Paediatric cardiology Flashcards

1
Q

Five features of innocent murmurs

A
Soft
Short
Systolic
Symptomless
Situation dependent (e.g. if quieter when standing or only appears when the child is unwell/feverish)
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2
Q

Features of murmurs that prompt further investigations

A

Louder than 2/6
Diastolic murmurs
Louder on standing

Symptoms such as failure to thrive, feeding difficulty, cyanosis or shortness of breath

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3
Q

Investigations to determine the cause of a murmur

A

ECG
Chest X-Ray
Echo

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4
Q

PDA murmur features

A

Continuous crescendo-decrescendo “machinery” murmur

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5
Q

Tetralogy of Fallot murmur features

A

Ejection systolic murmur loudest in the pulmonary area (second intercostal space, left sternal border) –arising from the pulmonary stenosis

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6
Q

VSD murmur features

A

Pan systolic murmur heard at the left lower sternal border

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7
Q

ASD murmur features

A

Mid-systolic crescendo-decrescendo murmur heard loudest at the upper left sternal border with a fixed split second heart sound

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8
Q

What is a fixed split second heart sound?

A

Splitting of the second heart sound can be normal with inspiration, however a “fixed split” second heart sound means the split does not change with inspiration and expiration.

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9
Q

What are the causes of cyanotic heart disease?

A

Anything that can result in a right to left shunt:

  • Transposition of the great arteries
  • TOF can also cause cyanosis
  • VSD
  • ASD
  • PDA
  • These three don’t usually cause cyanosis because the pressure in the left side of the heart is much greater than the right side, and blood will flow from the area of high pressure to the area of low pressure. This prevents a right-to-left shunt. If the pulmonary pressure increases beyond the systemic pressure blood will start to flow from right-to-left across the defect, causing cyanosis. This is called Eisenmenger syndrome.
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10
Q

Risk factors for PDA

A

Prematurity
Maternal infections (e.g. rubella)
Genetic element

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11
Q

PDA presentation

A
May be asymptomatic 
Shortness of breath
Difficulty feeding
Poor weight gain
Lower RTIs
Murmur
Heart failure symptoms in adulthood (rarely)
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12
Q

PDA murmur

A

continuous crescendo-decrescendo “machinery” murmur that may continue during the second heart sound, making the second heart sound difficult to hear.

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13
Q

Investigations to diagnose PDA

A

Echocardiogram - can also assess effect of PDA on the heart e.g. RVH or LVH
Doppler during the echo to assess size of left to right shunt

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14
Q

Management of PDA

A

Indomethacin to promote its closure

If that is ineffective:
Monitoring with Echo until 1 year of age
After 1 year old highly unlikely to close spontaneously so surgical/transcatheter closure

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15
Q

Complications of ASD

A

Stroke (VTE)
AF or atrial flutter
Pulmonary hypertension and right sided heart failure
Eisenmenger syndrome

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16
Q

Symptoms/presentation of ASD

A
May be asymptomatic in childhood 
SOB (dyspnoea)
Difficulty feeding
Poor weight gain/failure to thrive
Lower RTIs

May be picked up on antenatal scans or hearing a murmur in newborn baby check

In adulthood it can present with dyspnoea, heart failure or stroke

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17
Q

ASD murmur features

A

Mid systolic crescendo-decrescendo murmur loudest at upper left sternal border

Fixed split second heart sound

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18
Q

ASD management

A

Refer to paediatric cardiologist
If small can watch and wait
Surgical closure (open or transcatheter)
Anticoagulants (aspirin, warfarin, NOACs) used in adults to prevent VTE/Stroke

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19
Q

VSD is commonly associated with what conditions

A

Down’s syndrome

Turner’s syndrome

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20
Q

Presentation of VSD

A

Often initially symptomless and patients can present as late as adulthood
May be picked up on antenatal scans or hearing a murmur in newborn baby check

Poor feeding
Tachypnoea
Dyspnoea
Failure to thrive

21
Q

VSD murmur features

A

pan systolic murmur heard loudest at the left lower sternal border in 3rd and 4th intercostal spaces

22
Q

Treatment of VSD

A

Referral to paediatric cardiologist
Small VSDs with no symptoms can watch and wait - often close spontaneously

Surgery to close - either transcatheter or open heart surgery (with antibiotic prophylaxis to reduce risk of infective endocarditis)

23
Q

Risks of VSD, complications of VSD

A

Pulmonary hypertension and right sided heart failure
Eisenmenger syndrome
Infective endocarditis

24
Q

What is Eisenmenger syndrome?

A

ASD/VSD leads to left to right shunt

Which leads to pulmonary hypertension

When pulmonary pressure exceeds systemic pressure the shunt switches to right to left

Deoxygenated blood bypasses the lungs leading to cyanosis

25
Q

What is coarctation of the aorta?

