1/2: Cardio - Acaynotic Heart Defects Flashcards

1
Q

How is congenital heart disease in paediatrics divided

A

Into cyanotic or acyanotic congenital heart defects

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2
Q

What are 4 broad categories of causes of congenital heart disease

A
  1. Idiopathic
  2. Genetic
  3. Maternal illness - infection or underlying condition
  4. Maternal exposure to toxins in-utero
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3
Q

What 2 heart conditions is Turner’s Syndrome associated with

A
  • Bicuspid aortic valve

- Coarctation aorta

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4
Q

What 4 heart conditions is DiGeorge’s Syndrome associated with

A
  • TOF
  • PDA
  • VSD
  • CA
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5
Q

What heart condition is William’s syndrome associated with

A

AS

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6
Q

What 2 heart conditions is rubella associated with

A

PS

PDA

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7
Q

What does congenital HD increase the risk of in children

A

Infective Endocarditis

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8
Q

What are the 4 acyanotic congenital heart defects

A
  1. ASD
  2. VSD
  3. Coarctation of the aorta
  4. Patent ductus arteriosus
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9
Q

What are the three cyanotic congenital heart defects

A
  1. TOF
  2. Tricuspid atresia
  3. TGA
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10
Q

What is a way to remember 4 acyanotic CHD

A

4D’s

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11
Q

What is a way to remember 3 cyanotic CHD

A

3T’s

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12
Q

Describe circulation in a foetus

A
  • Oxygenated blood passes from mother into umbilical vein via placenta.
  • Umbilical vein enters IVC by ductus venous
  • Enters right atrium
  • Blood right atrium passes to left atrium via foramen oval
  • Some passes to right ventricle and out pulmonary.a
  • Ligamentum arteriosum in pulmonary.a shunts blood to aorta
  • Umbilical vein originates fro, internal iliac
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13
Q

Explain changes in circulation at birth

A
  • Clamping of the umbilical cord increases systemic vascular resistance
  • As baby breathes air, alveoli expand and surrounding vessels dilate due to oxygenation. This reduces pulmonary pressure. Reduction in pulmonary pressure increases blood flow to the lungs and hence decreases flow through ductus arteriosus which closes at 10-15h following birth
  • Increase systemic vascular resistance increases pressure in right atrium > left atrium causes closure of foramen ovale within 3 minutes
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14
Q

Is peripheral cyanosis a normal or abnormal finding in first 24h

A

Normal

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15
Q

Is central cyanosis normal or abnormal finding in first 24h

A

Abnormal

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16
Q

What does central cyanosis indicate

A

deoxygenated Hb concentration >5

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17
Q

What is used to distinguish respiratory from cardiac causes of neonatal cyanosis

A

Nitrogen wash out test

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18
Q

What is the nitrogen wash out test

A
  • Baby is given 100% oxygen for 10-minutes then an ABG given to measure PaO2
  • If respiratory cause, oxygen should mean deficit resolves. In cardiac cause, additional oxygen will not help
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19
Q

What does a PaO2 of <15kPa indicate on nitrogen wash out test

A

Cyanotic congenital heart disease is the cause of cyanosis

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20
Q

When is paediatric heart failure most common

A

3m life

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21
Q

How is does paediatric HF present

A
  • Poor Feeding
  • SOB
  • Sweating
  • FTT
  • Sacral oedema (due to lying down)
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22
Q

What type of shunt is present in acyanotic heart disease

A

Left-to-Right Shunt

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23
Q

What are 5 causes of acyanotic heart disease

A
ASD
VSD
Patent Ductus arteriosus
Coarctation of aorta 
Aortic stenosis
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24
Q

Explain general pathophysiology of acyanotic heart conditions

A
  • Due to increased pressure in left side (systemic system) compared to right side blood is shunted left to right
  • This means already oxygenated blood passes through pulmonary circulation again
  • This can increase pressure in pulmonary circulation causing pulmonary HTN
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25
Q

What can acyanotic heart conditions eventually lead to

A

Eisenmenger syndrome

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26
Q

What is Eisenmenger syndrome

A
  • Left-to-right shunt increases pulmonary pressure
  • Leads to remodelling of pulmonary vasculature which results in permanent pulmonary HTN
  • To compensate, right ventricle undergoes hypertrophy
  • This increases right-sided pressure reversing shunt from right-to-left causing cyanotic HD
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27
Q

