Cardio Flashcards

1
Q

How does the fetal circulation work?

A

Fetus receives oxygen and nutrients and disposes of waste via the placenta, bypassing the lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

How does the fetal circulation bypass the lungs?

A

3 fetal shunts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the 3 fetal shunts?

A

Ductus venosus
Foramen ovale
Ductus arteriosus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What does the ductus venosus allow blood to bypass in the fetus?

A

The liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What does the ductus venosus connect?

A

The umbilical vein and inferior vena cava

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What does the foramen ovale allow the fetal blood to bypass?

A

The right ventricle and pulmonary circulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What does the foramen ovale connect?

A

The right and left atria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What does the ductus arteriosus allow the fetal circulation to bypass?

A

The pulmonary circulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What does the ductus arteriosus connect?

A

The pulmonary artery and aorta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

From the umbilical vein, outline the passage of blood in the fetal circulation:

A

Placenta–> Umbilical vein–> Ductus venosus–> IVC–> RA–> (Foramen ovale–> Left atrium–> Left ventricle)/ (Right ventricle–> Pulmonary artery–> Ductus arteriosus)–> Aorta–> Internal iliac arteries–> Umbilical arteries–> Placenta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What happens to the pulmonary vascular resistance with the first breath and why?

A

The first breath expands the alveoli, decreasing the pulmonary vascular resistance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What does the decrease in pulmonary vascular resistance do to the pressure in the right atrium?

A

Causes it to fall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What does the fall in RA pressure on the first breath result in?

A

The left atrial pressure becomes greater than the RA, squashing the atrial septum and causing functional closure of the foramen ovale.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Outline the physiology of the closure of the foramen ovale?

A

On the first breath, the alveoli expand, reducing the pulmonary vascular resistance. This in turn reduces the RA pressure, meaning the LA pressure is greater, squeezing the septum and causing the foramen to close

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What does the foramen ovale become after birth and how long does it take to reach this point?

A

It gets sealed shut after a few weeks to become the fossa ovalis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What causes the closure of the ductus arteriosus at birth?

A

Increased blood oxygenation causes a drop in circulating prostaglandins which are needed to keep the ductus arteriosus open. This causes its closure.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What does the ductus arteriosus become at birth?

A

The ligamentum arteriosum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Why does the ductus venosus stop functioning immediately after birth?

A

Because the umbilical cord is clamped and there is no flow in the umbilical veins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What does the ductus venosum become?

A

Ligamentum venosum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the most common kind of heart murmurs in children?

A

Innocent murmurs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are innocent murmurs also known as?

A

Flow murmurs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What causes flow murmurs?

A

Fast blood flow through the heart during systole

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the typical features of innocent murmurs?

A
Soft
Short
Systolic
Symptomless
Situation dependent
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How might a situation dependent murmur change?

A

Quieter on standing

Only appear when the child is unwell/ feverish

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What features of a murmur would prompt further investigations and referral to paediatric cardiologist?

A

Murmur louder than 2/6
Diastolic murmurs
Louder on standing
Other symptoms (e.g. failure to thrice, feeding difficulty, cyanosis, SOB)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What are the key investigations to establish the cause of a murmur and rule out abnormalities?

A

ECG
CXR
Echo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What is S1?

A

The first heart sound

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What causes S1?

A

The closing of the atrioventricular valces and the start of ventricular contraction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What are the atrioventricular valves?

A

Mitral and tricuspid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is S2?

A

The second heart sound

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What causes S2?

A

The closing of the semilunar valves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What are the semilunar valves?

A

Pulmonary and aortic valves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Are S3 and S4 normal?

A

Always pathological

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What are the differentials of a pan-systolic murmur in children?

A

Mitral regurgitation
Tricuspid regurgitation
Ventricular septal defect

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Where would a ventricular septal defect be heard?

A

Left lower sternal border

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What are the differentials of an ejection-systolic murmur in children?

A

Aortic stenosis
Pulmonary stenosis
Hypertrophic obstructive cardiomyopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Where would hypertrophic obstructive cardiomyopathy be heard?

