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Paediatrics > Paediatric Cardiology > Flashcards

Paediatric Cardiology Flashcards

1
Q

Which congenital heart lesions cause a left to right shunt?

A

VSD
PDA
ASD

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2
Q

Which congenital heart conditions cause a right to left shunt ?

A

Tetralogy of fallout

Transposition of the great arteries

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3
Q

Which congenital heart condition causes common mixing of blood?

A

AVSD

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4
Q

Which congenital heart conditions will cause outflow obstruction in a ell child (asymptomatic murmur)?

A

Pulmonary stenosis

Aortic stenosis

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5
Q

Which congenital heart condition causes outflow obstruction, which will present as a sick neonate - collapsed with shock?

A

Coarctation of the aorta

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6
Q

How may congenital heart diseases be divided?

A

Acyanotic and Cyanotic

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7
Q

What is the mechanism which causes acyanotic heart disease?

A

L to R shunt

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8
Q

What is the mechanism that causes cyanotic heart disease?

A

R to L shunt

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9
Q

Causes of acyanotic?

A

ASD
PDA
VSD

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10
Q

Causes of cyanotic?

A 
ALL THE T's:
tetralogy of fallot
Transposition of thereat arteries
Truncal arteriosus
Tricuspid atresia
also AVSD
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11
Q

What are the causes of cyanosis (in a child)?

A 
Foreign body
Asthma
Effusions
Epiglottitis
Pneumonia 
Heart failure 
Tetralogy of fallout
Transposition of the great arteries
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12
Q

What is the management of cyanosis?

A

Warm up
Consider whether responsibility or cardiac cause
Pulse oximetry
give 100% O2 to see whether sats improve

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13
Q

How may central cyanosis present?

A

Blue mucous membranes and lips

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14
Q

How may peripheral cyanosis present?

A

Cold peripheries
Poor CO state –> sluggish circulation
Present with mottled skin

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15
Q

How do foetal RBCs get oxygen?

A

Transfer from mother’s RBCs as foetal RBCs have a higher affinity for oxygen due to the increased number of Hb molecules

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16
Q

How does the blood enter the foetus?

A

Umbilical vein

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17
Q

How saturated is the oxygenated blood to the foetus?

A

~80% oxygenated

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18
Q

How I the blood carried away from the foetus?

A

2 umbilical arteries

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19
Q

What are the adaptation sin the foetal circulation?

A 
Umbilical Vein
Ductus venosus
Foramen ovale
Ductus arteriosus
Two umbilical arteries
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20
Q

What does the ductus venous do?

A

Shunts blood away from the liver
Regulates the flow of blood through the liver sinusoids
Has a sphincter mechanism, which closes when a uterine contraction renders the venous return too high
Prevents sudden overloading of the heart

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21
Q

What is the role of the foramen ovale?

A

Shunts blood from the right atrium to the left atrium, aided by the IVC valve to bypass the lungs. (however, crista dividend prevents a little from going so some goes into RV)

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22
Q

What is the role of the ductus arteriosus?

A

Connects the pulmonary artery to the aorta so blood bypasses the lungs

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23
Q

After 10 minutes of being born what structures close in the foetal circulation?

A

Foramen ovale

Ductus arteriosus

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24
Q

When is a baby classed to have a ‘patent ductus arteriosus’?

A

when the ductus arteriosus has failed to close after 1 months of age (or 1 month after EDD)

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25
Q

What occurs regarding blood flow with a PDA?

A

Flows from aorta into the pulmonary artery (L to R shunt)

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26
Q

What is the usual cause for the ductus arteriosus remaining patent?

A

Defect in the constrictor mechanism of the duct

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27
Q

What are the Sx of PDA?

A

Usually asymptomatic..
•apnoea
•bradycardia
• Increased oxygen requirement

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28
Q

Signs of PDA?

A
  • continuous machinery murmur beneath the left clavicle
  • collapsing or bounding pulse
  • pulmonary HTN
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29
Q

What Ix are done for PDA?

A

CXR, ECG, ECHO*

  • best to diagnose
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30
Q

Mx of PDA?

A
  • Ibuprofen or Indomethacin can cause closure of PDA through prostaglandin E2 Synthase inhibition
  • surgical closure
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31
Q

Which drugs can keep PDA open?

A

Prostaglandins

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32
Q

What is an ASD?

A

Is a hole between two atria - the foramen ovale remains patent
allows for a L-R shunt

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33
Q

How is ASD classified?

A

Primary and secondary

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34
Q

What is primary ASD?

A

defect at the valce

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35
Q

What is secondary ASD?

A

simple ASD

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36
Q

What is the presentation usually?

A

Asymptomatic

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37
Q

What symptoms may be present for ASD?

A

recurrent chest infections or wheeze

Arrhythmias from the 4th decade onwards

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38
Q

What are signs of ASD?

A

Ejection systolic murmur on the upper left sternal edge
Shunt from L-R
Hear turbulence at the pulmonary valve
Hear a split S2

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39
Q

Ix for ASD?

A

CXR, ECG, ECHO**, Cardiac catheter

** gold standard

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40
Q

What may be seen on a CXR for ASD?

A

cardiomegaly
enlarged pulmonary arteries
increased pulmonary vascular markings

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41
Q

What may be seen on an ECG for ASD?

A

right axis deviation and RBBB

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42
Q

What is the Mx of ASD?

A

Corrective surgery

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43
Q

How are VSDs categorised?

A

By size - small and large

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44
Q

What is meant by a small VSD?

A

smaller in diameter than the aortic valve so up to 3mm

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45
Q

What is meant by a large VSD?

A

larger in diameter than aortic valve so greater than 3mm

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46
Q

What are the symptoms of VSD?

A

Usually asymptomatic when small
• heart failure symptoms - breathlessness, failure to thrive
•recurrent chest infections with a large VSD

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47
Q

What are signs of VSD?

A
  • tachypnoea
  • tachycardia
  • hepatomegaly
  • pansystolic murmur at LLSE - (volume depends on size - smaller is louder as more turbulence)
  • quiet P2
  • large VSD may result in an apical mid-diastolic murmur
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48
Q

Ix of VSD?

A
  • CXR
  • ESD
  • ECHO*
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49
Q

What might be seen on CXR for VSD?

A

Cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema

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50
Q

What might be seen on ECG for VSD?

A

When large –> biventricular hypertrophy

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51
Q

Mx of VSDs?

A

Expect small ones to close spontaneously

  • Good dental hygiene to prevent bacterial endocarditis
  • Heart failure drugs - ACE inhibitor (captopril) and diuretics
  • Diet: increase calorie intake
  • Surgical correction
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52
Q

What can occur in children with a large VSD?

A

They have pulmonary HTN which can lead to irreversible damage of the pulmonary capillary vascular bed –> Eisenmenger syndrome.

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53
Q

When is surgery usually performed on a VSD?

A

3-6 months

54
Q

Why is VSD performed when it is?

A

To manage HF and thrive

Prevent permanent lung damage form pulmonary HTN and high blood flow

55
Q

What is coarctation of the aorta (CoA)?

A

A narrowing in the aorta

56
Q

Where are 70% of the coarctations?

A

Preductal - so proximal to the insertion of the ductus arteriosus

57
Q

What is the pathophysiology of CoA?

A

Due to arterial duct tissue encircling the aorta just at the point of the duct insertion, so when the PDA closes, the aorta constricts and causes severe obstruction to LV outflow

58
Q

Who is CoA more common in?

A

females with turner’s syndrome

59
Q

What are the physical signs with CoA?

A

Sick baby with severe HF
Absent femoral pulses
Severe metabolic disease
May get peripheral cyanosis if v severe

60
Q

What is the commonest cause of collapse due to LV outflow obstruction?

A

coarctation of the aorta

61
Q

What may be seen on a CXR for CoA?

A

Cardiomegaly from heart failure and shock

62
Q

Ix for CoA?

A

CXR, ECG, ECHO

63
Q

What is the Mx of CoA?

A

Surgical repair ASAP

64
Q

What syndrome is AVSD commonest in?

