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Year 4 Paediatrics > Cardiology > Flashcards

Cardiology Flashcards

1
Q

What is Eisenmenger syndrome?

A

Eisenmenger syndrome describes the reversal of a left-to-right shunt (patent ductus arteriosus, atrial septal defect, or ventricular septal defect) to a right-to-left shunt

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2
Q

What are the three fetal circulatory shunts?

A

Ductus venosus
Foramen ovale
Ductus arteriosus

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3
Q

Role of the ductus venosus in fetal circulation?

A

Connects the umbilical vein to the inferior vena cava

Allows blood to bypass the liver

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4
Q

Which fetal shunt connects the umbilical vein to the inferior vena cava?

A

Ductus venosus

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5
Q

Which fetal shunt allows blood to bypass the liver?

A

Ductus venosus

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6
Q

Role of the ductus arteriosus in fetal circulation?

A

Connects the pulmonary artery with the aorta

Allows blood to bypass the pulmonary circulation

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7
Q

Which fetal shunt connects the pulmonary artery and the aorta?

A

The ductus arteriosus

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8
Q

Which fetal shunt allows blood to bypass the pulmonary circulation?

A

Ductus arteriosus

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9
Q

Role of the foramen ovale in fetal circulation?

A

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

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10
Q

Which fetal shunt connects the right atrium and left atrium?

A

Foramen ovale

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11
Q

Which fetal shunt allows blood to bypass the right ventricle and pulmonary circulation?

A

Foramen ovale

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12
Q

Why do the 3 fetal shunts exist?

A

So that blood can travel to the placenta and back

Blood does not need to pass through the pulmonary circulation as the fetal lungs are not fully developed of functional

Fetal blood needs to go via the placenta to collect oxygen and nutrients and dispose of waste products via the mother (lactate, CO2)

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13
Q

How does the foramen ovale become the fossa ovalis?

A

Baby takes first breaths - alveoli expand - DECREASE IN PULMONARY VASCULAR RESISTANCE

DECREASE IN PULMONARY VASCULAR RESISTANCE causes pressure to fall in the right atrium and the LEFT ATRIAL PRESSURE BECOMES GREATER THAN THE RIGHT ATRIAL PRESSURE

the change in pressure squshes the septum casuing the foramen ovale to close (similar to a closed valve with nothing flowing through it)

This then gets sealed shut structurally after a few weeks and becomes the fossa ovalis.

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14
Q

What does the foramen ovale become after birth?

A

The fossa ovalis

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15
Q

How does the ductus arteriosus become the ligamentum arteriosum?

A

Increased blood oxygenation after baby takes its first breath causes a drop in circulating prostaglandins.

Prostaglandins are required to keep the ductus arteriosus open.

This causes closure of the ductus arteriosus, which becomes the ligamentum arteriosum.

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16
Q

What does the ductus arteriosus become after birth?

A

Ligamentum arteriosum

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17
Q

How does the ductus venosus become the ligamentum venosum?

A

Immediately after birth the ductus venosus stops function as umbilical cord is clamed and there is no flow in the umbilical veins

The ductus venosus structurally closes a few days later and becomes the ligamentum venosum.

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18
Q

What does the ductus venosum become after birth?

A

Ligamentum venosum

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19
Q

Which fetal shunt was the ligamentum venosum?

A

Ductus venosus

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20
Q

Which fetal shunt was the ligamentum arteriosum?

A

Ductus arteriosus

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21
Q

Which fetal shunt was the fossa ovalis?

A

Foramen ovale

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22
Q

How does fetal circulation change at birth?

A
  1. Neonate takes first breaths - alveoli expand - decrease in in pulmonary vascular resistance leads to a fall in pressure in the right atrium, which squashed the atrial septum causing functional closure of the foramen ovale.

After a few weeks foramen ovale sealed structually becoming the ossa ovalis

  1. Increased blood oxygenation causes a drop in ciculating prostaglandins, causing closure of the ductus arteriosus (which requires prostaglandins to keep it open)

Closure of the ductus ateriosus occurs and it becomes the ligamentum arteriosum

  1. Umbilical cord clamped so flow ceases in umbilical veins, therefore ductus veonsus stops functioning

A few days later the ductus venosus closes structurally and becomes the ligamentum venosum

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23
Q

What are innocent murmurs?

A

'’Flow murmurs’’

Very common in children

Causes by fast blood flow through various areas of the heart during systole

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24
Q

Clear innocent murmurs with no concerning features may not require any investigations. Features that would prompt further investigations and referral to a paediatric cardiologist would include what?

A

Murmur louder than 2/6

Diastolic murmurs

Murmurs which are louder on standing

Other symptoms: failure to thrive, feeding difficulty, cyanosis, SOB

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25
Q

Typical features of innocent murmurs?

A

5 S’s

Soft (1/6 - 2/6)

Short

Systolic

Symptomless

Situation dependent, particularly if the murmur gets quieter with standing or only appears when the child is unwell or feverish

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26
Q

Key investigations to establish the cause of a murmur in children and r/o abnormalities?

A

ECG
Chest Xray
Echocardiography

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27
Q

Differentials of pansytolic murmurs?

A

Mitral regurgitation

Tricuspid regurgitation

Ventricular septal defect

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28
Q

Pansystolic murmur heard loudest at the fifth intercostal space, mid-clavicular line?

A

Mitral regurgitation

(5th ICS mid clavicular line = mitral area)

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29
Q

Pansystolic murmur heard loudest at the fifth intercostal space, left sternal border?

