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Paediatric rotation > Paediatric cardiology > Flashcards

Paediatric cardiology Flashcards

1
Q

How many shunts allow the blood in fetal circulation to bypass the lungs?

A

three

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2
Q

What are the three fetal shunts?

A

Ductus venosus
Foramen Ovale
Ductus arteriosus

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3
Q

What does the ductus venosus connect and what does this allow?

A

Connects the umbilical vein to the inferior vena cava and allows blood to bypass the liver

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4
Q

What does the foramen ovale connect and what does this allow?

A

Connects the right atrium with the left atrium and allows blood to bypass the right ventricle and pulmonary circulation

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5
Q

What does the ductus arteriosus connect and what does this allow?

A

Connects the pulmonary artery with the aorta and allows blood to bypass pulmonary circulation

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6
Q

What happens in the first breath a baby takes?

A

Alveoli expands
Pulmonary vascular resistance decreases
Pressure in right atrium falls
Left atrial pressure is greater than right atrial pressure
Atrial septum causes closure of the foramen ovale
After a few weeks the foramen ovale becomes the fossa ovalis

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7
Q

What is required to keep the ductus arteriosus open?

A

Prostaglandins

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8
Q

What causes a drop in prostaglandins in a newborn and what does this then cause?

A

Increased blood oxygenation. This causes closure of ductus arteriosus which becomes ligamentum arteriosum

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9
Q

What stops functioning immediately after birth and why?

A

Ductus venosus because the umbilical cord is clamped and there is no flow in the umbilical veins. This becomes the ligamentum a few days later

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10
Q

What are innocent murmurs also known as?

A

Flow murmurs

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11
Q

What are flow murmurs caused by?

A

Fast blood flow through various areas of the heart during systole

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12
Q

What are the typical features of flow murmurs? (S)

A

Soft
Short
Systolic
Symptomless
Situation dependent (quieter with standing or only appears in unwell child)

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13
Q

What features prompt further investigation in a flow murmur?

A

Murmur louder than 26
Diastolic murmur
Louder on standing
Other symptoms

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14
Q

What investigations may be done to establish cause of murmur in a child?

A

ECG
Chest xray
Echocardiography

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15
Q

What are the differentials of pan-systolic murmurs and where are they heard loudest?

A

Mitral regurgitation (mitral area- 5th intercostal space, mid-clavicular line)
Tricuspid regurgitation (tricuspid area- 5th intercostal space, left sternal border)
Ventricular septal defect (left lower sternal border)

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16
Q

What are the differentials of ejection-systolic murmurs and where are they heard loudest?

A

Aortic stenosis (aortic area- 2nd intercostal space, right sternal border)
Pulmonary stenosis ( pulmonary area- 2nd intercostal space, left sternal border)
Hypertrophic obstructive cardiomyopathy (4th intercostal space on the left sternal border)

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17
Q

What causes a splitting heart sound?

A

In inspiration, chest wall and diaphragm pulls the lungs open and heart causing negative intra-thoracic pressure, causing right side of heart to fill faster meaning right vebtricle takes longer to empty cause of increased volume causing a delay in pulmonary valve closing

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18
Q

What sound do atrial-septal defects cause?

A

mid-systolic, crescendo-decrescendo murmur that is loudest at the upper left sternal border with a fixed split second heart sound

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19
Q

What is the fixed split heart sound like in an atrial-septal defect?

A

The split second heart sound does not change with inspiration and expiration.

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20
Q

What are the heart sound changes in patent ductus arteriosus?

A

small one may not have any abnormal sounds. Big ones will cause a normal first heart sound with a continuous crescendo-decrescendo machinery murmur that may continue during the second heart sound, making it difficult to hear

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21
Q

What is the murmur like in tetralogy of fallot?

A

arises from pulmonary stenosis, giving an ejection systolic murmur loudest at the pulmonary area (2nd intercostal space, left sternal border)

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22
Q

When does cyanosis occur?

A

When deoxygenated blood enters the systemic circulation. Occurs in a right to left shunt

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23
Q

What heart defects cause a right to left shunt?

A

Ventricular septal defect
Atrial septal defect
Patent ductus arteriosus
Transposition of the great arteries

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24
Q

Are patients with VSD, ASD or PDA normally cyanotic?

