Tetrology of Fallot

What are the components of TOF?

Pulmonary Infundibular stenosis

High VSD
Overriding of Aorta
Right ventricular hypertrophy

What is Triology of Fallot?

ASD, PS, Right ventricular hypertrophy
What is pentology of Fallot?
TOF plus patent foramen ovale or ASD
What are the compensatory mechanisms in TOF?

Polycythemia
Development of extensive collateral circulation between
branches of aorta and pulmonary arteries
Persistent PDA

Conditions associated with TOF

Chromosomal anomalies deletion syndrome 10 q- , Downs

syndrome, trisomy 18
Syndromes Goldenhar syndrome, VATER syndrome,
DiGeorge syndrome, Laurence-moon-biedel syndrome,
CHARGE syndrome, Velocardiofacial syndrome, TAR syndrome,
Poland syndrome, Cornelia-delange syndrome, Alagille
syndrome, Fetal alcohol syndrome, fetal hydantoin syndrome
Maternal conditions phenylketonuria in mother
Teratogenic agents thalidomide, trimethadione,
carbamazepine, sex hormones.

What is Acyanotic Fallot and Cyanotic Fallot?

In acyanotic fallot, obstruction to pulmonary flow is minimal. So
blood flow to pulmonary artery is good and enough blood gets oxygenated
to prevent cyanosis. Thus even in the presence of defects, child is pink
and hence called pink fallot.

In cyanotic fallot, the pulmonary resistance is high and blood flow to

the pulmonary circulation is decreased. There is an increased shunting of
blood from right to left side aggravating the central cyanosis.
What are tet spells?
Cyanotic spells or tet spells occur due to abrupt reduction in
pulmonary blood flow or a decrease in systemic vascular resistance.
Characterised by paroxysms of hyperpnea, sudden marked increase in
cyanosis, irritability and prolonged crying, decreased intensity of heart
murmur. More common between 2 to 4 months of age. Precipitated by
exercise, sedation, fever, general anesthesia. Special postures such as
squatting or squatting equivalents such as knee chest position help in
alleviating the symptoms.
Treated with knee chest position, oxygen administration, morphine,
IV propranolol, Vasoconstrictors such as phenylephrine, treatment of
acidosis with sodium bicarbonate.
What is the natural history of TOF?
Children with acyanotic form of TOF gradually develop cyanosis by 1
3 years of age. Symptoms tend to progress because of increasing
severity of the infundibular stenosis. Infants developing cyanosis in the
first month of life usually have severe PS. These infants have symptoms
such as marked dyspnea and exercise intolerance. If these patients
survive, they improve after first or second year of life, possibly due to
development of collaterals.
Examination findings of TOF?

Pulse normal
BP normal, may be increased in later stages after several
years of marked cyanosis, polycythemia
Cyanosis, clubbing may be present
Heart sounds
o S1 is normal
o S2 is usually single, only aortic component is heard,
since aorta is place anteriorly, this is loud
o P2 is delayed and soft.
o Sometimes an aortic ejection click may be heard
Murmurs ESM in left third and fourth ICS due to flow from
right ventricle to stenosed pulmonary artery. The intensity and
duration of the murmur is inversely proportional to the
pulmonary artery obstruction.

Investigation findings in TOF?

ECG Right axis deviation, Right ventricular hypertrophy

X-ray Normal sized or small heart, clear lung fields due to
pulmonary oligaemia, boot shaped heart, right ventricular
enlargement, hypertrophy of right ventricle,
ECHO Infundibular stenosis, high VSD, RVH
Colour Doppler - flow of blood from right ventricle into the aorta

Timing of Surgery in TOF?

Surgery is done at birth or later in infancy depending on the severity
of defect, weight and general health of the baby and severity of
symptoms. When severe symptoms develop in the first or second month
of life, initial shunting operation should be done followed by repair within
12 months. When patient is asymptomatic or only mildly symptomatic,
repair can be deferred until 2 years of life. In severe TOF, surgery can be
done in the neonatal period. In babies who are too small or too weak,
palliative surgery can be done immediately and total corrective elective
surgery can done in later infancy.
What are the palliative shunt procedures in TOF?
Indications for palliative shunt procedures are recurrent spells, PCV
> 65, children who cannot tolerate total correction, infants who become
symptomatic in the first few months of life.
Few palliative surgeries are

Blalock Taussing shunt (Subclavian and pulmonary artery

anastomosis)
Modified Blalock taussing shunt
Waterston shunt
Potts Smith shunt
Glenns shunt
Barrets procedure

What are the total correction procedures in TOF?

Brocks procedure

Rastellis operation

What are the complications of TOF?

Anemia
Polycythemia (secondary to hypoxia)
Cyanotic spells
Growth retardation Failure to thrive
DIC
Bleeding disorders like thrombocytopenia
Hemoptysis due to DIC, Bronchopulmonary collaterals,
thrombocytopenia, polycythemia
Arthritis
CNS complications Brain infarction, Brain abscess
Gout and Hyperuricemia due to increased destruction of
RBCs
Infections Pulmonary tuberculosis
Paradoxical emboli
Metabolic acidosis