A

Tricuspid regurgitation

(5th ICS, left sternal border)

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30
Q

Pansystolic murmur hear loudest at the left lower sternal border?

A

Ventricular septal defect

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31
Q

Differentials of ejection-systolic murmurs?

A

Aortic stenosis
Pulmonary stenosis
Hypertrophic obstructive cardiomyopathy

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32
Q

Ejection systolic murmur heard loudest at the second intercostal space, right sternal border?

A

Aortic stenosis

(aortic area - 2nd ICS, right sternal border)

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33
Q

Ejection systolic murmur heard loudest at the second intercostal space, left sternal border?

A

Pulmonary stenosis

(Pulmonary area = second ICS, left sternal border)

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34
Q

Ejection systolic murmur heard loudest at fourth intercostal space on the left sternal border?

A

Hypertrophic obstructive cardiomyopathy

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35
Q

Why might there be splitting of the second heart sound?

A

When the pulmonary valve closes slightly later than the aortic valve:

During inspiration the chest wall and diaphragm pull the lungs and HEART open (negative intra-throacic pressure)

This causes RHS of heart to fill faster as it pulls in blood from the venous system

Increased volume in the RIGHT VENTIRCLE causes it to take longer for the right ventricle to empty in SYSTOLE causing a delay in the pulmonary valve

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36
Q

When is splitting of the second heart sound normal and when is it not?

A

Normal with inspiration

Abnormal - ‘‘fixed split’’, meaning second heart sound does not change with inspiration and expiration (ASD)

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37
Q

What is a ‘‘fixed split’’ and why might it occur?

A

Splitting of the second heart sound can be normal with inspiration, however a “fixed split” second heart sound means the split does not change with inspiration and expiration.

This occurs in an atrial septal defect because blood is flowing from the left atrium into the right atrium across the atrial septal defect, increasing the volume of blood that the right ventricle has to empty before the pulmonary valve can close.

This doesn’t vary with respiration.

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38
Q

What abnormalities can be found on ascultation in atrial septal defect?

A

MID-SYSTOLIC,
CRESCENDO-DECRESENDO
MURMUR
HEARD LOUDEST AT THE LEFT STERNAL BORDER

FIXED SPLIT SECOND HEART SOUND (does not change with inspiration or expiration?

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39
Q

What might be found on auscultation in a patient with a patent ductus arteriosus?

A

A small patent ductus arteriosus may not cause any abnormal heart sounds.

More significant PDAs cause a normal first heart sound with a continuous CRESCENDO-DECRESCENDO “machinery” murmur

that may continue during the second heart sound, making the second heart sound difficult to hear.

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40
Q

Where does the murmur in tetralogy of fallot arise from?

A

Pulmanory stenosis

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41
Q

Murmur in tetralogy of Fallot

A

The murmur in tetralogy of Fallot arises from pulmonary stenosis, giving an ejection systolic murmur loudest at the pulmonary area (second intercostal space, left sternal border).

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42
Q

When/why does cyanotic heart disease occur?

A

Cyanosis occurs when deoxygenated blood enters the systemic circulation.

Cyanotic heart disease occurs when blood is able to bypass the pulmonary circulation and the lungs.

This occurs across a right-to-left shunt. A right-to-left shunt describes any defect that allows blood to flow from the right side of the heart (the deoxygenated blood returning from the body) to the left side of the heart (the blood exiting the heart into the systemic circulation) without travelling through the lungs to get oxygenated.

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43
Q

What kind of shunt causes cyanotic heart diease?

A

Right-to-left shunt

A right-to-left shunt describes any defect that allows blood to flow from the right side of the heart (the deoxygenated blood returning from the body) to the left side of the heart (the blood exiting the heart into the systemic circulation) without travelling through the lungs to get oxygenated.

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44
Q

Cyanotic heart diseases?

A

Heart defects that can cause a right-to-left shunt, and therefore cyanotic heart disease, are:

Ventricular septal defect (VSD)
Atrial septal defect (ASD)
Patent ductus arteriosus (PDA)
Transposition of the great arteries

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45
Q

Which heart defect always causes cyanotic heart disease?

A

Patients with transposition of the great arteries will always have cyanosis because the right side of the heart pumps blood directly into the aorta and systemic circulation.

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46
Q

Which heart defects can but do not usually cause cyanosis - and why?

A

Patients with a VSD, ASD or PDA are usually not cyanotic.

This is because the pressure in the left side of the heart is much greater than the right side, and blood will flow from the area of high pressure to the area of low pressure.

This prevents a right-to-left shunt. If the pulmonary pressure increases beyond the systemic pressure blood will start to flow from right-to-left across the defect, causing cyanosis.

This is called Eisenmenger syndrome.

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47
Q

When does the ductus arteriosus stop functioning and then close completely?

A

The ductus arteriosus normally stops functioning within 1-3 days of birth, and closes completely within the first 2-3 weeks of life.

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48
Q

Why might PDA occur?

A

Genetics
Related to maternal infections such as rubella
Prematurity (key risk factor)

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49
Q

Do PDAs always cause problems in children?

A

A small PDA can be asymptomatic, cause no functional problems and close spontaneously.

Occasionally patients can remain asymptomatic throughout childhood and present in adulthood with signs of heart failure.

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50
Q

Pathophysiology of PDA?