A

No beacuse pressure prevents this. If pulmonary pressure becomes higher than systemic pressure then blood starts to flow right to left causing cyanosis- eisenmenger syndrome

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25
Q

Which heart defect always results in cyanosis?

A

Transposition of the great arteries because the right side of the heart pups blood directly into the aorta and systemic circulation.

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26
Q

When does the ductus arteriosus normally stop functioning?

A

1-3 days of birth and closes completely within the first 2-3 weeks of life

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27
Q

What might cause patent ductus arteriosus?

A

Prematurity
Maternal rubella
genetic

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28
Q

What is the pathophysiology of PDA?

A

Pressure in aorta is higher than pulmonary vessels so blood flows in left to right shunt. This increases pressure in pulmonary vessels causing pulmonary HTN leading to right sided strain and hypertrophy. This then leads to left ventricular hypertrophy

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29
Q

What are the symptoms of PDA in a newborn?

A

Murmur
SOB
Difficulty feeding
Poor weight gain
Lower respiratory tract infections

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30
Q

How is PDA diagnosed?

A

Echocardiogram- see left to right shunt and hypertrophy

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31
Q

What is the management for PDA?

A

Monitored till 1 year old until it is unlikly the hole will close- trans-catheter or surgical closure

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32
Q

What is the pathophysiology of atrial septal defects?

A

During development, left and right atria are connected. Two walls grow downwards from the top of the heart then fuse together, called the endocardial cushion. These two walls are called the septum primum and septum secondum. Holes in these walls are ASDs

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33
Q

What are the types of atrial septum defect from most common?

A

Ostium secondum- septum secondum fails to fully close.
Patent foramen ovale- foramen ovale fails to close (although not strictly an ASD)
Ostium primum- septum primum fails to fully close- tend to lead to atroventricular valve defects

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34
Q

What are the complications of ASD?

A

Stroke
Atrial fibrillation/flutter
Pulmonary HTN and right sided heart failure
Eisenmenger syndrome

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35
Q

What are the signs of ASD?

A

Murmur
SOB
Difficulty feeding
Poor weight gain
Lower respiratory tract infections

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36
Q

What did the PREMIUM trial find?

A

There is a possible link between migraine with aura and patent foramen ovale

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37
Q

What is the management for ASD?

A

If small can watch and wait
Can be closed surgically with transvenous catheter closure or open heart surgery

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38
Q

What medication is used to reduce risk of clots in adults?

A

Anticoagulants (aspirin, warfarin, NOACs)

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39
Q

What conditions are often associated with VSDs?

A

Down’s syndrome and Turner’s syndrome

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40
Q

What is the presentation of VSD?

A

often symptomless and can present in adulthood but often picked up on antenatal scan or newborn baby check
Poor feeding
Dyspnoea
Tachypnoea
Failure to thrive

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41
Q

what are the examination findings of VSD?

A

pan-systolic murmur heard more at the left lower sternal border in the third and fourth intercostal spaces.
There may be a systolic thrill on palpation

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42
Q

What are the causes of pan-systolic murmur?

A

VSD, mitral regurgitation and tricuspid regurgitation

43
Q

How is VSD managed?

A

Often they close spontaneously but can be treated with transvenous catheter via the femoral vein or open heart surgery

44
Q

What is there an increased risk of in VSD?

A

Infective endocarditis

45
Q

What underlying lesions can result in eisenmenger syndrome?

A

ASD, VSD, PDA

46
Q

When can Eisenmenger syndrome develop?

A

1-2 years with large shunts or adulthood with small shunts.

47
Q

When can eisenmenger syndrome develop more quickly?

A

During pregnancy

48
Q

What occurs due to cyanosis?

A

Bone marrow responds to low oxygen by producing more red blood cells an haemoglobin. This leads to polycythaemia. A high concentration of red blood cells and haemoglobin make the blood more viscous, making patients more prone to thrombus formation.

49
Q

What is polycythaemia?

A

High concentration of haemoglobin in the blood

50
Q

What symptom results from polycythaemia?

A

Plethoric complexion

51
Q

What are the examination findings in eisenmenger syndrome?

A

Pulmonary hypertension:
- Right ventricular heave
- Loud P2
- Raised JVP
- Peripheral oedema

Underlying septal defect symptom

Right to left shunt and chronic hypoxia
- Cyanosis
- Clubbing
- Dyspnoea
- Plethoric complexion

52
Q

What examination findings are there in eisenmenger syndrome related to underlying septal defect?

