LHM Filofax June2014RedesignDec2014 · visit the LHM website at or email us on [email protected]. uk. Verified by Dr Oliver Stümper October 2014 0121 455 8982. Little Hearts Matter is

InformationFilofax

IntroductionWelcome to the Little Hearts Matter Information Filofax. It has been written to support the doctors’ and nurses’ explanations of a child’s diagnosis. The information offers a broad overview of single ventricle heart conditions and the treatments available but it does not replace the personal treatment plan that will vary for each individual child. You may wish to take this file with you to all of your medical appointments so that doctors can use the contents to help explain the treatments they plan for your child.

The Filofax is one of many support and information services that Little Hearts Matter offers to families as they learn to understand single ventricle heart disease, its treatments and the lifestyle challenges that it creates.

If, having read the pack, you would like further information, support or an opportunity to talk with other parents experiencing life with single ventricle heart disease, please ring the Little Hearts Matter team on 0121 455 8982, visit the LHM website at www.lhm.org.uk or email us on [email protected].

Verified by Dr Oliver StümperOctober 2014

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Little Hearts Matter is a national charity which offers free support to children, and their families, affected by a diagnosis of a single ventricle congenital (a condition you are born with) heart condition, half a working heart. Hypoplastic Left Heart Syndrome, Tricuspid Atresia, Complex Pulmonary Atresia, Double Inlet/Double Outlet or Univentricular Heart are the most common single ventricle conditions but there are also other conditions that may need to be treated as a one pump heart.

The charity offers specialised support and information from the time of an antenatal diagnosis, through treatment decisions, hospital treatment and life at home. The charity works to alleviate the isolation, fear and lack of understanding that often follow a new diagnosis by offering one-to-one and group support and information written to help explain the medical diagnosis, treatment and lifestyle implications that having only half a working heart creates.

You can seek information through the support line which is available from 9am to 10pm seven days a week by ringing 0121 455 8982, by emailing [email protected] or via the Little Hearts Matter website at www.lhm.org.uk. A list of further publications can be found at the back of this Filofax.

The charity’s activities can be followed via the Little Hearts Matter Facebook (www.facebook.com/LittleHeartsMatter) and Twitter feeds (twitter.com/LHM_UK) with in-depth stories and children’s and families’ personal experiences available on the parent and youth blog: heartlog.tumblr.com or zipzipper.blogspot.co.uk. Other social media links can be found on our website.

Verifi ed by Dr Oliver StümperOctober 2014

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Little Hearts Matter

Membership Application FormChild’s Forename: .............................. Surname: ..............................

Date of Birth: .............................. Male/Female: ..............................

Diagnosis: .........................................................................................

Treatment Centre: .............................. Consultant: ..............................

Stage(s) of Surgery: .........................................................................................

Member Name: .........................................................................................

Relationship to Child: e.g. Mum ......................................................................

Signature: .........................................................................................

Member Name: .........................................................................................

Relationship to Child: e.g. Dad ......................................................................

Signature: .........................................................................................

Siblings’ Names & .........................................................................................

Dates of Birth: .........................................................................................

Address: .........................................................................................

.........................................................................................

.............................. Postcode: ..............................

Telephone Number: .........................................................................................

Email Address: .........................................................................................

Other Information: .........................................................................................

Please complete and return to Little Hearts Matter, 11 Greenfield Crescent, Edgbaston, Birmingham, B15 3AU or visit www.lhm.org.uk and click on ‘Register’.Full membership of Little Hearts Matter is open to anyone with a single ventricle heart condition, as well as their parents, grandparents, legal guardians and siblings. Members must be at least 18 years old and must be UK residents or citizens. Anyone who meets these criteria qualifies for full membership - please use a separate piece of paper if you would like to register additional members, making sure that we have their correct details. Membership is free.


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I would like to be put in touch with families in my local area.

I would be interested in sharing our story in the media.

Identifying Key Hospital And Community StaffWhen you first enter the world of congenital cardiac care it can be very difficult to know who everyone is and what they do.

In this section of the Filofax we hope to help you understand a little more about the people you might meet from the time a baby is diagnosed with a complex heart condition onwards.

Antenatal TeamThe antenatal team is usually made up of a number of obstetric (pregnancy) and cardiac staff.

Midwife The nurse specially trained to help with the pregnancy and delivery of the baby.

Obstetrician The pregnancy and delivery doctor.

SonographerThe member of staff who performs the ultrasound scan on mothers to look at the baby.

Fetal Obstetrician A doctor who specialises in looking at a baby before they are born, especially if there are problems with the way that the baby is developing. They will look at all parts of the baby and offer other tests if they detect any problems before birth.

Fetal Cardiologist A cardiac doctor who specialises in looking at heart problems in an unborn baby. They may also be a children’s cardiologist who looks after children with a heart condition. They will explain any problems with the baby’s heart and set out the treatment plan available to treat any condition.

Fetal Midwife A nurse who will help parents understand an antenatal diagnosis, plan tests and link families with local and specialist maternity services.


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Congenital Cardiac Specialist Nurse A nurse who specialises in supporting and informing families about the cardiac diagnosis and treatments. They provide a link from the obstetric team to the cardiac team.

Congenital Cardiac TeamCongenital Cardiologist The doctor who makes a diagnosis of congenital heart conditions and then looks after the child or adult patient throughout their life. They organise tests and see patients both in the hospital and at outpatient appointments. They also prescribe medications. Some cardiologists do interventional cardiac procedures (not surgery) during cardiac catheterisation, for example, closing off small holes in the heart.

Congenital Cardiac Surgeon

A doctor who offers surgical solutions to children and adults who have a congenital heart condition. They manage the care of a child if they are admitted to hospital for an operation.

Congenital Cardiac Specialist Nurse A nurse who has extra training and experience in looking after children and / or adults with congenital heart conditions.

Congenital Cardiac Ward Nurse A cardiac nurse who looks after a child or adult who has been admitted to the hospital to undergo tests or treatment for a congenital cardiac condition.

Anaesthetist A doctor who is responsible for keeping a child or adult asleep when they undergo certain tests or surgery.

Intensivist A special doctor who treats and observes a child or adult whilst they are in the Paediatric Intensive Care Unit (PICU).

PICU/ITU (Paediatric Intensive Care/Intensive Therapy Unit) Nurse

A nurse with special training to look after a very sick child or adult in the intensive care ward.

HDU (High Dependency Unit) Nurse

A nurse with special training to look after a child or adult who needs high dependency care. This is a step of care between intensive care and general care and usually takes place on a High Dependency Ward or a specially equipped area of a cardiac ward.

Sonographer and/or Cardiac Technician Specially trained staff who work with cardiologists to undertake medical tests like echos (heart scans) or ECGs (recordings of the heart rhythm).

Psychologist Specially trained staff who can help with the stress and strain that can be created when a child has a complex heart condition, for example, fear of being in the hospital or fear of needles.

Dietician An expert in knowing what food and drink is needed to keep healthy. They provide very important support for families learning how to help their children grow and thrive. Some children are referred to a Speech and Language Specialist for added help with feeding as they specialise in how the mouth and throat work.

Hospital Social Worker

A member of staff trained to support parents, families and patients with the social care they may need when they leave hospital. They can help with allowances and benefits.

Play Specialist Helps children recover from operations or prepare for treatment by encouraging activity and fun play. They are based in the ward or play department.


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The Normal HeartIn order to understand what a complex heart condition means, it is helpful to understand what the normal heart does. The heart’s job is to pump blood around the body. There are two separate circulations: the first takes blood to the lungs (the right side of the heart), and the second takes blood to the body (the left side of the heart).

