Tetralogy of

Fallot
 What is Tetralogy of Fallot?

It’s a congenital heart defect that is characterized by

FOUR structural defects of the heart.
 It’s the most common complex congenital heart defect.

 It occurs when the baby is developing in the womb.

 According to the CDC.gov, 1 in 2518 babies born in the US will have

TOF.

 It’s a cyanotic heart defect. This means the patient will have cyanosis
due to a decrease in pulmonary blood flow.
 When studying pediatric congenital heart defects, always
let the name of the defect help you.

 With TOF, there are FOUR structural problems that you

need to commit to memory.

 The word tetra or tetrad (tetralogy) means it’s a condition

that consists of FOUR problems.
What are the Heart Defects in
Tetralogy of Fallot?
Remember the mnemonic:

“RAPS”
Right ventricular hypertrophy

Aorta displacement

Pulmonary stenosis

Septal defect (ventricular)

To understand TETRALOGY OF FALLOT, let’s talk
about NORMAL blood flow because then the
defects make complete sense.
 It starts on the RIGHT SIDE

(goal of the right side is to get the UNOXYGENATED blood to the LUNGS)
1.The un-oxygenated blood
 (this is blood that has been “used-up” by your body that
needs to be resupplied with oxygen) enters to the heart
through the SUPERIOR AND INFERIOR VENA CAVA.

2. Blood enters the RIGHT ATRIUM

3. Then it is squeezed through the TRICUSPID VALVE

4. Blood then enters into the RIGHT VENTRICLE

5. Then it is squeezed into the PULMONIC VALVE

6. Blood is then shot-up through the PULMONARY ARTERY and then

enters the lungs for some oxygen
 Left Side of the Heart
(Goal of this side is to get the richly, oxygenated blood to the body to feed the
brain, tissue, muscles, organs etc.)
7. Blood enters from the lungs through the PULMONARY VEIN that
has just been refreshed with oxygen to go

8. into the LEFT ATRIUM

9. Down through the BICUSPID VALVE (also called
mitral valve)

10. Then blood is squeezed into the LEFT VENTRICLE

11. Up through the AORTIC VALVE

12. Lastly up through the AORTA, where it

pumped throughout the body
 Tetralogy of Fallot Blood Flow:

Let’s start on the right side:

o As blood enters in from the superior and inferior vena cava it goes into the
right atrium, down through the tricuspid, and then the left ventricular

But we have a problem at this point, and it’s called a

VENTRICULAR SEPTAL DEFECT (VSD).
This is a fancy word that means there is a hole in the ventricular wall.
Normally this wall separates the RIGHT and LEFT ventricle,
but in TOF the blood is mixing….

this is a problem because we will have

 Exhausted, unoxygenated blood mixing with

fresh oxygenated blood.
Unfortunately, the problem doesn’t stop here.

 The blood that is in the right ventricle has a strong desire to

reach the lungs to get oxygenated.

• To do this, it must go up through the pulmonic valve

and pulmonary artery.
In TOF

 there is NARROWING of the pulmonary valve and

artery (proper term STENOSIS).
 Therefore, blood can NOT easily go up through the pulmonary
artery to go to the lungs to get oxygenated.
 Now depending on the severity of the stenosis or
obstruction (if very severe),

 it will cause a right to left shunt of blood which

causes even more unoxygenated blood to enter
back into the system
SO, THE RIGHT VENTRICLE HAS TO WORK HARDER (when
you overwork a muscle it enlarges).

 Therefore, right ventricle hypertrophy occurs

(another way of saying enlargement of the right
ventricle), which can lead to heart failure in the long
run.
 Then on the left side of the heart, the aortic valve is enlarged, and this causes the
aorta to be displaced.

 Normally, the aortic valve just connects to the left ventricle, but now it is
connecting to the septal defect and is collecting all the blood that is mixing
at the ventricular septal defect.

 This will cause poorly oxygenated blood to go throughout the body.

 VENTRICULAR SEPTAL DEFECT (VSD)

 NARROWING of the pulmonary valve and artery

(proper term STENOSIS)

 right ventricle hypertrophy

 Displacement of the aorta

Signs and Symptoms
of
Tetralogy of Fallot

“Afflict”
Activity (feeding, crying, playing)

 Causes Cyanosis “TET spells”…

• Cyanosis
• Shortness of breath increased breathing
• Fainting…think “activity intolerance”
 Nurse’s role with a “Tet spell”:

 knee-to-chest position
 calm infant
 and apply oxygen
 Patient may need medication to calm or
 to increase systemic vascular resistance (IV fluids etc.)
Fingernail changes (clubbing due to hypoxia) noticeable at about 6 months
Fatigues or faints easily (especially during a “tet spell”….due to low oxygen
levels)

Lift knees-to-chest position or squatting (children)

 WHY? squatting and the knee-to-chest position increases systemic
vascular resistance,

• which will decrease the right to left shunting in the ventricles

and helps increase oxygen levels
Inability to grow properly due to hypoxemia
Cardiac: HARSH systolic murmur:
 WHY and WHERE? Due to pulmonary stenosis and is found left
of the sternal border at the 2nd intercostal space

• (where you would find the pulmonic valve) and a systolic

thrill may be present
Trouble feeding and thriving
 (not gaining weight, small for age, doesn’t
achieve developmental milestones etc.)…
think “failure to thrive”
Treatment for TOF
This heart defect will require surgery, which can be
temporary (a.k.a. palliative) or complete repair.
 Temporary surgery is performed to help with cyanosis and improve blood
flow.

 It can include shunt placement to shunt oxygenated blood through the heart,
so oxygen levels are increased

 or stent placement to open up the stenosis.

 In addition, complete repair can be performed when the child is older enough
to tolerate surgery
 (about 6-12 months of age).
 This includes placing a patch over the septal wall defect to prevent blood mixing
in the ventricles.
If a child has a severe case of TOF, where there is a severe
defect to the pulmonary valve (blood won’t be able to get to
the lungs) and the newborn is very cyanotic at birth,

 a temporary surgery may be performed

 and a prostaglandin infusion started.
Aprostadil (prostaglandin E)

 will keep the ductus arteriosus open (this normally

closes shortly after birth) to allow more blood to flow to
the lungs for oxygenation.
References:

Congenital Heart Defects | National Heart, Lung, and Blood Institute (NHLBI). (2018).
Retrieved from https://www.nhlbi.nih.gov/health-topics/congenital-heart-defects

Tetralogy of Fallot Facts | Congenital Heart Defects | NCBDDD | CDC. Retrieved from
https://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html
Thank you!