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#Phase 2.2: Paediatrics > Tetralogy of Fallot > Flashcards

Tetralogy of Fallot Flashcards

1
Q

What is Tetralogy of Fallot?

A

Most common cyanotic congenital heart disease

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2
Q

What is the pneumonic to remember Tetralogy of Fallot?

A

PROVe

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3
Q

What is Tetralogy of Fallot made up of? (4 things)

A
  1. Pulmonary Stenosis
  2. RV hypertrophy
  3. Overriding aorta
  4. Ventricular septal defect

(PROVe)

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4
Q

What is the prevalence of TOF?

A

3-6 in every 10,000 births

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5
Q

What does Overriding aorta mean? (2 points)

A
  1. Aorta entrance (aka aortic valve) more to R than normal
  2. So it’s right above VSD
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6
Q

What does Overriding aorta mean? (2 points)

A
  1. Aorta entrance (aka aortic valve) more to R than normal
  2. So it’s right above VSD
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7
Q

What is having Overriding aorta in TOF bad? (2 steps)

A
  1. When RV contracts –> send blood upwards
  2. More deoxygenated blood enters Aorta from R heart
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8
Q

Why is Pulm Stenosis bad in TOF? (2 steps)

A
  1. PS –> increased resistance against blood flow from RV
  2. Encourages deox blood to flow thru VSD –> Aorta (instead of thru pulm vessels to lungs)
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9
Q

Therefore, what phenomena does Overriding aorta + PS contribute towards?

A
  1. R-L shunting –> Cyanosis
  2. How do you get RV hypertrophy in TOF?
  3. RV straining to pump blood vs Pulmonary Stenosis
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10
Q

What are the RF for TOF? (5 things)

A
  1. Male
  2. Rubella
  3. Maternal age 40+
  4. Maternal DM
  5. Alcohol @ pregnancy
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11
Q

How are TOF Dx picked up early? (2 things)

A
  1. Antenatal scans (most cases Dx here)
  2. Newborn baby checks (will hear Ejection systolic murmur from PS)
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12
Q

What are the CF of TOF? (5 things)

A
  1. Cyanosis
  2. Clubbing
  3. Poor feeding / X weight gain
  4. Ejection systolic murmur
  5. Tet spells
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13
Q

Where will the Ejection systolic murmur be loudest?

A

In Pulmonary area

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14
Q

What are Tet Spells?

A

Episodes where R-L shunt becomes temporarily worse –> Cyanosis episode

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15
Q

What is the pathophysiological reason for a Tet Spell? (2 things)

A
  1. Pulm vasc resistance increases // OR //
  2. Systemic resistance decreases
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16
Q

What things can cause Tet Spells? (3 things)

A
  1. Waking up
  2. Physical exertion
  3. Crying
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17
Q

How can physical exertion cause a Tet spell? (6 things)

A
  1. CO2 produced @ exercise
  2. CO2 = vasodilator –> Systemic vasodilation
  3. Systemic resistance decreased
  4. Blood flow prefers low resistance path –> R-L shunting increases
  5. Deoxyg blood flows from RV –> Aorta (instead of Pulm vessels, so bypasses lungs)
  6. Cyanosis
18
Q

What are the CF of a Tet Spell? (3 things)

A
  1. Irritable
  2. Cyanosis
  3. SOB
19
Q

What can severe Tet Spells lead to? (3 things)

A
  1. LOC
  2. Seizures
  3. Death
20
Q

What is the GOLD standard investigation for TOF Dx?

A

Echocardiogram

21
Q

What investigations should you for do for sus TOF? (2 things)

A
  1. ECG
  2. Bloods
22
Q

What will an ECG show you in TOF?

A

R axis deviation / RVH signs

23
Q

When should you do Bloods in sus TOF?

A

When sus chromosomal abn

24
Q

What imaging should you do for sus TOF? (2 things)

A
  1. Echocardiogram (GOLD)
  2. CXR
25
Q

What will a CXR show you in TOF? (2 things)

A
  1. Boot shaped heart (RVH)
  2. Reduced pulm vasc marking (bc less pulm blood flow bc shunt)
26
Q

What are the CONSERVATIVE Mx options for TOF?

A

Squatting / knees to chest
(infants do it by themselves, n parents should do it for them as well)

27
Q

Why do TOF kids squat / bring knees to chest?

A

It increases venous return –> increases Systemic resistance

28
Q

What are the MEDICAL Mx options for TOF? (5 things)

A
  1. O2 (if hypoxic in Tet Spell)
  2. Prostaglandin infusion
  3. Beta blockers
  4. Morphine
  5. Saline 0.9% bolus
29
Q

How does Prostaglandin infusion work in TOF Mx? (2 steps)

A
  1. Maintains PDA (patent ductus arteriosus)
  2. Allows blood to flow bk from Aorta –> Pulm arteries
30
Q

When should Prostaglandin infusions be started for TOF?

A

Immediately after delivery
(to avoid newborn collapsing)

31
Q

What are the Side fx of Prostaglandin infusions? (3 things)

A
  1. Apnoea
  2. Bradycardia
  3. Hypotension
32
Q

How do Beta blockers work in TOF Mx? (2 steps)

A
  1. Relax RV
  2. Improves flow to Pulm vessels
33
Q

When can Beta blockers also be used during?

A

Tet spells

34
Q

What Beta blocker is commonly used in TOF?

A

Propranolol

35
Q

How does Morphine work in TOF Mx? (2 steps)

A
  1. Increases resp drive
  2. Reduces Hyperpnoea –> more effective breathing
36
Q

How does Saline 0.9% bolus work in TOF Mx? (2 steps)

A
  1. Increase pre-load
  2. Increases volume of pulm blood flow
37
Q

What are the SURGICAL Mx options for TOF?

A
  1. Temporary procedure (to reduce shunt)
  2. Definitive procedure
38
Q

What does the Definitive Surgery for TOF involve? (2 things)

A
  1. Enlarging PS
  2. VSD patch
39
Q

What are the complications of TOF? (6 things)

A
  1. Polycythaemia
  2. Cerebral abscess
  3. Stroke
  4. Inf endocarditis
  5. CHF
  6. Death
40
Q

What is the rate of death in TOF in 1st year of life?

A

25%

41
Q

What complications can post TOF surgery pt develop in long term? (4 things)

A
  1. Pulm regurg (PR) (common)
  2. Arrhythmias
  3. Exercise intolerance
  4. Sudden death

#Phase 2.2: Paediatrics (18 decks)

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