What are the two types of atrial septal defect

A
  1. Ostium primum

2. Ostium secondum

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28
Q

What is the most common type of ASD

A

Ostrium Secondum (70%)

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29
Q

What is the most common congenital HD found in adulthood

A

ASD

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30
Q

Which gender is ASD more common

A

Female (3:1)

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31
Q

What syndrome is osteum secondum ASD associated with

A

Holt-Oram Syndrome

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32
Q

What is holt-oral syndrome called

A

Hand-Heart Syndrome

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33
Q

What is heart-hand syndrome

A

Individual presents with ASD and tri-phalangeal thumb

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34
Q

How does ASD present clinically

A

Asymptomatic in majority.

May present with complications in 3rd-4th decade of life due to pulmonary HTN

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35
Q

How are ASD often detected

A

On incidental exam

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36
Q

How does osteum primum present

A

If large defect may present with heart failure

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37
Q

What is a palpable sign of ASD and why does this happen

A

Right ventricular heave - due to increased blood flow through right-side heart

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38
Q

What murmur is present in ASD and why does it happen

A

Ejection Systolic - due to increased/more blood flow through narrow (same-size) pulmonary valve causing turbulence

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39
Q

What HS is present in ASD and why does it happen

A

Split S2 - Due to more blood flow in left side of the heart it takes longer for pulmonary valve to close

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40
Q

Why is ostium primum more serious

A

As it involves endocardial tissue which is used to form mitral and tricuspid valves. Therefore is also associated with valve regurgitation.

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41
Q

Why is ostium secundum less serious than primum

A

Does not involve endocardial cushions used for valves

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42
Q

What is first-line investigation in suspected ASD

A

ECG

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43
Q

What will ECG show in a small shunt

A

Normal

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44
Q

What will ECG show in larger shunt

A
  • P pulmonale

- Right axis deviation

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45
Q

What may a CXR show in ASD

A

Pulmonary plethora - evidence increased blood flow to lungs

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46
Q

What is diagnostic of ASD

A

ECHO

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47
Q

How is ostium primum managed

A

surgical closure before pre-school

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48
Q

How is ostium secundum managed

A

surgical closure before school age

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49
Q

How are ASD closed

A

Either via cardiac catheterisation or open heart surgery

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50
Q

Without repair what will happen in ostium primum

A

Lead to heart failure in childhood

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51
Q

Without repair what will happen in ostium secundum

A

Lead to heart failure in 3-4th decade life

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52
Q

What is the most common congenital HD

A

VSD

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53
Q

What % of congenital HD is VSD

A

25%

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54
Q

What are VSD caused by

A
  • Chromosomal abberations
  • Maternal toxins in-utero
  • Congenital infection
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55
Q

What does clinical presentation of VSD depend on

A

Size

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56
Q

How will a small VSD present

A

Asymptomatic

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57
Q

How may a small VSD be found

A

Heart murmur on exam

58
Q

What % of VSD spontaneously close

A

75% close by 10y

59
Q

What is the main risk of small VSD

A

Infective endocarditis

60
Q

When will medium VSD cause clinical symptoms

A

In infancy/childhood

61
Q

What are the symptoms of medium VSD

A

Presents as heart failure

62
Q

How will heart failure in infants present

A
  • Poor feeding
  • Increased work of breathing
  • Failure to thrive
  • Sacral oedema
63
Q

What may precipitate symptoms in medium VSD

A

Infection as this increases cardiac demand

64
Q

Explain prognosis of medium VSD

A

As the child grows, the VSD will get smaller and majority will close spontaneously. Medications are used at the time to relieve symptoms.

65
Q

Explain symptoms of large-VSD

A

Presents in first-few days of life with heart failure

66
Q

What is the complication of large VSD

A

If not closed surgically, Eisenmenger syndrome will ensue

67
Q

What murmur is present in VSD

A

pan systolic

68
Q

What is paradoxical about VSD

A

small the VSD and therefore less severe, louder murmur, due to more turbulence.

