Tetralogy of Fallot (ToF)

Question 1

Main features of ToF

Answer 1

1. Ventricular septal defect (VSD)
2. Pulmonary stenosis
3. Right ventricular hypertrophy
4. Overriding aorta

Question 1

What is a ventricular septal defect (VSD)?

Answer 1

a hole between the left and right ventricles

Question 2

What is pulmonary stenosis?

Answer 2

Narrowing of the pulmonary artery

Question 3

What is right ventricular hypertrophy?

Answer 3

enlargement of the right ventricle

Question 4

What is the overriding aorta?

Answer 4

the aorta is positioned above both ventricles

Question 5

What causes a boot-shaped heart to appear?

Answer 5

Caused by right ventricular hypertrophy due to the heart working harder to pump blood through the narrowed pulmonary artery

Question 6

Symptoms of ToF

Answer 6

Cyanosis (blue skin), difficulty feeding, failure to thrive

Question 7

Treatment of ToF

Answer 7

Surgical correction is usually in early childhood

Question 8

Family role in ToF

Answer 8

1. Monitor for signs of hypoxia
   = blue lips, nails
2. Post-surgery care and support
3. Emotional and financial support for the family.

Question 9

How to diagnose ToF

Answer 9

Echocardiogram, chest X-ray (boot-shaped heart)

Question 10

Most common rhythm disturbance in children is what bitch?

Answer 10

Supraventricular Tachycardia

Question 11

Which of the following is not a feature of tetralogy of fallot?

A. Overriding Aortic Arch
B. Pulmonary Stenosis
C. Ventricular Septal defect
D. Right ventricular hypertrophy
E. Mitral regurgitation

Answer 11

E

Question 12

A 6-month-old female infant is being assessed by the Paediatric Cardiology Team. She was delivered by elective caesarean section without any complications. She cried immediately after birth, and her APGAR score was normal.

She was noted to have an ejection systolic murmur at the upper sternal border on the left side at birth. Over the next couple of months, her mother reports that she is gasping for breath, turning blue, fainting immediately after taking a feed and regaining normal colour and becoming alert only after she is picked up.
An ECG shows right-axis deviation and right ventricular hypertrophy. A subsequent chest X-ray shows a small heart with a concave main pulmonary artery segment

Given the clinical history and findings, which is the most likely diagnosis?

Answer 12

Tetralogy of Fallot

Question 13

You are a medical student sitting in on a paediatric cardiology clinic.
During a break between patients the consultant is giving some teaching on Tetralogy of Fallot and asks which anatomical anomaly determines the extent of cyanosis in Tetralogy of Fallot (TOF)?

Answer 13

Pulmonary stenosis