TETRALOGY

OF FALLOT
Arianna JasmineMabunga
BSN-2B
What is Tetralogy of Fallot?
Tetralogy of Fallot or TOF is a birth defect that affects normal blood flow
through the heart. It happens when a baby’s heart does not form correctly as
the baby grows and develops in the mother’s womb during pregnancy. It is
made up of the following four defects of the heart and its blood vessels.
Four defects of the heart
◦ Pulmonary valve stenosis. Pulmonary valve stenosis is a narrowing of the
pulmonary valve. Narrowing (constriction) of the pulmonary valve reduces
blood flow to the lungs. The narrowing might also affect the muscle beneath
the pulmonary valve. In some severe cases, the pulmonary valve doesn't form
properly (pulmonary atresia) and causes reduced blood flow to the lungs.
◦ Ventricular septal defect. A ventricular septal defect is a hole (defect) in the
wall (septum) that separates the two lower chambers of the heart. The hole
allows deoxygenated blood in the right ventricle to flow into the left ventricle
and mix with oxygenated blood fresh from the lungs. Blood from the left
ventricle also flows back to the right ventricle in an inefficient manner. This
ability for blood to flow through the ventricular septal defect reduces the
supply of oxygenated blood to the body and eventually can weaken the heart.
Four defects of the heart
◦ Overriding aorta. Normally the aorta branches off the left ventricle. In tetralogy
of Fallot, the aorta is shifted slightly to the right and lies directly above the
ventricular septal defect. The aorta receives blood from both the right and left
ventricles, mixing the oxygen-poor blood from the right ventricle with the
oxygen-rich blood from the left ventricle.
◦ Right ventricular hypertrophy. When the heart's pumping action is
overworked, it causes the muscular wall of the right ventricle to thicken. Over
time this might cause the heart to stiffen, become weak and eventually fail. This
heart defect can cause oxygen in the blood that flows to the rest of the body to be
reduced. Infants with tetralogy of Fallot can have a bluish-looking skin
color―called cyanosis―because their blood doesn’t carry enough oxygen. At
birth, infants might not have blue-looking skin, but later might develop sudden
episodes of bluish skin during crying or feeding. These episodes are called tet
spells.
Etiology
◦ The exact cause of tetralogy of Fallot is not known.
◦ Some studies suggest that the disorder may be due to the interaction of
several genetic and/or environmental factors (multifactorial). Therefore,
researchers suspect that something may affect the genes in the developing
fetus, causing this birth defect, but the exact nature of this trigger is not
known.
◦ Some conditions that may increase the risk of having a child with tetralogy of
Fallot are viral illnesses, alcohol use, diabetes, poor nutrition, and being
pregnant over the age of 40.
Symptomatology:
◦ Tetralogy of Fallot symptoms vary, depending on the extent of obstruction of
blood flow out of the right ventricle and into the lungs. Signs and symptoms
may include:
◦ A bluish coloration of the skin caused by blood low in oxygen (cyanosis)
◦ Shortness of breath and rapid breathing, especially during feeding or exercise
◦ Loss of consciousness (fainting)
◦ Clubbing of fingers and toes — an abnormal, rounded shape of the nail bed
◦ Poor weight gain
◦ Tiring easily during play or exercise
◦ Irritability
◦ Prolonged crying
◦ A heart murmur
Symptomatology:
Tet spells
◦ Sometimes, babies who have tetralogy of Fallot will suddenly develop deep
blue skin, nails and lips after crying or feeding, or when agitated.
◦ These episodes are called tet spells and are caused by a rapid drop in the
amount of oxygen in the blood. Tet spells are most common in young
infants, around 2 to 4 months old. Toddlers or older children might
instinctively squat when they're short of breath. Squatting increases blood
 GENERAL
PATHOPHYSIOLOG
Y
To better explain the pathophysiology of TOF, we should explore
the 4 lesions in greater depth:
Ventricular Septal Defect
◦ VSDs are categorised according to their location on the ventricular septum.
The smaller membranous septum is located on the superior aspect whilst the larger
muscular septum is on the inferior aspect.
◦ When the VSD involves parts of the membranous and muscular septum, this is called
a perimembranous VSD – this is the commonest type associated with TOF. Other
VSDs associated with TOF are muscular VSDs and doubly committed
VSDs (located near both pulmonary and aortic valves)
◦ The VSD is normally of a significant size, which causes the systolic pressures
between the ventricles to equalise. In mild TOF, the left ventricular pressures remain
higher than the right ventricle, thus blood shunts from left-to-right through the VSD.
These patients are normally acyanotic.
◦ In more severe disease, due to increased right ventricular pressure (secondary to
PS – see below) the shunt direction reverses from right-to-left allowing mixing of
deoxygenated (“blue”) and oxygenated (“red”) blood. This results in lower
oxygenated (“purple”) blood in the systemic circulation, hence patients are cyanotic.
Pulmonary Valve Stenosis
◦ Pulmonary stenosis can be classified according to its location. The
commonest site is the infundibular septum (50%). Some authors use the more
descriptive term right ventricular outflow tract (“RVOT”) but they
essentially refer to the same thing. The stenosis may also be valvular (10%)
or a combination of both (30%).