A

Fourth intercostal space, left sternal border

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Where do you listen for the aortic valve?

A

2nd intercostal space, right sternal border

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Where do you listen for the pulmonary valve?

A

2nd intercostal space, left sternal border

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Where do you listen for the tricuspid valve?

A

5th IC space, left sternal border

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Where do you listen for the mitral valve?

A

5th IC space, midclavicular line

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Why is the second heart sound split into two?

A

Due to the negative intra-thoracic pressure on inspiration, the right side of the heart fills up faster than the left. This increased volume means it takes longer for the right ventricle to empty during systole, causing a delay in the pulmonary valve closing compared to the aortic valve.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What is PDA?

A

Patent ductus arteriosus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is the action of the ductus arteriosus?

A

Shunts blood from the pulmonary artery straight to the aorta, bypassing the lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

How long after birth should the ductus arteriosus stop functioning and after how long will it close completely?

A

Stops functioning within 1-3 days

Closes completely in first 2-3 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What are the potential causes of PDA?

A

Unknown-

Genetic, maternal infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What is a key risk factor for developing PDA?

A

Prematurity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Are PDA’s always symptomatic?

A

No, may be asymptomatic and cause no functional problems and close spontaneously

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

If patients with PDA are asymptomatic throughout childhood, how may they present in adulthood?

A

With signs of heart failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What kind of shunt is created in PDA and why?

A

Pressure in aorta is higher than pulmonary vessels, so blood flows from left to right side of circulation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What does the increased pressure in the pulmonary vessels cause?

A

Pulmonary hypertension, leading to right sided heart strain.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Why do you get right ventricular hypertrophy in PDA?

A

Due to the pulmonary hypertension causing right sided heart strain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Why do you also get left ventricular hypertrophy in PDA?

A

Due to the increased blood flowing through the pulmonary vessels and returning to the left side of the heart

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

How might PDA present?

A
Murmur at newborn examination
SOB
Difficulty feeding
Poor weight gain
LRTI
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

What kind of murmur might you hear with more severe PDA?

A

Normal first heart sound, with continuous crescendo-decrescendo ‘machinery’ murmur that may continue during second heart sound

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

How is a PDA diagnosis confirmed?

A

Echocardiogram

Doppler flow studies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

How is PDA managed?

A

Patients monitored until 1 year using echo’s.

If not closed, trans-catheter or surgical closure can be performed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Why are patients with PDA monitored for the first year of life?

A

Because it may spontaneously close itself

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

What is an atrial septal defect?

A

Hole in the septum allowing blood to flow between the two atria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What happens to the atria, septum during development of the fetus?

A

Atria begin connected, then septum primum and septum secondum grow downwards and fuse with endocardial cushion to separate them.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Where is the foramen ovale?

A

In the second secondum between the atria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

What happens to the heart circulation in atrial septal defects?

A

Blood shunts from the left to right atrium due to the higher pressure in the LA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Does the patient become cyanotic with atrial septal defects?

A

No, the blood continues to flow to the pulmonary vessels and lungs to get oxygenated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

What does the increased flow to the right side of the heart in ASD’s lead to?

A

Right sided overload
Right heart strain
Can lead to right heart failure and pulmonary hyptersion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

What can pulmonary hypertension eventually lead to?

A

Eisenmenger syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What is Eisenmenger syndrome?

A

Where the pulmonary pressure is greater than the systemic pressure, so the shunt reverses and forms a right to left shunt across the ASD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What are the different types of atrial septal defect?

A

Ostium secondum
Patent foramen ovale
Ostium primum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

What is the most common ASD?

A

Ostium secondum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

What is ostium secondum

A

Where the septum secondum fails to close, leaving a hole in the wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

What is ostium primum?

A

Where the septum primum fails to fully close, leaving a hole in the wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

What does ostium primum usually lead to?

A

Atrioventricular valve defects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

What are the key complications of ASD?

A

VTE–> Stroke
AF
Pulmonary hypertension–> Right sided heart failure
Eisenmenger syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Why is ASD a cause of stroke in patients with a DVT?