69
Q

What is palpable in VSD

A

thrill

70
Q

Where does VSD usually occur

A

pars membranacea - membranous part of ventricular septum

71
Q

What shunt will defect in pars membranacea cause and why

A

left-to-right due to higher pressure in left side (systemic system)

72
Q

What is used to investigate VSD

A

ECHO with doppler

73
Q

How is small VSD managed

A
  • Follow up ECHO

- Usually spontaneous closure

74
Q

How is a symptomatic VSD managed

A

Treat HF: ACEi, B-blocker, Diuretics

75
Q

What are the indications for surgical closure of VSD

A
  • Pulmonary HTN and less than 1y

- Failure to control symptoms on medical management

76
Q

What are 4 complications of VSD

A
  1. Aortic regurgitation
  2. Pulmonary HTN
  3. Right HF
  4. Eisenmenger complex
77
Q

Why does aortic regurgitation occur in VSD

A

Defect in pars membranacea reduces support of aortic valve causing regurgitation

78
Q

What is contraindicated in adults with pulmonary HTN and why

A

Pregnancy. Due to 30-50% associated mortality

79
Q

What is patent ductus arteriosus

A

Failure of ductus arteriosus to close during post-natal period

80
Q

In which gender is a patent ductus arteriosus more common

A

F (2:1)

81
Q

What % of PDA is an isolated heart defect

A

90%

82
Q

What % of PDA is associated with another heart anomaly

A

10%

83
Q

What are three risk factors for PDA

A
  • Rubella infection during first trimester
  • Prematurity
  • Baby born at high altitude
84
Q

What are symptoms of a small PDA

A

Asymptomatic

85
Q

How will a large PDA present

A

HF in infancy

86
Q

What are the 2 symptoms of right-sided HF in infants

A
  • Sacral oedema

- Hepatosplenomegaly

87
Q

What are the symptoms of left-sided HF in infants

A
  • Breathlessness
  • Poor Feeding
  • Recurrent LRTI (due to pulmonary congestion)
  • Fatigue
  • FTT
  • Cold extremities (cyanosed)
88
Q

What can be felt in PDA

A
  • Heaving apex beat

- Left sub-clavicular thrill

89
Q

What are three characteristics of pulse in PDA

A
  • Bounding
  • Wide pulse pressure
  • Collapsing
90
Q

Why is pulse collapsing in PDA

A

As with each systole, blood passes from aorta to pulmonary.a (lower pressure)

91
Q

What murmur is heard in PDA

A

Continuous murmur through systole and diastole - described as machinery murmur

92
Q

Where is the murmur in PDA loudest

A

Infra-clavicular region

93
Q

What is the role of ductus arteriosus in the foetus

A
  • In the foetus oxygen is obtained through placenta not foetal lungs
  • Majority of blood in right atrium passes to left atrium by foramen ovale
  • Remaining blood enters pulmonary.a. Shunted to aorta by ductus arteriosus
  • Therefore bypass lungs
94
Q

What keeps ductus arteiosus open in-utero

A

PGE2 released from the placenta and foramen ovale

95
Q

What happens at birth to the ductus arteriosus

A

Placenta is removed. Foramen Ovale closes - reducing PGE2 secretion resulting in closure.

Once oxygenated, lungs release bradykinin that closes smooth muscle component of DA

96
Q

When does the ductus arteriosus collapse to form the ligamentous arteriosum

A

3W

97
Q

When is the ductus arteriosus regarded as patent

A

> 3W

98
Q

Why is PDA acyanotic

A

As blood shunts from aorta into pulmonary.a down a pressure gradient. Therefore already oxygenated blood cycles

99
Q

What is first-line investigation for PDA

A

ECG

100
Q

What will be seen on ECG in small PDA

A

Normal

101
Q

What will be seen on ECG in large PDA

A

Left axis deviation due to left ventricular hypertrophy

102
Q

What other investigations are ordered in suspected PDA

A

CXR, ECHO

103
Q

What will be seen on CXR in PDA

A
  • Prominent aortic knob

- Prominent pulmonary. a

104
Q

What is confirmatory diagnostic test in PDA

A

ECHO

105
Q

When is elective surgical PDA closure indicated

A
  • Symptomatic
  • Pulmonary HTN
  • Non-spontaneous closure
106
Q

What is used first-line to close PDA in pre-term infants

A

Indomethacin

107
Q

What is used to close PDA in infants >5Kg

A
  • Percutaneous catheter occlusion

- Surgical ligation

108
Q

How is PDA managed if associated with other heart defects

A

PGE2 given to keep it open then closed surgically later

109
Q

What is prognosis of PDA

A

Typically spontaneously closing

110
Q

What is timeline of PDA

A

If it has not closed by 2W in term-infants it is unlikely to

111
Q

What are three complications of PDA

A
  1. HF
  2. Infective endocarditis
  3. Eisenmenger
112
Q

What is coarctation of the aorta

A

Congenital narrowing of the aorta

113
Q

What are the two forms of coarctation of the aorta

A

Infant (70%)