A

Down’s syndome (trisomy 21)

65
Q

What do you get in AVSD? - with regards to defects

A

ASD
VSD
Tricuspid regurgitation
Mitral regurgitation

66
Q

Why do you get MR and TR in AVSD?

A

As part of the septum isn’t formed in the heart

67
Q

what is a complete AVSD?

A

Defect in the middle of the heart with a single 5-leaflet valve between the atria and the ventricles, which stretches across the whole AV junction - tends to leak

68
Q

what are the clinical features of AVSD?

A
  • Usually presents on antenatal screening
  • Cyanosis at birth or heart failure at 2-3 weeks of life
  • Lesion being detected on Echo screening in a baby with Down syndrome
  • ECG shows a superior axis
69
Q

What is the Mx of AVSD?

A
  • Treat heart failure medically

* Surgical repair at 3-6 months of age.

70
Q

What is the commonest cause of cyanotic congenital heart disease?

A

Tetralogy of Fallot (50-70%)

71
Q

What are the 4 cardinal signs of Tetralogy of Fallot?

A
  • A large VSD
  • Pulmonary Stenosis
  • Aorta overrides septal defect (allowing RV and LV to eject into it)
  • RV hypertrophy
72
Q

When does tetralogy of fallot present with cyanosis at birth?

A

When there is severe pulmonary stenosis

73
Q

When is tetralogy of fallot usually diagnosed?

A

•antenatally
OR
• following identification of a murmur in first 2 months of life

74
Q

What are the symptoms of Tetralogy of Fallot?

A

Cyanosis
•of lips
• fingertips
•hyper cyanotic spells (but these are super rare now)

75
Q

What are hyper cyanotic spells?

A

Rapid increase of cyanosis usually associated with irritability and inconsolable crying due to severe hypoxia There is breathlessness and pallor due to tissue acidosis. These spells are accompanied by a very short murmur

76
Q

What are signs of tetralogy of fallot

A
  • clubbing in older children
  • loud, harsh, ejection systolic murmur at LSE present from day 1 of life
  • failure to thrive
  • failure to develop normally
  • failure to gain weight
  • cyanosis
  • feeding difficulties
77
Q

What investigations would be done for tetralogy of fallot?

A

CXR, ECG, ECHO, cardiac catheterisation

78
Q

What would be seen on CXR for tetralogy of fallot ?

A
  • relatively small heart with untitled apex (boot shape) due to RV hypertrophy
  • decreased pulmonary vascular markings
79
Q

What would ECG of tetralogy of fallot show?

A

Normal at birth, RV hypertrophy in older children

80
Q

What is gold standard Ix for diagnosis?

A

ECHO

81
Q

What is the management plan for tetralogy of fallot?

A

Treat medically initially then definitive surgery around 3-6 months

82
Q

What medical management is given?

A
  • prostaglandin to maintain PDA (IV 5ng/kg per min)
  • oxygen therapy in very severely cyanosed newborns
  • unless severely ill infants - prevent dehydration and iron deficiency
83
Q

What is the treatment for hypercyanotic attacks ‘test spells’?

A
  • initially place child over parents shoulder or on abdomen
  • try keep child calm to abort the attack
  • prom treatment needed if lasts > 15 min
  • oxygen, morphine, bicarbonate, fluids, sedation, IV propanolol
84
Q

What surgical management is offered?

A

Initially can have palliative surgical procedure if not old enough for primary repair to either shunt from subclavian to pulmonary artery or to dilate the RV outflow tract.

Primary repair is then doe when 3-6 months of age - which closes the septal defect and the enlarges/dilates the RV outflow tract

85
Q

What is transposition the great arteries?

A

Where the aorta is attached to the RV and the pulmonary artery is attached to the LV. ( aorta and PA switch)

86
Q

Why is transposition of the great arteries a cyanotic congenital heart defect?

A

as deoxygenated blood enters from the IVC and then gets pumped to the body via the aorta, no interaction with oxygenated blood.
Oxygenated blood from the lungs goes to LV - then back into PA and all oxygenated.
NO COMMUNICATION BETWEEN PULMONARY AND SYSTEMIC CIRCULATIONS

87
Q

What must be present in order for an infant to survive with transposition of the great arteries?

