OVERVIEW OF THE DISEASE

INTRODUCTION
Tetralogy of Fallot is a rare condition caused by a combination of four heart
defects that are present at birth (congenital).These defects, which affect the structure
of the heart, cause oxygen-poor blood to flow out of the heart and to the rest of the
body. Infants and children with tetralogy of Fallot usually have blue-tinged skin
because their blood doesn't carry enough oxygen.Tetralogy of Fallot is often
diagnosed during infancy or soon after. However, tetralogy of Fallot might not be
detected until later in life in some adults, depending on the severity of the defects and
symptoms.

CAUSES
Tetralogy of Fallot occurs during fetal growth, when the baby's heart is
developing. While factors such as poor maternal nutrition, viral illness or genetic
disorders might increase the risk of this condition, in most cases the cause of
tetralogy of Fallot is unknown.
The four abnormalities that make up the tetralogy of Fallot include:
1.)Pulmonary valve stenosis. Pulmonary valve stenosis is a narrowing of the
pulmonary valve — the valve that separates the lower right chamber of the heart
(right ventricle) from the main blood vessel leading to the lungs (pulmonary
artery).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.
2.)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 left and right
ventricles. The hole allows deoxygenated blood in the right ventricle — blood that
has circulated through the body and is returning to the lungs to replenish its oxygen
supply — 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.
3.)Overriding aorta. Normally the aorta — the main artery leading out to the
body — 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.In this
position 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.
4.)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.Some children
or adults who have tetralogy of Fallot may have other heart defects, such as a hole
between the heart's upper chambers (atrial septal defect), a right aortic arch or
abnormalities of the coronary arteries.

RISK FACTORS
While the exact cause of tetralogy of Fallot is unknown, various factors might
increase the risk of a baby being born with this condition. These risk factors include:
 A viral illness during pregnancy, such as rubella (German measles)
Alcoholism during pregnancy
Poor nutrition during pregnancy
A mother older than age 40
A parent who has tetralogy of Fallot
The presence of Down syndrome or DiGeorge syndrome.

SYMPTOMS
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

COMPLICATIONS
All babies who have tetralogy of Fallot need corrective surgery. Without
treatment, your baby might not grow and develop properly. Your baby may also be at
an increased risk of serious complications, such as infective endocarditis — an
infection of the inner lining of the heart or heart valve caused by a bacterial
infection.Untreated cases of tetralogy of Fallot usually develop severe complications
over time, which might result in death or disability by early adulthood.
Tests that can aid in the diagnosis of TOF are:
a chest X-ray to check for structural abnormalities.
an echocardiogram to check for disruptions in heartbeats.
a heart MRI to check for structural problems.
a pulse oximetry test to measure the oxygen level in the blood.
a cardiac catheterization.

MEDICAL INTERVENTION/ MANAGEMENT INCLUDING MEDICATIONS

Tetralogy of Fallot must be repaired with open-heart surgery, either soon after
birth or later in infancy. The goal of surgery is to repair the four defects of tetralogy
of Fallot so the heart can work as normally as possible. Repairing the defects can
greatly improve a child's health and quality of life
Tetralogy of Fallot With Pulmonary Stenosis Medication: Vasodilators, Diuretics,
Alpha1 Agonists, Beta-Blockers, Beta-1 Selective.

NURSING INTERVENTION/ MANAGEMENT

In the preoperative period, we focus on preoperative examinations, monitoring,
fasting and premedication. In the postoperative period, intensive postoperative care
and education about post-operative life.
PREVENTION
What about preventing endocarditis? Children with tetralogy of Fallot are at
increased risk for endocarditis. Some children, including those have had a valve
replacement, still have a shunt or have leaks around surgical patches, and need to take
antibiotics before certain dental procedures to help prevent endocarditis.
 ANATOMY AND PHYSIOLOGY
OVERRIDING AORTA
The aorta is between the left and right ventricles, directly over the VSD. As a
result, oxygen-poor blood from the right ventricle flows directly into the aorta instead
of into the pulmonary artery to the lungs.

PULMONIC STENOSIS
A narrowing or thickening of the valve that connects the right ventricle to the
pulmonary artery, the blood vessels that carries low-oxygen blood from heart to the
lungs.

VENTRICULAR SEPTAL DEFECT

A hole in the septum, or wall, separating the two lower chambers, or ventricles
of the heart. The septum normally acts as a barrier that prevents blood from both side
of the heart from mixing.