A

Pressure in aorta > pulmonary vessels so blood flows FROM THE AORTA TO THE PULMONARY ARTERY - creating a LEFT TO RIGHT shunt (where blood from the left side of the heart crosses to the circulation from the right side)

This increases pressure in the pulmonary vessel causing pulmonary hypertension, leading to:
RIGHT SIDED HEART STRAIN - right ventricle struggles to contract against the INCREASED RESISTANCE

Pulmonary hypertension and right sided heart strain lead to RVH (right ventricular hypertrophy)

The increased blood flow through the pulmonary vessels and returning to the left side of the heart leads to LVH (left ventricular hypertophy)

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51
Q

PDA - presentation

A

A patent ductus arteriosus can be picked up during the newborn examination if a murmur is heard (continous crescendo-decrescendo ‘‘machinery’’ murmur that may continue during the second heart sound - making it difficult to hear)

It may also present with symptoms of:

Shortness of breath
Difficulty feeding
Poor weight gain
Lower respiratory tract infections

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52
Q

What is used to diagnose PDA?

A

The diagnosis of PDA can be confirmed by echocardiogram.

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53
Q

How is an echo useful in ?PDA

A

The diagnosis of PDA can be confirmed by echocardiogram.

The use of doppler flow studies during the echo can assess the size and characteristics of the left to right shunt.

An echo is also useful for assessing the effects of the PDA on the heart, for example demonstrating hypertrophy of the right ventricle, left ventricle or both.

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54
Q

Management of PDA

A

Patients are typically monitored until 1 year of age using echocardiograms.

After 1 year of age it is highly unlikely that the PDA will close spontaneously and:

trans-catheter or

surgical closure

can be performed.

Symptomatic patient or those with evidence of heart failure as a result of PDA are treated earlier.

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55
Q

Up to what age is a watch and wait approach appropriate management for PDA?

A

1 year - after this its highly unlikely it will close spontaneouslt

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56
Q

Methods of closing PDA?

A

Trans-catheter or surgical closure

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57
Q

What is ASD?

A

An atrial septal defect is a defect (a hole) in the septum (the wall) between the two atria. This connects the right and left atria allowing blood to flow between them.

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58
Q

Atrial septal defect - pathophysiology?

A
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59
Q

What ECG ‘abnormality’ is comman and unconcerning in children

A

RBBB

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60
Q

Pathophysiology of ASD

A

During the development of the fetus the left and right atria are connected. Two walls grow downwards from the top of the heart, then fuse together with the endocardial cushion in the middle of the heart to separate the atria. These two walls are called the septum primum and septum secondum.

Defects this these two walls lead to atrial septal defects, a hole connecting the left and right atria. There is a small hole in the septum secondum called the foramen ovale. The foramen ovale normally closes at birth.

An atrial septal defect leads to a shunt, with blood moving between the two atria. Blood moves from the left atrium to the right atrium because the pressure in the left atrium is higher than the pressure in the right atrium. This means blood continues to flow to the pulmonary vessels and lungs to get oxygenated and the patient does not become cyanotic, however the increased flow to the right side of the heart leads to right sided overload and right heart strain. This right sided overload can lead to right heart failure and pulmonary hypertension.

Eventually pulmonary hypertension can lead to Eisenmenger syndrome. This is where the pulmonary pressure is greater than the systemic pressure, the shunt reverses and forms a right to left shunt across the ASD, blood bypasses the lungs and the patient becomes cyanotic.

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61
Q

Development of the atrial septum

A

During the development of the fetus the left and right atria are connected. Two walls grow downwards from the top of the heart, then fuse together with the endocardial cushion in the middle of the heart to separate the atria.

These two walls are called the septum primum and septum secondum.

62
Q

What kind of shunt occurs in ASD and why?

A

An atrial septal defect leads to a shunt, with blood moving between the two atria.

Blood moves from the left atrium to the right atrium because the pressure in the left atrium is higher than the pressure in the right atrium.

However the increased flow to the right side of the heart leads to right sided overload and right heart strain. This right sided overload can lead to right heart failure and pulmonary hypertension.

Eventually pulmonary hypertension can lead to Eisenmenger syndrome. This is where the pulmonary pressure is greater than the systemic pressure, the shunt reverses and forms a right to left shunt across the ASD, blood bypasses the lungs and the patient becomes cyanotic.

63
Q

What congenital (non cardiac) syndrome is associated with aortic stenosis?

A

Turners syndrome

64
Q

How does ASD lead to a right to left shunt?

A

An atrial septal defect leads to a shunt, with blood moving between the two atria. Blood moves from the left atrium to the right atrium because the pressure in the left atrium is higher than the pressure in the right atrium. This means blood continues to flow to the pulmonary vessels and lungs to get oxygenated and the patient does not become cyanotic, however the increased flow to the right side of the heart leads to right sided overload and right heart strain. This right sided overload can lead to right heart failure and pulmonary hypertension.

Eventually pulmonary hypertension can lead to Eisenmenger syndrome. This is where the pulmonary pressure is greater than the systemic pressure, the shunt reverses and forms a right to left shunt across the ASD, blood bypasses the lungs and the patient becomes cyanotic.

65
Q

Most common type of atrial septal defect?

A

Ostium secondum, where the septum secondum fails to fully close, leaving a hole in the wall.

66
Q

Ostium secdonum ASD

A

The septum secondum fails to fully close, leaving a hole in the wall.

67
Q

Patent foramen ovale ASD

A

The foramen ovale fails to close (although this not strictly classified as an ASD).