A

ASD- mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border
VSD- pan-systolic murmur loudest at the left lower sternal border
PDA- continuous crescendo-decrescendo machinery murmur
Arrhythmias

53
Q

What is the prognosis of eisenmenger syndrome and what are the main causes of death?

A

Reduces life expectancy y around 20 years compared with healthy individuals. The main causes of death are heart failure, infection, thromboembolism and haemorrhage. The morality can be up to 50% in pregnancy

54
Q

What is the only definitive treatment of eisenmenger syndrome?

A

heart-lung syndrome

55
Q

What does medical management involve in eisenmenger syndrome?

A

Pulmonary HTN: Sildenafil
Arrhythmias
Polycythaemia: venesection
Thrombosis: anticoagulation
Infective endocarditis: Prophylactic antibiotics

56
Q

What is coarctation of the aorta?

A

Narrowing of the aortic arch, usually around the ductus arteriosus

57
Q

What is coarctation of the aorta associated with?

A

Turner’s syndrome

58
Q

What does coarctation of the aorta do to pressure of the blood?

A

Reduces pressure of blood flowing to the arteries distal to the narrowing and increases the pressure in areas proximal to the narrowing such as the heart and first three branches of the aorta.

59
Q

What is often the only indication of coarctation in a neonate?

A

Weak femoral pulses

60
Q

What should you do to see if the blood pressure indicates coarctation of the aorta?

A

Four limb blood pressure

61
Q

What murmur may be heard in coarctation of the aorta?

A

systolic murmur heard below the left clavicle and below the left scapula

62
Q

What signs might there be in infancy of coarctation of the aorta?

A

Tachypnoea
Poor feeding
Grey and floppy baby

Over time:
- Left ventricular heave due to left ventricular hypertrophy
- Underdeveloped left arm where there is reduced flow to the subclavian artery
- Underdevelopment of the legs

63
Q

What is the management in critical aortic coarctation where there is risk of heart failure and death shortly after birth?

A

Prostaglandin E is used to keep the ductus arteriosus open while waiting for surgery

64
Q

What are the leaflets that make up the aortic valve?

A

3 and they are the aortic sinuses of Valsalva

65
Q

How many leaflets does the aortic valve have in people born with aortic stenosis?

A

one, two, three of four leaflets

66
Q

What does significant aortic valve stenosis present as?

A

Fatigue, SOB, dizziness and fainting. Symptoms are worse on exertion

67
Q

What is the key examination finding in aortic valve stenosis?

A

Ejection systolic murmur heard loudest at the aortic area (second intercostal space, right sternal border). Crescendo-decrescendo character and radiates to the carotids.

68
Q

What signs might be present on examination in aortic valve stenosis?

A

Ejection click just before the murmur
Palpable thrill during systole
Slow rising pulse and narrow pulse pressure

69
Q

What is the gold standard management for aortic valve stenosis?

A

Echocardiogram

70
Q

What are the options for treating stenosis?

A

Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement

71
Q

What are the complications of aortic valve stenosis?

A

Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmias
Bacterial endocarditis
Sudden death, often on exertion

72
Q

How many leaflets is the pulmonary valve usually made up of?

A

3

73
Q

What other conditions are associated with pulmonary valve stenosis?

A

Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome

74
Q

What are the signs of pulmonary valve stenosis?

A

Often asymptomatic but may be fatigue on exertion, shortness of breath, dizziness and fainting

75
Q

What are the signs of pulmonary valve stenosis?

A

Ejection systolic murmur heard loudest at the pulmonary area (second intercostal space, left sternal border)
Palpable thrill in pulmonary area
Right ventricular heave due to right ventricular hypertrophy
Raised JVP with giant a waves

76
Q

What is the gold standard for diagnosing pulmonary valve stenosis?

A

Echocardiogram

77
Q

What is the management for symptomatic pulmonary valve stenosis?

A

Balloon valvuloplasty via a venous catheter through femoral vein and inferior vena cava into the right side of the heart to the pulmonary valve

78
Q

What are the four coexisting pathologies in tetralogy of fallot?

A

VSD
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy

79
Q

How does overriding aorta contribute to TOF?

A

aortic valve is placed further to the right than normal, above the VSD, meaning when the right ventricle contracts and sends blood upwards, the aorta is in the direction of travel of that blood. therefore a greater proportion of deoxygenated blood enters the aorta from the right side of the heart

80
Q

How does pulmonary valve stenosis contribute to TOF?

