TETRALOGY OF FALLOT

 refers to a type of congenital heart defect, Congenital means present at birth.

 Most common cyanotic heart disease present during pregnancy. ''blue babies''

 TOF/"TET" is a complex condition involving several associated congenital heart defects that occur due
abnormal development of the fetal heart.

 This affects the structure of the heart causing oxygen-poor blood to flow out of the heart and to rest of
the body.

 TOF occurs in approximately 400 per million live births or occurs in about 5 out of every 10,000 babies.

 occurs equally in girls and boys.

NORMAL HEART FLOW

Tetralogy of Fallot
Congenital heart defects have four structural defects which are:
P.R.O.V
1. PULMONARY STENOSIS
2. VENTRICULAR SEPTAL DEFECT
3. OVERRIDING AORTA
4. RIGHT VENTRICULAR HYPERTROPHY

F O U R (4 ) P R O B L E M S OR H E A R T P R O B L E M ( P . V . O . R . ) T H AT IS L E A D I N G RIGHT-LEFT-
SHUNT
 1. Pulmonary Stenosis
 A narrowed or stiffened pulmonary valve means the pulmonary artery has trouble sending enough
blood from the heart to the lungs.

2. Right Ventricular Hypertrophy

 The right lower chamber of the heart is bigger or thicker than normal, making it harder for blood to go
through the pulmonary valve

3. LARGE VENTRICULAR SEPTAL DEFECT

 VSD is a hole between the two lower chambers (ventricles) of the heart. The hole allows oxygen-rich
blood and oxygen-poor blood to mix, so the body does not receive enough oxygen-rich blood.
 4. OVERRIDING
AORTA
 The aorta, which is the artery that carries oxygen-rich blood to the body, is out of place and rises
above both ventricles. As a result, the body gets too much oxygen-poor blood.
 PATHOPHYSIOLOGY

RISK
FACTORS:
✓ Children with certain genetic syndromes, such as Down syndrome or Di George syndrome
 Di George Syndrome:
 a disorder caused when a small part of chromosome 22 is missing.
 This deletion results in the poor development of several body systems
 A.k.a 22q11.2 deletion syndrome

✓ Environmental factors, such as smoking or taking certain medications during pregnancy

✓ Family history
✓ Having certain medical conditions during pregnancy, such as diabetes or rubella

SIGNS AND SYMPTOMS

✓ Cyanosis (around lips & fingernail beds at birth)

✓ Difficult or rapid breathing
✓ Fatigue
✓ Fussiness
✓ Heart murmur
✓ Trouble feeding or gaining weight
✓ Clubbing of fingers and toes (1-2 yrs)
 difficulty feeding
 failure to gain weight and develop normally
 no severe ventricular outflow obstruction = baby might not be cyanotic

DIAGNOSIS
➢ The heart conditions may be seen and heard on
prenatal ultrasounds, while the baby is still in the womb.
Some babies are diagnosed with tetralogy of Fallot before birth.
➢ After birth, tests for TOF may include:
✓ Cardiac catheterization
✓ Chest X-ray
✓ Echocardiogram (echo)
✓ Electrocardiogram (ECG)
✓ Cardiac CT
✓ MRI

CLINICAL MANIFESTATION
1. Cl i ni c al m a n i f e s t a ti o n s are va r i ab l e and de pe nd on the size of the VSD and the de gr e e
of RVOTO .
2. Cy an os i s .
a) Ne onate may have nor m al ox yge n s a t u r a ti o n s ; as the infant gr ows , the RV OTO
in c r e a s es and the oxyge n s a t u r a ti o n f all s.
b) Ne onate with unac c e ptabl y low oxyge n s a t u r a ti o n nee ds PGE, i nf usi on to
maintain duc tal pate nc y and a de qu at e oxyg en s a t u r ati o n .
 c) Cy an os i s may i ni ti al l y be ob se r ve d only with cr yi ng and with e x e r ti o n.

3. Polycythemia.
4. D e c r e as e d e xe r c i se to l e r an c e
5. A common clinical manifestation years ago was squatting, a posture characteristically assumed by older
children to increase systemic vascular resistance and to encourage increased pulmonary blood
flow, Squatting is rarely seen currently because TOF is now surgically repaired during the first year
of life.
6. Hypercyanotic spells (formerly known as Tet spells):
 young infants with tetralogy of Fallot
 peak incidence:2-4 months
 crying, agitation, pain, injury, straining to defecate, fright or wailing from nap.
 Under GA- during induction & surgical stimulation

 HYPERCYANOTIC- occur in any children who has heart defect that includes obstruction
to pulmonary blood flow and increases right-to- left-shunting.
- an increase in oxygen requirements which infants are unable to meet, may cause a spell.
- hypoxia causes acidosis that increases pulmonary blood flow and in turn further decrease pulmonary
blood flow.
 spells rarely seen before 2 months of age and occurs in the first year of life and more often
in the morning.
 profound hypoxemia causes cerebral hypoxia.
TREATMENT
> place infant in knee-chest position
>employ calm, comforting approach
>administer oxygen
>give morphine subcutaneously or
through existing intravenous line.
>begin intravenous fluid replacement
and volume expansion, if needed
>Repeat morphine administration.
 - Babies who are not strong enough for a full repair surgery or who have other health problems may
have a shunt operation.
- A shunt is a tube that is sewn into place between the aorta and the pulmonary artery. It helps blood
flow to the lungs.
- A shunt operation is not permanent; babies with a shunt still need a full repair surgery later.

FULL REPAIR SURGERY

- A full tetralogy of Fallot repair surgery involves:
✓ Placing a patch over the ventricular
septal defect
✓ Removing thickened tissue from the
right ventricle
✓ Widening the pulmonary valve
NURSING INTERVENTIONS AND MANAGEMENT