68
Q

Ostium primum ASD

A

Ostium primum, where the septum primum fails to fully close, leaving a hole in the wall. This tends to lead to atrioventricular valve defects making it an atrioventricular septal defect.

69
Q

Ostium primum ASD

A

Ostium primum, where the septum primum fails to fully close, leaving a hole in the wall. This tends to lead to atrioventricular valve defects making it an atrioventricular septal defect.

70
Q

Types of ASD

A

Ostium secondum
Ostium primum
(Patent foramen ovale)

71
Q

ASD complications

A

Stroke in the context of venous thromboembolism

Atrial fibrillation or atrial flutter

Pulmonary hypertension and right sided heart failure

Eisenmenger syndrome

72
Q

How can atrial septal defects cause stroke in patients with a DVT?

A

Normally when patients have a DVT and this becomes an embolus, the clot travels to the right side of the heart, enters the lungs and becomes a pulmonary embolism.

In patients with an ASD the clot is able to travel from the right atrium to the left atrium across the ASD.
This means the clot can travel to the left ventricle, aorta and up to the brain, causing a large stroke.

73
Q

Atrial septal defects are often picked up how?

A

Atrial septal defects are often picked up through antenatal scans or newborn examinations.

74
Q

Typical symptoms of ASD in childhood?

A

Shortness of breath
Difficulty feeding
Poor weight gain
Lower respiratory tract infections

75
Q

How might ASD that has been asymptomatic throughout childhood appear in adults?

A

Dyspnoea
HF
Stroke

76
Q

Atrial septal defects - management

A

Patients with an ASD should be referred to a paediatric cardiologist for ongoing management.

If the ASD is small and asymptomatic, watching and waiting can be appropriate.

ASDs can be corrected surgically using a transvenous catheter closure (via the femoral vein) or open heart surgery.

Anticoagulants (such as aspirin, warfarin and NOACs) are used to reduce the risk of clots and stroke in adults.

77
Q

What is a ventricular septal defect?

A

A ventricular septal defect (VSD) is a congenital hole in the septum (wall) between the two ventricles.

This can vary in size from tiny to the entire septum, forming one large ventricle. VSDs can occur in isolation, however there is often an underlying genetic condition and they are commonly associated with Down’s Syndrome and Turner’s Syndrome.

78
Q

When a ventricular septal defect is present, there is often an underlying genetic condition, such as?

A

Down’s syndrome
Turner’s syndrome

79
Q

Why do patients with VSD remain ayanotic?

A

Due to the increased pressure in the left ventricle compared to the right, blood typically flows from left the right through the hole.

Blood is still flowing around the lungs before entering the rest of the body, therefore they remain acyanotic (not cyanotic) because their blood is properly oxygenated.

80
Q

What kind of shunt is present in VSD and what can this lead to?

A

LEFT TO RIGHT: Due to the increased pressure in the left ventricle compared to the right, blood typically flows from left the right through the hole.

A left to right shunt leads to right sided overload, right heart failure and increased flow into the pulmonary vessels.

81
Q

In VSD, blood is still flowing around the lungs before entering the rest of the body, therefore they remain acyanotic (not cyanotic) because their blood is properly oxygenated. Why might these patients become cyanotic and what is this called?

A

Initially left to right shunt (due to increase pressure in the left ventricle compared to the right) - pt is acynotic

The extra blood flowing through the right ventricle increases the pressure in the pulmonary vessels over time, causing pulmonary hypertension.

If this continues, the pressure in the right side of the heart may become greater than the left, resulting in the blood being shunted from right to left and avoiding the lungs.

When this happens the patient will become cyanotic because blood is bypassing the lungs. This is called Eisenmenger Syndrome.

82
Q

VSD presentation

A

Often VSDs are initially symptomless and patients can present as late as adulthood.

Antenatal scans

Murmur is heard during the newborn baby check (pan-systolic murmur more prominently heard at the left lower sternal border in the third and fourth intercostal spaces)

Typical symptoms include:

Poor feeding
Dyspnoea
Tachypnoea
Failure to thrive

83
Q

VSD examination findings?

A

Patients with a VSD typically have a pan-systolic murmur more prominently heard at the left lower sternal border in the third and fourth intercostal spaces. (Other causes of this murmur: mitral regurgitation and tricuspid regurgitation)

There may be a systolic thrill on palpation.

Usually acyanotic

84
Q

Treatment of VSD?

A

Treatment should be coordinated by a paediatric cardiologist. Small VSDs with no symptoms or evidence of pulmonary hypertension or heart failure can be watched over time. Often they close spontaneously.

SURGICAL CORRECTION: transvenous catheter closure via the femoral vein or open heart surgery.

There is an increased risk of infective endocarditis in patients with a VSD - Antibiotic prophylaxis should be considered during surgical procedures to reduce the risk of developing infective endocarditis.

85
Q

What should be considered in a patient with VSD undergoing surgery and why?

A

There is an increased risk of infective endocarditis in patients with a VSD - Antibiotic prophylaxis should be considered during surgical procedures to reduce the risk of developing infective endocarditis.

86
Q

What underlying lesions can cause Eisenmenger syndrome?

A

Atrial septal defect

Ventricular septal defect

Patent ductus arteriosus

87
Q

When might Eisenmenger syndrome occur in patients with VSD, ASD or PDA?

A

Large shunts: 1-2 years

Small shunts: adulthood

Pregnancy

88
Q

Concerns re: pregnancy in patients with VSD/ASD/PDA?