Adult (30%)

114
Q

What % of coarctation occurs during infancy

A

70%

115
Q

Where does coarctation often occur

A

Adjacent to ductus arteriosus

116
Q

What defect is coarctation associated with

A

Patent ductus arteriosus

117
Q

What % of coarctation is in adults

A

30

118
Q

What gender is coarctation more common

A

Males (2:1)

119
Q

What are 4 associations with coarctation

A
  • Berry aneurysms
  • Neurofibromatosis
  • Tuner’s Syndrome
  • Bicuspid aortic valve
120
Q

When will symptoms neonatal coarctation present

A

When the ductus arteriosus closes ~2w

121
Q

What are the two symptoms of coarctation in neonates

A
  • Heart Failure

- Cyanosis of lower extremities (differential cyanosis)

122
Q

What is a sign of coarctation in neonates

A
  • Radio-femoral delay.

- Difference in BP between upper and lower extremities.

123
Q

What are 5 symptoms of coarctation in children

A
  • Cold feet
  • Claudication
  • Chest pain
  • Radio-femoral delay
  • HTN in upper extremities. Hypotension in lower extremities
124
Q

What are 2 signs of coarctation in children

A
  • Ejection systolic murmur over left paravertebral region

- Continous murmur over left sternal edge radiates to between scapula

125
Q

What are 6 symptoms of coarctation in adults

A
  1. HTN
  2. Epistaxis
  3. Tinnitus
  4. Headache
  5. Claudication
  6. Radio-femoral delay
126
Q

Where does coarctation tend to occur in infant disease

A

Before ductus arteriosus

127
Q

Explain pathophysiology of coarctation of the aorta

A
  • In coarctation there is narrowing before the ductus arteriosus
  • This reduces blood flow through aorta
  • Causing back flow of pressure in left ventricle but a low pressure in aorta itself
  • This means pressure in the aorta is lower than the pulmonary artery
  • So this means deoxygenated blood passes from pulmonary/a to aorta in a right to left shunt and maintains blood flow
  • Once the duct closes, less blood can reach distal aorta and rest of the body
  • This causes hypo perfusion of organs and multi-organ failure.
  • If small segment defect - may be able to be compensated for by collaterals + hence present in childhood
128
Q

What is first-line investigation for coarctation

A

BP

129
Q

What will be seen on BP in coarctation

A

Difference in BP between upper and lower extremities.

Typically HTN in upper and Hypotension in lower

130
Q

What is second-line investigation of coarctation

A

ECHO

131
Q

What is third-line investigation of coarctation

A

X-ray

132
Q

What are 4 findings on CXR in coarctation

A
  • Cardiomegaly
  • Increased vascular markings
  • Figure of three sign
  • Rib notching
133
Q

What is a figure of three sign

A
  • Dilation pre and post-coarctation causing an hour glass appearance
134
Q

What is rib notching

A

Dilation of internal thoracic and intercostal arteries. This increases pressure on inferior ribs causing atrophy - resultant notching.

135
Q

When is rib notching seen

A

It is a sign of chronic disease and only seen in children >5Y

136
Q

How is coarctation managed in neonates

A
  • PGE1 to keep DA open

- Medical management of HF

137
Q

What is PGE1 called

A

Alprostadil

138
Q

How is coarctation managed in older children

A

Surgical correction

139
Q

How is coarctation managed in adults

A

Angioplasty and stending

140
Q

What is the prognosis of coarctation if untreated

A

20% would survive past 50y

141
Q

Give 6 complications of coarctation

A
  • Secondary HTN
  • HF
  • Aortic dissection
  • CAD
  • Endocarditis
  • Stroke
  • MI
142
Q

What can secondary HTN lead to

A

Cerebral aneurysms

IC haemorrhage