Y4 - Paediatric Cardiology Flashcards

1
Q

What sort of congenital heart defects can cause decompensation and heart failure?

A

All of them

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2
Q

In which situation will you get cyanosis?

A

Only if there is a right to left shunt

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3
Q

What investigations should you do for suspected congenital heart disease?

A

FBC, CXR, PaO2, ECG, echo, cardiac catheter/advanced imaging techniques

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4
Q

How should you manage heart failure in babies with congenital heart disease?

A

Sit up right, oxygen, calories via NG tube, diuretics (e.g. furosemide)
Duct dependent conditions req. prostaglandins to keep the duct patent (alprostadil)
Transfer to neonatal cardiac centre

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5
Q

What are the symptoms of a small VSD?

A

Usually mild

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6
Q

What are the symptoms of large VSDs?

A

Tachypnoea, hypoxia, difficulty feeding, irritability, tachycardia, growth faltering

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7
Q

When do kids with VSD tend to present and why?

A

Tend to present between 3-6 weeks
Lung vasculature in neonate is very stiff and so pressure in lungs is very high, but as child grows older, gradually the vasculature relaxes and the pressure falls (lowest at 6 weeks)
Between 3-6w reaches critical level and floods the lungs –> pulmonary oedema and hypoxia

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8
Q

What are the signs of VSD?

A

Loud harsh pansystolic murmur at L sternal edge + thrill

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9
Q

What is the course of a VSD?

A

Small ones & muscular ones tend to close on their own

Larger ones more likely to need surgery

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10
Q

What is a VSD?

A

A hole connecting the ventricles

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11
Q

True or false:

In VSD, smaller holes give louder murmurs.

A

True

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12
Q

What is a major complication of VSD?

A

Pulmonary hypertension as pulmonary vessels become stiff

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13
Q

How do you manage VSD?

A

Diuretics and vasodilators
Optimise feeding to improve weight
Open heart surgery if large and req.

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14
Q

What is an ASD?

A

Failure of closure of the septum between the atria

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15
Q

What does the atrial septum do?

A

Separates the right and left atria

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16
Q

Describe the development of the atrial septum

A
  1. Septum primum grows downward & leaves gap at bottom (ostium primum)
  2. Septum primum fuses with endocardial cushion closing gap between R & L atria completely
  3. Hole appears in upper part of septum primum (ostium secundum)
  4. Septum secundum grows down parallel & to the right of septum primum which covers the ostium secundum but leaves an opening (foramen ovale)
    (This all creates a one way valve allowing blood to go from the R to L atrium)
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17
Q

What is the function of the foramen ovale in the foetal circulation?

A

It allows the already oxygenated blood (from the placenta) to bypass the R ventricle and lungs and go into the L atrium

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18
Q

What happens to the septum secundum & primum at birth?

A

They snap closed and fuse to close the foramen ovale

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19
Q

What are most ASDs due to defects in?

A

Ostium secundum (secudum septum doesn’t form properly)

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20
Q

What conditions are ASDs related to?

A

Down’s syndrome

Foetal alcohol syndrome

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21
Q

What is the pathophysiology of an ASD?

A

Higher pressure in L side of heart means blood is shunted from L to R

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22
Q

True or false:

ASD is a cyanotic heart defect

A

False

Acyanotic

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23
Q

What sort of murmur do you get in ASD?

A

Ejection systolic murmur in pulmonary area
Extra blood in R atrium & therefore R ventricle so more blood and therefore turbulence as the blood is going through the pulmonary artery

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24
Q

What other sign may indicate ASD?

A

Wide and fixed second heart sound
P2 is widened as there is more blood in the RV and so more blood passing through the pulmonary artery on systole so valve takes longer to close than the aortic
Fixed as consistently more blood being pumped (unlikely physiological splitting of HS on inspiration due to increased intrathoracic pressure)

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25
Q

What causes physiological splitting of the second heart sound?

A

Inspiration increases intrathoracic pressure, which pulls more blood into the right side of the heart which means there is more blood pumping through the pulmonary artery and thus it closes later than the aortic valve

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26
Q

How do most people with ASD present?

A

Most asymptomatic

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27
Q

How would you diagnose as ASD?

A

Using echo

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28
Q

How do you Rx ASD?

A

If very small may close on its own
Ample T device (access through femoral)
Larger ones may req. open heart surgery

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29
Q

What condition is ASVD associated with?

A

Down’s (40-50% of Down’s babies will have one)

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30
Q

What parts of the heart does an ASVD involve?

A

Atrial and ventricle septa

AV valves

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31
Q

True or false:

ASVD is an acyanotic condition

A

True

32
Q

What does an ASVD present like?

A

Like a large VSD
If very big may get symptoms of congestive heart failure & pulmonary congestion
Poor feeding, tachycardia, tachypnoea, excessive sweating etc.

33
Q

A defect in which embryonic structure is responsible for ASVD?

A

Endocardial cushion

34
Q

How old are children when they present with ASVD & why?

A

3-6w

Same reason as VSD

35
Q

What signs will you see of ASVD on ECG?

A

L axis deviation and RV hypertrophy

36
Q

What is used to confirm the diagnosis of ASVD?

A

Echo

37
Q

What is the management of AVSD?

A

Vasodilators & diuretics

Try and wait until 4-12m old until surgery

38
Q

What does the ductus arteriosus do during development?

