiple VSDs w/i the muscular septum

87
Q

what do the clinical manifestations of VSD depend on?

A

size & assoc R-sided malformation of the VSD

88
Q

what happens w/ a small VSD?

A

well-tolerated

may close spontaneously

89
Q

what happens w/ a large VSD?

A

difficulties from birth

significant L>R shunting

90
Q

a large VSD is usually what type?

A

membranous or infundibular

91
Q

do you have to correct large VSDs?

A

YES

early!

92
Q

what is the purpose of the ductus arteriosus in a fetus?

A

shunts blood from the pulmonary artery to the aorta

93
Q

what happens in PDA?

A

the ductus arteriosus doesn’t close spontaneously after birth

94
Q

does PDA produce a murmur?

A

YES

harsh, machine-like

95
Q

what % of PDAs are isolated?

A

90%

10% are assoc w/ coarctation of the aorta or pulmonic or aortic valve stenosis

96
Q

what do clinical features of PDA depend on?

A

diameter & CV status

97
Q

T/F: PDA pts are asymptomatic at birth

A

TRUE

98
Q

what type of PDA does NOT affect a child’s growth & development?

A

narrow

99
Q

what can a larger PDA lead to?

A

Eisenmenger syndrome

starts as L>R shunt though

100
Q

when should the PDA be closed?

A

early in life

101
Q

how can you keep a PDA open?

A

prostaglandin E

102
Q

what is another name for atrioventricular septal defect?

A

atrioventricular canal defect

103
Q

how does AVSD happen?

A

embryologic failure of the superior & inferior endocardial cushions of the AV canal to fuse adequately

104
Q

how does AVSD manifest?

A

incomplete closure of the AV septum

malformation of tricuspid & mitral valves

105
Q

what are the 2 forms of atrioventricular septal defect?

A

partial & complete

106
Q

what is partial AVSD?

A

primum AVSD & cleft anterior mitral leaflet > mitral insufficiency

(idk what any of this means)

107
Q

what is complete AVSD?

A

large combined AVSD + large common AV valve

108
Q

what is a large common AV valve?

A

a big hole in the center of the heart

109
Q

what happens in complete AVSD?

A

all 4 chambers communicate so you get volume hypertrophy

110
Q

1/3 of complete AVSD pts have what disorder?

A

down syndrome

111
Q

can you repair AVSD?

A

yes

surgical repair is possible

112
Q

what are the 3 types of obstructive congenital heart disease?

A

coarctation of the aorta

aortic valvular stenosis

pulmonary valvular stenosis

113
Q

T/F: coarctation of the aorta is rare

A

FALSE

it’s a common structural abnormality

114
Q

what disorder is coarctation of the aorta assoc w/?

A

turner syndrome (XO)

115
Q

what are the 2 forms of coarctation?

A

infantile

adult

116
Q

what is infantile coarctation?

A

tubular hypoplasia of the aortic arch proximal to PDA

117
Q

are pts w/ infantile coarctation symptomatic?

A

yes

right after birth (slide 32)

in early childhood (slide 31)

118
Q

what is adult coarctation?

A

discrete ridgelike infolding of the aorta opposite of closed ductus arteriosus (ligamentum arteriosum) distal to arch vessels

119
Q

what does the clinical manifestation of coarctation depend on?

A

severity of narrowing of the lumen

patency of ductus arteriosus

120
Q

what happens if you don’t surgically intervene w/ infantile coarctation?

A

high mortality rate

121
Q

why does infantile coarctation cause lower extremity cyanosis?

A

delivery of unsaturated blood thru the PDA

122
Q

T/F: the adult form of coarctation is immediately life-threatening

A

false

children may go unrecognized until adulthood unless severe

123
Q

what are the sx of adult coarctation?

A

upper extremities: HTN

lower extremities: weak pulses, hypotension, claudication, coldness

Notching on radiograph

124
Q

what causes the Notching on radiograph of adult coarctation?

A

development of collateral circulation btwn pre & post coarctation arteries via enlarged intercostal & internal mammary arteries

125
Q

is surgical repair of adult coarctation possible?

A

yes

126
Q

T/F: pulmonary stenosis is common

A

TRUE

127
Q

T/F: pulmonary stenosis is always isolated

A

false

may be isolated or may be part of a syndrome (TOF, TGA)

128
Q

what is the result of pulmonary stenosis?

A

right ventricular hypertrophy

129
Q

what is the difference btwn mild & severe pulmonary stenosis?

A

mild can be asymptomatic

severe requires surgical repair

130
Q

what is pulmonary atresia?

A

no communication btwn R ventricle & lungs

131
Q

what are the results of pulmonary atresia?

A

hypoplastic R ventricle

ASD

132
Q

how does blood reach the lungs in pulmonary atresia?

A

PDA

133
Q

where can the aorta narrow?

A

valvular

subvalvular

supravalvular

134
Q

what are the cusps like in valvular aortic stenosis/atresia?

A

may be hypoplastic, thickened, nodular, or abnormal in number

135
Q

what causes subvalvular aortic stenosis/atresia?

A

thickened ring or collar of dense endocardial fibrous tissue BELOW the cusps

136
Q

what is subvalvular aortic stenosis/atresia assoc w/ clinically?

A

prominent systolic murmur

137
Q

what is the result of subvalvular aortic stenosis/atresia?

A

pressure hypertrophy of the L ventricle

138
Q

how do you manage mild aortic stenosis?

A

conservatively w/ abx to prevent endocarditis

139
Q

what is supravalvular aortic stenosis/atresia an inherited form of?

A

aortic dysplasia

140
Q

what happens to the ascending aorta in supravalvular aortic stenosis/atresia?

A

it is thickened > causes luminal constriction

141
Q

what does severe aortic stenosis/atresia cause?

A

obstruction to L ventricular outflow

hypoplasia of L ventricle & ascending aorta

142
Q

what is another name for severe aortic stenosis/atresia?

A

hypoplastic left heart syndrome

143
Q

what do you NEED to live w/ severe aortic stenosis/atresia?

A

PDA

144
Q

T/F: You can live w/ severe aortic stenosis/atresia w/ no repair

A

FALSE

nearly always fatal unless surgically repaired

145
Q

in what percentage of people does the foramen ovale stay open/patent?

A

20%

146
Q

what is this?

A

tricuspid atresia

147
Q

what is this?

A

transposition of the great vessels

148
Q

what is this?

A

tetralogy of fallot

149
Q

what is this?

A

patent foramen ovale

150
Q

what is this?

A

persistent truncus arteriosus

151
Q

what is this?

A

TAPVC

Block 9 Week 1 (4 decks)

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