A

Eisenmenger syndrome can develop more quickly during pregnancy, so women with a history of having a “hole in the heart” need an echo and close monitoring by a cardiologist during pregnancy.

89
Q

Eisenmenger syndrome: pathophysiology

A

Normally when there is a septal defect (ASD, VSD, PDA) blood will flow from the left side of the heart to the right - LEFT TO RIGHT SHUNT (because the pressure in the left side is greater than in the right)

A left to right shunt means blood still travels to the lungs and gets oxygenated, so the patient does not become cyanotic.

Over time the extra blood flowing into the right side of the heart and the lungs increases the pressure in the pulmonary vessels.
This leads to PULMONARY HYPERTENSION.

When the pulmonary pressure exceeds the systemic pressure, blood begins to flow from the right side of the heart to the left across the septal defect - RIGHT TO LEFT SHUNT.

Essentially it becomes easier for the right side of the heart to pump blood across the defect into the left side of the heart compared with pumping blood into the lungs.

This causes deoxygenated blood to bypass the lungs and enter the body. This causes cyanosis (blue discolouration of skin relating to a low level of oxygen saturation in the blood).

The bone marrow will respond to low oxygen saturations by producing more red blood cells and haemoglobin to increase the oxygen carrying capacity of the blood.

This leads to polycythaemia, which is a high concentration of haemoglobin in the blood.

Polycythaemia gives patients a plethoric complexion.

A high concentration of red blood cells and haemoglobin make the blood more viscous, making patients more prone to developing blood clots.

90
Q

What type of shunt causes cyanosis??

A

RIGHT TO LEFT SHUNT.

Essentially it becomes easier for the right side of the heart to pump blood across the defect into the left side of the heart compared with pumping blood into the lungs.

This causes deoxygenated blood to bypass the lungs and enter the body. This causes cyanosis (blue discolouration of skin relating to a low level of oxygen saturation in the blood).

91
Q

What are the physiological consqeuences of a right to left shunt?

A

DEOXYGENATED BLOOD BYPASSES THE LUNGS and enters the body, causing low levels O2 saturations

The bone marrow will respond to low oxygen saturations by producing more red blood cells and haemoglobin to increase the oxygen carrying capacity of the blood.

This leads to polycythaemia, which is a high concentration of haemoglobin in the blood.

(Polycythaemia gives patients a plethoric complexion)

A high concentration of red blood cells and haemoglobin make the blood more viscous, making patients more prone to developing blood clots.

92
Q

Eisenmenger syndrome: examination findings associated with pulmonary hypertension?

A

Right ventricular heave: the right ventricle contracts forcefully against increased pressure in the lungs

Loud P2: loud second heart sound due to forceful shutting of the pulmonary valve

Raised JVP

Peripheral oedema

93
Q

Eisenmenger syndrome: examination findings associated with underlying septal defects?

A

Atrial septal defect: mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border

Ventricular septal defect: pan-systolic murmur loudest at the left lower sternal border

Patent ductus arteriosus: continuous crescendo-decrescendo “machinery” murmur

Arrhythmias

94
Q

Eisenmenger syndrome: examination findings associated with the right to left shunt and chronic hypoxia?

A

Cyanosis
Clubbing
Dyspnoea
Plethoric complexion (a red complexion related to polycythaemia)

95
Q

What is the prognosis of Eisenmenger syndrome?

A

Eisenmenger syndrome reduces life expectancy by around 20 years compared with healthy individuals.

The main causes of death are heart failure, infection, thromboembolism and haemorrhage.

The mortality can be up to 50% in pregnancy.

96
Q

Management of Eisenmenger syndrome

A

Ideally the underlying defect should be managed optimally or corrected surgically to prevent the development of Eisenmenger syndrome, but once the pulmonary pressure is high enough to cause the syndrome, it is not possible to medically reverse the condition.

The only definitive treatment is a heart-lung transplant, however this has a high mortality.

Patients with Eisenmenger syndrome will be closely followed up by a specialist. Medical management involves:

Oxygen can help manage symptoms but does not affect overall outcomes

Treatment of pulmonary hypertension, for example using sildenafil

Treatment of arrhythmias

Treatment of polycythaemia with venesection

Prevention and treatment of thrombosis with anticoagulation

Prevention of infective endocarditis using
prophylactic antibiotics

97
Q

What is coarction of the aorta and what is it associated with?

A

Coarctation of the aorta is a congenital condition where there is narrowing of the aortic arch, usually around the ductus arteriosus. The severity of the coarctation (or narrowing) can vary from mild to severe. It is often associated with an underlying genetic condition, particularly Turners syndrome.

98
Q

How does coarctation of the aorta affect pressures within the arteries and heart?

A

Narrowing of the aorta reduces the pressure of blood flowing to the arteries that are distal to the narrowing.

It increases the pressure in areas proximal to the narrowing, such as the heart and the first three branches of the aorta.

98
Q

How does coarctation of the aorta affect pressures within the arteries and heart?

A

Narrowing of the aorta reduces the pressure of blood flowing to the arteries that are distal to the narrowing.

It increases the pressure in areas proximal to the narrowing, such as the heart and the first three branches of the aorta.

99
Q

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

A

Weak femoral pulses

100
Q

Presentation of coractation of the aorta

A

Often the only indication of coarctation in a neonate may be weak femoral pulses.

Performing a four limb blood pressure will reveal high blood pressure in the limbs supplied from arteries that come before the narrowing, and lower blood pressure in limbs that come after the narrowing.

