Paediatric Cardiology Flashcards

1
Q

What are the 4 defects of tetralogy of fallot

A

Ventricular septal defect
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy

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2
Q

RF for tetralogy of fallot

A

Rubella infection, maternal diabetes, increasing maternal age (>40), alcohol use during pregnancy

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3
Q

What determines the degree of cyanosis in tetralogy of fallot

A

The degree of pulmonary stenosis

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4
Q

Pathophysiology of tetralogy of fallot

A
  • pulmonary stenosis increases pulmonary resistance
  • blood shunts through VSD from right to left and into aorta bypassing the lungs
  • the increased pulmonary resistance also causes right ventricular hypertrophy
  • the blood does not get oxygenated at the lungs causing cyanosis
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5
Q

Presentation of tetralogy of fallot

A

Cyanosis, finger clubbing, poor feeding and poor weight gain, Tet spells, ejection systolic murmur in pulmonary area

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6
Q

What are Tet spells

A

Periods of worsening cyanosis due to decreases in systemic resistance or increases of pulmonary resistance
- for example during exertion there is increased CO2 causing systemic vasodilation
- this worsens the right to left shunt increasing cyanosis

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7
Q

How do Tet spells present

A

Worsening of cyanosis- irritability, shortness of breath, loss of consciousness, seizures and potentially death
May occur during physical exertion, crying etc
Child may squat or bring knees to chest to increase systemic resistance

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8
Q

How is tetralogy of fallot diagnosed

A

Echocardiogram and Doppler study of blood flow
Chest X ray- boot sign

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9
Q

How are Tet spells treated

A

Oxygen, beta blockers, IV fluids, morphine, sodium bicarbonate, phenylephrine infusions

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10
Q

Treatment of Tetralogy of fallot

A

Neonates- prostaglandin infusion to maintain the ductus arteriosus (aorta-> PA)
Definitive = surgery

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11
Q

Describe what atrial septal defects are

A

Defects in the septum between the atrium caused by failure of the septum primum or septum secondum to close

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12
Q

Two types of ASD

A

Ostium primum and ostium secondum

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13
Q

Are ASDs cyanotic?

A

No - may become if eisenmengers syndrome occurs

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14
Q

Describe how ASDs can become cyanotic

A

If the left to right shunt is big enough there can be right sided overload.
This can lead to RHF and pulmonary hypertension
This can cause reversal of the left to right shunt so blood bypasses the lungs

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15
Q

RF for ASD

A

Maternal smoking, family history, maternal diabetes, maternal rubella infection

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16
Q

What is the murmur that is associated with atrial septal defects ?

A

Mid systolic crescendo decrescendo murmur heard loudest on the upper sternal border

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17
Q

What can happen to the heart sounds in ASDs?

A

You can have a fixed split second heart sound due to difference in timing of the aortic and pulmonary valves (occurs due to delayed closing of the pulmonary valve as a result of increased blood flow) .
This will not vary with inspiration (its fixed)

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18
Q

How may a child with a ASD present

A

Shortness of breath, poor feeding, poor weight gain, lower respiratory tract infections

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19
Q

How may ASDs present in adulthood

A

Dyspnoea, heart failure, stroke

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20
Q

Diagnosis of atrial septal defects

A

Echo
ECG - RBBB

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21
Q

Treatment of atrial septal defects

A

If less than 1cm watch and wait
If larger than 1cm - surgical repair (either transvenous catheter closure or open heart surgery)

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22
Q

Treatment of atrial septal defects

A

If less than 1cm watch and wait
If larger than 1cm - surgery (transvenous catheter closure or open heart surgery

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23
Q

Why may strokes occur in atrial septal defects?

A

In DVTs emboli usually travel into the lungs and cause PE
In a right to left shunt they can travel into the brain and cause a stroke

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24
Q

Complications of ASDs

A

Strokes, atrial fibrillation, atrial flutter, right heart failure

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25
Q

Explain what a ventricular septal defect is?

A

A congenital hole in the the septum between the ventricles

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26
Q

What can cause ventricular septal defects

A

Genetic condition such as Down’s syndrome, Turner’s syndrome, Edward’s syndrome
Congenital infections
Post-myocardial infarction

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27
Q

Are VSDs cyanotic

A

No but then can become cyanotic in Eisenmengers

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28
Q

Presentation of a VSDs

A

Pan-systolic murmur heard loudest in the lower sternal border
Poor feeding
Dyspnoea
Tachypnoea
Failure to thrive

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29
Q

Differentials of a pan systolic murmur

A

VSDs, mitral regurgitation, tricuspid regurgitation

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30
Q

Diagnosis of VSDs

A

Echo

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31
Q

Treatment of VSDs

A

Small- watch and wait
Large - surgical repair (trans catheter, or open)

32
Q

What are potential ocmplications of VSDs

A

Aortic regurgitation
Infective endocarditis
RHF

33
Q

What should be given to patients with VSDs if they have surgery

A

Prophylactic antibiotics to prevent infective endocarditis

34
Q

How does a patent ductus arteriosus present on examination

A

machinery-like murmuer
bounding pulse
widened pulse pressure

35
Q

What can be given to close a patent ductus arteriosus

A

indomethacin or ibuprofen

36
Q

What type of pulse may be heard in a patent DA

A

collapsing pulse

37
Q

what type of murmur is not consisten with an innocent murmur

A

a diastolic murmur

38
Q

what is ebstein’s anomaly

A

a congenital cardiac condition where the tricuspif valve leaflets are attached to the walls of the septum of the right ventricle

39
Q

What are features of a innocent murmur?