This is how the journey begins: blood returns from the body, via veins, to the right side of the heart into a collecting chamber called the right atrium. This blood has a bluish tinge (blue blood) because the body has extracted oxygen from it (deoxygenated blood).

The blood is then passed through a valve (tricuspid valve) to a pumping chamber (right ventricle), which pumps the blood to the lungs via the lung arteries (pulmonary arteries).

The blood picks up oxygen as it passes through the lungs which turns the blood a red colour (oxygenated blood). This blood then returns to the left collecting chamber (left atrium) and it then passes through a valve (mitral) to the left pumping chamber (left ventricle). The left ventricle then pumps blood to the body through a valve (aortic valve) and to the body via the body artery (aorta). The body uses the oxygen from the blood, turning it blue again. And the journey starts again.


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Right Left

Aortic valve

Left ventricle

Right atrium

Right ventricle

Tricuspid valve

Mitral valve

Left atrium

Pulmonary artery

Aorta

The Circulation Before Birth(Fetal Circulation)

When the baby is still in the mother’s womb it does not need to breathe for itself as the mother is supplying all the oxygen to the baby via the umbilical cord.

The circulation before birth is different from that after birth. It is designed so that the oxygen-filled blood from the umbilical cord goes to the most important parts of the body, for example, the brain. Very little blood needs to go to the lungs.

There is a hole between the upper two collecting chambers (the left and right atrium) called the foramen ovale. Oxygen-filled blood from the umbilical cord is directed to the right collecting chamber (right atrium) and passes to the left collecting chamber (left atrium) through the hole and then on into the left pumping chamber (left ventricle), from where it is pumped around the body.

There is also a connection between the lung artery (pulmonary artery) and the body artery (aorta) called the ductus arteriosus (duct). Blood passes from the right pumping chamber (right ventricle) into the lung artery (pulmonary artery). Some of this blood then passes to the lungs.


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Right Left

Foramen ovale

Pulmonary artery

Umbilical cord attaches to baby’s circulation

AortaDuctus arteriosus


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When the baby is born and starts to breathe for itself, the bypass systems are no longer needed. Gradually over the first few days or weeks after birth, the duct (ductus arteriosus) and the hole (foramen ovale) between the upper two pumping chambers will close off and the baby’s circulation will be as described earlier. (See Normal Heart).

How The Diagnosis Of Single Ventricle Heart Disease Is MadeThere are two main opportunities for a diagnosis of complex heart disease to be made - either before or after birth.

Antenatal DiagnosisIn many cases it is now possible to make a diagnosis of a single ventricle condition when a pregnant woman undergoes her 16 - 20 week ultrasound scan. Scans before this stage of the pregnancy are unlikely to detect problems because the baby’s heart, lungs and other organs are not big enough to be seen clearly.

A sonographer (the member of staff who scans mothers to look at the baby) will be looking to see that the baby’s heart has four chambers, and two major crossing blood vessels leaving the heart. If there is any doubt that the baby has a normal heart or other organs the sonographer will seek a second opinion from the obstetrician or fetal medicine doctors (doctors who specialise in looking at unborn babies).

If a problem with the heart is seen clearly, the obstetric team will refer the family to a specialist in this field, a fetal cardiologist (a heart doctor who specialises in looking after unborn babies). If a complex condition is confirmed, the cardiologist and the obstetric team will talk to the family about the diagnosis and the possible treatments. They will offer further tests, such as amniocentesis, to see if the baby has any unseen problems with their genetic picture (to see if the blueprint of the baby has a fault - for example, something like Down’s syndrome). For more information on antenatal tests speak to your midwife.

Once all of the tests have been completed the family will be given the opportunity to discuss the future for their child. The team will support the family whilst they make their decision either to continue with the pregnancy or to end the pregnancy through termination.

Most babies with a single ventricle condition grow well in their mother’s womb. They are usually born between 38 and 40 weeks of pregnancy. It is only after birth, once their fetal circulation changes (see Fetal Circulation) that their defects begin to affect their bodies.

For more information on antenatal diagnosis refer to the LHM Antenatal Information Booklet.


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Postnatal DiagnosisAs long as the ductus arteriosus remains open, it is often difficult to detect that a newborn baby has a problem with its heart. (See Fetal Circulation).

As the ductus arteriosus closes the baby will become breathless and sweaty, sleep more and drink less milk. This can happen while the baby is still in hospital and may be detected by the midwife or doctor as they do their post-delivery checks or within a few days of going home when the midwife or health visitor should notice that the baby needs to see a doctor.

Once the baby has been referred to a paediatrician (baby doctor) tests will be done to try and find out what is causing the problem. Medicine called Prostaglandin will be given to the baby to try and keep the ductus arteriosus open, as this will keep the baby alive until treatment decisions can be made. (See Fetal Circulation).

The baby will be transferred with a medical team to the nearest hospital that treats congenital heart defects. Here the baby will have an ultrasound scan (echocardiogram or echo) similar to the scans done in pregnancy. The specialist heart team will be able to see what the heart looks like and will be able to make a diagnosis.

The Hypoplastic Left HeartHypoplastic Left Heart Syndrome is a congenital heart condition (a problem that a baby is born with). It is made up of a collection of problems on the left side of the heart. Usually, the pumping chamber (left ventricle) is small (hypoplastic) and the mitral and/or the aortic valve may be narrow, blocked or not formed at all. The body artery (aorta) is often small (hypoplastic) and there is a hole (atrial septal defect) between the two collecting chambers.

The blood’s journey through the heart is very different from normal. The blue (deoxygenated) blood flows into the right collecting chamber (right atrium), through the valve (tricuspid valve) into the right pumping chamber (right ventricle). From there it is pumped up to the lungs where the blood receives oxygen. The red oxygen-filled blood then flows from the lungs into the collecting chamber (left atrium) in the left side of the heart, but it will be unable to then pass into the left pumping chamber (left ventricle). As the valve will be blocked, it therefore passes through the hole between the two collecting chambers into the right side, where it mixes with the blue blood and follows the normal path to the lungs.

Whilst the ductus arteriosus is still open (patent), the blood will pass from the lung artery into the body artery and then around the body. When the duct closes, the baby will no longer have oxygen flowing to their body. Gradually, without medical intervention, the baby would become sicker and die.


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Small aorta

Atrial septal defectBlocked aortic valve

Blocked mitral valve

Hypoplastic left ventricle

Right Left


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Hypoplastic Left Heart Syndrome is a fairly rare problem that occurs in approximately 1 in 5,000 babies and accounts for 1% of all congenital heart disorders.

Complex Pulmonary Atresia

Pulmonary Atresia is a congenital heart condition (a problem that a baby is born with). As the heart forms, the pulmonary valve at the base of the lung artery (pulmonary artery) does not develop and the right ventricle does not develop normally and is often small (hypoplastic).

These defects mean that little or no blue (deoxygenated) blood passes through the lung artery to the lungs. If blood is unable to pass to the lungs through the right side of the heart it will pass through a hole (atrial septal defect) between the right and left collecting chambers (atria) into the left side of the heart where it mixes with red (oxygenated) blood and passes into the body artery (aorta). Some blood will then flow to the body and some will pass to the lungs via the connection (patent ductus arteriosus) between the body artery and the lung artery.

The children may be blue at birth, due to the lack of body oxygen and they usually need surgery within the first few days or months of life. It is important to keep the blood vessel between the lungs and the body open (patent ductus arteriosus) after birth as this gives the medical team and parents the opportunity to plan the baby’s treatment path before the child’s condition deteriorates. (See Fetal Circulation).


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Ductus arteriosus

Atrial septal defectAorta

Blocked pulmonary valve

Thick right ventricle

Right Left

Tricuspid AtresiaTricuspid Atresia is a congenital heart condition (a problem that a baby is born with). As the heart forms the valve between the right collecting chamber (right atrium) and the right pumping chamber (right ventricle) does not develop. This means that no blue (deoxygenated) blood passes through the right side of the heart to the lungs.