◦ This results in impaired flow of deoxygenated blood into the main
pulmonary artery. It may be severe enough to cause intermittent RVOT
obstruction (“RVOTO”) – this forms the basis of hypoxic episodes,
commonly known as tet spells.
Right Ventricular
Hypertrophy
◦ Hypertrophy of the right ventricle occurs in response to the high pressures it
must overcome to pump deoxygenated blood through the RVOTO. This
usually develops in utero and may be seen in chest x-rays as the ‘boot’ sign.
Overriding Aorta
◦ Compared to the normal heart, the aorta in TOF is dilated and displaced over
the intraventricular septum. Aortic dilatation is caused by an increase in blood
flow through the aorta as it receives blood from both ventricles via the VSD.
◦ In severe TOF, multiple aorto-pulmonary collateral arteries (“MAPCAs”)
may also form to help increase pulmonary blood flow.
 MEDICAL
MANAGEMEN
T
Lab Tests:
◦ During pregnancy, there are screening tests (also called prenatal tests) to
check for birth defects and other conditions. Tetralogy of Fallot might be
seen during an ultrasound (which creates pictures of the body).
◦ Physical exam may make the health care provider think a baby may have
tetralogy of Fallot, including bluish-looking skin or a heart murmur (a
“whooshing” sound caused by blood not flowing properly through the heart).
However, it is not uncommon for a heart murmur to be absent right at birth.
Lab Tests:
◦ Echocardiography. Echocardiograms use high-pitched sound waves to produce
an image of the heart. Sound waves bounce off the heart and produce moving
images that can be viewed on a video screen.
◦ This test is generally used to diagnose tetralogy of Fallot. It allows your or your
baby's doctor to determine if there is a ventricular septal defect and where it's
located, if the structure of the pulmonary valve and pulmonary artery is normal, if
the right ventricle is functioning properly, if the aorta is positioned properly, and if
there are any other heart defects. This test can also help your or your baby's doctor
to plan treatment for the condition.
Lab Tests:
◦ Electrocardiogram. An electrocardiogram records the electrical activity in the
heart each time it contracts. During this procedure, patches with wires
(electrodes) are placed on your or your baby's chest, wrists and ankles. The
electrodes measure electrical activity, which is recorded on paper.
◦ This test helps determine if the right ventricle is enlarged (right ventricular
hypertrophy), if the right atrium is enlarged and if the heart rhythm is regular.
◦ Chest X-ray. A chest X-ray can show the structure of the heart and lungs. A common sign
of tetralogy of Fallot on an X-ray is a "boot-shaped" heart, because the right ventricle is
enlarged.
Lab Tests:
◦ Oxygen level measurement (pulse oximetry). This test uses a small sensor that
can be placed on a finger or toe to measure the amount of oxygen in the blood.
◦ Cardiac catheterization. Doctors may use this test to evaluate the structure of
the heart and plan surgical treatment. During this procedure, your or your baby's
doctor inserts a thin, flexible tube (catheter) into an artery or vein in the arm,
groin or neck and threads it up to the heart.
◦ Your or your baby's doctor injects a dye through the catheter to make the heart
structures visible on X-ray pictures. Cardiac catheterization also measures
pressure and oxygen levels in the chambers of the heart and in the blood vessels.
Medications:
◦ Heart medications – Digitalis or Amiodarone - may be prescribed to help control
irregular heartbeats (arrhythmias).
◦ Administration of supplemental oxygen, morphine, and/or other drugs that
improve oxygen concentration- Episodes of severe symptoms or “blue spells”
(hypoxia).
◦ Sodium bicarbonate may be administered to lower abnormally high levels of
acid in the blood (acidosis).
Medications:
◦ Propranolol may be given to help prevent future tet spells and to reduce their
severity.
◦ Diuretics- Furosemide, Bumetanide, and Ethacrynic acid- are drugs that
help to remove excess fluid from the body.
◦ Antibiotics – Amoxicillin- may be prescribed to infants with tetralogy of
Fallot to help prevent infections (prophylaxis) because children with this
disorder are susceptible to bacterial infection of the heart (endocarditis)
Treatment / Surgery
Prior to surgery
◦ Treatment to control symptoms (palliative) may include the maintenance of
adequate fluid intake (hydration), monitoring of hemoglobin levels in the
blood, and the avoidance of strenuous exercise., rapid heartbeats, and/or heart
failure.
◦ The knee-chest position may also bring some symptomatic relief. dietary salt
restriction, and bed rest may be effective in treating congestive heart failure
◦ Dietary salt restriction, and bed rest may be effective in treating congestive
heart failure.
Treatment / Surgery
Temporary Surgery
◦ It is recommended babies are too weak or small to have the full repair
surgery; the full repair surgery will be performed when the baby is stronger. A
tube or a “shunt” is placed between a large artery branching off the aorta and
the pulmonary artery. It creates a pathway for blood to travel to the lungs to
get oxygen. This is not open-heart surgery and doesn't fix the inside of the
heart. The shunt is usually a small tube of synthetic material sewn between a
body artery (or the aorta) and the pulmonary artery. The tube is removed
during the full repair surgery.