A

In ASD the clot is able to travel from the right to the left atrium and up to the brain, whereas it would usually cause a pulmonary embolism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

What does a murmur sound like in ASD?

A

Mid-systolic, crescendo-decrescendo murmur, loudest at upper left sternal border, with fixed split second heart sound

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

What is a fixed split heart sound?

A

When the split between the aortic and pulmonary valves closing doesn’t change with inspiration or expiration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Why do you get a fixed split heart sound in ASD?

A

Because blood is flowing from the left atrium to the right atrium, increasing the volume of blood that the right ventricle has to empty before the pulmonary valve can close

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

When are ASD’s usually picked up?

A

On antenatal scans or newborn examinations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

How may ASD present in childhood?

A

SOB
Difficulty feeding
Poor weight gain
Lower respiratory tract infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

How may ASD present if asymptomatic in adulthood?

A

Dyspnoea
Heart failure
Stroke

80
Q

How do you managed patients with ASD?

A

Refer to paediatric cardiologist

Either watch or wait or correct surgically with transvenous catheter closure or openheart surgery

81
Q

What is a ventricular septal defect?

A

Congenital hole in the septum between the two ventricles

82
Q

What are VSD’s commonly associated with?

A

Down’s and Turner’s syndrome

83
Q

What kind of shunt does a VSD lead to?

A

Left to right

84
Q

Do patients become cyanotic with VSD and why?

A

No, blood still flows to the lungs before entering the rest of the body

85
Q

What does a left to right shunt lead to?

A

Right sided overload
Right heart failure
Increased flow to the pulmonary vessels

86
Q

How do VSD’s usually get picked up?

A

Antenatal cans

Murmur at newborn baby check

87
Q

When do VSD’s typically present?

A

Usually symptomless initially and can present in adulthood

88
Q

What are typical symptoms of VSD’s?

A

Poor feeding
Dyspnoea
Tachypnoea
Failure to thrive

89
Q

What murmur is heard with VSD?

A

Pan-systolic murmur more prominent at the left lower sternal border, 3/4th intercostal spaces.

90
Q

How are small VSD’s treated?

A

watch and wait- often close spontaneously

91
Q

How are larger/ symptom causing VSD’s treated?

A

Corrected surgically with transvenous catheter closure or open heart surgery

92
Q

What does having a VSD increase the risk of?

A

Infective endocarditis

93
Q

What is Eisenmenger syndrome?

A

When blood flows from the right side of the heart to the left across a structural lesion, bypassing the lungs

94
Q

What are the 3 lesions that can result in Eisenmenger syndrome?

A

Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus

95
Q

How long does it take Eisenmengers to develop with large shunts?

A

1-2 years

96
Q

How long does it take Eisenmengers to develop with small shunts?

A

Into adulthood

97
Q

When might Eisenmenger’s develop more quickly?

A

During pregnancy so they need close monitoring and an echo

98
Q

What is the pathophysiology of Eisenmengers?

A

Septal defect causes a left to right shunt due to increase pressure in left ventricle. However, over time the extra blood flowing into the right side increases the pressure in the pulmonary vessels. When this pressure exceeds the systemic pressure, blood begins to flow from right to left, causing deoxygenated blood to bypass the lungs and enter the body.

99
Q

What does Eisenmengers cause?

A

Cyanosis

100
Q

What is cyanosis?

A

Blue discolouration of the skin relating to a low oxygen saturation

101
Q

What does the bone marrow do in response to low oxygen saturations?

A

Produces more red blood cells and haemoglobin

102
Q

What does low oxygen saturations lead to?

A

Polycythaemia

103
Q

What is polycythaemia?

A

High concentration of haemoglobin in the blood

104
Q

What kind of complexion do you get with polycythaemia?

A

Plethoric- full of fluid

105
Q

What does polycythaemia increase the risk of?

A

Blood clots (blood is more viscous)

106
Q

What is found on examination of Eisenmenger?

A
Right ventricular heave 
Loud S2
Raised JVP 
Peripheral oedema
Murmurs associated with underlying septal defect 
Cyanosis
Clubbing
Dyspnoea
Plethoric complextion
107
Q

What is a thrill?