A

soft, systolic, short, symptomless, standing/sitting

40
Q

what medication should be given initially in babies born with cyanotic congenital heart diseases

A

prostaglandin E1

41
Q

what are the 5 cyanotic heart diseases?

A

tetralogy of fallot
transposition of the great vessels
tricuspid atresia
total anomalous pulmonary venous return
truncus arteriosus

42
Q

What test can be used to differentiate between cardiac and non-cardiac causes of cynosis in neonates

A

nitrogen washout test - neonate is given 100% O2 for 10 mins and then an ABG is taken- pO2 of less than 15 indicates cyanotic heart disease

43
Q

If a cyanotic heart defect presents in the first few days of life what is the likely cause

A

transposition of the great arteries

44
Q

if a cyanotic heart disease presents after 1-2 months of life, what is the most likely cause?

A

tetralogy of fallot

45
Q

when is indomethacin given to a newborn for PDA

A

afte an echo shows PDA one week after delivery

46
Q

is ventricular tachycardia a shockable rhythm

A

yes

47
Q

what size VSD can be treated conservatively

A

less than 3mm

48
Q

murmur of a patent ductus arteriosus

A

continuous machinery like murmer in the left sternal edge

49
Q

in tetralogy of fallot what defect causes the murmur

A

pulmonary stenosis

50
Q

what does transposition of the great arteries look like on x ray

A

egg on a string appearance

51
Q

What are the cyanotic congenital heart diseases?

A

transposition of the great arteries
tetralogy of fallot

52
Q

What is transposition of the great arteries?

A

a cyanotic heart disease where the origins of the aorta and the pulmonary artery are transposed

53
Q

RF of transposition of the great arteries

A

maternal diabetes
maternal rubella infection in pregnancy
alcohol use
increased maternal age
family history
other structural heart defects (VSD, ASD)

54
Q

Pathophysiology of transposition of the great arteries

A

normally in embryology there is spiralling of the great arteries however in GA the spiraling fails to occur

55
Q

how does transposition of the great arteries present?

A

cyanosis at birth- worsens after closure of the ductus arteriosus, improves if a septal defect is present
rapid breathing
poor weight gain
heart murmur- systolic if VSD also prsent
loud single S2

56
Q

How is transposition of the great arteries diagnosed

A

often antenatal diagnosis on USS
echo
xray- eggs on a string appearance.

57
Q

How is transposition of the great arteries treated?

A

surgical correction in the first few months

58
Q

what treatment can be given prior to surgery in transposition of the great arteries and why?

A

prostaglandin E1 - keeps the ductus arteriosus open

59
Q

What is coarctation of the aorta

A

a congential heart disease where there is narrowing of the aorta, typically just before the ductus arteriosus

60
Q

What genetic condition is coarctation of the aorta associated with?

A

Turner’s syndrome

61
Q

How does coarctation of the aorta present?

A

can be asymptomatic if mild
systolic murmur- loudest between the scapula
radiofemoral delay
hypertension of the upper limbs
can present as shock once the ductus arteriosus shuts

62
Q

How is coarctation of the aorta diagnosed?

A

Echo
BP - increased in upper and lower in the lower limbs
ECG- may show evidence of left ventricular hypertrophy

63
Q

How is severe coarctation of the aorta treated

A

1st line- balloon angioplasty and stent insertion
2nd line- surgical correction

64
Q

What is rheumatic fever?

A

an immunological response to recent streptococcus pyogenes infection

65
Q

How soon after infection does rheumatic fever occur?

A

2-4 weeks

66
Q

What condition often is associated with rheumatic fever

A

tonisilitis caused by strep pyogenes

67
Q

Pathophysiology of rheumatic fever

A

Infection with strep pyogenes activates the innate immune system causing IgM and IgG production and activation of CD4+ T cells

There is a type 2 hypersensitivity reaction where antibodies to a antigen on the cell wall of strep pyogenes (M protein) cross react with myosin and smooth muscle of arteries

68
Q

What diagnostic criteria can be used for rheumatic fever?

A

the Jones criteria

69
Q

What components make up the jones criteria

A

RF is diagnosed when there is evidence of a recent streptococcal infection (positive throat swab, raised ASOT, raised DNase B titre)
plus two major criteria or one major and 2 minor criteria

Major criteria:
- joint arthritis
- organ inflammation (carditis)
- Nodules
- Erythema marginatum rahs
- Sydenham chorea

Minor:
– Fever
- ECG changes without carditis (prolonged PR interval)
- arthralgia without arthritis
- raised inflammatory markers (CRP and ESR)

70
Q

How is arthritis associated with rheumatic fever describeD?

A

migratory arthritis of the large joints- joint are hot and swollen then improve nad move to next joint)

71
Q

What is sydenham’s chorea

A

irregular uncontrolled rapid movmements of the limbs along with emotional disturbance and muscle weakenss
Often worse on one side and ceases during sleep

72
Q

What ECG change may be associated with rheumatic fever

A

prolonged PR interval

73
Q

describe the rash associated with rheumatic fever

A

erythema marginatum - pink rings of varying sizes on torso and proximal limbs

74
Q

How is rheumatic fever treated

A

pen V 10 days when streptococcal infection
NSAIDs

prophylactic antibiotics may be used into adulthood to preent further strep infections- oral or IM penicillin

75
Q

what type of bacteria causes rheumatic fever?

A

group A beta haemolytic streptococci- strep pyogenes

76
Q

What complications can occur in rheumatic fever?

A

valvular heart disease- particularly mitral stenosis
chronic heart failure
recurrence

77
Q
A