This leaflet provides information for parents and carers about Tetralogy of Fallot in children and the management and treatment of this condition.
Tetralogy of Fallot
Tetralogy of Fallot is a serious heart abnormality in which there are two main problems:
- There is a narrowing in the right sided pumping chamber of the heart (the right ventricle) just below the outlet valve (the pulmonary valve) which lets blood pass from the heart to the artery which feeds the lungs with blood (the pulmonary artery). The outlet valve itself is also usually narrowed.
- There is a large hole (ventricular septal defect or VSD) between the two main pumping chambers (ventricles) of the heart.
Here is a video explaining the condition
Tetralogy of Fallot – YouTube transcript
this video explains the congenital heart
condition to travel G a fellow it may be
useful to watch our video on how the
normal heart works before watching this
one in tetralogy a fellow the heart
develops with two main abnormalities the
first of these is an obstruction to
blood being pumped from the heart to the
lungs the part of the heart that pumps
blood to the lungs is called the right
ventricle blood passes from the right
ventricle through the pulmonary valve to
the pulmonary artery which then divides
into right and left pulmonary arteries
to take blood to the right and left
lungs the obstruction to blood being
pumped to the lungs can occur at
different levels there may be a
narrowing within the right ventricle
just below the pulmonary valve due to an
excess or overgrowth of heart muscle
there may also be an obstruction at the
pulmonary valve itself it may be too
small or formed in such a way that it
doesn’t open very well the obstruction
can also be within the pulmonary artery
or its branches which may be narrow the
other main of normality in tetralogy of
Fallot in the wall that divides the
right and left ventricles this is called
a ventricular septal defect or VSD there
are two other characteristic features of
to travel gia follow the muscle of the
right ventricle is thicker than normal
and this occurs because of the
obstruction to blood flow to the lungs
lastly the position of the aorta the
main blood vessel that takes blood from
the left ventricle to the body is
shifted so that it lies over the
ventricular septal defect the
abnormalities in tetralogy a fellow
affects the way blood flows through the
heart blood that is low in oxygen enters
the right atrium from the veins and then
moves to the right ventricle as normal
however
destruction – blood entering the
pulmonary arteries means that some blood
is forced through the ventricular septal
defect into the aorta this means that
some blood that has low levels of oxygen
is pumped to the body rather than to the
lungs
the bluer blood that contains lower
levels of oxygen mixes with the red of
blood that contains higher levels of
oxygen that is being pumped from the
left ventricle to the aorta and to the
body and this causes the person’s oxygen
levels to be lower than normal this
lower level of oxygen causes their skin
to be slightly blue in color and this is
called cyanosis
you
In the normal circulation, blood passes through the lungs to collect oxygen from the air. In babies with Tetralogy of Fallot the narrowing in the heart causes blood flow to the lungs to be reduced, making the blood oxygen level low. This makes the baby (particularly the lips and hands) appear blue. How blue the baby looks depends on how severe the narrowing in the heart is; some babies appear pink or are just slightly blue when they cry but others may appear blue all the time. Some children with Tetralogy of Fallot may have attacks where they become suddenly very blue or very pale or floppy or faint. These are known as “hypercyanotic attacks” or are sometimes called “spells”. If your child has episodes like this you should tell us immediately as these can be very serious and may even be life threatening.
Hypercyanotic Spell/Blue Episode
A typical episode will involve your baby being very upset. They will then turn very blue or purple, and become pale and floppy afterwards. This is more likely to happen if they are unwell, especially if they are not feeding well. Some of these symptoms may be mild or less obvious, so please seek medical advice if you are unsure.
What to do if your child has a spell
- Keep them as calm as possible. Give them a cuddle and bring their knees up to their chest.
- If it stops quickly and your baby remains well, call the cardiac specialist nurses.
- If your baby goes pale and floppy, loses consciousness or the spell lasts longer than one minute call 999 stating ‘my child is having a hypercyanotic spell’.
Tests
Only a simple ultrasound scan (“echocardiogram”) is required. Sometimes additional imaging with a CT scan is required.
Treatment
Here’s a video explaining the surgery needed for Tetralogy of Fallot Repair.
