Signs & Symptoms
Surprisingly in newborn babies even a large hole may cause no problems until the circulation has adjusted fully to being out of the womb - a period of about a month. The diagnosis is usually readily made (except in the first week or two after birth) by examining the child and hearing a heart murmur due to the blood passing through the hole.
Larger holes allow enough blood to pass into the lungs to cause breathlessness, poor weight gain and an increased susceptibility to infections.
Breathlessness can occur in small babies - particularly when they feed. It occurs in some heart conditions for example a hole in the heart or if there is a leaky heart valve. Worrying signs are if the skin is sucked in between the ribs or below the lower border of the ribs (recession).
Lung problems can also cause breathing problems and so the advice of a doctor is essential.
Breathlessness on running or other exercise is normal unless it is out of proportion to the exercise taken - as a general rule children should be able to manage as well as their friends.
Blue colouration is common in babies and young children. It is usually confined to the area around the mouth ("muzzle area") and the hands and feet. The colour change usually occurs for no apparent reason and the child is completely well. The technical term for these colour changes is acrocyanosis. It is probably caused by local variation in blood flow through the skin - as oxygen is extracted from the blood by the tissues it changes to a blue colour. It is of no consequence and usually goes away as the child gets older.
Blue colour (cyanosis) can be a sign of heart disease and in others they develop - either as a result of untreated heart disease, in this case, a large Ventricular Septal Defect.
What causes it
In the developing baby the wall between the two main pumping chambers (left and right ventricles) is formed from separate tissues and sometimes they fail to meet properly in the middle.
People with DiGeorge Syndrome are usually rather small and may be slow developers. They may have a speech impediment and feeding difficulties. Some also have an immune deficiency and infections, vaccinations and blood transfusions can cause problems.
People with DiGeorge Syndrome may suffer from heart problems; including tetralogy of Fallot, pulmonary atresia - VSD, truncus arteriosus and sometimes simpler lesions like a ventricular septal defect. These are usually treatable with surgery.
Half of all children with Down syndrome have a heart problem. These include patent ductus arteriosus, atrial septal defect, ventricular septal defect, tetralogy of Fallot and atrioventricular septal defects. Some of these may not require surgery as some may close by themselves (patent ductus arteriosus, atrial septal defect, ventricular septal defect). Tetralogy of Fallot and atrioventricular septal defects always require surgery and sometimes more than one operation is necessary.
About the condition
This is a very common problem. A Ventricular Septal Defect (VSD) allows blood that has just been through the lungs to pass through the wall (ventricular septum) and go back through the lungs again. The effect of the hole depends upon how much blood is going around the lungs and this varies upon the size of the hole and age of the child. Small holes do not allow sufficient blood across the hole to cause any problems at all. Larger holes allow enough blood to pass into the lungs to cause breathlessness, poor weight gain and an increased susceptibility to infections.
Diagnosis and Treatment Options
The hole cannot get larger - it either stays the same size or gets smaller and in many instances can go away completely. For this reason holes are not operated upon at birth but left for a few months to see how much they affect the baby and whether or not they are going to get smaller. Whilst this observation period is going on medical treatment with diuretics (medicine to reduce the fluid) and extra strength feeds may be necessary.
Surgery is required if the hole is sufficiently large to cause long term damage to the heart and lungs. Another indication for surgery is if the hole is located near to the aortic valve (subaortic VSD) because over a prolonged period of time this may cause the valve to leak. Open heart surgery is necessary to close a VSD. This allows the surgeon to close the hole (usually with a Dacron patch - cloth like material) whilst the heart is still. Surgery is very successful and once the VSD is closed further operations are usually unnecessary.
If the hole is small and not causing a problem then it is usually left alone as although the risks of surgery are small they still outweigh any benefits.
Pulmonary Artery Band is used to narrow the pulmonary (lung) artery and thus reduce the amount of blood flowing to the lungs. It may be required in certain types of single ventricle or tricuspid atresia or when there are multiple ventricular septal defects. The operation is usually performed through the breastbone although sometimes a scar is made through the side of the rib cage. The narrowing is made by wrapping a piece of surgical tape around the artery and drawing it tight. It can be difficult to get the degree of narrowing right at the first attempt and occasionally the patient will require a re-operation within a few days.
Endocarditis prophylaxis on at risk occasions is necessary for life.
Children and adults 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 who had Pulmonary Artery Band are restricted to light exercise (like golf, cricket)
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 2.4k run as they often perform less well at endurance type activities.
As always parents or patients must seek the advice of their own doctor when deciding how much exercise and to what level is safe - particularly as there are no published guidelines for activity levels in children.
The Bottom Line
- Most children with heart conditions (even following surgery) can safely participate in sport at all levels
- Some children with complex heart problems should restrict themselves to certain sports only
- For a few children competitive sport should not be undertaken
- For all a certain level of fitness is healthy and should be encouraged
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 that you:
- have a recent medical check up
- have appropriate insurance cover
- have an adequate supply of medicine
- are aware of the quality and access to local health care
- carry the relevant documentation about the heart condition
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 and 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:
- reduce the body fluids
- assist the pumping action
- control rhythm problems
- thin the blood
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.
Most women with heart disease are able to undergo a normal pregnancy and delivery.
The exceptions are those with severe cyanosis when there is usually insufficient oxygen in the blood supply to the placenta to sustain the baby. Most of these pregnancies spontaneously abort.
Women with pulmonary hypertension (high lung artery pressure) are not usually advised to get pregnant as there is a serious danger to their life.
As with any medical problem it is essential that medical advise is taken - preferably before the event so that the pregnancy can be monitored and if treatment is necessary it can be undertaken early.
If a women herself has a heart problem then there is an increased risk that her child will have a problem too. The risk is around 6%. If a man has a heart problem then the risk is 2%. If the couple have had a previously affected child then the risk is about 2%. The risks quoted above are averages - the actual risk varies considerably between the various heart conditions. There is also some evidence that taking vitamins for 3 months prior to and the first 3 months of the pregnancy can reduce the chance of heart disease occurring in the baby.