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Tetralogy of Fallot (TOF) in Children

Tetralogy of Fallot (TOF) is a cardiac anomaly that refers to a combination of four related heart
defects that commonly occur together. The four defects are:

Ventricular septal defect (VSD)

Overriding aorta the aortic valve is enlarged and appears to arise from both the left and
right ventricles instead of the left ventricle as in normal hearts
Pulmonary stenosis narrowing of the pulmonary valve and outflow tract or area below the
valve that creates an obstruction (blockage) of blood flow from the right ventricle to the
pulmonary artery
Right ventricular hypertrophy thickening of the muscular walls of the right ventricle,
which occurs because the right ventricle is pumping at high pressure
A small percentage of children with tetralogy of Fallot may also have additional ventricular
septal defects, an atrial septal defect (ASD) or abnormalities in the branching pattern of their
coronary arteries. Some patients with tetralogy of Fallot have complete obstruction to flow
from the right ventricle, or pulmonary atresia. Tetralogy of Fallot may be associated with
chromosomal abnormalities, such as 22q11 deletion syndrome.
The pulmonary stenosis and right ventricular outflow tract obstruction seen with tetralogy of
Fallot usually limits blood flow to the lungs. When blood flow to the lungs is restricted, the
combination of the ventricular septal defect and overriding aorta allows oxygen-poor blood
("blue") returning to the right atrium and right ventricle to be pumped out the aorta to the body.
This "shunting" of oxygen-poor blood from the right ventricle to the body results in a reduction
in the arterial oxygen saturation so that babies appear cyanotic, or blue. The cyanosis occurs
because oxygen-poor blood is darker and has a blue color, so that the lips and skin appear blue.
The extent of cyanosis is dependent on the amount of narrowing of the pulmonary valve and
right ventricular outflow tract. A narrower outflow tract from the right ventricle is more
restrictive to blood flow to the lungs, which in turn lowers the arterial oxygen level since more
oxygen-poor blood is shunted from the right ventricle to the aorta.

2. Sign And Symptoms of tetralogy of fallot

Tetralogy of Fallot is most often diagnosed in the first few weeks of life due to either a
loud murmur or cyanosis. Babies with tetralogy of Fallot usually have a patent ductus
arteriosus at birth that provides additional blood flow to the lungs, so severe cyanosis is rare
early after birth.
As the ductus arteriosus closes, which it typically will in the first days of life, cyanosis can
develop or become more severe.
The degree of cyanosis is proportional to lung blood flow and thus depends upon the degree of
narrowing of the outflow tract to the pulmonary arteries.
Rapid breathing in response to low oxygen levels and reduced pulmonary blood flow can occur.
The heart murmur, which is commonly loud and harsh, is often absent in the first few days of
The arterial oxygen saturation of babies with tetralogy of Fallot can suddenly drop markedly.
This phenomenon, called a "tetralogy spell," usually results from a sudden increased
constriction of the outflow tract to the lungs so that pulmonary blood flow is further restricted.
The lips and skin of babies who have a sudden decrease in arterial oxygen level will appear
acutely more blue.
Children having a tetralogy spell will initially become extremely irritable in response to the
critically low oxygen levels, and they may become sleepy or unresponsive if the severe
cyanosis persists.
A tetralogy spell can sometimes be treated by comforting the infant and flexing the knees
forward and upward. Most often, however, immediate medical attention is necessary.

3. Diagnosis Of tetralogi Of Fallot

When a newborn with significant cyanosis is first seen, he or she is often placed in
supplemental oxygen. The increased oxygen improves the child's oxygen levels in cases of
lung disease, but breathing extra oxygen will have little effect on the oxygen levels of a child
with tetralogy of Fallot.
Failure to respond to this "hyperoxia test" is often the first clue to suspect a cyanotic cardiac
defect. Infants with tetralogy of Fallot can have normal oxygen levels if the pulmonary stenosis
is mild (referred to as "pink" tetralogy of Fallot). In these children, the first clue to suggest a
cardiac defect is detection of a loud murmur when the infant is examined.
Once congenital heart disease is suspected, echocardiography can rapidly and accurately
demonstrate the four related defects characteristic of tetralogy of Fallot.
Cardiac catheterization is occasionally required to evaluate the size and distribution of the
pulmonary arteries and to clarify the branching patterns of the coronary arteries.
Catheterization can also demonstrate whether patients have pulmonary blood flow supplied by
an abnormal blood vessel from the aorta (aortopulmonary collateral).