A

ASD or PDA or VSD

88
Q

How is transposition of the great arteries usually diagnosed?

A

Antenatally on anomaly scan

89
Q

Symptoms of transposition of the great arteries?

A

Cyanosis - usually presents on day 2 when the ductus arteriosus closes

90
Q

Signs of transposition of the great arteries?

A
  • cyanosis
  • second heart sound
  • usually no murmur
91
Q

What Ix for transposition of the great arteries?

A

CXR,ECG, ECHO

92
Q

What would be seen on a CXR for Transposition of the great arteries?

A
  • narrow upper mediastinum with an ‘egg on the side’ appearance of cardiac shadow
  • increased pulmonary vascular markings
93
Q

What is the management of Transposition of the great arteries?

A
  • first step keep ductus arteriosus patent

* then operate and switch the arteries when achieved 6kg in weight and 6 months old

94
Q

What techniques are used to improve mixing of oxygenated and deoxygenated blood in Transposition of the great arteries?

A
  • maintain PDA patency with IV prostaglandin

* cardiac catheterisation to perform an atrial septostomy

95
Q

What is Eisenmenger Syndrome?

A

Occurs when there is high pulmonary blood flow due to a L - R shunt. The pulmonary arteries become thick walled and resistant to flow increases. Gradually, the shunt decreases until it reverses and the child becomes cyanosed (usually after 10-15 years)
So L- R shunt turns into R-L shunt due to pulmonary vascular disease.

96
Q

What is the presentation of eisenmenger syndrome?

A
  • primarily cyanosis
  • dyspnoea, fatigue, syncope,
  • chest pain
  • haemoptysis
97
Q

What are signs of eisenmenger syndrome?

A
  • cyanosis
  • clubbing
  • plethora
  • RV heave with palpable, loud pulmonary component of the second heart sound
  • ejection systolic murmur audible along left sternal border
  • loud second heart sound with a narrow split
  • Graham Steel murmur
98
Q

What is the treatment of eisenmenger syndrome?

A

HEART TRANSPLANT IS THE DEFINITIVE Tx

Treat heart failure, and arrhythmias
Mainly Tx aims to prevent eisenmenger syndrome

99
Q

What is the preventative treatment for eisenmenger syndrome?

A
  • early intervention for high pulmonary blood flow

* prevent infective endocarditis

100
Q

What is hypoplastic left heart syndrome?

A

underdevelopment of the left side of the heart causing:
• small or atretic mitral valve
• diminutive LV
•aortic valve atresia
• ascending aorta v small- usually coarctation

101
Q

How is hypoplastic left heart syndrome usually diagnosed?

A

antenatally

102
Q

If hypoplastic left heart syndrome presents after birth what are the clinical features?

A
  • present with a duct-dependent systemic circulation
  • sickest of all neonates
  • no flow through the left side of the heart - ductal construction leads to profound acidosis and rapid cardiovascular collapse
  • weakness or absence of peripheral pulses
103
Q

What is the management of hypoplastic left heart syndrome?

A

surgery
• Norwood procedure
• glenn or heme-fontal procedure around 6 months
• fontan procedure at 3 years

104
Q

What type of heart disease is aortic stenosis?

A

acyanotic

outflow obstruction

105
Q

what is the pathology of aortic stenosis?

A

Cusps of aortic valve are partly fused - leading to restrictive exit from the left ventricle

106
Q

What is aortic stenosis often associated with?

A

Mitral valve stenosis and coarctation of the aorta

107
Q

What are the clinical features of aortic stenosis?

A 
• majority - asymptomatic murmur
when severe stenosis:
• reduced exercise tolerance
• chest pain on exertion
• syncope
108
Q

What are the physical signs of AS?

A
  • small volume, slow rising pulses
  • carotid thrill
  • ejection systolic murmur @URSE, radiates to the neck
  • delayed and soft A2 sound
  • apical ejection click
109
Q

What investigations are done for AS?

A

CXR, ECG, Echo

110
Q

What may be seen on CXR when aorta stenosis is present?

