Paediatric Cardiology - Embryology and Acyanotic Heart Defects Flashcards

1
Q

What are the five embryological origins of the heart?

A
Truncus arteriosus
Bulbus cordis
Ventricle
Atrium
Sinus venosus
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2
Q

What does the truncus arteriosus form?

A

Aorta and Pulmonary trunk

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3
Q

What does the bulbis cordis form?

A

Ventricles - smooth part (with primitive ventricle)

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4
Q

What does the primitive ventricle become?

A

Ventricles - trabecular part of wall (with bulbis cordis)

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5
Q

What does sinus venosus become?

A

Smooth part of atria
SA Node
Coronary sinus

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6
Q

What shunts are present in the foetus?

A

Ductus arteriosus
Foramen vale
Ductus venosus

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7
Q

What is the function of ductus arteriosus and what does it become?

A

Bypass lungs to protect them

Ligamentum arteriosum

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8
Q

What is the function of foramen ovale and what does it become?

A

Bypass lungs to protect them

Fossa ovalis

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9
Q

What is the function of ductus venosus and what does it become?

A

Bypass liver as it is very metabolically active

Ligamentum venosum

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10
Q

How does ductus arteriosus shut?

A

When the baby takes its first breath, the ductus arteriosus contracts due to high O2 sats

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11
Q

How does foramen ovale shut?

A

The first breath opens the pulmonary circulation causing LA pressure > RA pressure which pushes the septum shut

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12
Q

How does ductus venosus shut?

A

When placental support is removed

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13
Q

What are the acyanotic heart defects?

A

Left to Right shunts

ASD
VSD
Coarctation of the Aorta
Patent Ductus Arteriosus
Pulmonary stenosis
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14
Q

How do acyanotic defects present?

A

Most are picked up on antenatal screening
Asymptomatic mostly

Possibly
Fail to thrive
Resp infections
Heart failure

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15
Q

Why can you see heart failure with acyanotic heart defects?

A

Increased pressure in pulmonary circulation

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16
Q

What is Eisenmenger’s syndrome?

A

L –> R shunt swap to R –> L if pulmonary pressure rise to exceed systemic pressure

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17
Q

How are acyanotic heart defects commonly investigated?

A

Echo - flow and anatomy
ECG - identity cardiomegaly or pulmonary hypertension
Cardiac catheter - if severe

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18
Q

How would you see cardiomegaly or pulmonary hypertension on ECG?

A

Commonly Left Ventricular hypertrophy - lead I increase, lead III invert

Right ventricular hypertrophy, right axis deviation, p pulmonale, tall R in V1, right ventricular strain

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19
Q

How are acyanotic heart defects managed in the general term?

A

Loop diuretics if heart failure

Surgical closure of defect

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20
Q

What are the risk factors for developing an acyanotic heart defect?

A

Family history
Maternal smoking/toxins/infection/diabetes
Genetic conditions - Down’s

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21
Q

What are the types of Atrial Septal Defect?

A

Ostium primum ASD

Ostium secundum ASD

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22
Q

What is a patent foramen ovale?

A

Isolated defect in the ostium secundum (more common)

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23
Q

What does a ostium primum Atrial Septal Defect leave?

A

Left to right shunt

+

Tricuspid incompetence

Seen in downs

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24
Q

What murmur would you hear with an Atrial Septal Defect?

A

Ejection systolic

Best heard at left sternal edge - increased blood flow in right ventricular outflow

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25
Q

What clinical features can be suggest a patent foramen ovale?

A

Abnormal right ventricular pressure
Widely split and fixed second sound
Tricuspid flow murmur - mid diastolic rumble
Pulmonary flow murmur - ejection systolic

26
Q

What investigations are used to diagnose an Atrial Septal Defect?

A

CXR - pulmonary plethora
ECG - right ventricular hypertrophy
Echo

27
Q

How are Atrial Septal Defect’s managed?

A

Surgical - transcatheter closure

28
Q

What is the most common type of congenital heart defect?

A

VSD

29
Q

How common are Ventricular Septal Defect’s?

A

Seen in 1/3 of children with heart defects

2-6 per 1000 births

30
Q

What happens to 50% of Ventricular Septal Defect’s?

A

Close spontaneously

31
Q

What is the prognosis of a Ventricular Septal Defect dependent on?

A

Size and position of defect
Development of changes due to blood shunting - pulmonary hypertension/narrowing of pulmonary arteries reduce left to right shunt

32
Q

What chromosomal disorders are Ventricular Septal Defect’s associated with?

