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Year 4 Paediatrics > Cardiovascular Disease > Flashcards

Cardiovascular Disease Flashcards

1
Q

How do left to right shunts present? Name 3 congenital cardiac defects that involve left to right shunt

A

Breathlessness

1) Ventricular septal defect (30%)
2) Persistent ductus arteriosus (12%)
3) Atrial septal defect (7%)

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2
Q

How do right to left shunts present? Name 2 congenital cardiac defects that involve right to left shunt

A

Blue (cyanosed)

1) Teratology of Fallot
2) Transposition of the great arteries

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3
Q

Name a condition with common mixing i.e. leading to a breathless and blue baby

A

Complete atrioventricular septal defects (2%)

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4
Q

In what % of live births is there a an abnormality of the CV system.

A

1-2%

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5
Q

Name three conditions which cause outflow obstruction and how they present

A 
Pulmonary stenosis (7%) and aortic stenosis (5%). Asymptomatic with a murmur.
Coarctation of the aorta (5%) - presents collapsed with shock
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6
Q

When does the foramen ovale close and why?

A

Within the first few breaths.
Resistance to pulmonary blood flow falls, leading to increase in blood flowing to the lungs. More blood is thus returning to the left atrium, increasing pressure in the left atrium and therefore closing the foramen ovale.

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7
Q

When is the anatomy of the heart checked in the antenatal period?

A

During the fetal anomaly scan performed at 18 and 20 weeks gestation (in developed countries).

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8
Q

What happens if a cardiac abnormality is picked up during the antenatal checks?

A

A detailed fetal echocardiography will be performed by a paediatric cardiologist

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9
Q

What is the most common presentation of congenital heart disease?

A

Heart murmur

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10
Q

What % of children have an ‘innocent’ murmur i.e. not associated with any cardiac pathology?

A

30%

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11
Q

What are the hallmarks of an innocent murmur?

A

4S

1) aSymptomatic patient
2) Soft blowing murmur
3) left Sternal edge
4) Systolic murmur

Also, normal heart sounds with no added sounds; no parasternal thrill & no radiation

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12
Q

What can cause innocent/flow murmurs and why?

A

During a febrile illness or anaemia - this is due to increased cardiac output

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13
Q

Name three maternal conditions that can cause cardiac abnormalities in the fetus and what abnormalities may be caused

A

1) Rubella infection - can cause peripheral pulmonary stenosis and PDA
2) SLE - can cause complete heart block in babies with neonatal lupus (passage of anti-Ro and anti-La antibody across the placenta)
3) Diabetes mellitus - increased incidence overall

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14
Q

What cardiac abnormalities can result from a pregnant woman taking warfarin?

A

Pulmonary valve stenosis and PDA

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15
Q

What cardiac abnormalities may arise in fetal alcohol syndrome?

A

ASD, VSD, tetralogy of fallot (TOF)

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16
Q

What cardiac abnormalities does Down’s syndrome predisposed to?

A

VSD and AVSD

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17
Q

What cardiac abnormalities does Turner syndrome (XO) predispose to?

A

Aortic valve stenosis & coarctation of the aorta

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18
Q

What cardiac abnormalities does the chromosome 22q11.2 deletion predispose to? What % of people with this deletion have cardiac abnormalities?

A

Aortic arch abnormalities, tetralogy of Fallot, common arterial trunk. Cardiac abnormalities are present in 80% with this chromosome deletion.

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19
Q

What cardiac abnormalities does Williams syndrome (7q11.23) predispose to?

A

Supravalvular aortic stenosis; peripheral pulmonary artery stenosis.

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20
Q

What cardiac abnormalities does Noonan syndrome (PTPN11 mutation) predispose to?

A

Hypertrophic cardiomyopathy, ASD, pulmonary valve stenosis

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21
Q

What are the symptoms of heart failure in an infant?

A

Breathlessness, sweating, poor feeding, recurrent chest infections

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22
Q

What are the signs of heart failure in an infant?

A

Poor weight gain or ‘faltering growth’; tachypnoea; tachycardia; heart murmur, gallop rhythm; enlarged heart; hepatomegaly; cool peripheries

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23
Q

What signs of right heart failure are rare in children of developed countries? Under which conditions might they be seen?

A

Ankle oedema, sacral oedema and ascites.
May be seen with long standing rheumatic fever or pulmonary hypertension and also with tricuspid regurgitation and right atrial dilatation.