A hole (atrial septal defect) forms between the right and left collecting chambers (atria) and the blue blood passes into the left side of the heart and mixes with the red (oxygenated) blood and passes through the left side of the heart. Some of the blood then leaves the heart via the body artery (aorta) and some passes through a hole (ventricular septal defect) between the left and right pumping chambers (ventricles) into the right side of the heart where it is then pumped to the lungs via the lung artery (pulmonary artery). The circulation to the lungs is aided, before birth, by a connection between the lung and body arteries (patent ductus arteriosus). (See Fetal Circulation).

Some children with Tricuspid Atresia also have other heart problems, for example, the major arteries leaving the heart are swapped around (Transposition of the Great Arteries) and / or a hole between the two pumping chambers (ventricular septal defect). This altered circulation means that a reduced amount of blue (deoxygenated) blood reaches the


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Narrow pulmonary valve

Reduced pulmonary flow

Atrial septal defect

Absent tricuspid valve

Small right ventricleVentricular septal defect

Right Left


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lungs to collect oxygen for use by the body, and a mixture of blue and red blood passes around the body. The children may be blue at birth, due to a lack of body oxygen. They usually need surgery within the first few days or months of life. It is important to keep the blood vessel between the lungs and the body open (patent ductus arteriosus) after birth as this gives the medical team and parents the opportunity to plan the baby’s treatment path before the child’s condition deteriorates. (See Fetal Circulation).

Univentricular HeartDouble Inlet or Outlet Ventricle

Univentricular Heart occurs in a number of congenital heart conditions (a problem that a baby is born with). In each of these conditions there is a large pumping chamber (ventricle) which both collecting chambers (atria) empty their blood into. There may be a valve at the end of each collecting chamber or just one between the two.

One of the ventricles will be smaller than the other and there will be a large hole (ventricular septal defect) between them. The two major blood vessels leaving the heart, the pulmonary artery to the lungs and the aorta to the body, may leave from either the left or right sides of the ventricle and they may be swapped around (transposed).

Treatment for this condition depends on the exact position of all the blood vessels. Your cardiac team will explain the treatment path for your child. (See Possible Treatments).


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Transposed

Right atrium

Single ventricle

AortaPulmonary artery

Right Left

Left atrium

Small ventricular septum

Other Conditions Where A Child May Need To Go Down The Fontan Treatment Route

There are a number of congenital (something a baby is born with) heart conditions where the treatment plan is not always clear at the first diagnosis. Some children are born with a heart on the borderline between having two pumps or just one. If possible, cardiac doctors will plan to create a two-pump heart: this may mean creating a wall within the heart or creating a tube to take blood from one pump to the lungs.

It may be that surgeons will do a small amount of surgery to keep the child safe and give them an ability to grow and then wait to see if the heart shows it can support a two-pump circulation.

In some cases as the child grows it becomes clear that a two-pump heart is not possible and doctors will choose to follow the Fontan treatment path. (See The Fontan Procedure).

Conditions which might be borderline are described below.

Ebstein’s AnomalyThis condition means that there are problems with the tricuspid valve, the valve between the atrium and ventricle on the right side of the heart. It has not formed properly and may well have caused the right atrium (collecting chamber) to be bigger than normal and the right ventricle (the pumping chamber) to be much smaller than normal. The surgeons will always try to keep the valve and chambers working as normal but sometimes the ventricle is not big enough and they will offer a treatment path similar to that offered for Tricuspid Atresia.

Atrioventricular Septal Defect (AVSD)This condition means that there is a problem with the wall within the heart that separates the right and left sides of the heart. The septal defect (hole) runs from the atria (collecting chambers) in the lower part of the heart.

Surgeons can often create a wall so that the heart has four chambers as it should, but sometimes the defect is too big to repair and a surgical pathway similar to that for a Univentricular Heart will be planned.


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One and a Half VentriclesSome children are born with a heart condition where there is a difference in the size of the ventricles (heart pumps) from normal and there is a large hole where the wall should be between the two pumps. Where possible, the surgeon will close the hole and create a two-pump heart but this is only possible if each pump is big enough to do its job properly. Having offered treatment that will allow the baby to grow, an assessment will be made of the ventricle size and the ability of the ventricle to pump. Where one of the two pumps is not adequate in size, the treatment path will be to a Fontan circulation. These hearts have the advantage of having one pump that is stronger than normal because it is supported by the action of the other small ventricle, hence the one-and-a-half-pump heart.


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Questions Commonly AskedAt Time Of DiagnosisWhat causes the heart to form this way?As yet the causes of single ventricle heart conditions are unknown. It cannot be linked with mild drinking or the taking of ‘over the counter’ or most prescribed medications.

There are some indications that it can be linked to heavy smoking, alcohol or drug abuse, and anyone with diabetes has an increased risk of having a child with a heart condition.

It is very likely that one or more abnormalities are present before the third month of pregnancy but they may become more obvious as the heart is growing through the last part of the pregnancy.

Heart abnormalities can be present in babies that have other congenital problems, for example, genetic disorders like Down’s syndrome or Di-George syndrome (22q Deletion).

Why was the problem not detected during pregnancy?It is possible to detect single ventricle heart disease during pregnancy but it may not be obvious during scans due to the size or position of the baby. In some cases the condition can become more evident later in pregnancy after the routine scans have been performed. (See Antenatal Diagnosis).

Can my baby have treatment anywhere in the country?Over the past few years, an increasing number of centres around the country have begun offering surgery for these conditions, but not all, so it may be necessary for your baby to move hospital to receive treatment.


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Genetics

Many families who have a child with congenital heart disease ask why their child was born with the condition. In some cases the malformation will have occurred because of a genetic problem that has affected the heart as it formed in the womb.

A BlueprintWhen a new house is built the architect draws up a plan of what the house will look like and how it is going to be built.

When a new child is being created, information is drawn from the mother and the father to make a plan of how the child will look and how its body will be put together to work.

A Genetic BlueprintEvery person in the world has a genetic blueprint of their own. The blueprint is stored in every cell in their body and holds the information required to help the body grow, develop and work properly. The information is made up of lots of messages which we call genes. We have about 30,000 genes in each cell of our body. Different genes have different messages that are responsible for instructing our body to do specific things. For instance, genes determine the colour of our hair or eyes or how cells work in different organs, for example, the liver, heart or lungs. It is our very own information computer.

The genes are stored in coils and split into chromosomes. Humans have 23 pairs of chromosomes, so 46 in total. 23 chromosomes (one of each pair) come from the mother and the other 23 from the father. They are transferred in the egg and the sperm that make a child.

The picture overleaf shows what the chromosomes look like - if you look at the last set of chromosomes in this picture you will see that they are labelled X and Y. These are the chromosomes that decide if you are male or female. The chromosomes overleaf must come from a man because there is an X and a Y chromosome. A woman has two X chromosomes.


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Genetics and Heart DiseaseSome congenital heart conditions are linked to a genetic disorder such as Down’s syndrome or 22q Deletion. These disorders are caused when one of the 46 information chromosomes is malformed. As the baby grows in the womb, the genetic malformation will cause a particular part of the heart to develop incorrectly. In some cases the genetic condition can be detected before birth.

Geneticists (genetics doctors) are always looking for genetic causes for congenital conditions; however, there are many heart conditions that do not have a specific genetic cause. Many of the single ventricle heart conditions fall into this group.

There may be many factors, as yet unknown, that cause a baby’s heart to develop abnormally. The problems can occur early in pregnancy as the heart forms or later as it grows.

