TETROLOGY OF FALLOT

R.SAKTHI ABIRAMI
FACULTY OF ALLIED HEALTH SCIENCES
 TOF

• COMMON CYANOTIC CONGENITAL HEAT DISEASE .

• PRESENTING ABOVE 2 YRS .
• ANATOMICALLY IT IS CHARACTERIZED BY THE CLASSIC TETRAD:
• severe right ventricle outflow obstruction,
• large malalligned vsd,
• aorta that overrides the vsd
• right ventricular hypertrophy.
 COMPONENTS OF
TOF

1. MALALLINGED VSD

2. PS

3. OVERRIDING AORTA

4. RVH
HEMODYNAMICS (FALLOTS PHYSIOLOGY )
• Physiologically the pulmonic stenosis causes concentric right ventricular hypertrophy without
cardiac enlargement and an increase in right ventricular pressure .
• When the right ventricular pressure is as high as the left ventricular or the aortic pressure, a right
to left shunt appears to decompress the right ventricle.
• Once the right and left ventricular pressures have become identical, increasing severity of
pulmonic stenosis reduces the flow of blood into the pulmonary artery and increases the right to
left shunt.
• As the systolic pressures between the two ventricles are identical there is little or no left to right
shunt and the VSO is silent
• The right to left shunt is also silent since it occurs at insignificant difference in pressure between
the right ventricle and the aorta
• The flow from the right ventricle into the pulmonary artery occurs across the pulmonic stenosis
producing an ejection systolic murmur.
• The more severe the pulmonic stenosis, the less the flow into the pulmonary artery and the
bigger the right to left shunt.
• Thus the more severe the pulmonic stenosis, the shorter the ejection systolic murmur and the
more the cyanosis.
• Thus the severity of cyanosis is directly proportional to the severity of pulmonic stenosis, but the
intensity of the systolic murmur is inversely related to the severity of pulmonic stenosis.
 CLINICAL FETAURES

Patients with TOF may become symptomatic any time after birth.
Neonates as well as infants may develop anoxic spells (paroxysmal attacks of dyspnea).
Cyanosis may be present from birth or make its appearance some years after birth
The commonest symptoms are dyspnea on exertion and exercise intolerance
 ANOXIC SPELL

• Anoxic spells occur predominantly after waking up or following exertion.

• The child starts crying, becomes dyspneic, bluer than before and may lose consciousness.
Convulsions may occur.
• The frequency varies from once in a few days to numerous attacks every day
 SQUATTING
The patients assume a sitting posture-squatting-as soon as they
get dyspneic.
• PHYSICAL EXAMINATION –
• Cyanosis
• Clubbing
• Slightly prominent 'a' waves in the jugular venous pulse
• Normal sized heart with a mild parasternal impulse
• A systolic thrill in less than 30% patients
• Normal first sound
• Single second sound and an ejection systolic murmur which ends before the audible single second sound
 DIAGNOSIS

• The diagnosis of TOF is confirmed by echocardiography; cardiac catheterization is seldom

necessary
 COURSE AND COMPLICATIONS

• The pulmonic stenosis becomes progressively severe with age. The dyspnea and increasing
exercise intolerance limit Patient activities.
• Each attack of paroxysmal dyspnea or anoxic spell is potentially fatal.
• Anemia, by decreasing the oxygen carrying capacity of blood, reduces the exercise tolerance still
further.
• It can result in cardiac enlargement and congestive cardiac failure making diagnosis difficult.
• Patients are prone to infective endocarditis.
 MANAGEMENT

MEDICAL PALLIATIVE CORRECTIVE

 MANAGEMENT OF CYNAOTIC SPELL

check airway

• deliver oxygen by face mask or nasal cannula

• knee chest position

• morphine (0.2 mg/kg subcutaneously or ketamine 3-5 mg/kg/ dose intramuscular)

• sodium bicarbonate at 1-2 ml/kg (diluted 1:1 or in 10 ml/kg N/5 in 5% dextrose)

• correct hypovolemia (10 ml/kg of dextrose normal saline)

• keep child warm

• transfuse packed red cell if anemic (hemoglobin <12 g/ dl)

• beta blockers unless contraindicated by bronchial asthma or ventricular dysfunction;

metoprolol is given at 0.1 mg/kg IV slowly over 5 min and repeated every 5 min for maximum 3 doses; may be followed by infusion at 1-2
µg/kg/min

• monitor saturation, heart rates and blood pressure; keep heart rate below 100/minute
 Definitive surgery for TOF

• involves closure of the VSD and relief of the RVOT obstruction.

• Often the relief of the RVOT obstruction involves the placement of a transannular patch across
the pulmonary valve and valvectomy resulting in severe pulmonary regurgitation.
VARIANTS OF TOF
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