RIGHT VENTRICULAR HYPERTROPHY

Also called right ventricular enlargement. Happens when the muscle on the right
side of your heart becomes thickened and enlarged.
 PATHOPHYSIOLOGY
IF TREATED IF NOT TREATEDE

PREDISPOSING FACTOR: PRECIPITATING FACTORS:

-GENETICS -ENVIRONMENTAL
 DE GEORGE SYNDROME -STRESS
-AGE= AT BIRTH -RUBELLA ALCOHOLISM
TRANSIENT INCREASE IN
DIAGNOSTIC EXAM:
RESISTANCE TO BLOOD
-CBC
FLOW F THE LUNGS
-2D ECHO
-ABG

TREATMENT ALTERATION IN FETAL DEVELOPMENT

-MEDICATIONS -UNEQUAL GROWTH OF THE
-02 THERAPHY AORTICOPULMONARY SEPTUM
-SURGERY

AORTA TOO LARGE C. OVERRIDING AORTA

STEALS SPACE
FROMPULMONARY B. PULMONARY STENOSIS
ARTERY

PREVENTS VENTRICULAR A. VENTRICULAR SEPTAL

WALL CLOSURE DEFECT

RIGHT VENTRICULAR
OUTFLOW OBSTRUCTION

MIXING OXYGENATED
INCREASE VENTRICULAR
AND DEOXYGENATED
CONTRACTION
BLOOD

DECREASE O2 SUPPLY TO D.RIGHT VENTRICULAR

SYSTEMIC CIRCULATION HYPERTROPHY

-CYANOSIS
-TET SPELLS
HYPOXIA -DYSPNEA ON EXERTION
-CLUBBING OF FINGERS AND
TOES
-HEART MURMURS
-LOW BIRTH WEIGHT
-POOR FEEDING
-GROWTH AND DEVELOPMENT
MAY BE DELAYED
-POLYCYTHEMIA
 WORSEN TET SPELLS

DECREASE ORGAN
PARTIALLY IMPROVED PERFUSION INCLUDING
OXYGENATION BRAIN

-HYPOXIC BRAIN INJURY

MAY IMPROVED LENGTH -ORGAN FAILURE
AND QUALITY OF LIFE

NURSING CARE PLAN DEATH

Assessmen Diagnosis Planning Implementation Rationale Evaluation
t
-Cyanosis Risk for After 4 hours  Assess and  If the patient Baby's
-dyspnea Decreased of nursing record the experience cardiac condition
-delay in cardiac intervention vital sign. output the cardiac was
growth and output the patient,  Administer and respiratory improved.
developme related to will have cardiac drugs rate will increase
-nt structural adequate as ordered. and Blood
-blue abnormalit cardiac output  Assess pressure will
anoxia ies of the as evidenced dypsnea, decrease.
attacks heart by cardiac rate exertion skin   Cardiac
within normal color during drugs are given to
range rest and when increase the
active. strength of cardiac
 Avoid contractions.
allowing the  Indicates
infant to cry hypoxia and
for a long increase oxygen
period of need.
time,  Conserves
  Use soft energy,cross cut
nipple when nipple requires
feeding less energy for
infant to feed.

Assessment Diagnosis Planning Implementation Rationale Evaluation

Objectives: Ineffective  Monitor  Monitored  Cool, The
 bluish tissue skin color skin color blanched, mottled condition of
discolor perfusion and temp and temp skin and cyanosis the baby
ation of related to every 2 every 2 may indicate tissue was
lips. decrease hours. hours. perfusion. improved.
 Clubbin oxygen Assess for Assessed  Decreased
g of cellular signs of for signs of heart rate and
finger. exchange skin skin blood pressure
 Nasal secondary breakdow breakdown. may indicate
flaring to n.  Monitored increase
use of congenital  Monitor VS of arteriovenous
accessor heart VS of patient exchanged, which
y disease t/c patient every 2 leads to decrease
muscles. teralogy every 2 hours. tissue perfusion.
 With of fallot hours.  Kept  Warmth aids
capillar  Keep patient vasodilation which
y refill patient warm. improve tissue
time of warm.  Elevated perfusion.
3  Elevate lower  To increase
seconds. lower extremeties. arterial blood
 Weak in extremetie  Changed supply and
appeara s position improve tissue
nce.  Change regularly perfusion.
position and  To avoid
regularly inspected decrease in tissue
and skin every perfusionand risk
inspect shift. for skin
 skin every breakdown.
shift.