There may be a systolic murmur heard below the left clavicle (left infraclavicular area) and below the left scapula.

Coarctation may have other signs in infancy:

Tachypnoea and increased work of breathing
Poor feeding
Grey and floppy baby

Additional signs may develop over time:

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

101
Q

Management of coarctation of the aorta?

A

The severity of the coarctation varies between patients. In mild cases patients can live symptom free until adulthood without requiring surgical input, and in severe cases patients will require emergency surgery shortly after birth.

In cases of critical coarctation where there is a risk of heart failure and death shortly after birth Prostaglandin E is used keep the ductus arteriosus open while waiting for surgery.

This allows some blood flow flow through the ductus arteriosus into the systemic circulation distal to the coarctation.

Surgery is then performed to correct the coarctation and to ligate the ductus arteriosus

102
Q

What is aortic stenosis?

A

Patients with congenital aortic valve stenosis are born with a narrow aortic valve that restricts blood flow from the left ventricle into the aorta. The severity of the stenosis varies between patients and will determine the symptoms.

The aortic valve is normally made up of three leaflets, called the aortic sinuses of Valsalva, which allow blood to flow from the left ventricle into the aorta, but prevent blood from flowing back into the left ventricle. Patients with aortic stenosis may have one, two, three or four leaflets.

103
Q

How many leaflets is the aortic valve normally made up of and what are they called?

A

Aortic sinuses of Valsalva

104
Q

How many leaflets may the aortic valve have in a patient with aortic stenosis?

A

1-4

105
Q

What signs may be present O/E in aortic stenosis?

A

The key examination finding is an EJECTION SYSTOLIC MURMUR, HEARD LOUDEST AT THE AORTIC AREA, which is the 2N ICS, right sternal border.

It has a CRESCENDO-DECRESENDO character and radiates to the CAROTIDS

Other signs that may be present on examination are:
- Ejection click just before the murmur
- Palpable thrill during systole
- Slow rising pulse and narrow pulse pressure

106
Q

Presentation of moderate aortic stenosis?

A

Fatigue
Shortness of breath
Dizziness
Fainting

Symptoms are typically worse on exertion as the outflow from the left ventricle cannot keep up with demand.

107
Q

Presentation of severe aortic stenosis?

A

Heart failure within months of birth

108
Q

Presentation of mild aortic stenosis

A

Can be completely asymptomatic, discovered as an incidental murmur during a routine examination

109
Q

Why are symptoms of aortic stenosis typically worse on exertion?

A

Outflow from the left ventricle cannot keep up with increasing demand

110
Q

Management of congenital aortic stenosis?

A

Congenital aortic stenosis tends to be a progressive condition that worsens over time.

Patients need regular follow-up under a paediatric cardiologist, with echocardiograms, ECGs and exercise testing to monitor the progression of the condition.

Patient with more significant stenosis may need to restrict physical activities.

Options for treating the stenosis are:

  • Percutaneous balloon aortic valvoplasty
  • Surgical aortic valvotomy
  • Valve replacement
111
Q

What is the gold standard for diagnosing aortic stenosis?

A

Echocardiogram

112
Q

Potential complications of congenital aortic stenosis?

A

Left ventricular outflow tract obstruction

Heart failure

Ventricular arrhythmia

Bacterial endocarditis

Sudden death, often on exertion

113
Q

What is congenital pulmonary valve stenosis?

A

The pulmonary valve usually consists of three leaflets that open and close to let blood out and prevent blood from returning to the heart.

These leaflets can develop abnormally, becoming thickened or fused.

This results in a narrow opening between the right ventricle and the pulmonary artery.

This is called congenital pulmonary valve stenosis.

114
Q

Congenital pulmonary valve stenosis?

A

Congenital pulmonary valve stenosis often occurs without any associations. It can be associated with other conditions such as:

Tetralogy of Fallot

William syndrome

Noonan syndrome

Congenital rubella syndrome

115
Q

Presentation of congenital pulmonary valve stenosis?

A

Often pulmonary stenosis is completely asymptomatic, and it is discovered as an incidental finding of a murmur during routine baby checks.

More significant pulmonary valve stenosis can present with symptoms of:
Fatigue on exertion
Shortness of breath
Dizziness and fainting.

116
Q

Signs of pulmonary valve stenosis O/E

A

EJECTION SYSTOLIC murmur heard loudest at the pulmonary area (2ND ICS, left sternal border)

Palpable thrill in the pulmonary area

Right ventricular heave due to right ventricular hypertrophy

Raised JVP with giant a waves

117
Q

Tetralogy of Fallot is a congenital condition where there are which four coexisting pathologies?

A

Ventricular septal defect (VSD)

Overriding aorta

Pulmonary valve stenosis

Right ventricular hypertrophy

118
Q

Tetralogy of Fallot: VSD and overriding aorta pathophysiology

A

The VSD allows blood to flow between the ventricles.

The term “overriding aorta” refers to the fact that the entrance to the aorta (the aortic valve) is placed further to the right than normal, above the VSD.

This means that 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.

119
Q

Tetralogy of fallot: pathophysiology

A

VSD + overriding aorta + pulmonary valve stenosis + right ventricular hypertrophy

The VSD allows blood to flow between the ventricles. The term “overriding aorta” refers to the fact that the entrance to the aorta (the aortic valve) is placed further to the right than normal, above the VSD. T
his means that 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.

