Tetralogy of Fallot Flashcards
When are most cases of Tetralogy of fallot picked up?
In an antenatal scan, before the baby is born
When does Tetralogy of Fallot usually present?
Around 1-2 months
When is Tetralogy of fallot usually picked up?
Around 6 months
What is Tetralogy of fallot?
Anterior malalignment of the aorticopulmonary septum
What are the 4 key characteristics of Tetralogy of fallot?
- ventricular septal defect (VSD)
- right ventricular hypertrophy
- right ventricular outflow tract obstruction, pulmonary stenosis (pulmonary valve stenosis)
- overriding aorta
What determines the severity of the disease?
The severity of the right ventricular outflow tract obstruction
(pulmonary valve stenosis)
What will severe cases of teratology present with?
Heart failure before one year of age.
How would mild Tetralogy of fallot present?
Older children develop signs and symptoms of heart failure.
What are the signs and symptoms of Tetralogy of fallot (heart failure)?
- Cyanosis (blue discolouration of the skin due to low oxygen saturations)
- Clubbing
- Poor feeding
- Poor weight gain
What murmur can be heard with Tetralogy of fallot?
Ejection systolic murmur- heard loudest in the pulmonary area
What is the ejection systolic murmur due to?
Pulmonary stenosis
What might an unrepaired TOF cause?
Hypercyanotic tet spell
What is a tet spell?
Intermittent symptomatic periods where the right to left shunt becomes temporarily worsened, precipitating a cyanotic episode.
What are the main features of tet spells?
- Tachypnoea
* Severe cyanosis that may occasionally result in loss of consciousness
When do tet spells typically occur?
When an infant is upset, is in pain or has a fever
What is the main diagnostic test for Tetralogy of fallot?
Echocardiogram
What can be used to assess the severity of the abnormality and shunt in someone with Tetralogy of fallot?
Doppler ultrasound- produced by echocardiogram
What other imagine can you used for tetralogy of fallot?
Chest x-ray
What might a chest x-ray show?
Characteristic “boot shaped” heart due to right ventricular thickening
What is the non-pharmacological management of tet spells?
- Older children may squat when a tet spell occurs.
* Younger children can be positioned with their knees to their chest
What is seen in 25% of patients with tetralogy of fallot?
A right-sided aortic arch is seen in 25% of patients
What medical management can be given?
Beta blockers
What is the function of beta blockers in tetralogy of fallot?
Reduce infundibular spasm
What is the definitive management of Tetralogy of fallot?
Total surgical repair by open heart surgery
What is the management of a neonate with tetralogy of fallot?
A prostaglandin infusion can be used to maintain the ductus arteriosus-
