Lightning Learning: Tetralogy of Fallot

““Tetralogy of Fallot is the most common cyanotic congenital heart disease, making up 10% of all CHDs.””

— advice is based on local guidelines & procedures

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STOP!

Tetralogy of Fallot is the most common cyanotic congenital heart disease (CHD) and makes up 10% of all CHDs. Consists of a tetrad of defects (see image).

Three categories:

1. Mild (“Pink”) – asymptomatic until age 1-3

2. Moderate-severe – cyanotic and respiratory distress age
   <1 week

3. Extreme – cyanotic and respiratory distress <few hours

Risk factors?

• Male

• 1st degree FH of CHD

• Teratogens

• Genetic conditions

LOOK

Hypoxic ‘Tet’ spells…

Mild disease:

• Left ventricular pressure (LVP) > right ventricular pressure (RVP)

• Acyanotic

Severe disease:

• Pulmonary Stenosis causes RVP > LVP

• Cyanosis

Investigations?

ECG: RVH and RAD

CXR: Boot shaped heart

ECHO: Gold standard

Other: e.g. Cardiac MRI

LEARN

• Teach Me Paediatrics: Tetralogy of Fallot

Managed with medication and surgery. Definitive repair surgery between 3 months to 4 years. Following surgery >85% survive to adulthood.