A

Provides greater resistance against the flow of blood from the right ventricle. This encourages blood to flow through the VSD and into the aorta rather than taking the normal route into the pulmonary vessels.

81
Q

What direction is the shunt in TOF?

A

Right to left meaning blood bypasses the child’s lungs, causing cyanosis

82
Q

What are the risk factors for TOF?

A

Rubella infection
Increased age of the mother (over 40)
Alcohol consumption in pregnancy
Diabetic mother

83
Q

What is the investigation of choice for all structural congenital cardiac abnormalities?

A

Echocardiogram

84
Q

What might a chest xray show in tetralogy of fallot?

A

Boot shaped heart due to right ventricular thickening

85
Q

What are the signs and symptoms of TOF?

A

Cyanosis
Clubbing
Poor feeding
Poor weight gain
Ejection systolic murmur heard loudest in the pulmonary area (second intercostal space, left sternal border)
Tet spells

86
Q

What are tet spells?

A

Intermittent symptomatic periods where the right to left shunt becomes temporarily worsened precipitating a cyanotic episode.

87
Q

When do tet spells occur?

A

When the child is physically exerting themselves, crying or waking.

88
Q

Why do tet spells occur on exertion?

A

They generate a lot of carbon dioxide which is a vasodilator causing systemic vasodilation and therefore reduces the systemic vascular resistance. Blood flow will choose the path of least resistance, so blood will be pumped from the right ventricle to the aorta rather than the pulmonary vessels, bypassing the lungs

89
Q

What might children do when they experience a tet spell?

A

Older children may squat, younger children can be positioned with their knees to their chest. Squatting increases the systemic vascular resistance encouraging blood to enter the pulmonary vessels.

90
Q

What is the medical management for a tet spell?

A

Oxygen
Beta blockers
IV fluids to increase pre-load
morphine can decrease respiratory drive
sodium bicarbonate can buffer any metabolic acidosis that occurs
Phenylephrine infusion can increase systemic vascular resistance

91
Q

In neonates, what can help with TOF?

A

Prostaglandins to maintain the ductus arteriosus

92
Q

What is the definitive treatment for TOF?

A

Total surgical repair however mortality is around 5%

93
Q

What is the prognosis of TOF with corrective surgery?

A

90% of patients will live into adulthood

94
Q

What is Ebstein’s anomaly?

A

A congenital heart condition where the tricuspid valve is set lower in the right side of the heart, causing a bigger right atrium and a smaller right ventricle. Leading to poor flow from right atrium to right ventricle.

95
Q

Which way is the shunt in Ebstein’s anomaly?

A

right to left shunt

96
Q

What is Ebstein’s anomaly associated with?

A

Wolff-Parkinson-White syndrome

97
Q

What is the presentation of Ebstein’s anomaly?

A

Evidence of heart failure
Gallop rhythm heard on auscultation with third and fourth heart sounds
Cyanosis
SOB and tachypnoea
Poor feeding
Collapse or cardiac arrest

98
Q

When do symptoms of Ebstein’s anomaly typically appear?

A

A few days after birth when the ductus arteriosus closes

99
Q

What is the diagnosis for Ebstein’s anomaly?

A

Echocardiogram

100
Q

What is the management for Ebstein’s anomaly

A

Medical management- treat arrhythmia and heart failure
Prophylactic antibiotics to prevent infective endocarditis
Surgical correction

101
Q

What is transposition of the great arteries?

A

Attachments of the aorta and pulmonary trunk are swapped so the right ventricle pumps blood to the aorta and left ventricle pumps blood to the pulmonary trunk and they don’t mix.

102
Q

What can transposition of the great arteries also be associated with?

A

VSD
Coarctation of the aorta
Pulmonary stenosis

103
Q

When is transposition of the great arteries often diagnosed?

A

Antenatal ultrasound scans

104
Q

What can be used to manage transposition of the great arteries?

A

VSD will allow some time until definitive treatment
Prostaglandin to keep ductus arteriosus open
Balloon septostomy involves inserting a catheter into the foramen ovale via the unbilicus and inflating a balloon to create a large ASD.
Open heart surgery is the definitive management . A cardiopulmonary bypass machine is used to perform an arterial switch procedure within a few days of birth.

Paediatric rotation (14 decks)

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