A

Connect aortic arch to the pulmonary artery
Foetuses do not rely on the lungs & req. oxygenated blood from the placenta (most of this blood flows from foramen ovale and that which doesn’t goes to pulmonary artery (where most of blood shunted to aorta via DA))

39
Q

When does the ductus arteriosus usually close?

A

After birth

40
Q

What does the ductus arteriosus become after birth?

A

Ligamentum arteriosum

41
Q

What is patent ductus arteriosus?

A

When the ductus arteriosus stays open after birth

42
Q

What keeps the ductus arteriosus patent during development?

A
Prostaglandin E2 (vasodilator)
Made by placenta and DA
43
Q

After birth what causes the DA to close?

A

Fall in levels of prostaglandin E2

Lungs also produce bradykinin which constricts smooth muscle walls of DA

44
Q

When does the DA usually close?

A

Between 48-72h

45
Q

Why is a PDA a problem?

A

L to R shunt from aorta into pulmonary artery as pressure in L side of heart higher than R

46
Q

How do most people with PDA present?

A
Usually asymptomatic 
Continuous murmur (harsh)
47
Q

What complications may occur later in life from a PDA?

A

Pulmonary hypertension due to increased pulmonary volume

48
Q

In which group of individuals is PDA most problematic?

A

Premature babies

49
Q

How do manage PDA?

A

If premature or persistent PDA (>6m)
Indomethacin/ibruprofen
Surgery - e.g. T. coil closure/simple device closure

50
Q

What must you do before administering ibruprofen to treat an PDA?

A

Monitor for IVH and check kidney function

51
Q

What is coarctation of the aorta?

A

Narrowing of the ventricles

52
Q

What are the two forms of coarctation of the aorta?

A

Infant/preductal coarctation - coarctation comes after aortic arch & before ductus arteriosus

Adult - no PDA, no mixing of blood, blood flow increases to head and upper extremities (increases risk of berry aneurysms), dilation of aortic valve & aortic dissection, lower BP in extremities (claudication)

53
Q

What is the typical presentation of coarctation of the aorta?

A
Cyanosis of lower limbs
Collapse, pale/grey
Poor sats and circulation 
Absent femorals 
If critical won't feel any pulse
54
Q

Without intervention what can occur in aortic coarctation?

A

Death in the neonatal period

55
Q

What condition is coarctation of the aorta strongly associated with?

A

Turner’s syndrome (45 X)

56
Q

How is aortic coarctation managed?

A

Prostaglandins to keep PDA open

Operation within 48h at specialist centre (e.g. balloon dilatation)

57
Q

What is transposition of the great arteries?

A

Aorta coming from RV
Pulmonary artery coming from LV
Creating two closed circuits where the systemic and pulmonary circuits don’t link
Blood on right side ever gets oxygenated and is pumped to the body
Blood on left side never gets deoxygenated

58
Q

What is the presentation of transposition of the great arteries?

A

Cyanosis
Dyspnoea
Distressed
Oxygen doesn’t help

59
Q

What are the great arteries?

A

Pulmonary artery and aorta

60
Q

What is the ductus venosus?

A

Duct containing the umbilicus to the inferior vena cava

61
Q

What is lifesaving in TGA?

A

Giving prostaglandins early until surgery can occur at a cardiac centre

62
Q

What defects may be lifesaving in TGA?

A

PDA
VSD
ASD

63
Q

True or false:

TGA is a cyanotic condition

A

True

64
Q

What surgery is used for TGA?

A

Switch procedure

65
Q

What are the conditions in tetralogy of fallot?

A
  1. Stenosis of the right ventricular outflow tract (narrowing of valve/area below valve)
  2. Right ventricular hypertrophy
  3. VSD
  4. Overriding aorta
66
Q

In ToF what does stenosis of the RV outflow tract lead to?

A

Difficulty for the blood to get into the pulmonary artery which leads to RVH in order to push blood against the stenosed pulmonary valve

67
Q

What finding will you see on CXR in ToF and why?

A

Boot shaped heart (due to RVH)

68
Q

What is different about VSD in ToF and VSD in patients with isolated VSD?

A

In ToF, pulmonary stenosis leads to really high pressures in RV so there is a R to L shunt

69
Q

How do people with ToF present?

A

Cyanosis
Cyanotic spells (cry/feeding leads to increased cyanosis & can lose consciousness/have seizures)
FTT
Feeding difficulties

70
Q

What kind of murmur do you get in ToF?

A

Ejection systolic murmur

P2 very quiet

71
Q

What component of ToF causes cyanosis?

A

RV outflow tract obstruction as it increases pressure in RV leading to the R to L shunt

72
Q

How do you manage ToF?

A

Oxygen, IV fluids, beta-blockers (e.g. propranolol)

Corrective surgery at 6m with shunt in interm

73
Q

What is the prognosis of ToF based on?

A

Degree and extent of RV outflow tract obstruction

74
Q

What are the 6 grades for loudness systolic murmurs?

A
  1. Just audible
  2. Quiet, but easily audible
  3. Loud but no thrill
  4. Loud with thrill
  5. Audible even if stethoscope only makes partial contact w. skin
  6. Audible without stethoscope
75
Q

What does inspiration augment?

A

Systemic venous return negative pressure draws blood from abdomen into thorax) & so pulmonary stenosis and tricuspid regurg murmurs

76
Q

What does expiration augment?

A

Pulmonary venous return therefore VSD, mitral incompetence, aortic stenosis