A

Palbable murmur that feels like a vibration

108
Q

What is a heave?

A

An abnormally large beating of the heart, usually as a result of right ventricular hypertrophy

109
Q

Why do you get right ventricular heave with Eisenmenger?

A

Because the right ventricle has to contract forcefully against the increased pressure in the lungs

110
Q

Why do you get a loud S2 in Eisenmenger?

A

Due to the forceful shutting of the pulmonary valve

111
Q

What is the prognosis of Eisenmenger?

A

Reduces life expectancy by around 20 years

112
Q

What are the main causes of death due to Eisenmenger?

A

Heart failure, infection, thromboemboilsm, haemorrhage

113
Q

How is Eisenmenger treated?

A

Manage/ surgically correct underlying defect

Only definitive treatment= heart-lung transpolant

114
Q

How is Eisenmenger managed?

A
Oxygen 
Treatment of pulmonary hypertension
Treatment of arrhythmias
Venesection for polycythaemia
Anticoagulation for thrombosis
Prophylactic antibiotics for infective endocarditis
115
Q

What is coarctation of the aorta?

A

Congenital condition causing narrowing of the aortic arch

116
Q

Where is the most common site of narrowing in coarctation of the aorta?

A

Around the ductus arteriosus

117
Q

What is coarctation of the aorta often associated with?

A

An underlying genetic condition (particularly Turners syndrome)

118
Q

What does narrowing of the aorta do to the blood pressure in the vessels proximal and distal to the narrowing?

A

Increases the pressure in the proximal areas (heart and aortic branches) and decreases the pressure distally

119
Q

What is the main indication of coarctation of the aorta in a neonate?

A

Weak femoral pulses

120
Q

What will a four limb blood pressure reveal in coarctation of the aorta?

A

High BP in the limbs supplied from arteries before the narrowing, and low BP in limbs that come after the narrowing

121
Q

What murmur may be heard in coarctation of the aorta?

A

Systolic murmur below left clavicle and scapula

122
Q

What signs may there be of coarctation in infancy?

A

Tachypnoea
Poor feeding
Grey, floppy baby

123
Q

What additional signs of coarctation may develop over time?

A

Left ventricular heave
Underdeveloped left arm
Underdeveloped legs

124
Q

Does coarctation range in severity?

A

Yes, may be mild and asymptomatic or severe and require emergency surgery shortly after birth

125
Q

How is critical coarctation after birth treated?

A

Prostaglandin E given to keep ductus arteriosus open while waiting for surgery to correct the coarctation and ligate the ductus arteriosus

126
Q

What is congenital aortic valve stenosis?

A

When babies are born with a narrow aortic valve

127
Q

What is the aortic valve made up of?

A

Three leaflets- the aortic sinuses of the Valsalva

128
Q

What may happen to the aortic sinuses of Valsalva in aortic stenosis?

A

They may have an abnormal number of leaflets (1-4)

129
Q

How may mild aortic stenosis present?

A

Completely asymptomatic with incidental murmur found on routine examination

130
Q

How might more severe aortic stenosis present?

A

With symptoms of fatigue, SOB, dizziness and fainting

131
Q

When are symptoms of aortic stenosis usually worse?

A

On exertion

132
Q

How will severe aortic stenosis present?

A

With heart failure within months of birth

133
Q

What are the signs of aortic stenosis on examination?

A
Murmur
Ejection click
Palpable thrill
Slow rising pulse
Narrow pulse pressure
134
Q

What kind of murmur do you get with aortic stenosis?

A

Ejection systolic murmur, loudest at aortic area.

135
Q

What is the character of the aortic stenosis murmur and where might it radiate to?

A

Cresendo-decrescendo character. Radiates to carotids.

136
Q

How is aortic stenosis diagnosed?

A

Echocardiogram

137
Q

How are patients with congenital aortic stenosis managed?

A

Regular follow up with paediatric cardiologist

Refular echos, ECGs & excercise testing

138
Q

What are the treatment options for congenital aortic stenosis?