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24
Q

What are the causes of HF in neonates (first week of life)?

A

Anything that causes LEFT heart obstruction (obstructed duct dependent systemic circulation):

1) Hypoplastic left heart syndrome
2) Critical aortic valve stenosis
3) Severe coarctation of the aorta
4) Interruption of the aortic arch

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25
Q

What condition may develop in children with untreated left to right shunt? What is it?

A

Eisenmenger syndrome.
(irreversibly raised pulmonary vascular resistance resulting from chronically raised pulmonary HTN. Eisenmengers develops when there is reversal of the shunt, (from L to R to R to L) causing the teenager to be cyanotic.

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26
Q

What three conditions can result in Eisenmenger’s syndrome and what treatment options are available for it?

A

1) VSD (most commonly)
2) ASD
3) PDA

Medication can palliate the symptoms but the only surgical option is a heart-lung transplant

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27
Q

What may cause peripheral cyanosis in a child?

A

Can occur when the child is unwell or cold. Can also be a sign of polycythaemia.

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28
Q

Where do you check for central cyanosis? What does it suggest? When might it be less pronounced?

A

Central cyanosis is seen on the tongue as a blue slate colour. It suggests a fall in arterial blood oxygen tension and it may be less pronounced if a child is anaemic.

(Why? central cyanosis is only clinically apparent if the reduced Hb in the blood i.e. deoxyHb, exceeds 5g/dL. In anaemia there is less Hb altogether so less of it will be reduced/deoxygenaed.)

29
Q

Define respiratory distress in a neonate in breaths per min

A

> 60 breaths/min

30
Q

Summary card: presentation of congenital heart defects

A
  • Antenatal ultrasound screening -increasing proportion detected
  • Detection of a heart murmur - need to differentiate innocent from pathological
  • Cyanosis - if duct dependent, prostaglandin to maintain ductal patency is vital for initial survival
  • Heart failure - usually from left to right shunt when pulmonary vascular resistance falls.
  • Shock - when duct closes in severe left heart obstruction (although bare in mind non cardiac causes e.g. septicemia)
31
Q

What investigation should be carried out if a congenital heart defect is suspected?

A
  • CXR and ECG (rarely diagnostic but helpful for establishing an abnormality of the CV system and for a baseline to asses future changes)
  • Echocardiography combined with doppler US enables almost all causes of congenital heart disease to be diagnosed.
32
Q

What are the two main types of ASD?

A

1) Secundum ASD (80%)

2) Partial atrioventricular septal defect (primum ASD and pAVSD)

33
Q

Describe the anatomy of a secundum ASD

A

A defect in the center of the of the arterial septum involving the foramen ovale

34
Q

Describe the characteristic anatomical features of a partial AVSD

A

1) An inter-atrial communication between the bottom end of the atrial septum and the atrioventricular valves (primum ASD).
2) Abnormal AV valves, with a regurgitant left (mitral) valve which has three cusps and tends to leak.

35
Q

What are the symptoms of ASD?

A

Commonly no symptoms are present…

Or they may experience recurrent chest infections/wheeze and arrhythmia from the fourth decade onwards.

36
Q

What are the physical signs of secundum ASD?

A
  • An ejection systolic murmur best heard at the upper left sternal edge - due to increased flow across the pulmonary valve
  • A fixed widely spilt second heart sound (difficult to hear)
37
Q

What are the physical signs of a partial AVSD?

A

An apical pansystolic murmur from atrioventricular valve regurgitation.

38
Q

What might a chest x-ray show in ASD?

A

Cardiomegaly, enlarged pulmonary arteries & increased pulmonary vascular markings

39
Q

What ECG changes may be seen in secundum ASD?

A

Partial RBBB is common but may occur in normal children

Right axis deviation due to right ventricular hypertrophy

40
Q

What ECG changes may be seen in partial AVSD?

A

A superior QRS axis

41
Q

How do you treat secundum ASD?

A

Cardiac catheterisation with insertion of an occlusive device

42
Q

How do you treat partial AVSD?

A

Surgical correction

43
Q

VSDs can be classified according to size: small or large. Considering small VSD, up to what size does this include and what are the symptoms?

A

Small VSDs are smaller than the aortic vlve in diameter… (up to 3mm) and are asymptomatic

44
Q

What are the signs of a small VSD?