Stenosis of the pulmonary valve 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.
Therefore, the overriding aorta and pulmonary stenosis encourage blood to be shunted from the right heart to the left, causing cyanosis.

The increased strain on the muscular wall of the right ventricle as it attempts to pump blood against the resistance of the left ventricle and pulmonary stenosis causes right ventricular hypertrophy, with thickening of the heart muscle.

120
Q

What type of shunt occurs in tetralogy of Fallot and is there therefore cyanosis present?

A

RIGHT TO LEFT CARDIAC SHUNT - CYANOSIS

The overriding aorta and pulmonary stenosis encourage blood to be shunted from the right heart to the left.

This means blood bypasses the child’s lungs. Blood bypassing the lungs does not become oxygenated. Deoxygenated blood entering the systemic circulation causes cyanosis.

121
Q

Managing congenital pulmonary valve stenosis

A
  • In mild pulmonary stenosis without symptoms patients generally do not require any intervention.

They are followed up by a cardiologist with a “watching and waiting” approach.

  • If the patient is symptomatic or the valve is more significantly stenosed, BALOON VALVULOPLASTIC VIA A VENOUS CATHETER IS THE TREATMENT OF CHOICE

This involves inserting a catheter under xray guidance into the femoral vein, through the inferior vena cava and right side of the heart to the pulmonary valve, and dilating the valve by inflating a balloon.

  • If valvuloplasty is not appropriate or fails open-heart surgery can be performed.
122
Q

Tetralogy of Fallot is a congenital condition where there are four coexisting pathologies

A

Echocardiogram

123
Q

The degree to which the right to left shunting happens in tetralogy of Fallot is related mostly to what?

A

the severity of the patients pulmonary stenosis

124
Q

Risk factors for tetralogy of fallot?

A

Rubella infection

Increased age of the mother (over 40 years)

Alcohol consumption in pregnancy

Diabetic mother

125
Q

Investigating tetralogy fallot?

A

As with all structural congenital cardiac abnormalities, an echocardiogram is the investigation of choice for establishing the diagnosis.

  • ECHOCARDIOGRAM + DOPPLER STUDIES - SEVERITY OF ABNORMALITY AND SHUNT
    During the echocardiogram, the machine can produce coloured pictures that demonstrate the direction of flow of blood. This is called doppler flow studies. This is useful in assessing the severity of the abnormality and shunt.

A chest xray may show the characteristic “boot shaped” heart due to right ventricular thickening if performed

126
Q

What might a CXR show in tetralogy of fallot and why?

A

A chest xray may show the characteristic “boot shaped” heart due to right ventricular thickening if performed

127
Q

Presentation of Tetralogy of Fallot

A

Most cases are picked up before the child is born during the antenatal scans.

Additionally, an EJECTION SYSTOLIC MURMUR caused by the pulmonary stenosis may be heard on the newborn baby check.

Severe cases will present with heart failure before one year of age. In milder cases, they can present as older children once they start to develop signs and symptoms of heart failure.

128
Q

Presentation of Tetralogy of Fallot

A

Most cases are picked up before the child is born during the antenatal scans.

Additionally, an EJECTION SYSTOLIC MURMUR caused by the pulmonary stenosis may be heard on the newborn baby check.

Severe cases will present with heart failure before one year of age. In milder cases, they can present as older children once they start to develop signs and symptoms of heart failure.

129
Q

Before what age will Tetralogy of Fallot present by in severe cases?

A

Before one year of age, with HF

130
Q

Signs and symptoms of Tetralogy of Fallot

A

Cyanosis (blue discolouration of the skin due to low oxygen saturations)

Clubbing

Poor feeding

Poor weight gain

Ejection systolic murmur heard loudest in the pulmonary area (second intercostal space, left sternal border)

“Tet spells”

131
Q

What are Tet Spells?

A

“Tet Spells” are intermittent symptomatic periods where the right to left shunt becomes temporarily worsened, precipitating a cyanotic episode.

This happens when the pulmonary vascular resistance increases or the systemic resistance decreases.

For example, if the child is physically exerting themselves they are generating a lot of carbon dioxide.
Carbon dioxide is a vasodilator that causes 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.

These episodes may be precipitated by waking, physical exertion or crying. The child will become irritable, cyanotic and short of breath. Severe spells can lead to reduced consciousness, seizures and potentially death.

132
Q

What might precipitate a Tet spell?

A

Waking
Physical exertion
Crying

133
Q

Tet spell signs/symptoms?

A

Irritability
Cyanotic
Short of breath

Severe spells can lead to:
Reduced/loss of conciousness
Seizures
Death

134
Q

“Tet Spells” are intermittent symptomatic periods where the right to left shunt becomes temporarily worsened, precipitating a cyanotic episode.

Why do they occur?

A

This happens when the pulmonary vascular resistance increases or the systemic resistance decreases.

For example, if the child is physically exerting themselves they are generating a lot of carbon dioxide.

Carbon dioxide is a vasodilator that causes 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.

135
Q

Treatment of a tet spell?

A

Older children may squat when a tet spell occurs. Younger children can be positioned with their knees to their chest.
Squatting increases the systemic vascular resistance. This encourages blood to enter the pulmonary vessels.

Any medical management of a tet spell should involve an experienced paediatrician, as they can be potentially life threatening:

Supplementary oxygen is essential in hypoxic children as hypoxia can be fatal.

Beta blockers can relax the right ventricle and improve flow to the pulmonary vessels.

IV fluids can increase pre-load, increasing the volume of blood flowing to the pulmonary vessels.