A

Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement

139
Q

What are the complications of congenital aortic stenosis?

A
Left ventricular outflow tract obstruction 
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death
140
Q

What is the pulmonary valve made up of?

A

Three leaflets

141
Q

What is congenital pulmonary valve stenosis?

A

Abnormal development of the pulmonary valve leaflets, leading them being thickened or fusing and resulting in a narrow opening between the right ventricle and pulmonary artery

142
Q

What other conditions may pulmonary valve stenosis be associated with?

A

Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome

143
Q

How does pulmonary valve stenosis present?

A

Often asymptomatic

144
Q

How is pulmonary valve stenosis often discovered?

A

Incidental finding of murmur during routine baby checks

145
Q

How may more severe pulmonary valve stenosis present?

A

With symptoms of fatigue on exertion, SOB, dizziness and fainting

146
Q

What are the signs of pulmonary valve stenosis on examination?

A

Murmur
Palpable thrill
Right ventricular heave
Raised JVP

147
Q

What kind of murmur is found with pulmonary valve stenosis?

A

Ejection systolic murmur, heard loudest at pulmonary area

148
Q

How is pulmonary valve stenosis diagnosed?

A

Echocardiogram

149
Q

How is mild pulmonary valve stenosis managed?

A

Monitoring with cardiologist- often need no intervention

150
Q

How is more severe pulmonary valve stenosis managed?

A

Balloon valvuloplasty via venous catheter

151
Q

What does balloon valvuloplasty involve?

A

Inserting catheter into femoral vein undery X-ray guidance, going through IVC and right side of heart to the pulmonary valve and dilating the valve by inflating a balloon

152
Q

What is Tetralogy of Fallot?

A

Congenital condition where there are 4 coexisting pathologies

153
Q

What are the 4 pathologies in Tetralogy of Fallot?

A

Ventricular septal defect
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy

154
Q

What is an overriding aorta?

A

When the aortic valve is further right than normal, and lies above the VSD.

155
Q

What does an overriding aorta cause?

A

A greater proportion deoxygenated blood entering the aorta from the right side of the heart, due to the positioning of the aorta

156
Q

What further encourages deoxygenated blood through the VSD and into the aorta in tetralogy of fallot?

A

Pulmonary valve stenosis

157
Q

Why do you get cyanosis in tetralogy of fallot?

A

Because the overriding aorta and pulmonary stenosis cause blood to be shunted from right to left, meaning it bypasses the lungs

158
Q

What happens to the right ventricle in Tetralogy of Fallot?

A

Right ventricular hypertrophy

159
Q

What kind of shunt do you get in Tetralogy of Fallot?

A

Right to left

160
Q

What are the risk factors for Tetralogy of Fallot?

A

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

161
Q

What is the investigation of choice for diagnosing Tetralogy of Fallot?

A

Echocardiogram

162
Q

What can be done during an echo to look at the direction of blood flow?

A

Doppler flow studies

163
Q

What may a CXR show in Tetralogy of Fallot?

A

Boot shaped heart due to right ventricular hypertrophy

164
Q

When is Tetralogy of Fallot usually picked up?

A

During antenatal scans

165
Q

What may be picked up on the newborn baby check that indicates Tetralogy of Fallot?

A

Ejection systolic murmur caused by pulmonary stenosis

166
Q

How will severe cases of Tetralogy of Fallot present?

A

With heart failure before age 1

167
Q

What are the signs and symptoms of Tetralogy of Fallot?

A
Cyanosis
Clubbing
Poor feeding
Poor weight gain
Ejection systolic murmur
Tet spells
168
Q

What are Tet spells?

A

Intermittent symptomatic periods where the right to left shunt becomes temporarily worsened, causing a cyanotic episode

169
Q

What causes Tet spells?

A

When pulmonary vascular resistance increases or systemic resistance decreases

170
Q

What are some causes of Tet spells?

A

Physical exertion
Waking
Crying

171
Q

Why can physical exertion precipitate a Tet spell?

