Tetralogy

of
fallot
INTRODUCTION
qOne of the first types of congenital heart
defects


qInvolves four anatomical anomalies

 pulmonary stenosis
 ventricular septal defect
 overriding of aorta
 right ventricular hypertrophy


qMost common cyanotic heart defect (55-70%)

q
qDescribed in 1672 by Niels Stensen
 1773 by Edward Sandifort
 1888 by Etien-Louis Arthur Fallot

 EPIDEMIOLOGY


Occurs 400/million
live births

 Etiology

Chromosome 22
deletions
 ANATOMY
and
PHYSIOLOGY
 Fetal Circulation
The circulatory system of a
human fetus works differently
from that of born humans,
mainly because the lungs are
not in use:
the fetus obtains oxygen and 
 nutrients from the mother
through the placenta and
the umbilical cord.
Blood from the placenta is
carried to the fetus by
the umbilical vein.

About half of this enters the
fetal ductus venosus and is
carried to the inferior vena
cava, while the other half
enters the liver proper from
The ductus venosus then
merges to the inferior vena
cava, mixes with the
deoxygenated blood, and
travels to the right atrium.
In the fetus, there is an opening
between the right and left
atrium (the foramen ovale),
and most of the blood flows
through this hole directly into
the left atrium from the right
The continuation of this blood
flow is into the left ventricle,
and from there it
is pumped through
the aorta into the body.
Some of the blood moves from
the aorta through the internal
iliac arteries to the umbilical
arteries, and re-enters the
placenta, where carbon
dioxide and other waste
Some of the blood entering the
right atrium does not pass directly
to the left atrium through
the foramen ovale, but enters
the right ventricle and is pumped
into the pulmonary artery.
In the fetus, there is a special
connection between the pulmonary
artery and the aorta, called
the ductus arteriosus, which directs
most of this blood away from the
lungs (which aren't being used
PATHOPHYSIOLO
GY
FORAMEN OVALE


 Normally this opening closes in

the first three months of life. When
the lungs become functional at
birth, the pulmonary pressure
decreases and the left atrial
pressure exceeds that of the right.
This forces the septum primum
against the septum secundum,
functionally closing the foramen
ovale. In time the septa eventually
fuse, leaving a remnant of the
PULMONARY STENOSIS
narrowing of the right
ventricular outflow tract and
can occur at the pulmonary
valve(valvular stenosis) or just
below the pulmonary
valve (infundibular stenosis).
The pulmonic stenosis is the
major cause of the
malformations, with the other
associated malformations
acting as compensatory
mechanisms to the pulmonic
OVERRIDING AORTA
An aortic valve with
biventricular connection, that
is, it is situated above the
ventricular septal defect and
connected to both the right and
the left ventricle. The degree to
which the aorta is attached to
the right ventricle is referred to
as its degree of "override." right
ventricle.
VENTRICULAR SEPTAL DEFECT
A hole between the two
bottom chambers (ventricles)
of the heart. The defect is
centered around the most
superior aspect of the
ventricular septum (the outlet
septum), and in the majority
of cases is single and large. In
some cases thickening of the
septum (septal hypertrophy)
can narrow the margins of the
RIGHT VENTRICULAR
HYPERTROPHY
The right ventricle is more
muscular than normal, causing a
characteristic boot-shaped
(coeur-en-sabot) appearance as
seen by chest X-ray. Due to the
misarrangement of the external
ventricular septum, the right
ventricular wall increases in size
to deal with the increased
obstruction to the right outflow
tract. This feature is now
generally agreed to be a
  PULMONARY STENOSIS-RIGHT
VENTRICULAR HYPERTROPHY-
VENTRICULAR SEPTAL DEFECT-
OVERRIDING OF THE AORTA


 mixing of oxygenated and deoxygenated

blood in the left ventricle via the VSD 


 preferential flow of the mixed blood from

both ventricles through the aorta because
of the obstruction to flow through the
pulmonary valve

Diagnostic Tests and
Procedures
 Echocardiography
Echocardiography (echo) is a painless test that
uses sound waves to create a moving picture
of the heart. During the test, the sound waves
(called ultrasound) bounce off the structures
of the heart. A computer converts the sound
waves into pictures on a screen.


Echo is an important test for diagnosing

tetralogy of Fallot because it shows the four
heart defects and how the heart is responding
to them. This test helps the cardiologist
decide when to repair these defects and what
type of surgery is needed.
EKG
( Electrocardiogram )

An EKG is a simple, painless test that records
the heart’s electrical activity. The test shows
how fast the heart is beating and its rhythm
(steady or irregular). It also records the
strength and timing of electrical signals as
they pass through each part of the heart.

Chest X Ray
The abnormal "coeur-en-sabot" (boot-like)
appearance of a heart with tetralogy of Fallot
is easily visible via chest x-ray, and before
more sophisticated techniques became
available, this was the definitive method of
diagnosis. Congenital heart defects are now
diagnosed with echocardiography, which is
quick, involves no radiation, is very specific,
and can be done prenatally.

Pulse Oximetry

For this test, a small sensor is attached to a
finger or toe (like an adhesive bandage). The
sensor gives an estimate of how much
oxygen is in the blood.