Congenital heart conditions occur in 1 in 100 pregnancies. If you have previously had a child with a single ventricle heart problem, the risks of it happening again to parents, their other children or the child affected themselves rises to between 5 and 8%. Although the risk is higher, over 90% of future babies will not have a problem with their heart.

1

6

13

19 20 21 22 23X

14 15 16 17 18

7 8 9 10 11 12

2 3 4 5

Y

Human Chromosomes


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Possible Treatments ForHypoplastic Left Heart SyndromeHaving explained your child’s diagnosis, the doctor will go on to discuss what treatments are available. None of these treatments provide a long-term cure but, for many children, they can offer a reasonable quality of life.

It is vital that parents are fully included in the treatment discussions and decisions taken for their child.

These treatment options may include:

Surgical TreatmentThe aim of this treatment is to re-route the blood through the right side of the heart, bypassing the blockages on the left side. This surgery is offered in three stages over three or more years and involves extremely high-risk open-heart surgery. Your doctors will explain the risks for your child. Even after all the surgery has been performed, your child’s heart will not look normal or work normally, and it may be that it will gradually fail and your child may need a heart transplant in teenage years or early adult life. More information on the surgery follows.

Supported Comfort CareAs the long-term outcome is unclear for children with Hypoplastic Left Heart Syndrome, it is important to know that there is the option not to put a child forward for surgery. The baby is allowed to die peacefully either in the hospital or at home; the family would be fully supported by hospital and community nursing and medical staff.

TransplantationHeart transplantation is one of the possible treatments for single ventricle heart disease, but it is not offered as a first treatment within the United Kingdom for the following reasons.

There are very few donor hearts small enough for a baby available in the United Kingdom.

Transplanted hearts do not last for ever and there are many risks involved throughout the recipient’s life. Offering surgery as a first treatment path and retaining transplant as a future option offers a greater chance of a longer life for a child.


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Modified Norwood Procedure(Stage One)

This is normally performed within a few days of birth or diagnosis of Hypoplastic Left Heart Syndrome, while the ductus arteriosus (connection which joins the lung artery and the body artery) is being kept open with Prostaglandin therapy. (See Fetal Circulation).

The aims of the first operation are:

YTo improve the flow of red blood (oxygenated blood) around the body by attaching the base (trunk) of the lung artery (pulmonary artery) to the body artery (aorta). It may be necessary to enlarge the body artery with a patch.

Y To provide a blood flow to the lungs through a passage (shunt), creating a link between the lung artery (pulmonary artery) and the body artery (aorta). The passage is made out of soft plastic (Gore-Tex).

Y To create a permanent passage (septectomy) between the collecting chambers (left and right atrium) ensuring that a mix of red (oxygenated) blood and blue (deoxygenated) blood is flowing around the body.


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(a) Trunk of the pulmonary artery is attached to the aorta

(b) Shunt is formed

(c) Enlarge the atrial septal defect

Right Left

This operation is extremely complicated and holds a high risk (chance of the baby dying). Ask your surgeon about the risk for your child. The baby may need to be in hospital for some weeks following this surgery to ensure that their condition is stable before being sent home.


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Modified Norwood Procedure(Norwood with a Sano Shunt)

The reconstruction of the main artery leaving the right side of the heart (pulmonary / aorta vessel) remains as described on the previous page; this provides a flow of blood to the body.

The lung (pulmonary) flow of blood is provided by a Gore-Tex tube that leads from the right pumping chamber (right ventricle) to the left lung vessel (pulmonary artery). This connection ensures that blood reaches the lungs where it collects oxygen that is then passed around the body.

This, like the previously described procedure, is an extremely complicated operation and holds a high risk (chance of the baby dying). Ask your surgeon about the risks for your child.

Pulmonary trunk attached to aorta (major vessel leaving the heart)

Enlarge the atrial septal defect

Right ventricle to pulmonary artery connection

Right Left

The Hybrid ProcedureIn the past many newborn babies were not suitable for the Norwood Procedure for Hypoplastic Left Heart Syndrome because they were too small or too sick. Medical teams have been working to develop a procedure that will support a newborn baby’s heart until he or she is big enough to undergo more complex reconstructive operations. This procedure is called the Hybrid Procedure.

For some babies born with a diagnosis of Hypoplastic Left Heart Syndrome, the Norwood Procedure, the first stage of surgery, may not be possible or may have an exceptionally high risk.

The Hybrid Procedure has been developed as a way to help a baby survive until it is strong enough and big enough for the more complex Norwood Procedure.

The aim of the Hybrid Procedure is to allow flow of oxygen-filled red blood (oxygenated blood) to reach the body and to allow used blue blood (deoxygenated blood) to reach the lungs where it can collect oxygen again.

This is done by keeping open the arterial duct and holes that are in the heart whilst the baby is still inside the womb (fetal circulation), at the same time protecting the lung blood vessels so that they can still be used in


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Banded pulmonary artery

Blocked aortic valve

Blocked mitral valve

Hypoplastic left ventricle

Patent ductus arteriosus stent

Banded pulmonary artery

Small hypoplastic aorta

Atrial septal defect

Right Left

later surgery. (See Fetal Circulation).

The Hybrid Procedure is performed under a general anaesthetic within the cardiac catheter suite or the operating room or possibly both.

The procedure is performed by both a cardiac surgeon and a cardiologist.

There are three parts to this procedure:

1 Small bands are tightened around the left and right lung arteries (pulmonary arteries) to control the flow of blood and pressure in the lungs.

2 A mesh of fine metal (stent) is placed in the patent ductus arteriosus (between the pulmonary arteries and the aorta) to keep it open.

3 At the same time the hole between the upper collecting chambers (atria) may be made bigger and permanent (atrial septostomy). This may be delayed for about a week.

This procedure has less initial risk than the original Stage One Norwood Procedure but children undergoing the Hybrid Procedure are usually sicker or smaller so their personal risks may be high.

Children who have undergone the Hybrid Procedure will need a more complex operation at Stage Two that will incorporate the reconstructive surgery of the usual Stage One Norwood Procedure as well as the Stage Two Cavo-Pulmonary Connection.


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Possible Treatments ForRight-Sided Single Ventricle ConditionsHaving explained your child’s diagnosis, the doctor will go on to discuss what treatments are available. Although the surgery offered can bypass the heart abnormalities, it is unable to provide a long-term cure.

It is vital that parents are fully included in the treatment discussions and decisions taken for their child.

These treatment options may include:

Surgical TreatmentIncluded here are a variety of operations that may be offered in the treatment of single ventricle heart conditions. Most children go through a series of three operations over the first three to five years of their life. Even after all the surgery has been performed, your child’s heart will not look normal or work normally, and it may be that it will gradually fail to function. If this occurs your doctors may suggest heart transplantation. More information on the surgery follows.

Supported Comfort CareAs the long-term outcome is unknown and some children’s conditions are extremely severe, it may not be possible or advisable to put the child forward for surgery. The baby would be allowed to die peacefully either in the hospital or at home; the family would be fully supported by the hospital and community care teams.

TransplantationHeart transplantation is one of the possible treatments for single ventricle heart disease, but it is not offered as a first treatment within the United Kingdom for the following reasons.

There are very few donor hearts small enough for a baby available in the United Kingdom.

Transplanted hearts do not last for ever and there are many risks involved throughout the recipient’s life. Offering surgery as a first treatment path and retaining transplant as a future option offers a greater chance of a longer life for a child.


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Surgical Treatments forRight-Sided Single Ventricle Conditions

It is often difficult to predict which early procedure will be most helpful in supporting a newborn baby with a complex right-sided single ventricle condition. Some children are born with too much blood flowing to their lungs, others with not enough. In some conditions it is not until after birth that the doctors are able to decide exactly which of the following procedures will be needed by each child.

ShuntsMany single ventricle conditions cause a restriction of the blood supply to the lungs. As it is essential that blood flows to the lungs to collect oxygen for the body, connections must be created to re-route blood around any blockages within the heart. Surgeons will insert tubes made of either donated heart tissue or Gore-Tex (a specialised plastic) to form connections from the body circulation to the lung circulation. This treatment is often used within the first few weeks of life, as it is a simple solution to the circulation problem. Further more complex surgery will be done as the child grows.


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BandingSome children are born with a circulation that causes too much blood flow to the lungs. If the lungs are left with too high a flow, this can damage the lung blood vessels over time. To prevent this damage a band is placed around the base of the lung artery (pulmonary artery). This is usually removed at a later stage of surgery.

Septostomy or SeptectomyMany of the children are born with valves that are malformed so that they block the flow of blood around the heart. It is important to divert blood around the blockages by creating extra channels within the heart. Doctors may choose to create a hole between the two collecting chambers (atria) of the heart either during a cardiac catheterisation or during surgery.

Septectomy is the surgical procedure. Septostomy is a cardiac catheterisation procedure. (See Cardiac Tests).


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Left atrium

Aorta

Right atrium

Right ventricle

Pulmonary artery

Band

Mitral valve

Aortic valve

Left ventricle

Right Left

Septostomy at the atrial septal defect

The Damus ProcedureChildren who are born with only one of the chambers (ventricles) needed to pump blood around their body and who may also have a narrowing of the body artery (aorta) may need to have surgery to aid circulation of blood from the heart to the body.

The base of the lung artery (pulmonary artery trunk) is attached to the base of the body artery (aorta) to ensure that all the blood that enters the one functioning pumping chamber will be directed around the body.

To ensure that blood also reaches the lungs a connection (shunt) is made between the body circulation and the lung artery (pulmonary artery).


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Single ventricle

Damus connection

Shunt

Right Left

Cavo-Pulmonary Connection(Stage Two) for all single ventricle conditions

Bi-directional Cavo-Pulmonary Shunt (Glenn Shunt) or The Hemi-Fontan OperationAs the children grow, they will require a more permanent supply of blood to their lungs than earlier surgical procedures can provide. A Cavo-Pulmonary Connection is normally performed between three and twelve months of age.

The aim of this operation is to redirect the flow of blue blood (deoxygenated blood) to the lungs by attaching the upper body vein (superior vena cava) directly on to the lung artery (pulmonary artery). The Gore-Tex shunt or band that was inserted at the last operation will be taken away.

In some centres the stump of the lower body vein (inferior vena cava) is attached to the base of the lung artery (pulmonary artery). This is known as the Hemi-Fontan operation. Although blood does not flow through this attachment at this time, it prepares the child for the completion of a Fontan procedure at Stage Three.

These operations have fewer risks than the first stage but it is important to talk with the medical team to find out the risks for your child.


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Right pulmonary artery

Superior vena cava

Right Left

The Fontan Procedure(Stage Three) for all single ventricle conditions

Or completion of the Cavo-Pulmonary ConnectionThe final stage of surgery will be performed as the child starts to show that they need more blood flow to the lungs. They may become more breathless on exercise or their growth may slow down. For some children this will occur before they start school. Others may be able to wait a little longer. For more information see the LHM booklet The Fontan.

The Fontan procedure aims to separate the blue (deoxygenated) blood supply and the red (oxygenated) blood supply. Although this does not make the heart function normal, it does allow the children to grow and enjoy more physical activity. The procedure can be performed in two ways.

External Fontan or External Conduit

This operation is done by attaching a tube of a special plastic (a conduit of Gore-Tex) from the lower body vein (inferior vena cava) to the base of the lung artery (pulmonary artery), diverting blue (deoxygenated) blood away from the heart straight to the lungs.


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Superior vena cava

Fenestration

Conduit

Inferior vena cava

Pulmonary artery

Right Left

A hole (fenestration) may be created between the tube and the right collecting chamber (right atrium). As with the Internal Fontan (see below) there can be a rise in pressure in the lung arteries after surgery and the hole acts as a pressure valve.

Children may be in hospital for some weeks after the third operation as it is important to give them time to adjust to their new circulation.

Internal Fontan

This is done by creating a wall (baffle) in the right collecting chamber (right atrium) and then attaching the chamber to the base of the lung artery (pulmonary artery). This may have been partially completed at stage two, the Hemi-Fontan.

All the returning blue (deoxygenated) blood will now be flowing to the lungs, without a pump behind it. This causes an increase in pressure within the lung blood vessels, so, to aid circulation, a small hole (fenestration) may be created in the wall (baffle). This acts as a pressure release valve whilst the child’s body adjusts to the new circulation.


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Superior vena cava

Right atrium connected to the pulmonary artery

Fenestration

Inferior vena cava

Pulmonary artery

Baffle (wall) created in the right atrium

Right Left

Other InterventionsAlthough the plan set out when a child has their first diagnosis of a single ventricle heart condition usually involves three stages of surgery, many children require other procedures to improve their heart function as they get older. Here we briefly describe some of the possible interventions that may be needed.

Ballooning a Blood VesselChildren who have malformations within the heart requiring reconstruction of blood vessels often develop a narrowing around the site of surgery or the malformation. To ensure that blood can flow freely through the blood vessels cardiologists often balloon the area of the narrowing to stretch it back to normal or nearly normal size. The ballooning takes place during a cardiac catheterisation (see Cardiac Tests). Most children have this procedure done under a light anaesthetic and they will be in hospital for an overnight stay.

Inserting a StentFollowing the ballooning of a blood vessel it may be necessary to insert a stent (a mesh of fine wire in a tube shape) that can be used to keep the blood vessel open after it has been stretched. This is inserted during a cardiac catheterisation.

Surgery to a Heart ValveChildren with only one side of their heart working normally have more blood pumping through that side until their Fontan operation has been completed. This added blood flow increases the strain on the valve between the collecting and pumping chambers. If the valve shows signs of damage that will not be relieved by the Fontan operation surgeons may decide to repair and on rare occasions replace the valve. This involves open heart surgery and a post operation trip to PICU (Paediatric Intensive Care Unit).

Closing a FenestrationMany children undergoing the Fontan operation will have a fenestration created to help blood flow smoothly through the lungs and to reduce the pressure on the circulation. The fenestration creates a hole that allows oxygen-filled red blood and deoxygenated blood (blue blood) to mix; this reduces oxygen saturations. As the children grow it may become clear that the fenestration is no longer needed so a plan will be made to close it with an umbrella device placed in the heart during a cardiac catheterisation (see Cardiac Tests). Most children have this procedure done under a light anaesthetic and they will be in hospital for an overnight stay.


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Tying Off CollateralsIf a child is very blue (cyanosed) the body tries to correct the problem by creating extra blood vessels to take blood to the lungs where it can pick up oxygen. These blood vessels are known as collaterals. Often these extra vessels create problems for the child’s Fontan circulation and they need to be closed off. This may happen during planned surgery or during a cardiac catheterisation. For more information see section on Collaterals.

Heart RhythmAll hearts have a heartbeat, which is the electrical message that passes through the heart and tells the heart muscle to push blood from one section of the heart to another and then out of the heart to the circulation. The beat (pulse) created depends on the body’s activity so at rest the rate is lower and on exercise it becomes faster. Children and adults with congenital heart conditions may have a disturbed heart rhythm (arrhythmia). Sometimes they are born with an arrhythmia, sometimes one develops.

Many children who have had major heart surgery have a disturbed heart rhythm after their operations. These are usually temporary and are solved as the heart recovers from the surgery but in some cases the heart rhythm does not recover and a more permanent solution will need to be found. The first treatment offered will often be medications to control the heartbeat. This may solve the problem and no further intervention will be needed. For some children and young adults other solutions will be explored.

Ablating an Electrical PathwayIn some patients the electrical pathway will be disturbed so that heart rhythm messages will be sent to the wrong part of the heart, which creates an imbalance with the pumping of the heart muscle. In these cases cardiologists may offer ablation as a treatment. This is a treatment undertaken under anaesthetic in the cardiac catheterisation theatre. Cardiologists identify the pathways of faulty electrical activity and block the ones that are creating an imbalance. This may be done by either a freezing or burning process.

PacemakerIf an arrhythmia (abnormal heart rhythm) persists and affects the heart function doctors may decide to insert a pacemaker. There are a number of different pacemakers that solve different problems. Some help to regulate the heartbeat, others send an impulse to the heart when there are long pauses between beats. Surgery will be needed to insert the pacemaker but it is not as complex as open heart operations. The pacemaker box is either placed in the abdomen, especially in smaller


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children, or under the arm or chest muscles in an older/bigger patient.

Following a pacemaker insertion regular pacemaker checks will be set in place. Changes to the pacemaker settings can be made externally so there is no need for further invasive intervention until the pacemaker box needs changing - usually at around ten-year intervals.


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ECMO or ECLSExtracorporeal Membrane Oxygenation or Extracorporeal Life Support

Babies and children with congenital heart conditions sometimes have periods of time when they have difficulty pumping enough oxygen-filled blood around their body. This may be because their heart is failing as they await surgery or in the period after an operation when their heart is tired. If doctors feel that a period of added support for the heart will aid recovery they may suggest ECMO.

The ECMO machine is similar to the heart-lung bypass machine used for open heart surgery, although often smaller. Extracorporeal means ‘outside the body’, and a membrane oxygenator is a piece of equipment which delivers oxygen into your child’s blood.

Therefore, ECMO is the use of an artificial lung (membrane) located outside the body (extracorporeal) that puts oxygen into the blood (oxygenation) and continuously pumps this blood into and around the body.

Most congenital heart units are able to offer ECMO for cardiac patients but occasionally a child will be transferred to another cardiac team for the specialised care.


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Cardiac Tests

Oxygen SaturationThis is a simple painless test where a plastic strip or clip is attached to a finger or foot to measure the amount of oxygen circulating in the blood of babies, children and adults. It is particularly important for children with complex heart conditions who are blue (cyanosed) because of the low oxygen levels as it helps the medical team to plan their treatment and care.

Blood TestsBlood is a very important part of the circulation because it carries all sorts of things around the body: oxygen, nutrients and chemicals. Taking samples of blood can be a very helpful way of monitoring how well a baby, child or adult is and how the body is coping with an illness or long-term condition.

Chest X-raysFor doctors to see if a baby, child or adult’s chest is healthy, especially before and after surgery, the easiest test is to take an x-ray of the chest so that they can see how the chest is healing but also to look for infections or fluid collections in the lung area. These tests help plan treatment and post-surgery recovery support.

Echocardiogram (Echo)Echocardiography uses sound waves to scan the heart which is then shown as a picture. This is the same type of scan that mothers have during pregnancy. The test does not hurt, but children sometimes have a light sleeping medicine (sedative) so that they stay still. This is the first test that will be done on the baby, as it is the easiest and least stressful way of making a diagnosis of congenital heart disease.

ECGElectrocardiography (ECG) is the recording of the electrical activity of the heart. The heart has its own electrical system, which passes the message to beat through the heart’s muscle tissue. Sometimes the message breaks down and so by recording the rhythm, the medical team can see if the messages are being passed properly. It is also possible to assess the size of the pumping chamber and the thickness of the heart wall.


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Cardiac CatheterisationIt is often necessary for the doctors to gain more information about the heart than an echo or ECG can provide, so the children may need to undergo cardiac catheterisation. This investigation is best done with the child asleep, so they will be given a light general anaesthetic.

A long thick tube (catheter) is passed into the heart from a big blood vessel, either from the groin or the shoulder. Through this tube a dye (radio-opaque fluid) is injected that can be seen on x-ray. The dye passes into the heart and x-ray pictures can be recorded. The doctor is then able to see the structures of the heart more clearly.

It is also possible to measure the blood pressure in the different chambers and blood vessels. This information is extremely important when deciding what further treatments can be offered safely. This test is often performed whilst planning the next stage of surgery.

MRI ScanMRI (Magnetic Resonance Imaging) scans use radio-frequency radiation to gain a picture of the soft tissues of the heart from different angles. This can be very useful when looking at the results of surgery. Children need to lie still for this scan and so many of them need some sedation or a light general anaesthetic.

CT ScanComputerised tomography, also known as a CT or CAT scan, uses x-rays and a computer to create detailed images of the inside of the body.


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Heart FailureThis is a commonly used term, which means that the heart is failing to do its job properly. It can occur:

Yif a child has a congenital heart defect

Yfollowing surgery as the heart is healing

Yas the heart muscle grows tired because it has to work too hard.

It means that the heart muscle is unable to pump blood around the body efficiently, often leaving fluid in the body tissues (e.g. the lungs and liver).

Signs and symptoms of heart failure:

BreathlessnessEspecially when feeding or taking exercise.

Poor feedingBecause the heart is working hard, eating and drinking become hard work.

TirednessBecause all the child’s energy is being used to keep the heart working.

Puffiness (oedema)Especially around the eyes, because fluid is not being passed around the body properly.

Small amounts of urineBecause fluid is not moving around the body to the kidneys.

IrritabilityBecause the child feels unwell or uncomfortable.

If your child is showing any of the above signs, especially when feeding or taking gentle exercise (e.g. walking), contact your local doctor. It may be that your child has a simple cold but it is always important that someone assesses your child.


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If the child is very tired and not completing feeds it may be necessary for them to have a short stay in hospital and some help with feeding. Medications are also given to help the heart function and to help the child pass more urine.

This treatment will give the heart an opportunity to rest and recover. (See Medications). Most children are able to live at home on medication once their condition has stabilised.


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MedicationsA very important part of the treatment of complex congenital heart disease is the medical support given to the heart and circulation. This support will be altered as the needs of each child and young adult change as they undergo surgery and grow. Part of the ongoing maintenance of stable heart function will include prescribing medications.

DiureticsDiuretics are medications that make the kidneys work more efficiently to create urine from the fluid circulating in the blood. The more fluid in the blood, the harder the heart has to work to pump it around the body. It is important to encourage children to continue drinking normally whilst on these medications.

Frusemide (Furosemide) is the most commonly used diuretic; it works well in all age groups.

Amiloride is often used as it keeps the body’s salts (electrolytes) in balance as the child passes out more urine.

Spironolactone is another form of diuretic.

On some occasions these medications are used together.

Medications that Strengthen or Support the Heart’s FunctionDigoxin is a drug that slows, strengthens and steadies the heart. It is often used in the treatment of heart failure as it makes the heart beat more efficiently.

Captopril, Lisinopril, Ramipril and Enalapril - ACE inhibitors are medications that open up the body’s blood vessels, making it easier for the heart to pump blood through them. This relieves pressure/workload on the heart pumps (ventricles).

Carvedilol is a drug that slows down the resting heart rate and increases the efficiency of the heart muscle.

Anti-ArrhythmicsThese are medications that help a heart that is not beating in rhythm. These drugs strengthen the heartbeat and make the beat more regular.

Digoxin is a drug that slows, strengthens and steadies the heart. It is


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often used in the treatment of heart failure as it makes the heart beat more efficiently.

Amiodarone helps treat heartbeats that are irregular by blocking some defective electrical signals. Avoid eating grapefruit if prescribed this medication because it alters the function of Amiodarone.

Sotalol is prescribed to block irregular electrical messages as they pass through the heart. This steadies the heartbeat.

AnticoagulantsThese are drugs that lengthen the time it takes for the blood to clot. They are used to ensure that the blood flows freely through the tubes and passages that have been created in the heart. For more information see the LHM booklet Living with Anticoagulation.

Aspirin is a gentle anticoagulant that stops blood cells sticking together. It is often the drug of choice in young children.

Warfarin is a medication that slows down the blood’s ability to clot (form scabs). Regular blood tests are required to ensure that the correct dose of Warfarin is given.

Dipyridamole works to interrupt the formation of clots.

Clopidogrel works to hinder the formation of clots. It sometimes replaces Aspirin or can be used in partnership with Aspirin.

Anti-Pulmonary HypertensivesSildenafil works to relax blood vessels so that they widen more easily, making it easier for the heart to push blood through them.

Dipyridamole works to reduce blood pressure by opening up blood vessels. It works especially well in the lungs.


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Possible Complications

This section explores the sort of complications that can occur before, during or after heart surgery when a child or young adult has a single ventricle heart.

When doctors discuss a new baby’s diagnosis and the treatment path that they plan for them to travel, they also discuss the risks that go hand in hand with a complex heart problem and the treatments available.

Most parents understand that there are major problems when you only have one heart pump.

Here we briefly discuss a broad range of problems that can occur. It is very important that parents, and patients themselves as they get older, talk to their medical team about the risks as not all problems are a risk for each child.

Low Oxygen LevelsAll children with a cyanotic heart condition (blue children) have lower-than-normal oxygen levels. It is safe for them to be moderately low for short periods of time, especially when there is a treatment plan in place and the children are carefully monitored, but if left too low for long periods of time it can affect energy levels and brain function.

There is also a risk of low oxygen levels when a child undergoes surgery or in the post-operative recovery time, especially if the team use the heart-lung bypass machine. Low oxygen levels leading to problems with the brain are known as neurological incidents.

Doctors will explain the risk of low oxygen levels but they will also work to reduce that risk as much as they can.

InfectionAll children get coughs and colds which need treating in the normal way but post-operatively there is a risk that the chest scar wound both inside the body and on the skin will become infected. The doctors work to prevent this by giving antibiotics but it can still occur after surgery. If the scar is weeping or red a test will be done to identify the type of infection and treatment will begin.


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There is also a risk of something called bacterial endocarditis. This is an infection that starts in the mouth and can travel to the heart. For more information about dental care and endocarditis see Dental Care section.

Blood LossAfter any operation there is a risk of bleeding. Blood loss will be measured and replacements will be given if the medical staff feel that the loss is compromising blood flow. If the blood pressure remains low for long periods the kidneys are unable to work properly and ultimately blood containing oxygen is unable to reach the brain.

If bleeding occurs between the heart muscle and the outside skin of the heart it can cause problems with the pumping of the heart. If this happens the surgeons may need to remove the blood that has collected there.

Chest Drain LossesAfter chest surgery children and adults have chest drains inserted to ensure that any fluid or blood in the chest can drain away. In some cases the drainage becomes excessive. This could be increased blood loss or because fluid fat is leaking in the chest. If the drainage is excessive, and doctors are unable to keep fluid intake in balance, children can go into shock and collapse.

ClotsThe heart is responsible for pumping blood around the body. If the heart is not working efficiently, especially around the time of surgery, there is a risk that the blood will clot on areas where surgery has been performed or in pockets of the heart where blood moves slowly. There are also changes to the liver’s function in patients who have a Fontan circulation which makes the risk of clotting greater than normal. There are a number of risks associated with this clotting. The heart’s pumping can be compromised if clots grow within the heart. Small bits of the clot can travel into the lungs, causing problems with breathing, or to the brain where they could cause a stroke. Children and young adults undergoing heart surgery or living with a Fontan circulation are often prescribed anticoagulation medication to reduce the risk of clots. See LHM’s booklet Living with Anticoagulation.

StrokesA stroke is a disruption to the circulation of blood to the brain either because of a bleed or because of a blockage, usually a clot. This disruption causes damage to the brain tissue, a condition that is called a stroke. Strokes can take different forms. They can affect mobility, sight, speech and memory, and can cause developmental problems. Medical teams will be working to avoid strokes by making sure that the risk of


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clots is low, and that the heart is supported to pump well and maintain the oxygen circulating to the brain.

ArrhythmiasAn arrhythmia is a problem with the rate or rhythm of the heartbeat. During an arrhythmia, the heart can beat too fast, too slowly, or with an irregular rhythm. Children with single ventricle hearts have a risk of arrhythmias because of the structure of their heart. The risk is increased after surgery when the circulation has been altered. Arrhythmias can be treated with medications or the use of a pacemaker but often a post-operative arrhythmia will disappear once the heart has recovered from the operation.

A number of these complications can occur routinely around the time of surgery or treatment and so the medical and nursing staff will be watching for them and will intervene with treatment swiftly to help resolve the problems.


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Collaterals

Children who are born with complex congenital heart disease associated with a reduced blood fl ow to their lungs can sometimes develop collateral vessels.

What are collaterals?If a baby is born with a malformation of the heart and a lack of blood fl owing to the lungs to collect oxygen, the child will have low oxygen saturations (the amount of oxygen in their blood). They will be cyanosed (have blue-coloured lips and fi ngernails) and may be breathless on mild exercise or feeding. Collaterals are connections, like normal blood vessels, that can develop in children with cyanotic heart disease such as single ventricle heart conditions.

There are two types of collaterals:

Systemic Arterial CollateralsThese abnormal vessels originate from the body blood vessels, in particular the aorta, and grow towards the lungs. They can form when a child has had a long period of cyanosis (low oxygen levels in the blood which create blue lips and fi ngernails). The collaterals aim to take more blood to the lungs where it can collect oxygen. This is the body’s response to the long-standing low oxygen saturations. These collaterals make the child less blue but create more work for the heart!


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Collateral

AortaTo lung

Systemic Venous CollateralsThese are abnormal blood vessels that originate from the veins taking the blue (deoxygenated) blood back to the heart. They normally develop after the second operation, the Cavo-Pulmonary Connection (or Stage Two). After this operation the pressure in the veins in the upper body is greater than the pressure in the veins in the lower body. With that, small veins can enlarge and can allow blue (deoxygenated) blood from the upper body to run down to the lower body rather than having to squeeze through the lungs. These collaterals make the child more blue, but do not increase the work for the heart.

How are collaterals diagnosed?When children with single ventricle disorders undergo cardiac catheterisation or MRI scanning investigations it is possible to clearly see the collateral vessels. When there is a large collateral vessel it may be seen during a routine scan (echocardiogram). (See Cardiac Tests).

How are collaterals treated?Once collateral vessels have been found the cardiac team will assess if they need closing (occlusion). Small systemic arterial collaterals will normally disappear after the Fontan procedure has been performed. Large systemic arterial collaterals should normally be closed by a catheter procedure as they put strain on the heart and raise the pressure in the lung arteries. The child may be more cyanosed after this, and the Fontan operation may have to be performed earlier.

Large systemic venous collaterals in young children after the Cavo-Pulmonary Connection operation (Stage Two) should be closed by catheter. This will make the child less cyanosed, as more blue blood goes to the lungs, and frequently the Fontan operation can be delayed. Small systemic venous collaterals identifi ed just before the Fontan operation can be ignored.


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Collateral

Understanding Common Terms

The following are terms that you may hear whilst your child is in hospital or at home receiving treatment. There will always be some terms that are not covered so ask your cardiac team to explain things if they use terms that you do not understand.