Assessmen Diagnosis Planning Implementation Rationale Evaluation

t
Objective: Impaired  Esta  Establishe  To gain The condition
 V/S: gas blish rapport. d rapport. trust. of the baby
BP- exchanged  Mon  Monitored  Indicators was
80/50 related to itor respiratory rate, of adequacy of improved.
PR- altered respiratory use of accessory respiratory function
124bp oxygen rate, use of muscles, areas of or degree of
m RR- supply as accessory cyanosis. compromise and
28cp evidenced muscles,  Auscultate therapy needs.
m T- by dyspnea, areas of d breathe sounds.  Developme
37.1 tachypnea, cyanosis.  Monitored nt of atelectasis and
 With tachycardia  Ausc VS. stasis of secretion
O2 and fatigue ultate breathe  Helped can impair gas
inhala secondary sounds. with breathing exchange.
tion to  Mon exercise.  Can affect
@ congenital itor VS.  Elevated the gas exchange.
2lpm heart  Help head of bed to  Helps
via disease with moderate or high improve oxygen
nasal breathing back rest. inspiration in the
canula exercise. lungs.
as  Elev  Helps the
ordere ate head of lungs expand.
d. bed to
 Circu moderate or
moral high back
cyano rest.
sis
noted.
 DRUG STUDY
Name of Mechanism of Indication Contraindicatio Side/ adverse effects Nursing
drug action n respons
Generic Cephalosporins Susceptible mild Cefuroxime  Diarrhea,  Mo
name: exert to moderate (cefuroxime  Dizziness, hep
Cefuroxime bactericidal infections (cefuroxime  Headache, hem
sodium activity by including injection)  Drowsiness, fun
Brand interfering with pharyngitis/tonsill injection) for  itching/swelling, per
name: bacterial cell itis, acute Injection USP  Rash, wit
Zinacef wall synthesis maxillary and Dextrose  Nausea, pro
Classificati and inhibiting sinusitis, chronic Injection USP is  vomiting, the
on: cross-linking of bronchitis, acute contraindicated  abdominal pain,  Mo
is in a class the otitis media, in patients with  stomach upset, pro
of peptidoglycan. uncomplicated known allergy  Gas, tim
medications The skin and skin to the  Headache, pat
called cephalosporins structure, UTIs, cephalosporin  itching or rash, risk
cephalospor are also thought gonorrhea, early group of  Hives, pro
in to play a role in Lyme disease. antibiotics.  Cough, du
antibiotics. the activation Solutions  stuffy nose, cep
It works by of bacterical containing  stiff or tight the
stopping cell autolysins dextrose may be muscles, (nu
the growth which may contraindicated  muscle pain, def
of bacteria. contribute to in patients with  joint pain or pro
bacterial cell hypersensitivity swelling, tre
lysis. to corn products  Restlessness, ren
 Irritability, hep
 hyperactivity, dis
 vaginal itching  Ob
or discharge sig
 unusual or sym
unpleasant taste ana
in the mouth , du
 white patches or do
sores in the
 mouth, and
 diaper rash (in
infants taking
liquid
cefuroxime).

Name of drug Mechanism Indication Contraindicati Side/ adverse Nursing

of action on effects responsibilities
Generic name: Diazepam is s used to atients with  drowsiness Assess blood
Diazepam a treat severe pressure, pulse
Brand name: benzodiazepi anxiety, respiratory  tiredness or and respiration if
Valium ne that exerts alcohol insufficiency, fatigue IV administration.
Classification: anxiolytic, withdraw myasthenia - Provide frequent
belongs to a sedative, al, and gravis, sleep  muscle sips of water for
class of drugs musclerelaxa seizures. apnea weakness dry mouth. -
known as nt, It is also syndrome, and Provide fluids and
 inability to
benzodiazepin anticonvulsan used to severe hepatic fibre for
es. t and relieve insufficiency. control constipation. -
amnestic muscle It is muscle Evaluate
effects. Most spasms permissible in therapeutic
of these and to patients with movements response, mental
effects are provide open-angle (ataxia) state and physical
thought to sedation glaucoma who dependency after
 headache
result from a before are receiving long-term use.
facilitation of medical appropriate  tremor
the action of procedure therapy but is  dizziness
gamma s. This contraindicate
aminobutyric medicatio d in acute  dry mouth
acid n works narrow-angle or
(GABA), an by glaucoma
inhibitory calming excessive
neurotransmit the brain saliva
ter in the and  nausea
central nerves.
nervous  constipatio
system. n
Name of Mechanis Indication Contraindication Side/ adverse Nursing
drug m of action effects responsibilities
Generic Exerts Treatment of A very serious  Upset  Determine
name: bactericida bacterial allergic reaction stomach, previous
Cloxacillin l activity infections to this drug is  nausea, exposure and
Brand name: via including unlikely, but seek  vomiting, sensitivity to
Cloxapen inhibition endocarditis, immediate  diarrhea, penicillins and
Classificatio of bacterial pneumonia, medical attention  gas, cephalosporins
n: penicillin cell wall bone and joint if it occurs.  and mouth and other
synthesis infections, skin Symptoms of a sores allergic
by binding and soft-tissue serious allergic reactions of any
one or infections, and reaction may kind before
more of the sepsis that are include: rash, treatment is
penicillin caused by itching/swelling initiated.
binding susceptible (especially of the  Monitor for
proteins strains of face/tongue/throa S&S of
(PBPs). penicillinase- t), new fever, anaphylactoid
Exerts producing chills, severe reaction (see
bacterial staphylococci. dizziness, trouble Appendix G) or
autolytic breathing. other signs or
effect by symptoms of
inhibition hypersensitivity
of certain reaction (see
PBPs Appendix F) as
related to with other
the penicillins.
activation  Lab tests:
of a Periodic
bacterial assessments of
autolytic renal, hepatic,
process. and
hematopoietic
function are
advised in
patients on
long-term
therapy.