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
Which heart defect always results in cyanosis?
Transposition of the great arteries because the right side of the heart pups blood directly into the aorta and systemic circulation.
26
When does the ductus arteriosus normally stop functioning?
1-3 days of birth and closes completely within the first 2-3 weeks of life
27
What might cause patent ductus arteriosus?
Prematurity Maternal rubella genetic
28
What is the pathophysiology of PDA?
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
29
What are the symptoms of PDA in a newborn?
Murmur SOB Difficulty feeding Poor weight gain Lower respiratory tract infections
30
How is PDA diagnosed?
Echocardiogram- see left to right shunt and hypertrophy
31
What is the management for PDA?
Monitored till 1 year old until it is unlikly the hole will close- trans-catheter or surgical closure
32
What is the pathophysiology of atrial septal defects?
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
33
What are the types of atrial septum defect from most common?
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
34
What are the complications of ASD?
Stroke Atrial fibrillation/flutter Pulmonary HTN and right sided heart failure Eisenmenger syndrome
35
What are the signs of ASD?
Murmur SOB Difficulty feeding Poor weight gain Lower respiratory tract infections
36
What did the PREMIUM trial find?
There is a possible link between migraine with aura and patent foramen ovale
37
What is the management for ASD?
If small can watch and wait Can be closed surgically with transvenous catheter closure or open heart surgery
38
What medication is used to reduce risk of clots in adults?
Anticoagulants (aspirin, warfarin, NOACs)
39
What conditions are often associated with VSDs?
Down's syndrome and Turner's syndrome
40
What is the presentation of VSD?
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
41
what are the examination findings of VSD?
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
42
What are the causes of pan-systolic murmur?
VSD, mitral regurgitation and tricuspid regurgitation
43
How is VSD managed?
Often they close spontaneously but can be treated with transvenous catheter via the femoral vein or open heart surgery
44
What is there an increased risk of in VSD?
Infective endocarditis
45
What underlying lesions can result in eisenmenger syndrome?
ASD, VSD, PDA
46
When can Eisenmenger syndrome develop?
1-2 years with large shunts or adulthood with small shunts.
47
When can eisenmenger syndrome develop more quickly?
During pregnancy
48
What occurs due to cyanosis?
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
What is polycythaemia?
High concentration of haemoglobin in the blood
50
What symptom results from polycythaemia?
Plethoric complexion
51
What are the examination findings in eisenmenger syndrome?
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
What examination findings are there in eisenmenger syndrome related to underlying septal defect?
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
What is the prognosis of eisenmenger syndrome and what are the main causes of death?
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
What is the only definitive treatment of eisenmenger syndrome?
heart-lung syndrome
55
What does medical management involve in eisenmenger syndrome?
Pulmonary HTN: Sildenafil Arrhythmias Polycythaemia: venesection Thrombosis: anticoagulation Infective endocarditis: Prophylactic antibiotics
56
What is coarctation of the aorta?
Narrowing of the aortic arch, usually around the ductus arteriosus
57
What is coarctation of the aorta associated with?
Turner's syndrome
58
What does coarctation of the aorta do to pressure of the blood?
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
What is often the only indication of coarctation in a neonate?
Weak femoral pulses
60
What should you do to see if the blood pressure indicates coarctation of the aorta?
Four limb blood pressure
61
What murmur may be heard in coarctation of the aorta?
systolic murmur heard below the left clavicle and below the left scapula
62
What signs might there be in infancy of coarctation of the aorta?
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
What is the management in critical aortic coarctation where there is risk of heart failure and death shortly after birth?
Prostaglandin E is used to keep the ductus arteriosus open while waiting for surgery
64
What are the leaflets that make up the aortic valve?
3 and they are the aortic sinuses of Valsalva
65
How many leaflets does the aortic valve have in people born with aortic stenosis?
one, two, three of four leaflets
66
What does significant aortic valve stenosis present as?
Fatigue, SOB, dizziness and fainting. Symptoms are worse on exertion
67
What is the key examination finding in aortic valve stenosis?
Ejection systolic murmur heard loudest at the aortic area (second intercostal space, right sternal border). Crescendo-decrescendo character and radiates to the carotids.
68
What signs might be present on examination in aortic valve stenosis?
Ejection click just before the murmur Palpable thrill during systole Slow rising pulse and narrow pulse pressure
69
What is the gold standard management for aortic valve stenosis?
Echocardiogram
70
What are the options for treating stenosis?
Percutaneous balloon aortic valvoplasty Surgical aortic valvotomy Valve replacement
71
What are the complications of aortic valve stenosis?
Left ventricular outflow tract obstruction Heart failure Ventricular arrhythmias Bacterial endocarditis Sudden death, often on exertion
72
How many leaflets is the pulmonary valve usually made up of?
3
73
What other conditions are associated with pulmonary valve stenosis?
Tetralogy of Fallot William syndrome Noonan syndrome Congenital rubella syndrome
74
What are the signs of pulmonary valve stenosis?
Often asymptomatic but may be fatigue on exertion, shortness of breath, dizziness and fainting
75
What are the signs of pulmonary valve stenosis?
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
What is the gold standard for diagnosing pulmonary valve stenosis?
Echocardiogram
77
What is the management for symptomatic pulmonary valve stenosis?
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
What are the four coexisting pathologies in tetralogy of fallot?
VSD Overriding aorta Pulmonary valve stenosis Right ventricular hypertrophy
79
How does overriding aorta contribute to TOF?
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
How does pulmonary valve stenosis contribute to TOF?
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
What direction is the shunt in TOF?
Right to left meaning blood bypasses the child's lungs, causing cyanosis
82
What are the risk factors for TOF?
Rubella infection Increased age of the mother (over 40) Alcohol consumption in pregnancy Diabetic mother
83
What is the investigation of choice for all structural congenital cardiac abnormalities?
Echocardiogram
84
What might a chest xray show in tetralogy of fallot?
Boot shaped heart due to right ventricular thickening
85
What are the signs and symptoms of TOF?
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
What are tet spells?
Intermittent symptomatic periods where the right to left shunt becomes temporarily worsened precipitating a cyanotic episode.
87
When do tet spells occur?
When the child is physically exerting themselves, crying or waking.
88
Why do tet spells occur on exertion?
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
What might children do when they experience a tet spell?
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
What is the medical management for a tet spell?
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
In neonates, what can help with TOF?
Prostaglandins to maintain the ductus arteriosus
92
What is the definitive treatment for TOF?
Total surgical repair however mortality is around 5%
93
What is the prognosis of TOF with corrective surgery?
90% of patients will live into adulthood
94
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 a smaller right ventricle. Leading to poor flow from right atrium to right ventricle.
95
Which way is the shunt in Ebstein's anomaly?
right to left shunt
96
What is Ebstein's anomaly associated with?
Wolff-Parkinson-White syndrome
97
What is the presentation of Ebstein's anomaly?
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
When do symptoms of Ebstein's anomaly typically appear?
A few days after birth when the ductus arteriosus closes
99
What is the diagnosis for Ebstein's anomaly?
Echocardiogram
100
What is the management for Ebstein's anomaly
Medical management- treat arrhythmia and heart failure Prophylactic antibiotics to prevent infective endocarditis Surgical correction
101
What is transposition of the great arteries?
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
What can transposition of the great arteries also be associated with?
VSD Coarctation of the aorta Pulmonary stenosis
103
When is transposition of the great arteries often diagnosed?
Antenatal ultrasound scans
104
What can be used to manage transposition of the great arteries?
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.