Morphine can decrease respiratory drive, resulting in more effective breathing.

Sodium bicarbonate can buffer any metabolic acidosis that occurs.

Phenylephrine infusion can increase systemic vascular resistance.

136
Q

Management of Tetralogy of Fallot

A

In neonates, a prostaglandin infusion can be used to maintain the ductus arteriosus. This allows blood to flow from the aorta back to the pulmonary arteries.

Total surgical repair by open heart surgery is the definitive treatment, however mortality from surgery is around 5%.

Prognosis depends on the severity, however it is poor without treatment. With corrective surgery, 90% of patients will live into adulthood.

137
Q

What is Ebstein’s Anomaly?

A

Ebstein’s anomaly is a congenital heart condition where the tricuspid valve is set lower in the right side of the heart (towards the apex), causing a bigger right atrium and a smaller right ventricle.

This leads to poor flow from the right atrium to the right ventricle, and therefore poor flow to the pulmonary vessels.

It is often associated with a right to left shunt across the atria via an atrial septal defect.

When this happens blood bypasses the lungs, leading to cyanosis. It is also associated with Wolff-Parkinson-White syndrome.

138
Q

Presentation of Ebstein’s anomoly?

A

Typical presenting features include:

Evidence of heart failure (e.g. oedema)

Gallop rhythm heard on auscultation characterised by the addition of the third and fourth heart sounds

Cyanosis

Shortness of breath and tachypnoea

Poor feeding

Collapse or cardiac arrest

Symptoms in patients with an associated atrial septal defect often present a few days after birth, when the ductus arteriosus closes. Where there is a right to left shunt across an atrial septal defect the ductus arteriosus allows blood to flow from the aorta into the pulmonary vessels to get oxygenated. This minimises the cyanosis. When the duct closes the patient becomes cyanotic and symptomatic.

139
Q

What is the investigation of choice for confirming the diagnosis and assessing the severity of Ebstein’s anomaly?

A

Echocardiogram

140
Q

Management of Ebstiens Anomaly?

A

Medical management includes treating arrhythmias and heart failure.

Prophylactic antibiotics may be used to prevent infective endocarditis.

Definitive management is by surgical correction of the underlying defect.

141
Q

What other cardiac syndrome is Ebstein’s anomaly associated with?

A

. It is also associated with Wolff-Parkinson-White syndrome.

142
Q

What is transposition of the great arteries?

A

Transposition of the great arteries is a condition where the attachments of the aorta and the pulmonary trunk to the heart are swapped (“transposed”).

This means the right ventricle pumps blood into the aorta and the left ventricle pumps blood into the pulmonary vessels. In this scenario are two separate circulations that don’t mix: one travelling through the systemic system and right side of the heart and the other traveling through the pulmonary system and left side of the heart.

143
Q

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

A

Ventricular septal defect
Coarctation of the aorta
Pulmonary stenosis

144
Q

Why does transposition of the great arteries become immediately life threatening at birth?

A

During pregnancy there is normal development of the fetus. The gas and nutrient exchange happens in the placenta, therefore it is not necessary for blood to flow to the lungs. After birth the condition is immediately life threatening as there is no connection between the systemic circulation and the pulmonary circulation. The baby will be cyanosed.

Immediate survival depends on a shunt between the systemic circulation and pulmonary circulation that allows blood flowing through the body an opportunity to get oxygenated in the lungs. This shunt can occur across a patent ductus arteriosus, atrial septal defect or ventricular septal defect.

145
Q

How might transposition of the great arteries present?

A

The defect is often diagnosed during pregnancy with antenatal ultrasound scans.

Close monitoring is necessary during the pregnancy and arrangements should be made so that the woman gives birth in a hospital capable of managing the condition after birth.

Where the defect was not detected during pregnancy it will present with cyanosis at or within a few days of birth.

A patent ductus arteriosus or ventricular septal defect can initially compensate by allowing blood to mix between the systemic circulation and the lungs, however within a few weeks of life they will develop respiratory distress, tachycardia, poor feeding, poor weight gain and sweating.

146
Q

When might a neonate with transposition of the great arteries survive birth without intervention?

A

A patent ductus arteriosus or ventricular septal defect can initially compensate by allowing blood to mix between the systemic circulation and the lungs, however within a few weeks of life they will develop respiratory distress, tachycardia, poor feeding, poor weight gain and sweating.

147
Q

Management of transposition of the great arteries?

A

Where there is a ventricular septal defect, this will allow some mixing of blood between the two systems and provide some time for definitive treatment.

A prostaglandin infusion can be used to maintain the ductus arteriosus. This allow blood from the aorta to flow to the pulmonary arteries for oxygenation.

Balloon septostomy involves inserting a catheter into the foramen ovale via the umbilicus, and inflating a balloon to create a large atrial septal defect. This allows blood returning from the lungs (on the left side) to flow to the right side of the heart and out through the aorta to the body.

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. If present, a VSD or ASD can be corrected at the same time.

148
Q

A neonate with with a significant difference in saturations between the right upper and lower limb (>3%) and radio-femoral delay most likely has which abnormality?

A

co-arctation of the aorta

149
Q

Paediatric BLS: In an infant, the appropriate places to check for a pulse are where?

A

brachial and femoral arteries

150
Q

What should be given to maintain a patent ductus arteriosus in cyanotic congenital heart diseases

A

Prostaglandin E1

Year 4 Paediatrics (18 decks)

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