Cardiac
Catheterization


The doctor also can use cardiac catheterization

to measure the pressure and oxygen level
inside the heart chambers and blood vessels.
This can help the doctor determine whether
blood is mixing between the two sides of the
heart.

 MEDICAL
MANAGEMENT
 GOALS OF TREATMENT


Improve the baby’s symptoms

Increase the level of oxygen in
the baby’s blood
Repair the defects



Digoxin
Indication: cardiac failure accompanied
by atrial fibrillation; management of
chronic cardiac failure where systolic
dysfunction is dominant
D: 25/35 mcg/kg
CI: intermittent complete heart block or
2nd degree AV block ; arrhythmia
caused by cardiac glycoside
intoxication; hypersensitivity to other
digitalis glycosides
SP:severe respiratory distress;hypoxia
AR: CNS disturbances, dizziness,
visual disturbances ; arrhytmia,
conduction disturbances, sinus
bradyccardia, nausea, vomiting,
diarrhea



 SURGICAL
MANAGEMENT
Corrective Surgery-Closing
the VSD
Opening and enlarging the
area that blood flows through
as it leaves the lower right
side of the heart
Opening or widening the
pulmonary valve
Temporary or Palliative Surgery -
As small opening can be made
between the ribs.
Place a tube/shunt between a
large artery branching off the
aorta and the pulmonary artery
The shunt is removed when the
baby’s heart defects are repaired
during the corrective surgery
NURSING
CARE
PLAN
CUES: CR –more than 160 bpm
DIAGNOSIS: Decreased cardiac output r/t ineffective
circulation
BACKGROUND
KNOWLEDGE
OBJECTIV INTERVENTION RATIONAL EVALUATION
E E
Tetralogy of The patient Assess and
 If the
Fallot will have record the patient
results in adequate vital signs experiences
low cardiac decreased
oxygenation output as cardiac
of blood due evidenced output, the
to mixing of by cardiac cardiac
oxygenated rate within rate,
and normal respiratory
deoxygenated range rate will
blood in the increase
left and the bp
ventricle
through the will
VSD decrease.
BACKGROUND
KNOWLEDGE
OBJECTIV INTERVENTION RATIONAL EVALUATION
E E
and Administer Cardiac
preferential cardiac drugs are
flow of both drugs as given to
oxygenated ordered increase
and the
deoxygenated strength of
blood from cardiac
the contraction
ventricles s and/or
through the increase
aorta return of
because of blood flow
obstruction
to flow to the
through the heart,
pulmonary thereby
valve. increasing
CO.
BACKGROUND
KNOWLEDGE
OBJECTIV INTERVENTION RATIONAL EVALUATION
E E
Monitor and Digoxin is
record a potent
digoxin medication
levels. that needs
Notify careful
physician monitoring.
if levels If digoxin
are out of levels are
acceptable high, the
range. patient
will
experience
s/s of
toxicity
such as
vomiting.
BACKGROUND
KNOWLEDGE
OBJECTIV INTERVENTION RATIONAL EVALUATION
E E
Keep Decreased The


output may patients

accurate indicate cardiac
record decreased rate is
of CO possibly within
due to a acceptable
intake shift of range.
and the
output intravascul
ar fluid
into the
interstitia
l space.
CUES: Abnormal heart rate/blood pressure response to
activity; exertional dyspnea
DIAGNOSIS: Activity intolerance related to imbalance oxygen
supply and demand.
BACKGROUND
KNOWLEDGE
OBJECTIV INTERVENTION RATIONALE EVALUATION
E
Because of the
shunting
The Assess Indicates
between the child dyspnea on hypoxia and
ventricles, the
mixing of the will exertion, increase
oxygenated and tolerate skin color oxygen need
unoxygenated
blood results increase changes during
to less oxygen d during energy
supplied for
the tissues. activity . rest and expenditure.
This results to
easy
when
fatigability active.
and cyanosis
whenever the
infant exerts
effort.
BACKGROUND
KNOWLEDGE
OBJECTIV INTERVENTION RATIONALE E VALUATION
E
Allow rest Promotes
periods
between rest and
cares; conserves
disturb energy.
only for
care and
necessary
procedure.
BACKGROUND
KNOWLEDGE
OBJECTIV INTERVENTION RATIONALE E VALUATION
E
Avoid Conserves
allowing energy.
infant to cry
for a long Cross-cut
period of nipple
time; use requires
soft nipple
for feeding; less energy
cross-cut for infant
nipple; if to feed.
unable for
infant to
ingest
sufficient
calories by
mouth,
gavage-feed
infant.
BACKGROUND
KNOWLEDGE
OBJECTIVE INTERVENTION RATIONALE EVALUATION

Provide Avoid
neutral extremes
environmental heat and
temperature; cold that
when bathing increases
exposed only oxygen and
area being energy
bathed and needs.
keep the
infant
covered to
prevent heat
loss.
BACKGROUND
KNOWLEDGE
OBJECTIVE INTERVENTION RATIONAL EVALUATIO N
E
Explain to Avoids The
patient ’ s
parents need
to conserve fatigue activity
level is
energy and optimal
encourage rest. within
the
Assist Provides limitatio
parents to rest and ns of the
avoids over disease .
plan for exertion,
care and minimizes
rest periods. energy
expenditure.