Tetralogy of fallot (TOF)

By : - Dr. Sanjeev
Tetralogy of fallot
Commonest cyanotic congenital heart
disease in children above the age of two
years constituting almost 75 % of all blue
patients.
Four constituents of tetra logy as described
originally by Fallot consist of :-
1. Ventricular septal defect (VSD)
2. Pulmonic stenosis
3. overriding or dextroposed aorta, and
4. Right ventricular hypertrophy
.
Pathophysiology :-
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 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
two ventricle are identical there is little or no
left to right shunt and the VSD is silent -------
-----
Cont
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 -- more severe the
pulmonic stenosis, the less the flow into the
pulmonary artery and the bigger the right
to left shunt -- more severe the pulmonic
stenosis, the shorter the ejection systolic
murmur and the more the cyanosis ---
Cont
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
The VSD of TOF is always large enough to
allow free exit to the right to left shunt ---
since the right ventricle is effectively
decompressed by the VSD --- congestive
failure never occurs in TOF.
Cont
the right ventricular outflow obstruction
results in the delay in the P2 -- since
the pulmonary artery pressure is reduced,
the P2 is also reduced in intensity -- the
late and soft P2 is generally inaudible in
TOF -- the S2 is , therefore, single and
the audible sound is A2 -- since the aorta
is somewhat anteriorly displaced, the
audible single A2 is quite loud --- the
ascending aorta in TOF is large and may
result in an aortic ejection click
Cont
Right ventricular hypertrophy reduced the
distensibility of the right ventricle during
diastole ---- the right atrial contraction at
the end of diastole causes a relatively large
`a ` wave (prominent in the jugular venous
pulse).
Clinical features : -
Become symptomatic any time after birth
Paroxysmal attack of dyspnea
Cyanosis may be present from birth or make
its appearance some years after birth
Commonest symptoms are dyspnea on
exertion and exercise intolerance
Patients assume a sitting posture squatting
as soon as they get dyspneic. Although
squatting is not specific for TOF, it is the
commonest congenital lesion in which
squatting is noted
Anoxic spells occur predominantly after
waking up or following exertion.
.
Normal growth and development depend on a
normal workload for the heart and normal flow of
oxygen-rich blood to all parts of the body. Babies
who have tetralogy of Fallot may not gain weight
or grow as quickly as children who have healthy
hearts because they tire easily while feeding.
Squatting (a compensatory mechanism) is
uniquely characteristic of a right-to-left shunt that
presents in the exercising child. Squatting
increases the peripheral vascular resistance,
which diminishes the right-to-left shunt and
increases pulmonary blood flow.

Child becomes more cyanosed while crying, these
are called cyanotic spellsor Fallots spells.


What causes a spell ?

Due to "spasm" or contraction of a band of
muscle in the right ventricle just under the
pulmonary valve. When this muscle
contracts, it further narrows the channel for
blood flow into the lungs. As a result,
oxygen delivery becomes further reduced.
This causes a spell

On physical examination : -

Cyanosis , clubbing, slightly prominent `a
wave in the jugular venous pulse, normal
sized heart with parasternal impulse, a
systolic thrill in less than 30 % patients.
Normal first sound,
Single second sound and
An ejection systolic murmur.
Investigations : -
ECG : right axis deviation with right
ventricular hypertrophy
Echocardiography : identify the large
overriding aorta, right ventricular
hypertrophy and outflow obstruction.
Chest X ray :

