Fallots Tetralogy

Question 1

What is Fallots Tetralogy?

Answer 1

a congenital heart defect which involve 4 abnormalities of the heart:

• ventricular septal defect (VSD)- hole in the heart
• pulmonary stenosis- a narrowing of the pulmonary valve
• overriding aorta- aorta position is over the right as well as the left ventricle
• right ventricular hypertrophy- more muscular right ventricle due to high pressure

Question 2

What are the signs and symptoms?

Answer 2

From birth:

• low blood oxygen saturation
• cyanosis
• difficulty feeding
• failure to gain weight
• retarded growth and physical development
• dyspnoea (breathlessness) on exertion
• heart murmur
• finger and toe clubbing
• polycythemia (large amount of RBC)
• “Tet spells”

Question 3

What are “Tet spells”?

Answer 3

a sudden, rapid marked increase in cyanosis (caused by drop in blood oxygen) followed by syncope, it may result in hypoxic brain injury and death.
Older children often squat during a “tet spell” which increases vascular resistance and allows for a temporary reversal of the shuntt

Question 4

What are the abnormal test results?

Answer 4

• abnormal anatomy of the heart demonstrated on an ultrasound, also a shunt of blood passing initially left to right then right to left layer (this is eisenmenger complex)
• abnormal “couer-en-sabot” (boot like) appearance of the heart on a chest x-ray

Question 5

What is the medical/surgical intervention?

Answer 5

• oxygen
• surgery for early management of a baby which many include forming an anastomosis between the subclavian artery and pulmonary artery
• curative surgery to relieve the right ventricular outflow tract stenosis by careful removal of muscle and repair of the VSD. However patients still remain at increased risk of sudden cardiac death and heart failure.

Question 6

How does oxygen help treat “tet spells”?

Answer 6

it is a potent pulmonary vasodilater and systemic vasoconstricter. this allows more blood flow to the lungs