A

Where there is narrowing of the aortic arch

26
Q

What is coarctation of the aorta associated with

A

Often associated with an underlying genetic condition - particularly Turner’s syndrome

27
Q

Presentation of coarctation of the aorta

A

Weak femoral pulses
Systolic murmur heard below the left clavicle

Difference in blood pressure between limbs

Tachypnoea and increased work of breathing
Poor feeding
Grey floppy baby

28
Q

Management of coarctation of the aorta

A

Mild cases - can live symptom free until adulthood without need for surgery
Severe cases - emergency surgery

In critical cases where there is a risk of heart failure and death shortly after birth - can give prostaglandin E to keep ductus arteriosus open

29
Q

Presentation of congenital aortic stenosis

A

May be incidental finding due to murmur with no symptoms

Fatigue
SOB
Dizziness and fainting
Worse on exertion

30
Q

Murmur and signs in Aortic stenosis

A

Ejection systolic murmur heard loudest in second intercostal space, right sternal border (aortic area)

Crescendo-decrescendo murmur which radiates to the carotids

Other signs:

  • Ejection click before the murmur
  • Palpable thrill during systole
  • Slow rising pulse and narrow pulse pressure
31
Q

Investigation for Aortic stenosis

A

Echo

Also will need ECG and exercise testing at follow up with a paeds cardiologist

32
Q

Management for aortic stenosis

A

Regular follow up as it is a progressive condition usually
May need to restrict physical activities

Treatment options:

  • Percutaneous balloon aortic valvuloplasty
  • Surgical aortic valvotomy
  • Valve replacement
33
Q

Complications of Aortic stenosis in children

A
Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death, often on exertion
34
Q

Congenital pulmonary valve stenosis associations

A

Congenital pulmonary valve stenosis often occurs without any associations. It can be associated with other conditions such as:

Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome

35
Q

Symptoms in pulmonary valve stenosis

A

Often asymptomatic

More severe cases can present with fatigue on exertion, SOB, dizziness and fainting

36
Q

Signs that may be present in pulmonary valve stenosis

A

Ejection systolic murmur heard loudest at the pulmonary area (second intercostal space, left sternal border)
Palpable thrill in the pulmonary area
Right ventricular heave due to right ventricular hypertrophy
Raised JVP with giant a waves

37
Q

Investigations in suspected pulmonary valve stenosis or any congenital heart disease for that matter

A

Echocardiogram

ECG

38
Q

Management of P valve stenosis

A

Mild - can watch and wait with regular follow up by cardiologist

Symptomatic or more significant stenosis - balloon valvuloplasty or open heart valve repair

39
Q

Four pathologies in tetralogy of Fallot

A

PROV

Pulmonary valve stenosis
Right ventricular hypertrophy
Overriding aorta
Ventricular septal defect

40
Q

Type of shunt in TOF

A

Right to left cardiac shunt

Therefore cyanosis can occur

41
Q

Risk factors for TOF

A

Rubella infection
Increased age of the mother (>40 years)
Alcohol consumption in pregnancy
Diabetic mother

42
Q

How does TOF present?

A

Most cases picked up on antenatal scans
Ejection systolic murmur caused by pulmonary stenosis

Cyanosis
Poor feeding
Poor weight gain
Tet spells - Spells where r-L shunt worsens and cyanosis occurs. These episodes may be precipitated by waking, physical exertion or crying. The child will become irritable, cyanotic and short of breath. Severe spells can lead to reduced consciousness, seizures and potentially death.
Clubbing
43
Q

Treatment of tet spells

A

Encourage child to squat or bring knees to chest in younger children

Oxygen
Beta blockers
IV fluids

44
Q

Management of TOF

A

Prostaglandin infusion in neonates to maintain PDA then total surgical repair by open heart surgery

45
Q

Ebstein’s anomaly - what is it and what is the presentation?

A

Tricuspid valve is set lower in the right side of the heart causing a bigger right atrium and small right ventricle and therefore poor flow to the pulmonary vessels

Cyanosis (due to right to left shunt)
Evidence of heart failure e.g. oedems
SOB and tachypnoea
Poor feeding

Associated with WPW syndrome

46
Q

What is transposition of the great arteries?

A

Attachments of the aorta and pulmonary trunk are swapped

47
Q

What can transposition of the great arteries also be associated with?

A

VSD
Coarctation of the aorta
Pulmonary stenosis

48
Q

How does transposition of the great arteries present?

A

Often diagnosed during antenatal ultrasound scans

As cyanosis at birth or in first days of life - PDA can initially compensate for this

49
Q

Management

A

Prostaglandin infusion can be used to maintain the ductus arteriosus

Balloon septostomy involves inserting a catheter into the foramen ovale via the umbilicus, and inflating a balloon to create a large atrial septal defect. This allows blood returning from the lungs (on the left side) to flow to the right side of the heart and out through the aorta to the body.

Open heart surgery is the definitive management - ASAP