A

They will be generating a lot of CO2 which is a vasodilator and therefore reduces systemic vascular resistance. This means blood will be pumped from the right ventricle to the aorta in the path of least resistance.

172
Q

What will happen to the child during a Tet spell?

A

They will become irritable, cyanotic and short of breath. May have reduced consciousness, seizures and potentially death

173
Q

What is the mainstay of treatment for a Tet spell?

A

Squatting in older children or knees to chest in younger children

174
Q

Why does a squat position help prevent a Tet spell?

A

It increases the systemic vascular resistance so encourages blood to enter the pulmonary vessels

175
Q

What does medical management of a Tet spell involve?

A
Supplementary oxygen
Beta blockers
IV fluids
Morphine
Sodium bicarbonate
Phenylephrine
176
Q

How do IV fluids help in a Tet spell?

A

Increase pre-load, increasing the volume of blood flowing to the pulmonary vessels

177
Q

How is Tetralogy of Fallot treated?

A

Prostaglandin infusion used to maintain the ductus arteriosus, followed by total surgical repair by open heart surgery

178
Q

What is the prognosis of Tetralogy of Fallot?

A

Depends on severity- poor without treatment. 90% make it to adulthood with corrective surgery

179
Q

What is Ebstein’s anomaly?

A

Congenital heart condition where the tricuspid valve is set lower in the right side of the heart, causing a bigger right atrium and smaller right ventricle

180
Q

What does Ebstein’s anomaly do to the flow in the heart?

A

Lead to poor flow from the right atrium to the right ventricle, causing poor flow to the pulmonary vessels

181
Q

What is Ebstein’s anomaly often associated with?

A

A right to left shunt across the atria via an atrial septal defect, and therefore cyanosis

182
Q

What syndrome is Ebstein’s anomaly associated with?

A

Wolff-Parkinson-White syndrome

183
Q

What is the presentation of Ebstein’s anomaly?

A
Heart failure
Gallop rhythm
Cyanosis
SOB
Tachypnoea
Poor feeding
Collapse/ cardiac arrest
184
Q

What is heard on auscultation of Ebstein’s anomaly?

A

Gallop rhythm caused by addition of third and fourth heart souds

185
Q

How is Ebstein’s anomaly diagnosed?

A

Echo

186
Q

How is Ebstein’s anomaly managed?

A

Treating arrhytmias and heart failure
Prophylactic antibiotics for IE
Surgical correction of underlying defect

187
Q

What is transposition of the great arteries?

A

Condition where the attachments of the aorta and pulmonary trunk to the heart are swapped

188
Q

Explain what transposition of the great arteries causes?

A

Two separate circulations: The right ventricle pumps blood into the aorta and systemic circulation, and the left ventricle pumps blood into the pulmonary vessels and pulmonary circulation

189
Q

What is transposition of the great arteries associated with?

A

Ventricular septal defect
Coarctation of the aorta
Pulmonary stenosis

190
Q

How will the baby present at birth with transposition of the great arteries?

A

Cyanosed

191
Q

What does immediate survival of a neonate with transposition of the great arteries depend on?

A

Shunts between systemic and pulmonary circulation (e.g. patent ductus arteriosus, atrial septal defect or ventricular septal defect)

192
Q

When is transposition of the great arteries usually diagnosed?

A

During pregnancy with antenatal USS

193
Q

What happens if a fetus is diagnosed with transposition of the great arteries?

A

Close monitoring during pregnancy and admission to a hospital capable of managing condition after birth

194
Q

If the condition is not detected during pregnancy, how will transposition of the great arteries present?

A

With cyanosis at or soon after birth

195
Q

How is transposition of the great arteries managed?

A

Prostaglandin infusion to maintain ductus arteriosus
Balloon septostomy
Open heart surgery

196
Q

What is a balloon septostomy?

A

Inserting a catheter into the foramen ovale via the umbilius, and inflating the balloon to create a large atrial septal defect, allowing blood from the lungs to flow to the right side of the heart.

197
Q

How is open heart surgery performed to treat transposition of the great arteries?

A

Cardiopulmonary bypass machine used to perform an arterial switch procedure within a few days of birth