A

Normal or prominent left ventricle with post-stenotic dilatation of the ascending aorta

111
Q

What is the management of aortic stenosis ?

A

For younger children:
- regular clinics and echo appointments to assess and evaluate when to intervene.
Thenbaloon valvotomy

112
Q

When do you interfere to do surgical management for aortic stenosis?

A
  • symptoms on exercise

* high resting pressure gradient (>64mmHg) across the aortic valve

113
Q

What type of heart disease is pulmonary stenosis?

A

Acyanotic

Outflow obstruction

114
Q

What is pulmonary stenosis usually associated with?

A

patent ductus arteriosus

115
Q

How can PDA partially compensate for pulmonary stenosis?

A

As PDA shunts blood from aorta into the pulmonary artery so that blood gets to the lungs

116
Q

What is the pathology of pulmonary stenosis?

A

Pulmonary valve leaflets are partly fused together and leads to restrictive exit from the RV

117
Q

What are the symptoms of pulmonary stenosis?

A

MOstly asymptomatic

However when severe in neonates - present cyanosed within first few days esp. if still a patent foramen ovale

118
Q

Signs of pulmonary stenosis?

A
  • ejection systolic at ULSE
  • may have a thrill present
  • prominent RV heave when severe
119
Q

Ix of pulmonary stenosis?

A

CXR, ECG, ECHO

120
Q

What may be present in ECG of pulmonary stenosis ?

A

Show RV hypertrophy - upright T wave in V1 lead

121
Q

What is the management of pulmonary stenosis?

A

Conservative when asymptomatic until pressure gradient across pulmonary valve is > 64mmHg and then do percutaneous balloon valvuplasty

122
Q

What is congestive cardiac failure?

A

+LV and RV failure

when the heart is unable to pump sufficiently to maintain blood flow to meet the body’s needs

123
Q

What are the symptoms of left heart failure?

A 
•dyspnoea
•poor exercise tolerance
•fatigue
• cold peripheries
• orthopnoea
•nocturnal cough
•wheeze
•muscle wasting 
• tachycardia
 tachypnoea
124
Q

What are the causes of left heart failure?

A
  • aortic stenosis
  • mitral valve disease
  • large L - R shunts
  • leaky mitral valve
  • coarctation of the aorta
125
Q

What are the hearts compensatory mechanisms?

A
  • increased heart rate (controlled by neural and humeral input)
  • pumps harder (increases ventricular contractility)
  • increases preload
126
Q

What happens when the compensatory mechanisms are no lone able to cope?

A

heart failure

127
Q

What are Sx of Right heart failure?

A 
Hepatomegaly
Peripheral oedema (pittig)
ascites
anorexia
epistaxis
raised JVP
128
Q

What are the causes of Right heart failure?

A

Progressive pulmonary disease
Defects/pathology of myocardium
Epstein’s anomaly

129
Q

What are the clinical signs of CCF in infants?

A
  • tachycardia >150 beats/min
  • tachypnoea >50 breaths/min
  • gallop rhythm
  • hepatomegaly
  • diaphoresis
  • poor weight gain
  • growth problems
  • oedema of face and limbs
130
Q

What are clinical signs of CCF in older children?

A
  • fatigue
  • poor exercise tolerance
  • dyspnoea
  • orthopnoea
  • abdo pain
  • oedema
  • ascites
131
Q

What are the major criteria of the framingham criteria?

A
  • Paroxysmal nocturnal dyspnoea
  • Crepitations
  • Jugular vein distension
  • S3 Gallop
  • Acute pulmonary oedema
  • Cardiomegaly
  • Hepatojugular reflux
  • Increased central venous pressure
  • Weight loss (>4.5kg in 5 days in response to treatment)
132
Q

What are the minor criteria of the framingham criteria?

A
  • Bilateral ankle oedema
  • Dyspnoea (on ordinary exertion)
  • Nocturnal cough
  • Hepatomegaly
  • Tachycardia (>120bpm)
  • Pleural effusion
  • Decrease in vital capacity by 1/3 from maximum recorded

Paediatrics (2 decks)

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