A

*Loud pansystolic murmur at the lower left sternal edge - loud murmur implies smaller defect
(makes sense that its lower left sternal edge as ventricles are lower!)

*Quiet pulmonary 2nd sound (P2)

45
Q

There is no pulmonary HTN in small VSDs. True or false?

A

True

46
Q

What is the management of small VSDs?

A

No intervention needed as these lesions close spontaneously. Advice about good dental hygiene should be given as a preventative measure for invective endocarditis

47
Q

What size are large VSDs?

A

Same size or bigger than the aortic valve

48
Q

What are the symptoms of large VSDs?

A
  • Heart failure with breathlessness and failure to thrive after one week old
  • Recurrent chest infections
49
Q

What are the signs of a large VSD?

A
  • Tachypnoea, tachycardia and hepatomegaly from HF
  • Active precordium
  • Soft pan-systolic murmur or no murmur - implying large defect
  • Apical mid-diastolic murmur (from increased flow across the mitral valve after the blood after circulated through the lungs)
  • Loud pulmonary second sound (from raised pulmonary arterial pressure)
50
Q

What might a chest x-ray show in a) small VSDs and b) large VSDs

A

a) CXR normal in small VSD
b) In large VSDs the CXR may show:
- Cardiomegaly
- Enlarged pulmonary arteries
- Increased pulmonary vascular markings
- Pulmonary oedema

51
Q

What might an ECG show in a) small VSDs and b) large VSDs

A

a) ECG normal in small VSD
b) In large VSD:
- Biventricular hypertrophy by 2 months of age

52
Q

What is the management for large VSDs?

A

Drug therapy for HF: diuretics often combined with catopril (ACE inhibitor)
Additional calorie input

53
Q

There is always pulmonary HTN in children with a large VSD and left-to-right shunt. True or false?

A

True

54
Q

Define persistent ductus arteriosus

A

Failure of the ductus arteriosus to close by 1 month after expected date of delivery. This is due to a defet in the constrictor mechanism of the duct.

55
Q

Which way does the blood flow in a PDA?

A

From the aorta to the pulmonary artery (as pulmonary vascular resistance falls after birth)

56
Q

When is PDA not sure to a congenital abnormality?

A

In the preterm infant

57
Q

How do most children with a PDA present?

A

With a continuous murmur beneath the left clavicle

Pulse pressure is increased causing a collapsing or bounding pulse

58
Q

What are the symptoms of PDA?

A

Symptoms are unusual but when the duct is large there will be increased pulmonary blood flow with HF and pulmonary HTN

59
Q

What do investigations show?

A

ECG and CXR are usually normal however, if the PDA is large then they will show signs indistinguishable from a large VSD.
The duct is easily identified by echocardiography

60
Q

What does closing the duct decrease risk of?

A

Bacterial endocarditis and pulmonary vascular disease

61
Q

What is the management of a PDA?

A

With a coil or occlusion device introduced via a cardiac catheter at about 1 year of age

Occasionally surgical ligation is required

62
Q

What is the hyperoxia (nitrogen washout) test?

A

It is a test used to help determine the presence of heart disease in a cyanosed neonate

63
Q

Describe the hyperoxia (nitrogen washout) test

A

The infant is placed in 100% oxygen for 10 mins. If the right radial arterial partial pressure of oxygen from a blood glass remains low after this time (

64
Q

How do you manage a neonate presenting with a cyanotic congenital heart defect in the first week of life?

A
  • Stabilise the airway, breathing & circulation (artificial ventilation if necessary)
  • Start prostaglandin infusion (PGE 5ng/kg/min)

Note: most infants with cyanotic heart disease presenting in the first few days of life are duct dependent

65
Q

What are the four features of the tetralogy of Fallot

A

Remembered by the mnemonic PROVe:

Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta
Ventricular septal defect

66
Q

How are most cases of TOF diagnosed?

A

Antenatally of following diagnosis of a murmur in the first couple of months of life

67
Q

What is the classical description of TOF (although now rare in developed countries)

A
  • Severe cyanosis
  • Hypercyanotic spells
  • & squatting on exercise (developing in late infancy)
68
Q

What is the reason for squatting on exercise in children with TOF?

A

It compresses the femoral arteries and therefore increases systemic vascular resistance

69
Q

What complications can result from hypercyanotic spells

A

MI, CVA & death if left untreated

Year 4 Paediatrics (17 decks)

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