4. Patofisiology

In TOF, the pathophysiology and management are dictated by 3 specific anatomical factors:

1. Degree of right ventricular outflow tract obstruction

Typically occurs at multiple levels, including below the pulmonary valve (subvalvar or
infundibular stenosis), at the level of the valve (valvar pulmonary stenosis), and above
the valve (supravalvar stenosis). The degree of pulmonary obstruction determines
whether the infant is cyanotic or acyanotic by affecting the amount of blood that shunts
right to left at the ventricular septal defect (VSD).
TOF with mild pulmonary obstruction is typically not a cyanotic lesion. There is no
significant restriction to the flow of blood into the pulmonary arteries and therefore the
infant is well saturated.
TOF and significant pulmonary obstruction result in a cyanotic infant. This is because
blood in the right ventricle has a higher resistance to overcome in order to enter the
pulmonary circulation. Blood is shunted from the right ventricle to the aorta through
the VSD and into the systemic circulation without being oxygenated in the pulmonary

2. Pulmonary artery anatomy

Pulmonary artery anatomy can dramatically affect the physiology.

Pulmonary atresia with VSD (TOF with pulmonary atresia) and absent pulmonary
valve syndrome (TOF with absent pulmonary valve) are very different physiologically
and are considered different disease processes from TOF. For that reason, they are not
discussed here.

3. Non-restrictive, mal-alignment VSD

Anterior mal-alignment VSD in TOF is nearly always non-restrictive. With a large,

non-restrictive VSD, the pressure in the right ventricle and left ventricle equalises. In
this case, the VSD does not determine the degree of shunting. The degree of shunting
in TOF is therefore due to the relative resistance to flow of the pulmonary versus
systemic circulations.
Additional VSDs may be present and should be looked for as these may complicate the
postoperative course.

Hypercyanotic spells, or tet spells, are episodes of severe cyanosis associated with hyperpnoea.
They result from an increase in right ventricular outflow tract obstruction causing a decrease
in the pulmonary blood flow and an increase in right-to-left shunting across the VSD. The exact
aetiology of hypercyanotic spells is unclear, but they are thought to be initiated by increases in
the right ventricular infundibular contractility. Hypercyanotic spells may be self-limited;
however, if sustained, they can result in brain ischaemia or death.

5. Physical Examination

Most infants with tetralogy of Fallot (TOF) are smaller than expected for age. Cyanosis of the
lips and nail bed is usually pronounced at birth; after age 3-6 months, the fingers and toes show
A systolic thrill is usually present anteriorly along the left sternal border. A harsh systolic
ejection murmur (SEM) is heard over the pulmonic area and left sternal border. When the right
ventricular (RV) outflow tract obstruction (RVOTO) (eg, from pulmonary atresia) is moderate,
the murmur may be inaudible (more cyanotic patients have greater obstruction and a softer
murmur). The S2 is usually single (the pulmonic valve closure is not heard). During cyanotic
episodes, murmurs may disappear, which is suggestive of lessened RV outflow to the
pulmonary arteries. In individuals with aortopulmonary collaterals, continuous murmurs may
be auscultated. Thus, an acyanotic patient with tetralogy of Fallot (pink tet) has a long, loud,
systolic murmur with a thrill along the RVOT
The following may also be noted:
RV predominance on palpation
May have a bulging left hemithorax
Aortic ejection click
Squatting position (compensatory mechanism)
Scoliosis (common)
Retinal engorgement
1. Indications intrakranial pressure
The most common indication for invasive ICP monitoring is closed head injury. the Guidelines
for the Management of Severe Traumatic Brain Injuri an ICP monitor should be placed in
patients with a Glasgow coma score less than 8T (after resuscitation) and after reversal of
paralytics or sedatives that may have been used during intubation. (See the Glasgow Coma
Scale calculator.)
Other candidates for ICP monitoring are as follows:
A patient who is awake yet at risk for increased ICP under general anesthesia for a
necessary nonneurosurgical procedure (eg, orthopedic limb-saving procedure), rendering
clinical observation impossible
Patients who have nonsurgical intracranial hemorrhage but are intubated for
nonneurosurgical reasons, preventing clinical examination
Patients with moderate head injury who have contusions to the brain parenchyma that are
at risk of evolving (Extreme caution and clinical judgment must be exercised for lesions
in the temporal fossa, since their proximity to the brainstem can lead to catastrophic
herniation and brainstem compression with little change in the global ICP.)
Perioperative ICP monitoring is indicated in patients who have just undergone tumor or
arteriovenous malformation resection and are at risk for cerebral edema with an inability to
follow a clinical neurological examination.
Raised ICP is the final common pathway that leads to death or disability in most acute
cerebral conditions. It is also potentially treatable. The two major consequences of increased
ICP are:
brain shifts
brain ischaemia.
Cerebral perfusion pressure (CPP) is the calculated difference between the mean arterial
pressure (MAP) and the ICP. CPP = MAP ICP.

