Marfan Syndrome is a genetic condition, which may affect more than one member of the same family.
The underlying problem involves the elasticity of body tissues. Fibrillin is the substance which gives tissues their strength and flexibility, and this is defective in people with Marfan Syndrome.
​People with Marfan Syndrome are usually exceptionally tall with long fingers and rather flat feet. They may have a spinal curve (scoliosis) and be shortsighted. The skin may develop stretch marks. The two heart problems that are most serious are enlargement of the main body artery and leaky heart valves.
When the main body artery stretches (aortic aneurysm) it becomes weaker and blood can leak through the wall (dissection or rupture). This causes severe chest pain and if the blood leak is rapid the circulation can fail and the patient may die. To prevent this happening it is important to monitor the size of the aorta with regular scans and to reduce the pressure within it with medication. If the artery is so large that it in danger of leaking it is replaced with a special tube. These measures have vastly reduced the chances of aortic rupture occurring.
Another heart problem that can occur is leaking of the heart valves. One of the most common heart problems is called mitral valve prolapse. The leaks are often mild and require no treatment but sometimes surgery is necessary.
Marfan Syndrome is a variable condition with some people only very mildly affected. In childhood, it rarely causes difficulties. Sometimes it can be difficult to be sure whether or not the condition is present as not everyone has all the signs and there is no reliable test. If this is the case, then it may be necessary to review the children as they grow older to see if they truly have the condition or not.
Children with congenital heart disease are at an increased risk of having a heart infection. Whilst this is rare the chances of it occurring can be reduced by taking precautions.
Infections in the heart can occur for no apparent reason but are more common if the teeth are rotten. Germs spread into the blood stream and infect the heart. Good dental hygiene is therefore important as are regular visits to the dentist.
If dental treatment is required then some procedures can cause germs to spill into the blood and infect the heart.
It is therefore important that the dentist is informed about the heart condition before treatment. The usual method of avoiding this problem is to give a single dose of antibiotics one hour prior to the treatment to kill any germs beforehand.
Children with Marfan Syndrome are restricted to light exercise (like golf, cricket), and should exclude contact sports.
Exercise is important even in those with heart disease. It improves the heart function and general sense of well being. It is associated with increased life expectancy and a reduced risk of heart disease in later life. In addition, physical activity helps with controlling weight and reducing blood pressure.
There are different types of exercise. In static exercise the muscles contract but there is little joint movement eg weight lifting. In dynamic exercise the muscles contract and also move the joints eg running. Each places a different stress on the body and cardiovascular system. In general most types of sports are a mix of the two.
Children usually take part in more rigorous exercise at school as they grow older. In Singapore, physical education (PE) tends to teach games skills rather than competitive sports. However, pupils may take sport for their co-curricular activities (CCA) when training is more intense and competitive. Training for the NAPFA test is also intensive, and some pupils with heart problems may have difficulty with the 2.4km run as they often perform less well at endurance type activities.
Parents should seek the doctor's advice when deciding how much exercise and to what level is safe - particularly as there are no published guidelines for activity levels in children.
Most children with heart disease can have all the normal vaccinations at the appropriate time. However some children with an immune deficiency (DiGeorge syndrome or an isomerism) and those who are receiving immunosuppression - for example following transplantation require a different vaccination schedule.
Before travelling anywhere unusual or a long distance, make sure your child has:
Those with cyanotic heart disease ("blue" due to reduced oxygen in the blood) can still travel but aircraft at altitude have less oxygen in the air than at ground level, so the blueness may be more apparent. This does not usually cause symptoms but if necessary, airlines can arrange for additional oxygen to be available on the aircraft.
For long aircraft flights it is sensible to use the support stockings and take aspirin or an equivalent unless your doctor advises against it.
Special diets are not normally necessary for those with heart disease. As with everyone it is important to have a balanced diet and not to eat to excess. It is however important to maintain a normal weight. Excess weight means more work for the heart.
Most children with heart disease are no more prone to infections than any other children. Some however are likely to get chest infections - particularly those with holes in the heart (ASD, VSD, PDA).
In addition, some heart diseases are also associated with an immune deficiency and infections therefore are more common. The majority of childhood infections are viruses and get better without antibiotics. In any case of doubt professional medical help should be sort and the doctor will decide if antibiotics are necessary.
Most children with heart disease do not require medication. Some however need them to:
The majority of these medicine have been used for many years and are very safe but like all drugs side effects may occur, especially if there is another illness or a change in other medication. If unusual symptoms or side-effects occur whilst on medication, it is important to inform the doctor immediately.
Click here to access our Find A Doctor directory for a list of doctors treating this condition across our NUHS institutions.
