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Paediatrics finals > Teratology of fallot > Flashcards

Teratology of fallot Flashcards

1
Q

What type of congenital heart condition is teratology of Fallot?

A

Cyanotic

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2
Q

What percentage of congenital heart diseases is teratology of Fallot?

A

10%

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3
Q

List the tetrad which comprises teratology of fallot

A

Ventricular septal defect
Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta

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4
Q

List some risk factors for teratology of fallot

A

1st degree FH
Males
Teratogens - alcohol, warfarin, trimethadione
Genetics - CHARGE (coloboma, heart defects, atresia choanae, retardation of growth/development, GU anomalies, ear anomalies), Di George syndrome, VACTREL association (vertebral anomalies, anorectal malformations, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities)
Associated congenital defects - right aortic arch and congenital diaphragmatic hernia

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5
Q

Describe the shunt direction in VSD

A

Left to right if small

If severe then shunt reversal right to left

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6
Q

What happens when there is a shunt reversal?

A

Child becomes cyanotic

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7
Q

What is the most common site of pulmonary stenosis?

A

Infundibular septum/Right ventricular outflow tract

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8
Q

What does pulmonary stenosis lead to?

A

Intermittent right ventricular outflow tract obstruction - tet spells

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9
Q

Describe right ventricular hypertrophy in teratology of fallot

A

Occurs in response to high pressures needed to overcome and pump deoxygenated blood through RVOTO

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10
Q

When does RVH usually develop?

A

In utero

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11
Q

Describe the overriding aorta in teratology of fallot

A

Compared to the normal heart, the aorta is dilated and displaced over the intraventricular septum
Aortic dilation is caused by an increase in blood flow through the aorta as it receives blood from both ventricles via the VSD
In severe TOF, multiple aorto-pulmonary collateral arteries may also form to help increase pulmonary flow

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12
Q

List the 3 classes of TOF

A

Mild
Moderate/severe
Extreme

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13
Q

Describe mild TOF

A

Mild PS/RVH
Asymptomatic
Disease progresses as the child grows - age of 1-3yrs they develop cyanosis

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14
Q

Describe moderate/severe TOF

A

Present within the first weeks of life with cyanosis and respiratory distress
Prone to recurrent chest infections and failure to thrive

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15
Q

Describe extreme TOF

A

Present within the first hours of life with respiratory distress and cyanosis

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16
Q

How can extreme TOF be divided?

A

TOF with pulmonary atresia

TOF with absent pulmonary valves

17
Q

How can deoxygenated blood flow into the lungs in extreme TOF?

A

Through patent ductus arteriosus

18
Q

What is the peak age of incidence of tet spells?

A

2-4 months

19
Q

How do tet spells present?

A

Paroxysms of hyperpnoea
Irritability
Increasing cyanosis

20
Q

What might precipitate a tet spell?

A

Dehydration
Anaemia
Prolonged crying (induces tachycardia and systemic vascular resistance)

21
Q

What is seen on examination of TOF?

A

Central cyanosis
Clubbing
Thrill (depends on intensity of murmur) or heave (RVH)
Auscultation - Loud, single S2, pansystolic murmur, ejection click, continuous machinery murmur, signs of congestive heart failure

22
Q

What investigations do you do for TOF?

A

ECG - right axis deviation and right ventricular hypertrophy
Microarray - genetic disorders
CXR - boot shaped heart and reduced pulmonary markings
Echocardiogram - standard for diagnosis
Cardiac CT angiogram - Anatomy
Cardiac MRI - anatomy and cardiac function
Cardiac catheter - performed under GA, done as part of pre-op assessment to measure haemodynamic and cardiac function

23
Q

Describe the management of TOF

A

Medical
- Squatting - increases venous return and systemic resistance
- Prostaglandin infusion - Helps maintain the PDA and must be started urgently after delivery to avoid the neonate collapsing - PGE1 (alprostadil) or PGE2 (dinoprostone)
Beta blockers- propranolol - used in tet spells
Morphine - reduces resp drive
Saline 0.9% bolus - used in tet spells as a volume expander

Surgical

  • Palliative - transcatheter RVOT stent insertion or Modified BlalockTaussig shunt (helps mimic a PDA)
  • definitive repair - performed under cardiopulmonary bypass via median sternotomy involving RVOT stenosis resection, RVOT/pulmonary artery augmentation and VSD patch closure
24
Q

List the side effects of prostaglandin infusion

A

Apnoea
Bradycardia
Hypotension

25
Q

List the complications of untreated TOF

A 
Polycythaemia
Cerebral abscess 
Stroke
Infective endocarditis
Congestive cardiac failure 
Death
26
Q

List the post corrective surgical complications those with TOF are at risk of?

A

Pulmonary regurgitation
Arrhythmias
Exercise intolerance
Sudden death

27
Q

When is definitive surgery carried out?

A

3months - 4 yr

28
Q

What percentage of those with TOF survive till adulthood after corrective surgery?

A

85%

Paediatrics finals (37 decks)

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