2. Pathophysiology

There are four ways in which the contents of the cranium can increase and result in increased pressure
on the brain:

CSF production is increased and/or reabsorption is decreased

Blood flow to the brain is increased or venous drainage of blood is decreased

A mass in the brain tissue caused by an abscess, tumor, or hematoma develops

Edema of the brain from tissue trauma occurs The production of CSF is constant and increased
production rarely occurs. An obstruction to the flow of CSF can occur as the result of a tumor, edema
of the brain, hematoma, or infection. The arachnoid villi which absorb CSF into the blood stream can
become blocked by blood, byproducts of infection or inflammation of the meninges causing an
increase in the amount of CSF present, increasing pressure on the brain. Hemorrhages can cause a
hematoma that acts as a mass in the brain causing pressure. Injury to brain tissues causes a breach in
the blood-brain barrier and extra fluids leak into the brain causing edema of the tissues. Compression
of the jugular vein or a clot in a vein causes engorgement of the blood vessels in the brain. Abnormally
high pressure in the thorax or abdomen restricts venous drainage from the brain to the heart. All of
these mechanisms result in an increased amount of blood in the brain and increased pressure upon
brain tissues. The resultant increase of pressure within the cranium constricts the flow of blood to the
cells. Brain cell ischemia causes vasodilation with an increase in blood supply to the brain and even
more pressure within the cranium leading to even more ischemia and cellular death. Cellular necrosis
allows fluids to leak out of cells causing more edema.

3. Signs and Symptoms

Signs and symptoms of increased intracranial pressure depend on the status of brain compliance and
autoregulation and the patient's place along the continuum of brain damage. When ICP increases
slowly papilledema may be the first sign. Papilledema is a swelling of the optic nerve at its entrance
into the back of the eyeball. When ICP rises rapidly, papilledema occurs late in the process. Other signs
and symptoms of increased intracranial pressure depend on the cause and can include:


Blurred or double vision, pupil dilation and lack of response to light, deviated gaze, nystagmus



Changes in level of consciousness: anxiety, agitation, disorientation, or sleepiness progress to

decreased responsiveness and unconsciousness.

Loss of motor function or abnormal function, unilateral or bilateral depending on the location of the
injury, which can include involuntary movements, tremors, attempts to remove noxious stimulus,
withdrawal from stimulus, decorticate or decerebrate posturing, or hemiparesis and hemiplegia.

Hypo or hyperthermia Abnormal respiratory patterns

Cardiac dysrhythmias



Widened pulse pressure

4. Diagnostic Tests

The patient with signs of increasing ICP needs emergency evaluation and rapid treatment to limit brain
damage. An eye exam with an ophthalmoscope will check for signs of papilledema. LOC and
neurological function are measured. A CT scan of the brain is the quickest way to look for brain
pathology. If the CT scan is indecisive, an MRI is done. The cervical spine is also x-rayed or scanned to
check for injury. If needed an EEG or lumbar puncture is done along with serum electrolytes and a
toxicology screen.

5. Nursing Care

Prepare the patient and family for the insertion of an ICP monitoring catheter.

Explain the procedure, risks, answer questions, and obtain consent according to policy.
Medicate the patient as ordered. Assess vital signs and neurological status to be used as a baseline.
Prepare equipment and shave the insertion site.

Assist as needed with the procedure, maintaining sterile conditions. Chart the procedure, type of
transducer system, locations of burr holes, tunneling and exit sites of the catheter, and initial ICP level
and waveform.

Do not apply triple antibiotic or betadine gel to the insertion/exit sites. Dress these sites with clear
dressings, taped gauze, or a full head dressing according to policy.

Secure catheters and tubing to the patient's clothing without tension and check bolt and other
connections frequently to maintain a closed system.

Restrain extremities as needed according to facility policy to protect the ICP catheter from being pulled
out during patient agitation.

Change the dressing over the insertion site as ordered and monitor for CSF drainage.
Kelompok Usia Tekanan Intrakranial normal (mmH
Dewasa dan anak-anak lebih besar < 10 - 15
Anak-anak 37
Bayi baru lahir 1,5 6
Nilai tekanan intrakranial normal

Pathophysiology Increased Pressure

Intracranial on Stroke
The main pathophysiology of TTIK, among others, is
cerebral edema, CSS flow obstruction,
increased blood brain volume (CBV),
and the expansion of brain mass. Cerebral edema
is one of the complications of all types
stroke. The time varies from 24 hours
first until about 72 hours after onset. 9
Cerebral edema may be divided into edema
interstitial, cytotoxic, and vasogenic