A

Down’s
Edward’s
Patau

33
Q

What examination features can be seen with a Ventricular Septal Defect?

A

Harsh pan systolic murmur at left lower sternal border

Heard louder in smaller defects

Parasternal thrill

34
Q

What does a Ventricular Septal Defect cause?

A

Pulmonary congestion - more blood through pulmonary circulation

High output cardiac failure - increased blood return from pulmonary circulation

35
Q

What is a small Ventricular Septal Defect called and what is it its prognosis?

A

Maladie de Roger

Spontaneous closure

36
Q

What is warranted if a small Ventricular Septal Defect doesn’t close within 12 months?

A

Echo to look for complications

37
Q

How does a medium Ventricular Septal Defect present?

A

Infancy symptoms:

Weight gain
Difficulty feeding
Recurrent chest infections

38
Q

What can be heard if pulmonary blood flow is affected by a Ventricular Septal Defect?

A

Mid diastolic murmur

39
Q

What investigations are done for a mid sized Ventricular Septal Defect and what will they show?

A

CXR - moderate cardiac enlargement
Echo - position of defect
Doppler - shunt

40
Q

What are the complications of a Ventricular Septal Defect?

A
Aortic regurgitation
Infective endocarditis
Eisenmenger's syndrome
Right heart failure
Pulmonary hypertension
41
Q

Why can you get aortic regurgitation with a Ventricular Septal Defect?

A

Poorly supported right coronary cusp lead to cusp prolapse

42
Q

How is heart failure treated if present?

A

Diuretics

ACE-Inhibitor

43
Q

What is the management plan for a mid sized Ventricular Septal Defect?

A

Spontaneous improvement in most

Can do surgical correction - case by case

44
Q

What is more likely to develop in a large Ventricular Septal Defect?

A

Heart failure develops early

45
Q

What is the management plan for a large Ventricular Septal Defect?

A

Treat heart failure
Surgical closure under cardiopulmonary bypass

Banding of pulmonary artery in young infants for temporary respite until definitive surgery

46
Q

What is coarctation of the aorta?

A

Narrowing of the aorta

47
Q

Where can coarctation of the aorta occur?

A

Preductal or post ductal (ductus arteriosus)

98% at level of ductus arteriosus

48
Q

Who does coarctation of the aorta affect?

A

Males 2:1 Females

If preductal - symptomatic infants
If post ductal - asymptomatic children

49
Q

How do preductal coarctations normally get picked up?

A

Antenatal diagnosis due to abnormal circulation

Sick neonate with absent femoral pulses - MUST CHECK FEMORAL PULSE WHEN EXAMINING NEONATES

50
Q

What happens in a preductal coarctation?

A

When ductus arteriosus is open, right ventricle adequately maintain CO

When it closes heart failure occurs

51
Q

How is preductal coarctation of the aorta managed?

A

Prostaglandin infusion to maintain ductal patency

Transfer to cardiac centre for surgical intervention

52
Q

How do post ductal coarctations present?

A

Asymptomatic usually

Possible leg pains or headache
Hypertension
BP difference in arms and leg
May have ejection click murmur due to biscupid aortic valve
Systolic ejection murmur in left inter scapular area

53
Q

How can coarctation of the aorta be managed surgically?

A

Catheter balloon dilatation

Resection of coarcted segment with end to end anastomosis

54
Q

What is patent ductus arteriosus?

A

Failure of the ductus arteriosus to close following birth

55
Q

How common is a Patent Ductus Arteriosus?

A

Very common in pre-term children

56
Q

When does the ductus arteriosus close? (functionally and anatomically)

A

Functionally - 12-18 hours

Anatomically - 2-3 weeks

57
Q

What are the risk factors for Patent Ductus Arteriosus?

A

Preterm
Down’s syndrome
High altitudes

58
Q

What clinical features are seen in a Patent Ductus Arteriosus?

A

Bounding pulses - wide pulse pressure

Systolic machinery type murmur at left sternal edge radiating to the back

59
Q

How is a Patent Ductus Arteriosus diagnosed?

A

CXR can show increased pulmonary markings

ECG - left ventricular hypertrophy, prolong PR

60
Q

How else can a Patent Ductus Arteriosus be visualised?

A

Echo

Doppler - shunt

61
Q

How is a Patent Ductus Arteriosus managed?

A

Surgical closure - ligation, division or transvenous umbrella occlusion

Pharmacological closure - indomethacin (not for term infants)

62
Q

Why are Patent Ductus Arteriosus’s closed?

A

Much higher risk of infective endocarditis or pulmonary vascular disease