This leaflet provides information for parents and carers about aortic stenosis in children and the management and treatment of this condition.
Aortic stenosis is a narrowing of the aortic valve. This valve lets blood flow from the heart to the artery feeding the body with blood (the aorta).
Almost all children with aortic stenosis will need major heart surgery at some stage in their life, but the age at which surgery is necessary is very variable.
If the valve is severely narrowed treatment may be necessary within the first few weeks of life, but if the narrowing is mild, treatment may not be necessary until later in life.
The timing of treatment depends on how badly narrowed the valve is, how it changes as time goes by (it almost always gets worse with increasing age), and how well the heart is coping with the extra work of pumping blood through the narrow valve.
When the aortic valve is narrow the muscle of the main pumping chamber of the left side of the heart (the left ventricle) has to work harder than normal and the muscle gets thickened, just like other muscles in the body if they are worked hard. If the valve is only mildly narrowed the heart copes very well, but if the narrowing is very severe the heart cannot pump normally and this can limit how much exercise a child can manage and can even cause death if it is not treated.
Tests
Usually only simple tests such as an ultrasound scan of the heart (“echocardiogram”) and electrical recordings of the heart’s activity (electrocardiogram or ECG) are needed to make a diagnosis.
Treatment
Most children with aortic stenosis will need an operation to replace the valve at some stage in their life, but avoiding this during childhood if possible is the best option as valves do not grow and repeated operations would be needed to put a larger valve in place. When the valve is badly narrowed in childhood, we can often help to delay valve replacement by stretching the narrow valve open with a balloon (called “balloon valvuloplasty”).
This is done under general anaesthetic and involves passing a long tube with a collapsed balloon on the end of it (a balloon catheter) into the artery at the top of the leg (or the arm in babies) and feeding it up into the heart and across the narrow valve. Using Xray pictures, the balloon is positioned in the narrow valve and is blown up, stretching the valve open.
This never makes the valve normal and it is not always successful, but in many cases it can reduce the severity of the narrowing so surgery can be delayed until the child is older, possibly an adult. However, it can also make the valve leak which may necessitate valve replacement. Sometimes balloon valvuloplasty can be repeated as the patient grows to further delay the need to replace the valve. Usually only one night’s stay in hospital is necessary after the balloon procedure.
If the valve cannot be stretched open using a balloon, open heart surgery will be needed. This involves opening the chest and the function of the heart and lungs are taken over by a machine so the surgeon can open the heart and cut the narrow valve open (called a “valvotomy”) or replace the valve. Usually about a week’s stay in hospital is necessary after this surgery.
If the valve needs to be replaced, there are two main options:
1) Metal Valve Replacement
To replace the aortic valve with an artificial metal one, because they last longer. Following a metal valve replacement, a drug called warfarin, will need to be taken to reduce the risk of a blood clot forming across the metal valve.
2) Ross Procedure
Surgery to replace the valve using an animal or human tissue valve. This is called the Ross Procedure. The pulmonary valve replaces the narrowed aortic valve, then a ‘tissue valve’ (usually from a pig, or a human valve) is used to replace the pulmonary valve. This video explains more about this operation.
Ross Procedure
This video explains the ros procedure an
operation that can be used to treat
congenital aortic valve disease in some
patients for an explanation of
congenital aortic valve disease you may
wish to watch our video on bicuspid
aortic valve when aortic valve surgery
is required the most common operation is
to implant a new valve either mechanical
or tissue we explained these in our
video on bicuspid aortic valve in some
patients the ros operation is an
alternative option to standard valve
replacement
on the left side of the heart blood is
pumped out of the left ventricle to the
body via the aorta
the aortic valve opens to let the blood
out and closes to prevent blood
returning into the heart
on the right side of the heart blood is
pumped out of the right ventricle to the
lungs via the pulmonary artery the
pulmonary valve opens to let blood out
and closes to prevent blood returning to
the heart
in a ros operation the diseased aortic
valve and a small amount of the aorta
are removed
the patient’s own pulmonary valve and a
small amount of the pulmonary artery are
also then removed and sewn into where
the aortic valve used to be to become
the new aortic valve
a short tube containing a valve called a
homograft is then sewn in to become a
new pulmonary valve the homograft
originally comes from a human donor
having been preserved until required
the advantages of the ros operation are
that there is no requirement to take
blood-thinning warfarin treatment and in
children the new aortic valve can grow
as the child grows
lifelong follow-up is required after a
ros operation to monitor the function of
the new aortic valve and the homograft
All treatment for aortic stenosis has potentially serious risks for the patient. If your child requires surgery or treatment in the catheter lab, the surgeon or cardiologist will talk to you about this in detail.
General advice for the future
Regular check-ups in the outpatient clinic are very important even if the patient appears perfectly well.
Unless the valve is only slightly narrowed, it is usually best to avoid competitive sporting activities. In most cases PE at school can continued to be enjoyed but more intensive exercise should be avoided. Your cardiologist, specialist nurse, or physiotherapist will answer any questions you have about individual recommendations for your child.
Children with aortic stenosis will be at risk of infection in the heart (called endocarditis), both before and after treatment.
Such infections may be caused by infections of the teeth or gums. It is important to visit the dentist regularly (every 6-12 months) and to follow good dental hygiene.
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:
