Congenital heart disease means that there is an abnormality. of the heart which has been present since birth. It occurs in

Transcription

1 Congenital heart disease 1General information Congenital heart disease means that there is an abnormality of the heart which has been present since birth. It occurs in about 8 of every 1,000 children born alive. About a third of these children have mild heart abnormalities and never require any treatment. In the normal heart the blue blood on the right side and the red blood on the left side are separated by partitions or septa so the blue and red blood do not mix. The valves in the heart, and in the arteries arising from the heart, open completely in the normal heart to allow the blood to flow freely through them. They then close completely to prevent any blood leaking backwards. Cause It is important to emphasise that the abnormality is not the fault of the parents. In most cases the cause of heart disease is unknown. The heart forms in the early weeks of pregnancy between the 6th and 12th weeks, often before a mother realises that she is pregnant. There is a small family (genetic) component and a high incidence in various syndromes such as Down s and Turner s. Some viral infections in the pregnant mother such as German measles, may cause abnormalities of the heart. We know that if the mother has diabetes there is an increased risk of the child having a heart defect. We also know that smoking, excessive intake of alcohol or drug taking can also lead to cardiac abnormalities. In the majority of our patients we do not find a cause. Function of the heart The normal heart works like a pump. On the right side of the heart there is a filling part (the right atrium) which receives blue blood from the body and this blood then passes through the tricuspid valve to the pumping part of the heart on the right side (the right ventricle) and the blood is then pumped through the pulmonary valve into the pulmonary artery and goes to the lungs. The blood receives oxygen and returns in the pulmonary veins to the filling part of the heart on the left side (the left atrium). It then passes through the mitral valve to the pumping part of the heart on the left side (the left ventricle) and is pumped through the aortic valve to the aorta which is the main artery supplying the head, body and limbs. You read in the newspapers of a baby having a hole in the heart as though only one abnormality existed, but in fact there are a lot of different abnormalities which can occur and you will be supplied with information only about the defect which your child has. Immunisation It is very important that your child is immunised and vaccinated in the routine way at the usual times and this should only be postponed if the child is ill. Development Babies with heart disease may be slower to sit up and walk but they make normal progress mentally. Even though a child is blue, the development of the brain is normal.

2 Further children The risks to parents who have had one child with heart disease having another child with heart disease are about 1 in 50. It is now possible to examine the heart of the fetus in the womb so, with subsequent pregnancies, if you would like this to be done, you should feel free to ask for a fetal echocardiogram. The best time for this is between 14 and 18 weeks. Out-patient visit When your child comes to the clinic s/he will be examined by a specialist in heart disease in children (a paediatric cardiologist). A heart murmur is a noise which the doctor hears with a stethoscope and he can sometimes tell what is wrong from the character of the noise. The child may have a chest x-ray and an electrocardiogram (ECG). If necessary s/he will also have an ultrasound scan of the heart (echocardiogram). This allows the doctor to look at the inside of the heart on a television screen and see the movement of the heart valves and the heart muscle. The doctor may also see holes between the two sides of the heart. If the heart abnormality is very mild, the scan may appear normal. Care of teeth It is important that you look after your child s teeth well from the very beginning. Avoid sweet foods and drinks between meals or at bedtime. Use a fluoride toothpaste and brush the teeth and gums thoroughly especially at night. Regular checks at the dentist should be made. It is a good idea to take children along even as babies so that they get used to the idea of a dentist looking in their mouths. You should consult your family doctor and dentist about giving fluoride. Endocarditis Some children with heart defects may develop a serious infection inside the heart. This is called endocarditis. It is usually caused by germs entering the blood from the mouth either spontaneously or sometimes after dental treatment or following an infection in the throat, ears or chest. Prevention of endocarditis is very important. An endocarditis card is available free of charge from the British Heart Foundation, which you should show to your doctor and dentist. If it does occur the child needs to come into hospital for treatment with antibiotics. A series of three videos, Right from the start is available from the British Heart Foundation for parents of children with congenital heart disease. These videos look at Children and heart disease, Children in surgery and intensive care, and Children and catheterisation. No charge is made but a donation of 5 per video towards the Foundation s costs is invited. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/1 Updated & reprinted 05/01

3 Congenital heart disease 2Intensive care unit Babies and children with heart problems can be treated with medicines, by catheter methods like balloon dilatation of narrow valves or by surgery. Most patients are nursed in an intensive care unit immediately after surgery and some who are having medical treatment or who have catheter interventions also require intensive care.the length of stay depends on the type of surgery but the majority of patients only stay for 1-3 days and are then returned to the cardiology ward. If surgery has been performed inside the heart and the heart/lung bypass machine has been used, the patient usually requires assisted ventilation. This means that s/he has a tube in his/her windpipe and this is connected to a ventilator which helps the child to breathe and get adequate amounts of oxygen into his/her blood. It is necessary to monitor the child s heartbeat on an electrocardiogram. Tubes are inserted into a vein and an artery both to measure the pressure in them and to take samples of blood for chemical analysis. Drugs can be given through these tubes to help the function of the heart when necessary. There are also drainage tubes coming from the chest so that blood oozing from the tissues can drain freely and does not accumulate and embarrass the heart. When you first see your child on the intensive care ward, s/he will seem to be surrounded by tubes and machines but these are essential to ensure the best possible intensive care. The tube to the lungs is removed as soon as possible when the doctor knows that the child can breathe adequately without help. The other tubes are removed at times indicated by the child s progress and s/he will then return to the ward. Doctors and nurses may want to discuss your child s progress among themselves and you may be asked to wait outside while this is done. The sisters and nurses will tell you when you may come and go. We try to keep you informed about your child s progress but if you want to ask questions or see a particular doctor or nurse, please say so. We are all busy but will always find time to speak to parents about a sick child.

4 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/2 Updated & reprinted 05/01

5 Congenital heart disease 3Tetralogy of Fallot Definition There are four things wrong with the heart (tetra = Greek for four) 1 A narrowing in the way out from the right side of the heart to the lungs (pulmonary stenosis) 2 A hole in the partition between the two pumping chambers of the heart (ventricular septal defect) 3 The aorta is over-riding the hole and receives blood from both ventricles therefore allowing some deoxygenated (blue) blood to go round the body. 4 The right ventricular muscle gets thickened as it has to work harder trying to push blood through the narrowed pulmonary valve.the greater the narrowing the more blue blood goes around the body.this leads to the baby looking dusky. Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Narrow pulmonary valve Narrow outlet Superior vena cava Inferior vena cava Normal Aorta Reduced pulmonary blood flow Pulmonary artery Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Large over-riding aorta Ventricular septal defect Thick right ventricle Tetralogy of Fallot

6 Symptoms The condition presents in two ways. a A gradual increase in the degree of blueness (cyanosis) and breathlessness. At birth the baby may appear pink and have no symptoms. The blueness may first be noticed when the child cries or exercises. b The baby has a sudden attack of blueness and breathlessness around the age of four months and may appear to lose consciousness. These attacks commonly occur in the mornings after breakfast. Further investigations A chest x-ray, an electrocardiogram and an echocardiogram will be performed to confirm the diagnosis. A cardiac catheterisation may be necessary to show the degree of narrowing of the branch pulmonary arteries. Treatment An operation is always needed but sometimes medicines may be given to relieve the blue attacks temporarily. Operation There are three ways of helping the child by operation and the decision about which to do depends on the child s size when operation is necessary and also the type of narrowing and the size of the arteries supplying the lungs. 1 A shunt operation which is a helping operation and will be followed at some time by a corrective operation (see point 2). In this operation some of the blood which normally goes round the body is diverted to the lungs and this makes the child less blue and less breathless. The operation is done without a heart-lung machine and the risk of it is relatively small. The scar is usually at the side of the chest. An additional heart murmur will be heard after the operation due to the extra blood flowing to the lungs. 2 Corrective operation. A heart-lung machine has to be used and the scar is in the middle of the chest. The narrowing is removed and the hole closed. There is a change in the heart murmurs heard after the operation because the narrowed valve is cut and often leaks a little afterwards but this usually does not matter. Later in life some patients require further operations because of this leak. Follow-up The patient will be seen at outpatients after surgery at first frequently and then at yearly, two yearly or three yearly intervals. Prevention of endocarditis This must be continued even after successful surgery. Always show your endocarditis card, which is available free of charge from the British Heart Foundation, to your doctor and dentist. 3 Balloon dilatation of the narrowed pulmonary artery is rarely performed. A balloon catheter is inflated in this region and stretches the narrowed portion. This allows more blood to get to the lungs and the procedure is performed during cardiac catheterisation. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/3 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

7 Congenital heart disease 4Aortic stenosis Definition This is a narrowing of the outlet valve from the left side of the heart.the pumping part of the heart on the left side (the left ventricle) has to work harder to force blood through the narrowed area.the degree of narrowing may be mild, moderate or severe. Symptoms Most patients do not have symptoms but they may occur with severe narrowing. The patient may have dizzy attacks or lose consciousness or experience chest pain. If these occur the child should see the cardiologist again. Restrictions Are only necessary when narrowing is moderate or severe. The child should not take part in competitive sports or cross country running. Further investigation Echocardiography is helpful in assessing the degree of narrowing. Cardiac catheterisation is advised if surgery is thought necessary. The pressure change across the valve is measured and dye injected to look at the movement of the valve. Superior vena cava Aorta Pulmonary artery Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Narrowed aortic valve Inferior vena cava Normal Aortic valve stenosis

8 Treatment The usual first line of treatment is balloon dilatation of the valve which is performed during cardiac catheterisation. The valve may leak a little after the procedure. If ballooning is unsuccessful open heart surgery is sometimes necessary. Later in life the valve may need to be replaced even if the initial ballooning or surgery was a success. Follow-up Children with mild aortic stenosis are seen at infrequent intervals at the outpatient clinic to ensure that the degree of narrowing does not increase with age. Children who have had ballooning or surgery must also be reviewed at outpatients, and will have echocardiography tests. Cardiac catheterisation may have to be repeated if the echocardiogram suggests that the narrowing has become severe enough to need relief. Prevention of endocarditis Must always be practised before and after the operation. Always show the endocarditis card, which is available free of charge from the British Heart Foundation, to the doctor and dentist. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/4 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

9 Congenital heart disease 5Transposition of the great arteries Definition The main arteries come from the wrong side of the heart.the aorta which goes round the body comes from the right sided pumping part (instead of the left) so blue blood is pumped round the body.the pulmonary artery which goes to the lungs comes from the left sided pumping part (instead of the right) and red blood goes again to the lungs.the baby can only survive if some of the red blood crosses over to join the blue blood to go round the body.the red blood can cross through a hole between the filling parts of the heart or between the pumping parts of the heart or through the ductus arteriosus. Superior vena cava Aorta Pulmonary artery Atrial septum Right atrium Pulmonary valve Left atrium Mitral valve Aortic valve Aorta from the right ventricle Pulmonary artery from the left ventricle Tricuspid valve Left ventricle Right ventricle Ventricular septum Inferior vena cava Normal Transposition of the great arteries

10 Symptoms These occur very early in life, usually in the first few days. The baby is blue and breathless and unable to feed. If there is a good opening between the two sides of the heart, symptoms will be delayed. Treatment The baby s condition may be improved by a balloon septostomy. In the newborn baby there is always a small hole between the two upper chambers of the heart. When a balloon septostomy is performed, this hole is enlarged by passing a balloon catheter through a vein in the groin into the heart and across this little hole. The balloon is inflated and pulled back thus enlarging the hole and thereby allowing more red blood to reach the body. This may be performed in the intensive care unit or in the catheter laboratory. This will tide the baby over until the 'arterial switch' operation can be performed. Operation The usual operation is one in which the aorta and pulmonary artery are disconnected and 'switched' to the correct place. The arteries supplying the heart, the coronary arteries, also have to be transferred. Any holes within the heart are also repaired. This surgery is done using the heart-lung machine and the scar is in the middle of the chest. The best time for this operation is within the first month of life. Follow-up The patient will be seen at regular intervals in the outpatient department. Prevention of endocarditis This must be practised for life. Always show the endocarditis card, which is available free of charge from the British Heart Foundation, to the doctor and dentist. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/5 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

11 Congenital heart disease 6Hypoplastic left heart syndrome Definition In this condition, the left side of the heart has not developed properly and is very small.the main artery supplying blood to the body (the aorta) is minute and blood can only reach it through a patent ductus arteriosus.when this duct closes, as it usually does, the baby dies. Symptoms The baby often seems normal at birth but sometime between the first and third day, the baby collapses, becomes grey and breathless and is unable to feed. Without treatment most babies die within the first week of life. Investigations The diagnosis is made by echocardiography. It can be made as early as the 14th-18th week of pregnancy and may be seen on the usual obstetric ultrasound scan although it can develop later in pregnancy. Treatment Unfortunately there is not a curative operation for this condition but now most centres are able to offer a palliative (helping) procedure. This is known as the Norwood procedure and involves three stages. Superior vena cava Aorta Pulmonary artery Ductus Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Small aorta Atrial Hole Blocked aortic valve Narrow mitral valve Small left ventricle Inferior vena cava Normal Hypoplastic left heart syndrome

12 (a) The first stage involves using the large lung vessel (pulmonary artery) to enlarge the small aorta and then attaching the right subclavian artery to a branch of the pulmonary artery with a tube. The hole between the two atria is also enlarged. This is a complex operation which requires the use of the heart/lung bypass machine and carries significant risk. (b) The next stage is carried out at about 5-6 months of age. The superior vena cava (upper body vein) is connected to the pulmonary artery. (c) In the third stage, at about a year later, the inferior vena cava (the lower body vein) is connected to the pulmonary artery. An alternative treatment would be to perform heart transplantation but unfortunately donor organs are in very short supply for this age group. Follow-up The child will have to be seen frequently in the outpatient department (the long term outlook is unknown). Prevention of endocarditis This must be practised for life. Always show the endocarditis card, which is available free of charge from the British Heart Foundation, to the doctor and dentist. Support Group Left Heart Matters is a UK support group for families with children who have Hypoplastic Left Heart Syndrome. Their helpline number is Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/6 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

13 Congenital heart disease 7 Ventricular septal defect (small) Definition This is a hole in the muscular partition between the two pumping chambers (ventricles) of the heart. Blood passes through the hole from the left sided chamber to the right sided chamber and, as a result, the blood flow to the lungs is slightly increased. Defects of this sort usually become smaller and may close by themselves as the child grows.this may take several years - sometimes even into adult life. Symptoms Further investigation Treatment Children with small defects do not have The condition can usually be identified by No treatment or operation is required symptoms and their activities need not be listening to the heart with a stethoscope. except for the prevention of endocarditis. restricted in any way. Simple tests such as an echocardiogram (ultrasound scan) can show the hole, its size and position and usually measure the pressures across it. Superior vena cava Aorta Pulmonary artery Atrial septum Left atrium Mitral valve Aortic valve Right atrium Pulmonary valve Increased bloodflow to the lungs Tricuspid valve Left ventricle Right ventricle Ventricular septum Enlarged left ventricle Ventricular septal defect Enlarged right ventricle Inferior vena cava Normal Ventricular septal defect

14 Prevention of endocarditis There is a small risk of an infection developing around the hole. This illness is called infective endocarditis and it is usually caused by germs which live in the mouth. It is very important to take good care of your child s teeth and to visit the dentist regularly. Make sure that you have a card which gives advice about precautions to prevent this illness. This risk is also present during some surgical operations including ear-piercing. Mention the hole in the heart if your child goes into hospital for any operation. An endocarditis card is available free of charge from the British Heart Foundation, which you should show to your doctor and dentist. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/7 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

15 Congenital heart disease 8 Ventricular septal defect (large) Definition This is a hole in the muscular partition between the two pumping chambers (ventricles) of the heart. Blood passes from the left sided chamber to the right sided chamber and as a result, the blood flow to the lungs is increased and the heart has to do more work than usual.the blood pressure in the lung artery is usually much higher than normal. Symptoms size and position can usually be seen on which is required. If the hole does not Babies with a large defect are usually an ultrasound scan (echocardiogram) of become smaller as the child grows, as it breathless, difficult to feed and do not the heart. may do, then an operation will be gain weight as quickly as they should. needed. If surgery is necessary, a further Treatment test, (called a cardiac catheterisation) may Further investigation At first the treatment will be with be needed to measure the A chest x-ray and an electrocardiogram medicines which help the heart to cope pressures inside the heart. This test (ECG) will be performed and the hole, its with the increased amount of work involves an overnight stay in hospital. Superior vena cava Aorta Pulmonary artery Atrial septum Left atrium Mitral valve Aortic valve Right atrium Pulmonary valve Increased bloodflow to the lungs Enlarged left ventricle Ventricular septal defect Tricuspid valve Left ventricle Right ventricle Ventricular septum Enlarged right ventricle Inferior vena cava Normal Large ventricular septal defect

16 The baby is sedated and a tube is passed from a vein at the top of the leg into the heart chambers. Pressures are measured and a special dye can be injected to obtain X-Ray pictures of the hole. Operation The operation is done using a heart-lung bypass machine to take over the circulation whilst the surgeon opens the heart and closes the hole with a patch. The operation is normally done before 2 years of age, with a midline chest incision. The child may be in hospital for 7-10 days with a couple of days in the intensive care unit. Prevention of endocarditis This should be practised before the operation and for a few years afterwards but may not be necessary as long as there is no residual leak. An endocarditis card is available free of charge from the British Heart Foundation, which you should show to your doctor and dentist. Further supervision Often children may be discharged after a few years. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/8 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

17 Congenital heart disease 9Atrial septal defect Definition This is a hole (or defect) in the partition between the two filling parts of the heart (the atria). Some of the red blood from the left side of the heart flows through the hole to the right side and there is an increased amount of blood flowing to the lungs. Symptoms The defect does not usually cause symptoms during childhood but in adult life or even as late as middle age, symptoms are common. This is due to the effects of the increased amount of blood flowing through the heart to the lungs gradually damaging the heart muscle over the years. Further Investigation The diagnosis can usually be confirmed by echocardiogram (ultrasound scan). Treatment Some atrial septal defects may be suitable for closure with a device introduced through a vein in the groin at the time of cardiac catheterisation. The device is shaped like a clam shell and the two halves of the shell are placed on either side of the defect thus closing the hole. If the defect is not suitable for this method of closure then surgery will be necessary. Superior vena cava Aorta Pulmonary artery Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Atrial septal defect Inferior vena cava Normal Atrial septal defect

18 Operation This is performed usually before school age but may be delayed until later. It is wise to close the defect before the heart becomes too enlarged and the muscle stretched. The heart will then gradually return to normal size. The operation requires the use of the heart/lung bypass machine and the scar may either be in the middle of the chest or under the arm. Follow-up Often children may be discharged after a few years of follow up. Prevention of endocarditis This should be practised for a year after closure of the defect but then as long as there is no residual hole, it may not be necessary. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/9 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

19 10 Coarctation of the aorta in older children Definition Congenital heart disease This is a narrowing of the main artery (the aorta). It is present at birth but becomes more significant as the patient grows. Symptoms Usually none. Sometimes children tire easily and have cramps in the legs. It is usually diagnosed because a heart murmur is heard or a high blood pressure is found in the arms, and the pulses in the legs are weak. Investigations The diagnosis is usually made by echocardiography but occasionally other tests, such as magnetic resonance scanning, may be necessary. This is done as an out-patient and is not painful nor does it have any risk. Treatment The narrowing needs to be relieved. There are two ways of doing this. Surgery is performed, without the heart-lung machine, via an incision in the left side of the chest and has been performed for many years. Recently balloon dilatation of the narrowed part has been introduced. A balloon catheter is introduced through the artery in the groin and the aorta is stretched from the inside. For balloon dilatation the stay in hospital is usually only 1 or 2 nights. Follow-up Infrequently at outpatient clinics. Prevention of endocarditis This should always be practised before and AFTER operation. An endocarditis card is available free of charge from the British Heart Foundation, which you should show to your doctor and dentist. Superior vena cava Aorta Pulmonary artery Coarctation Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Thick left ventricle Inferior vena cava Normal Coarctation of the aorta

20 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/10 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

21 11 Coarctation of the aorta in babies Definition This is a tight narrowing of the main artery (the aorta). Congenital heart disease Symptoms The left side of the heart (the left ventricle) has to pump at a higher pressure to force the blood through the narrowed artery and it usually fails to do this. The baby becomes breathless, is unable to feed normally and becomes shocked and pale. Urgent admission to hospital is usually necessary in the first weeks of life. Further investigation The narrowing of the aorta will be seen on the echocardiogram (ultrasound examination). If there are other heart defects then cardiac catheterisation may be necessary. Treatment This is usually required urgently and most often an operation has to be performed via an incision in the left side of the chest. This operation does not need the heart-lung machine. Sometimes the coarctation may reoccur as the child grows but this can often be treated by dilatation with a balloon (without an open surgical operation). Follow-up This is necessary at the outpatient clinic, frequently at first and then less often as the child improves and grows. Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Superior vena cava Aorta Pulmonary artery Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Region of aortic narrowing Patent ductus Arteriosus Inferior vena cava Normal Coarctation of the aorta

22 Associated defects Some babies have other abnormalities in the heart, most commonly ventricular septal defects and aortic stenosis. The management of these is the same as when these defects occur alone but their presence will delay recovery from operation on the coarctation. Prevention of endocarditis This should always be practised before and after operation. An endocarditis card is available free of charge from the British Heart Foundation, which you should show to your doctor and dentist. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/11 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

23 12 Pulmonary stenosis Definition Congenital heart disease This is a narrowing of the valve in the artery going to the lungs.the pumping part of the heart on the right side (the right ventricle) has to work harder to force the blood through the narrowed area.the degree of narrowing may be mild, moderate or severe. Symptoms Usually none. Diagnosis The diagnosis is made, and the severity of the narrowing assessed, by echocardiography. Supervision If surgery is not required the child is observed at outpatients every 2 or 3 years as s/he grows, because the narrowing may become more significant with growth. Superior vena cava Aorta Pulmonary artery Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Inferior vena cava Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Thickened pulmonary valve Thick right ventricle Normal Pulmonary valve stenosis

24 Treatment When the narrowing is severe enough to need treatment this is performed by balloon dilatation. A special balloon catheter is inserted into the vein in the groin and passed into the heart, being positioned inside the narrowed valve. It is then inflated and the valve stretched from the inside. There is no incision in the chest and the hospital stay is only 1 or 2 nights. Follow-up This will continue at the outpatient clinic. Prevention of endocarditis This should always be practised before and after the procedure. An endocarditis card is available free of charge from the British Heart Foundation, which you should show to your doctor and dentist. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/12 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

25 Congenital heart disease 13 Persistent ductus arteriosus Definition In the normal heart the ductus arteriosus (which is open in the infant before it is born) closes after birth. In some children, however, it remains open and allows blood to flow from the aorta (the main artery going around the body) to the lungs.the lungs receive an increased amount of blood and the heart has to do extra work. Symptoms Diagnosis umbrella-like device or wire coils are Most children do not have symptoms, The diagnosis is made by positioned in the duct using a special but if the ductus is large the infant echocardiography. catheter introduced via the vein in the becomes breathless and unable to finish groin and passed into the heart. When in feeds and does not thrive. Older children Treatment position, the umbrella is opened and may be easily tired and more breathless The duct should always be closed. released or the coils are introduced on exertion than their friends. Surgery is rarely necessary except perhaps thereby closing the duct. When the duct is in premature babies. Nowadays an large a second umbrella or more coils Superior vena cava Aorta Ductus Pulmonary artery Increased blood flow to the lungs Atrial septum Left atrium Mitral valve Aortic valve Right atrium Pulmonary valve Tricuspid valve Left ventricle Right ventricle Ventricular septum Enlarged left ventricle Inferior vena cava Normal Persistent ductus arteriosus

26 may have to be put in later if complete closure has not been achieved. There is no incision in the chest and the hospital stay is only 1 or 2 nights. Operation If surgery is required to close the duct, a small incision is made in the left side of the chest. The duct is either clipped or tied thereby blocking the flow of blood. The by-pass machine is not required and recovery is usually rapid. Follow-up After the duct has been closed for 1-2 years, the patient may be discharged. Prevention of endocarditis This is not necessary after the first year following the procedure. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/13 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

27 14 Innocent heart murmur Congenital heart disease An innocent heart murmur is a noise which occurs in a perfectly normal heart. If we listen to children s hearts in ideal quiet conditions, innocent murmurs can be heard in about 3 /4 of them. Such murmurs come and go and vary with breathing, with the heart rate and with the position of the child. An experienced cardiologist recognises the noise of an innocent murmur as being quite different from a murmur associated with a heart defect. When you are told that the murmur is innocent there is no need for any further visits to the cardiologist and you must treat the child as normal and forget about the murmur, which usually disappears at puberty. No special care is necessary for dental treatment. These noises are sometimes called: Vibratory murmurs Venous hums Functional murmurs but these terms all refer to innocent murmurs.

28 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/14 Updated & reprinted 05/01

29 15 Heart catheterisation Congenital heart disease Heart catheterisation, usually called cardiac catheterisation, is needed in some babies and children either to obtain more detailed information on the heart problem or to treat the heart problem, for instance by balloon dilatation. The child is admitted to hospital the day before the test and discharged the day after, so s/he is in hospital for two nights. Accommodation for parents of young children is arranged whenever possible. The child is examined in hospital by the doctor who will perform the test. The test is done in the X-Ray Department and the patient will be given a sedative before going there. S/he will be looked after by nurses in the room and will either have a general anaesthetic or the doctor will put some local anaesthetic at the top of the patient s leg. S/he will feel a slight sting but then afterwards the test is not painful. The catheter is a fine flexible hollow tube and it is inserted into a vein and/or an artery at the top of the leg and passed into the heart. There is no sensation from the catheter being in the heart and the child usually goes to sleep. Samples of blood are taken from the heart to measure the oxygen in it and pressure measurements in the heart are recorded. An angiocardiogram is then done in which dye (which shows up on an X-Ray) is injected through the catheter and a cine film is taken to show the passage of the dye through the heart. This shows structural abnormalities. The test usually lasts 1 to 2 hours. When the catheterisation is done to treat a heart abnormality the procedure is very similar. Special catheters for instance those with a balloon at the tip are used but the child is not aware of any difference from the diagnostic procedure. It lasts about the same time and, in most instances has a similar low risk. The risk of heart catheterisation is very small. It is not done unless it is essential. The risks are greater in babies who are very blue and breathless with complex abnormalities. It takes some time for all the information from the heart catheterisation to be analysed. The results of the test and possible treatment plans are discussed with the surgeon and then together with the parents the management is planned.

30 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/15 Updated & reprinted 05/01

31 16 Cardiomyopathy There are a group of diseases which affect the heart muscle. Among them are Hypertrophic Congenital heart disease Cardiomyopathy otherwise known as Hypertrophic Obstructive Cardiomyopathy (HOCM) and Dilated Cardiomyopathy. Hypertrophic Cardiomyopathy Definition In this condition the heart muscle becomes very thick and in some cases obstructs the flow of blood from the heart. Superior vena cava Symptoms Many cases are mild and have no symptoms but others have dizziness, fainting attacks, abnormal heart rhythm and chest discomfort. New born babies with this condition may have more severe symptoms. Cause The cause is often unknown but it may well be familial and it is advisable to have other children checked. Investigations Chest x-ray, electrocardiogram and echocardiogram will confirm the diagnosis. Blood tests will be carried out to try and discover the cause. Aorta Pulmonary artery Treatment Long-term medications to help improve relaxation of the heart muscle and to control the abnormalities of heart rhythm may be necessary. Transplantation may be considered. Follow-up Regular checks in the outpatient department are required. Prevention of endocarditis This must be practised for life. Always show the endocarditis card, available from the British Heart Foundation, to your doctor and dentist. Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Thick right ventricle Thick left ventricle Thick septum Inferior vena cava Normal Hypertrophic cardiomyopathy

32 Dilated Cardiomyopathy Definition In this condition the heart muscle becomes weak, loses its elasticity and results in the left ventricle, especially, becoming dilated. Cause The cause is often unknown, but it may be as a result of a previous viral infection. Sometimes it is familial. It may be advisable to have other family members screened. Symptoms Children present with breathlessness, feeding difficulties, poor weight gain and recurrent chest infections. Occasionally, babies are born with this condition and have severe symptoms. Investigations Chest x-ray, electrocardiogram and echocardiogram will confirm the diagnosis. Blood tests will also be undertaken to try and discover the cause. Treatment Long-term medications to help the heart work more efficiently and to remove extra fluid are needed. Most children gradually improve, but in some, the damage to the heart muscle is more severe and their condition gets worse. If this happens despite large doses of various medicines, then transplantation may be considered. Follow-up Regular checks in outpatient department are required. Endocarditis prophylaxis may be necessary in certain cases. For further information please contact: The Cardiomyopathy Association on Superior vena cava Aorta Pulmonary artery Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Enlarged left atrium Dilated thin walled left ventricle Inferior vena cava Normal Dilated cardiomyopathy 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Adelaide Tunstill RSCN and Philip Rees FRCP M27/16 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

33 17 Complete atrio-ventricular septal defect Definition Congenital heart disease There is a large hole in the middle of the heart between the upper chambers (the atria) and the lower chambers (the ventricles).there is only one valve between the atria and the ventricles.this large hole allows too much blood to flow from the left to right side of the heart and through into the lungs.this increased blood flow at high pressure gives the heart more work to do and can also damage the lung blood vessels. If the valve leaks, this adds to the work load of the heart. (This is a common problem in patients with Down s Syndrome). Symptoms Breathlessness will occur in the first weeks of life, causing difficulty in feeding, poor weight gain and repeated chest infections. Further investigations A chest x-ray, electrocardiogram and echo-cardiogram will confirm the diagnosis. Only occasionally a cardiac catheterisation is required. Treatment Medicines are used to help keep the lungs dry. An operation is required early in life before damage occurs to the lung arteries. This is a big operation and requires the heart/lung machine. The large hole is Superior vena cava Aorta Pulmonary artery Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Atrial septal defect Single valve Enlarged right ventricle Increased pulmonary blood flow Ventricular septal defect Enlarged left ventricle Inferior vena cava Normal Complete atrio ventricular septal defect

34 closed with either one or two patches and the valve divided, with the middle portion of the valves being attached to the patch. Occasionally in small babies, the pulmonary artery is narrowed (banded) as a first stage. This will protect the lungs and allow the baby to grow before having the major operation. Follow-up Medicines are often necessary, and long term follow-up is required in the outpatient department. Occasionally repaired valves may leak significantly and further surgery may be necessary. 660 babies. It is a genetic disorder which cannot be cured. About half of the babies born with Down s Syndrome have heart defects, most of which can be corrected. For further information and support please contact: THE DOWN S HEART GROUP on tel Prevention of endocarditis This should be practised for life. Always show the endocarditis card, which is available free of charge from the British Heart Foundation, to your doctor or dentist. Down s syndrome This is a syndrome affecting 1 in 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Adelaide Tunstill RSCN and Philip Rees FRCP M27/17 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.

35 18 Partial atrio-ventricular septal defect Definition Congenital heart disease There is a hole in the lower part of the wall between the two receiving chambers of the heart and an abnormal valve between the left atrium and ventricle.this valve is attached directly to the top of the wall between the two ventricles.the hole allows an increased amount of blood to flow from the left side of the heart to the right side and if the valve leaks, additional blood is pumped backwards into the atria from the left ventricle. Symptoms Children may remain free of symptoms and the problem is picked up because of a murmur. Others may have increased chest infections and breathlessness, and this is more likely if the valve is very leaky. Further investigations A chest x-ray, an electrocardiogram and echocardiogram will confirm the diagnosis. Very rarely a cardiac catheterisation is required. Superior vena cava Aorta Pulmonary artery Atrial septum Right atrium Pulmonary valve Tricuspid valve Right ventricle Left atrium Mitral valve Aortic valve Left ventricle Ventricular septum Atrial septal defect Right sided valve Enlarged right ventricle Increased pulmonary blood flow Abnormal left sided valve Inferior vena cava Normal Partial atrio - ventricular septal defect

36 Treatment Medicines to help keep the lungs dry may be required. An operation involves patching the hole between the two upper chambers of the heart and repairing the abnormal valve. The operation is performed through the breast bone, and is usually undertaken in early childhood. Follow-up Long term follow-up is required in the outpatient department. Occasionally repaired valves may leak further and additional operations are necessary. Prevention of endocarditis This should be practised for life. Always show the endocarditis card, which is available free of charge from the British Heart Foundation, to the doctor or dentist. 14 Fitzhardinge Street London W1H 6DH Telephone British Heart Foundation is a Company Limited by Guarantee. Registered in England No Registered Charity No British Heart Foundation Compiled by Dr Olive Scott MD FRCP Dr Leon Gerlis FRCPath Adelaide Tunstill RSCN and Philip Rees FRCP M27/18 Updated & reprinted 05/01 Heart diagrams from Heart Children published by HeartLine Association.