Treatment / Surgery
Complete Repair
◦ The surgeon closes the ventricular septal defect with a patch and opens the
right ventricular outflow tract by removing some thickened muscle below the
pulmonary valve, repairing or removing the obstructed pulmonary valve and,
if needed, enlarging the branch pulmonary arteries that go to each lung.
◦ Sometimes a tube is placed between the right ventricle and the pulmonary
artery. This is sometimes called a Rastelli repair. It's similar to the type of
repair used for some other heart defects.
 NURSING
DIAGNOSIS
Diagnosis:
Decreased Cardiac Output related to Structural factors of congenital heart
defect as evidenced by Cyanosis.
Interventions:
1. Assess heart rate and blood pressure. Rationale: Most patients have
compensatory tachycardia and significantly low blood pressure in response to
reduced cardiac output.
2. Note skin color, temperature, and moisture. Rationale: Cold, clammy, and
pale skin is secondary to a compensatory increase in sympathetic 
nervous system stimulation and low cardiac output and oxygen desaturation.
3. Monitor electrocardiogram (ECG) for rate, rhythm, and ectopy. Rationale:
Cardiac dysrhythmias may occur from low perfusion, acidosis, or hypoxia.
Tachycardia, bradycardia, and ectopic beats can further compromise cardiac
output. Older patients are especially sensitive to the loss of atrial kick in atrial
fibrillation.
Diagnosis:
Activity Intolerance related to Imbalance between oxygen supply and
demand as evidenced by Abnormal heart rate or blood pressure response to
activity
Interventions:
1. Assess level of fatigue, ability to perform ADL and other activities in
relation to severity of the condition. Rationale: Provides information
about energy reserves and response to activity.
2. Assess dyspnea on exertion, skin color changes during rest and when
active. Rationale: Indicates hypoxia and increased oxygen need during
energy expenditure.
3. Allow for rest periods between care;  disturb only when necessary for care
and procedures. Rationale: Promotes rest and conserves energy.
Diagnosis:
Compromised Family Coping related to Situational and developmental crises of
family and child as evidenced by Family expresses concern and fear about
infant/child’s disease and condition.
Interventions:
1. Encourage expression of feelings and provide factual information about
infant/child. Rationale: Reduces anxiety and enhances family’s understanding
of the condition.
2. Clarify any misinformation and answer questions regarding disease process.
Rationale: Prevents unnecessary anxiety resulting from inaccurate knowledge
or beliefs.
3. Encourage parents to include ill infant/ child in family activities rather than
family revolving around needs of infant/child. Rationale: Promotes normal
growth and development of family and infant/child.
Diagnosis:
Risk for Injury related to Cardiac function compromised by congenital defects
and medication administration.
Interventions:
1. Assess for risk of drug toxicity, a cardiac complication of heart failure.
Rationale: Early identification of signs and symptoms of complications
allows preventive measures and adjustments to be made.
2. Monitor orders for diagnostic tests and procedures. Rationale: Allows for
preparation and support of parents and infant/child.
3. Prepare parents and child (use play doll) for diagnostic procedures and/or
surgery; should be extensive, consistent, and comprehensive, including a
surgical procedure to be performed and expected results, prognosis and
whether corrective, palliative, temporary, or permanent. Rationale: Assists
in allaying anxiety and understanding that diagnostic tests are usually done
before surgery.
Diagnosis:
Risk for Infection related to Chronic illness; Tetralogy of Fallot
Interventions:
1. Assess temperature, IV site if present,  increased WBC, increased pulse and
respirations (specify when). Rationale: Provides information
indicating potential infection.
2. Avoid allowing those with infections to have contact with infant/ child.
Rationale: Prevents transmission of infectious agents to infant/child with
compromised defense.
3. Provide adequate rest and nutritional needs for age. Rationale: Protects
against potential infection by increasing body resistance and defenses.
PROGNOSIS
◦ After successful surgery, children generally don't have any symptoms and lead
normal lives with few, if any, restrictions. However, the surgery itself may have
some long-term complications. These include the following:
◦ Right ventricular failure: Right ventricular failure is possible, especially if surgery
created severe pulmonary valve insufficiency, which is regurgitation of blood
backwards from the pulmonary artery into the right ventricle.
◦ Electrical conduction abnormalities: Every patient with tetralogy of Fallot has
right bundle branch block secondary to the congenital ventricular septal defect.
But sewing the patch into the ventricular septum can create heart block or failure
of the upper atria to conduct/communicate with the lower ventricles. A permanent
pacemaker is occasionally needed.
◦ Arrhythmias: Because of surgery on the ventricles, postoperative 
ventricular tachycardia (VT) is an infrequent risk. This is a life-threatening 
arrhythmia, so follow-up detection of risk for ventricular tachycardia is important.
◦ Residual hole in the ventricular septum: This is also possible, with oxygenated
blood passing from the left side of the heart to the right (shunting).