Tetralogy of Fallot Repair – YouTube transcript
this movie explains the initial
treatment of tetralogy of Fallot with is
by open-heart surgery before you watch
this you may wish to view our movie that
explains what to charge EF is the aims
of the operation are to allow blood to
be pumped to the lungs more easily and
to close the hole in the heart the
ventricular septal defect exactly what
is required to reduce the obstruction to
blood being pumped to the lungs depends
on where the obstruction is in an
individual patient any excess heart
muscle under the pulmonary valve is
removed it may be possible to improve
the function of the poorly valve so that
it opens more effectively the pulmonary
valve and pulmonary artery may need to
be enlarged and this is achieved by
sewing in a patch of material over the
valve and into the pulmonary artery if
the right or left palm the arteries are
too small these can also be enlarged by
sewing in a patch the ventricular septal
defect is closed by sewing in a patch of
material once the heart is repaired
blood that has low levels of oxygen no
longer passes from the right side of the
heart to the left side of the heart and
this means the patient’s oxygen levels
become normal it also becomes much
easier for the heart to pump blood to
the lungs and so the thickening of the
right ventricle improves here you can
see the difference between tetralogy
fellow before repair on the left and
after repair on the right
you
a common consequence of surgery to
repair two trilogy a fellow is that the
pulmonary valve leaks a function of the
pulmonary valve is to prevent blood from
returning to the right ventricle once it
has been pumped into the pulmonary
arteries when the valve leaks some of
the blood being pumped from the right
ventricle to the pulmonary artery
returns to the right ventricle and this
is called pulmonary incompetence or
pulmonary regurgitation we explain more
about pulmonary competence in another
movie
you
Surgery is usually planned at 6-12 months of age, but the type and timing of treatment depends on how blue an individual baby becomes as well as how well the pulmonary arteries (the lung arteries) grow. If the pulmonary arteries do not grow well, it may be necessary to perform a “shunt” or “keyhole” operation to improve the blood supply to the lungs before we can consider major surgery to repair the heart. If the arteries have grown well we usually plan a single operation to close the hole (VSD) by sewing a patch over it and to open up the narrowing (pulmonary stenosis) in the heart. This is often referred to as “corrective” surgery, but it never makes the heart completely normal. You will meet the surgeon prior to the operation who will discuss surgery in detail including the risks and potential complications of the procedure.
After the operation
Most children are in hospital for 5-7 days after their operation, Most children have medicines to take at home after leaving hospital but these are only necessary for a month or so. Visits to the outpatient clinic will be necessary after the operation.
Other abnormalities
Some children with Tetralogy of Fallot have other abnormalities. The commonest of these is a genetic abnormality, where a small part of one chromosome is missing. This abnormality is called “22q11 deletion syndrome”.
Although some children with 22q11 deletion grow and develop normally, some will have learning or speech difficulties and if your child has 22q11 deletion it is important that his or her development is monitored so that help can be given early if necessary.
A blood test to check for this abnormality can be taken, but it may take some weeks for the result to come back.
The long-term future
All patients who have had Tetralogy of Fallot repair operations have an abnormal pulmonary valve.
This means further surgery may be necessary in older children and adults to replace the valve, so regular check-ups are required. Even many years after surgery new problems may arise which may require treatment.
Here’s a short video explaining pulmonary valve incompetence and valve replacement
Pulmonary Incompetence & Pulmonary Valve Replacement – YouTube transcript
this movie is about pulmonary
incompetence also called pulmonary
regurgitation and replacement of the
pulmonary valve the hearts right
ventricle pumps blood to the lungs
through the pulmonary arteries
as blood leaves the right ventricle it
passes through the pulmonary valve
the function of the pulmonary valve is
to prevent blood from returning to the
right ventricle from the pulmonary
artery
if the pulmonary valve leaks some blood
that’s been pumped out to the heart
returns from the pulmonary artery back
into the right ventricle and this is
called pulmonary incompetence or
pulmonary regurgitation there are a
number of possible causes of pulmonary
incompetence but it occurs most commonly
as a result of treatment of congenital
heart conditions such as tetralogy a
fallow or pulmonary stenosis you may
wish to view our separate movies on
these conditions the main effect that
the pulmonary incompetence has on the
heart if the leak is severe enough is to
cause the right ventricle to dilate
which means it becomes larger the heart
generally copes well with pulmonary
incompetence often for many years
however significant pulmonary
incompetence usually requires treatment
at some point and the treatment required
is replacement of the pulmonary valve
pulmonary valve replacement may require
open-heart surgery in which new valve is
sewn into place directly once the valve
has been implanted blood that is pumped
to the pulmonary artery no longer
returns to the right ventricle
it may be possible to implants a new
pulmonary valve in a keyhole technique
which avoids the need to open the chest
at an operation in the keyhole technique
a new valve is passed to the hearts
through the veins and is expanded once
it is in the correct position once a new
pulmonary valve is in place the right
ventricle usually becomes smaller
possibly returning to a normal size
regular follow-up is required after a
pulmonary valve replacement to check on
the function of the valve over time
you
One of the commonest late problems is the development of abnormal heart rhythms. These can usually be treated but can be serious and can even rarely be fatal, so regular review in outpatients by a specialist is very important even if the child appears well.
General advice for the future
Most patients with repaired Tetralogy of Fallot will lead normal active lives after their operation but may not manage as much physical exercise as a normal child. We do not usually stop children with heart disease from doing exercise but it is probably best to avoid really hard physical exercise (such as long distance competitive running) even after a successful operation.
All children with Tetralogy of Fallot will be at risk of infection in the heart (called endocarditis), before and after surgery. Such infections may be caused by infections of the teeth or gums. It is important to look after your child’s teeth and visit the dentist regularly (every 6-12 months).
Ear or body piercing and tattooing are best avoided as they also carry a small risk of infection which may spread to the heart.
For more information about endocarditis please see the link below:
