Paeds: Tetralogy of Fallot Dr Clarke

Question 1

Pathology

Answer 1

Cyanotic
Dec pulmonary outflow with R-L shunt
Most common cause of cyanotic hear disease
4 features
-overriding aorta (on V) -large VSD -sub-pulmonary stenosis (R outflow obstruction) -RVH (due to above) Most diagnosed antenatally or after birth

Question 2

Symptoms

Answer 2

Symptoms
-severe cyanosis -hypercyanotic spells on exercise leading to squatting (late infancy, now rare.
Rapid onset cyanosis, inconsolable cry, irritability, SOB + pallor due to tissue lactic acidosis)
-hypercyanotic spells—MI or CVA

Question 3

Signs

Answer 3

clubbing

-Harsh ESM L sternal edge from day 1 + A2 (pulmonary stenosis)

Question 4

CXR

Answer 4

-Usually normal
-older child- small heart with up tilting apex due to RVH. Pulmonary artery ‘bay’- concavity of L heart border, dec pulmonary vascular marking and blood flow.
Boot Heart

Question 5

ECG

Answer 5

Normal at birth Later-RVH

Question 6

ECHO

Answer 6

Show key features but catheterisation maybe need for detailed anatomy

Question 7

Mx

Answer 7

Initial management is medical
-Bicarbonate -Morphine
-Oxygen
Corrective surgery at 6m -close VSD
-relieve RV outflow
Cyanotic neonates require shunt to increase pulmonary blood flow