Boot shaped heart (it
means apex is lifted up
& there is a concavity
in the region of
pulmonary artery)
Oligaemic lung fields
Hilar vessels are few,
lung vessels also few,
large rt. Venricle.
Treatment :
Management of complications and correction
of anemia
Treatment of Anoxic spells :
Knee chest position to increase aortic
resistance. The increased aortic and left
ventricular pressure reduces the rush of
blood through the septal hole from the right
ventricle and improves blood circulation to
the lungs, decreasing the right to left shunt
thus decreasing the amount of deoxygenated
blood entering the systemic circulation.
Oxygen through a face mask to increase the
amount of oxygen in the blood.
Cont
beta-blockers such as propranolol
acute episodes may require rapid
intervention with morphine to reduce
ventilatory drive and a vasopressor
such as epinephrine, phenylephrine,
or norepinephrine to increase blood
pressure.
Correction of anemia
Consider operation
Surgery : -
Blalock-Taussig operation : connection
between the right subclavian artery, and
the right pulmonary artery, which increases
the amount of red oxygenated blood
reaching the lungs, relieving cyanosis.
Pott`s shunt : descending aorta is
anastomosed to the pulmonary artery
Waterstont`s shunt : ascending aorta
right pulmonary artery anastomosis
Cont.
Total correction: The hole in the
ventricular septum is closed with a patch
and the obstruction to right ventricular
outflow, pulmonic stenosis, is opened.
These corrections allow blood flow to the
lungs for oxygenation before being pumped
out into the body.
Coarctation of the aorta
There is localised narrowing of
the aortic arch, just distal or
proximal to the ductus or
ligamentum arteriosus and also
the left subclavian artery.
40 80 % patients have a
bicuspid aortic valve.
There are three types :
A. Ductal coarctation
B. Preductal coarctation
C. Postductal coarctation (most
common)
Cont
Pathophysiology :-
Physiologically the difference between the
preductal and postductal coarctation depends
on the absence or presence of collateral
anastomosing vessels -- in the fetal life, the
right ventricular output passes down the
descending aorta through a wide ductus
arteriosus and left ventricular output into the
left carotid and left subclavian arteries ---
very little of the left ventricular output reaches
the descending aorta --- the portion of the
aorta distal to the left subclavian and before the
portion where the ductus arteriosus joins called
the isthmus -------
Cont
At birth, normally the isthmus is the most
narrow part of the aorta -- if the fetus
has a preductal coarctation it does not
interfere with his normal hemodynamic and
collaterals are not formed --- on the other
hand if a postductal coarctation is present it
is operative in the fetal life as it interfere
with right ventricular output reaching the
descending aorta --- this stimulates the
formation of collaterals even in the fetal life
-------------
Cont.
after birth when the right ventricular output
is directed into the pulmonary arteries and
there is no flow from the pulmonary into the
aorta, the descending aorta must receive its
total supply from the left ventricle via the
ascending aorta -- since in preductal
coarctation there are no collaterals, the
neonate becomes symptomatic immediately
hypertension resulting in LVF -- neonates
who have a postductal coarctation already
have some collaterals and are spared from
developing severe hypertension and
congestive cardiac failure ---------
Cont
the exact mechanism for the
production of systemic hypertension
in coarctation is not known --- the
narrowed pulse pressure in the
descending aorta distal to the
coarctation has been implicated in the
renal mechanism for the causation of
hypertension in coarctation.
Cont
the obstruction stimulates growth of
collateral vessels between the proximal and
distal segments --- the intercostal vessels
also participate in decompressing the
hypertension upper segment --- they
enlarge and become palpable at the lower
borders of the ribs --- palpable collaterals
are also felt at the medial and inferior angle
or scapula --- because of the
decompression of the upper segment by the
collaterals, the resting blood pressure in
the upper extremities may be even normal
(on exercise, systolic pressure gets
accentuated).
Clinical features : -
Uncomplicated coarctation :
Intermittent claudication (due to a temporary
inadequate supply of oxygen to the muscles of the
leg)
Pain and weakness of legs and
Dyspnea on running
Physcial examination shows delayed and weak
or impalpable femorals compared to strong
brachial arteries.
Dorsalis pedis palpable (because distal pulse
pressure is wider)
Diagnosis can be excluded if femoral pulse is
well felt.
Cont
Rarely it is possible for the patient to continue to
have pulmonary arterial hypertension ---- a
patent ductus arteriosus may support the distal
segment of the aorta -- in such a situation
good femoral pulsations may be present in spite
of coarctation which would be preductal in
location and cyanosis in toes --- it is
important to remember that the site of
coarctation does not determine whether the flow
through the PDA is from left to right or from right
to left --------
Cont.
whether the coarctation is preductal or
postductal, the flow is from left to right since
the distal segment of the aorta in coarctation
almost never has a mean pressure below 50
mm of Hg. ----- if the flow through the PDA
is from right to left, it indicates that there is
severe pulmonary arterial hypertension.
Heart size remains normal
Systolic thrill may be palpable in the
suprasternal notch
Prominent arterial pulsation in the
suprasternal notch and the carotid vessels
Cont
Second sound is normally split with a loud
aortic component

Late systolic murmur and/or early diastolic
murmur
Investigations :-
Electrocardiography : may show left
ventricular hypertrophy
MRI : ideal
Chest X ray :
X ray features : -

Heart is enlarged in
transverse diameter due
to left Ventricular
enlargement
3 figures
Rib notching ( mostly 4
th

8
th
) due to their
erosion by enlarged
collateral intercostal
arteries


Cont
red arrows : - rib
notching
3 figures:
yellow arrow : - the
aortic knob,
blue arrow : - the
actual coarctation
and
green arrow: - the
post-stenotic
dilation of the
descending aorta.
Assessment of severity : -
Degree of systemic hypertension
determines the severity of coarctation.

Cardiac enlargement indicates LVF and
severe coarctation
Treatment : -
Medical : control of cardiac failure

Surgery : -
Resection and end-to-end anastomosis.
Subclavian flap angioplasty
Tubular graft can be used if long segment is
involved.
Baloon dilatation can be used if recurrence
occurs.


Complications : -
Congestive cardiac failure
Infective endocarditis
Rupture of an intercostal aneurysm
Dissection of aorta