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Paediatrics > Congenital heart defects > Flashcards

Congenital heart defects Flashcards

1
Q

Tetralogy of fallout?

A

Congenital condition

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2
Q

4 co- existing defects in tetralogy of fallot?

A

Ventricular septal defect
Overriding aorta
Pulmonary valve stenosis
Right ventricular hypertrophy

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3
Q

Ventricular septal defect?

A

Hole between 2 ventricles

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4
Q

Overriding aorta?

A

Allows blood from both ventricles to enter aorta

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5
Q

Pulmonary valve stenosis?

A

Narrowing of exit from right ventricle

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6
Q

Right ventricular hypertrophy

A

Thickening of right ventricular muscle

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7
Q

What does tetralogy of fallot cause?

A

Right to left cardiac shunt

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8
Q

What is right to left cardiac shunt?

A

Blood bypasses lungs & does not become oxygenated
Deoxygenated blood entering systemic circulations -> cyanosis
Degree of cyanosis is related to severity of pulmonary stenosis

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9
Q

Risk factors for TOF?

A

Rubella infection
Inc age of mother (>40)
Alcohol consumption in pregnancy
Diabetic mother

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10
Q

Presentation of TOF?

A

Usually at birth (up to 1 year)
Antenatal scans
Ejection systolic murmur
Heart failure (<1 year)

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11
Q

Symptoms of TOF?

A 
Cyanosis
Clubbing
Poor feeding
Poor weight gain
Ejection systolic murmur (loudest in pulmonary area)
Tet spells
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12
Q

Tet spells?

A

Intermittent symptomatic periods - becomes worse
Precipitates cyanotic periods
Becomes irritable, cyanotic and shortness of breath

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13
Q

Severe tet spells?

A

Reduced consciousness
Seizures
Death

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14
Q

Why do tet spells happen?

A

When pulmonary vascular resistance increases or systemic resistance decreases

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15
Q

What precipitates tet spells?

A

Waking
Physical exertion
Crying

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16
Q

Treatment for tet spells?

A 
Older = squat
Younger = knees to chest
Oxygen
Beta blockers
IV fluids
Morphine 
Sodium bicarbonate
Phenylephrine infusion
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17
Q

Investigations for TOF?

A

Echocardiogram
Doppler flow
CXR

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18
Q

What are chest x-ray signs of TOF?

A

‘Boot shaped heart’

Due to right ventricular thickening

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19
Q

Management of TOF?

A 
Prostaglandin infusion (in neonates) -> maintains ductus arteriosus
Total surgical repair
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20
Q

Transposition of great arteries?

A

Attachments of aorta and pulmonary trunk to heart are swapped (transposed)
So right ventricle pumps blood into aorta & left ventricle pumps blood into pulmonary vessels

21
Q

What can transposition of great arteries be associated with?

A

VSD
Coarction of aorta
Pulmonary stenosis

22
Q

How does transposition of GA affect baby during pregnancy/ birth?

A

Normal development of foetus during pregnancy but after birth is immediately life threatening as no connection between systemic and pulmonary circulation

23
Q

Immediate survival in transposition of GA?

A

Shunt between systemic and pulmonary circulation that allows blood flowing through body to be oxygenated by lungs
Shut can occur across a PDA, atrial septal defect or ventricular septal defect

24
Q

Presentation of transposition of great arteries?

A 
Pregnancy/ antenatal scans
PDA or VSD can initially compensate but within few weeks:
- Respiratory distress
- Tachycardia
- Poor weight gain
- Sweating
25
Q

Management of transposition of great arteries?

A 
Prostaglandin infusion (maintain ducts arterioles)
Balloon septostomy 
Open heart surgery = definitive management
26
Q

Balloon septostomy?

A

Insert catheter into foramen ovale via ubilicis and inflating balloon to create large atrial septal defect

27
Q

Coarctation of aorta?

A

Narrowing of aortic arch (usually around ductus arteriosus)

28
Q

What is coarctation of aorta often associated with?

A

Turner’s syndrome

29
Q

What does coarctation of aorta cause?

A

Reduced BP of blood flowing to arteries distal to narrowing

Inc pressure in areas proximal to narrowing e.g. heart and first 3 branches of aorta

30
Q

Presentation of coarctation of aorta in neonates?

A

Weak femoral pulses
4 limb BP - high BP in limbs supplied from arteries before narrowing
Low BP in arteries after narrowing
Systolic murmur

31
Q

Other signs of coarctation of aorta in infants?

A

Tachypnoea & inc work of breathing
Poor feeding
Grey and floppy baby

32
Q

Additional signs of coarctation of aorta?

A

Left ventricular heave
Underdeveloped left arm
Underdevelopment of legs

33
Q

Management of coarctation of aorta?

A

Varies
Asymptomatic -> can live symptom free
Severe -> emergency surgery after birth
Correct coarctation and ligate ductus arteriosus

34
Q

When is prostaglandin E used in coarctation of aorta and why?

A

Critical coarctation with risk of heart failure and death

Keeps ductus arteriosus open until surgery

35
Q

Total anomalous pulmonary venous drainage (TAPVD)?

A

All 4 pulmonary veins drain to systemic veins, right atrium or coronary sinus
Pulmonary & systemic venous blood mix in right atrium causing volume loading of right atrium and right ventricl

36
Q

What is always present with TAPVD?

A

Atrial septal defect or patent foramen ovale & is crucial for left ventricular output

37
Q

What does clinical presentation of TAPVD depend on?

A

Whether pulmonary venous return is obstructed or unobstructed

38
Q

Clinical presentation of unobstructed TAPVD?

A

Insidious with failure to thrive & recurrent chest infections in infancy

39
Q

Clinical presentation of obstructed TAPVD?

A 
Acutely unwell neonate
Tachypnoea
Tachycardia
Severe cyanosis
Death within weeks to months
40
Q

Investigations for TAPVD?

A

ECG

Chest radiology

41
Q

Management of TAPVD?

A

Surgical repair

42
Q

What is hypo plastic left heart syndrome?

A

Left side of heart does not form properly

43
Q

What structures does hypo plastic left heart syndrome affect?

A

Theleft ventricleis underdeveloped and too small.
Themitral valvesis not formed or is very small.
Theaortic valveis not formed or is very small.
The ascending portion of theaortais underdeveloped or is too small.
Often have atrial septal defect

44
Q

Diagnosis of hypo plastic left heat syndrome during pregnancy?

A

Prenatal screening
Ultrasound
Fetal echocardiogram if suspected

45
Q

Diagnosis of hypo plastic left heat syndrome after birth?

A 
Problems breathing
Pounding heart
Weak pulse
Blue skin 
Murmur
46
Q

Other diagnostic tests for hypo plastic left heart syndrome?

A

Echocardiogram

Newborn pulse oximetry screening

47
Q

Treatment of hypo plastic left heart syndrome?

A

Meds

  • Prostaglandin infusion
  • Diuretics
  • Inotropes
  • Afterload reduction

Nutrition or feeding tube

Surgery

48
Q

3 stages of surgery for hypo plastic left heart syndrome?

A

Norwood procedure
Bi- directional Glenn Shunt procedure
Fontan procedure

Paediatrics (1 decks)

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