Hypoplastic: .....................................................small or underdeveloped

Atrium: ....................................................collecting chamber of the heart

Ventricle: ..................................................pumping chamber of the heart

IVC: ..................................................inferior vena cava - lower body vein

SVC: ..............................................superior vena cava - upper body vein

Stenosed: ..................................................................................narrowed

Atretic: ...............................................................not fully formed / closed

Patent: ..............................................................................................open

Cyanosed / Cyanotic: .........................blue colour due to lack of oxygen

Acyanotic: ......................................................................not looking blue

Oedema: .............................................................fluid retention, puffiness

Pyrexia: .........................................................................high temperature

Apyrexia: ..................................................................normal temperature

ECG: ...........................electrocardiogram (see section on Cardiac Tests)

Echo: .............................echocardiogram (see section on Cardiac Tests)

ASD (Atrial Septal Defect): .............hole between the top two collecting chambers

VSD (Ventricular Septal Defect): ................hole between the bottom two pumping chambers


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Living Life With Half A HeartWhen you are first told that your baby has a heart problem it is very difficult to know what sort of life your child will lead. This section of the Filofax hopes to answer some of the most commonly asked questions about life at home.

It is extremely important to remember that each child is different and that not all of the information below will necessarily apply to your child.

AppearanceChildren with heart disease look very normal. They may have a slightly blue tinge around the lips and fingernails (cyanosis). This is because they have a reduced amount of oxygen flowing around their bodies. After the final operation when the blue (deoxygenated) blood and the red (oxygenated) blood have been separated, they should be pink.

DevelopmentChildren who recover well after surgery will normally be able to crawl, walk and run as children of their age will do, although they may become tired on exercise more easily. They are likely to go to mainstream school and be able to take part in normal learning but they may find concentrating for long periods difficult and the physical parts of school life challenging. Some children need extra support in school to help them reach their full educational potential. Socially the children will be able to develop normal friendships and take part in normal family life.

Diet and GrowthChildren with heart disease need more calories than other children because they need energy to fuel their inefficient heart and to grow. However, when they are small, feeding is a huge challenge because it takes a great deal of energy to take in food, especially from a bottle.

For many babies it is normal to need some sort of help to gain weight. This may mean adding calories into a breast or bottle feed or mixed feeding that includes the use of a bottle and naso-gastric feeding tube. The hospital dietician and specialist nurses will help families to learn different ways to feed and support their baby when they go home. As the child grows older feeding becomes easier but children will always need help to gain weight so planned small meals spread regularly through the day will help to provide the energy needed each day. For more information see the LHM Feeding at Home booklet.


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ActivityChildren with complex single ventricle disorders can normally walk and run. Many will be able to ride a bicycle and play football in the back garden, but they will not be able to play a full game of football or run in a competitive race as they will tire easily. Contact sports are not possible because of surgical scarring and anticoagulation medication. See LHM Sport and Exercise booklet.

Long-Term TreatmentIf the surgery has been successful the children should not need to spend a great deal of time in hospital. They will need to have regular outpatient check-ups to monitor their progress, with the occasional hospital admission for tests.

Most children are on some of the drugs described in the Medications section, but these are given at home and the children become used to having them.

Little Hearts Matter has a number of information booklets that provide in-depth support and information on specific aspects of life with a single ventricle heart condition. (See Other Support and Information Available).


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The FutureAdvances in surgery have enabled many children to enjoy life, joining with their peers in their education and play. They may have to learn to compromise on physical activity but they are able, within the restrictions that their heart condition creates, to achieve their full potential, reaching adulthood and striving to lead independent adult lives.

It is always important to balance the improvements in care with the knowledge that for a small number of children and young people their heart will gradually tire and begin to fail despite the described treatment. It may be that transplantation will be their only treatment at that time.

“She is like any other two-year-old and certainly keeps us on our toes. She still manages to give us the odd fright now and then. We know that Éabha has a different, and possibly difficult road ahead but with the family and friends that we have around us, the support available to us and not to mention the knowledge we now have of what Éabha can accomplish, we will continue to face whatever may lie ahead with all that we have.” Amie


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“I’ve finished the first year of college with a

double merit which is good. I also joined a band;

we’re called Memories of Shadows and play original hard

rock. We just had our first gig, opening for a couple of bands at the 02 Academy in Birmingham, which was an incredible experience. It went really well for our first gig together; I felt no pressure and didn’t feel ill. I was living my dream and my heart had no problems with it. We’ve got a few more gigs coming up and I’m really looking forward to them.” Ryan

“It was vital to prepare Charlie for the surgery. He has always known about his ‘special’ heart and is a child who likes to know what’s what, so it was important that he understood what his operation would entail. I told him stories about children having heart operations, including the procedures that would happen and what it might feel like. We read hospital books and looked at photos of other children post-op so he could understand what would be happening.I never told Charlie that things wouldn’t hurt; I’d say they would be sore, but there was medicine to help. By the time we got to hospital, we had eradicated the fear of the unknown.” Alison

heart operations, including the procedures that would happen and what it might feel like. We read hospital books and looked at photos of other children

things wouldn’t hurt; I’d say

the time we got to hospital, we had eradicated the fear of the

heart operations, including the procedures that would happen and what it might feel like. We read hospital books and looked

the time we got to hospital, we had eradicated the fear of the


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ImmunisationsYou may want to know if your child should have routine immunisations and whether there could be an increased risk of complications because of the heart condition.

We have sought the opinions of cardiologists and paediatricians to try to bring you a clear overview of why immunisations are important and what added precautions may need to be taken.

Your health visitor should be able to let you know where and when routine immunisation sessions are held in your area. If your child misses a vaccination appointment don’t worry; he or she does not have to start the course of vaccines again. But you will want to call your health centre to let them know why you were unable to attend, and to arrange another appointment.

Protection

Immunisations are given as injections. They help the body to protect itself against some infections. There is a recommended schedule for childhood immunisations. Routine immunisations start from two months old and are given throughout childhood. The final doses are usually given at fifteen years old.

AGE WHAT IS IT FOR? DOSE2 months Diphtheria; Tetanus; Whooping Cough; Hib

& Polio1st dose

Pneumococcal Conjugate Vaccination 1st doseRotavirus Vaccine 1st dose

3 months Diphtheria; Tetanus; Whooping Cough; Hib & Polio

2nd dose

Rotavirus Vaccine 2nd doseMeningitis C 1st dose

4 months Diphtheria; Tetanus; Whooping Cough; Hib & Polio

3rd dose

Pneumococcal Conjugate Vaccination 2nd dose


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AGE WHAT IS IT FOR? DOSE12/13months

Meningitis C and Hib boosterMMR (Measles, Mumps and Rubella) 1st dosePneumococcal Conjugate Vaccination 3rd dose

2 years Polysaccharide Pneumococcal Vaccination (PPV)

3 - 5 years MMR 2nd doseDiphtheria; Tetanus; Whooping Cough and Polio

booster

12 - 13 years

HPV Vaccine 2 doses between 6 months and 2 years apart

13 - 18 years

Low dose Diphtheria; Tetanus and Polio 2nd boosterMeningitis C booster

16 - 25 years

Older children may be offered Meningitis C if not previously immunised

Flu Vaccination - InfluenzaFrom six months old children with complex heart disease will be offered a flu vaccination once a year.

6 months - 2 years Inoculation2 - 17 years Nasal spray18 years upwards Inoculation

The yearly influenza immunisation may be offered as an intra-nasal preparation (nasal spray) when your child reaches two years of age. Most congenital heart disease patients can have this effective alternative except for those on Aspirin, those allergic to egg, those with significant immune compromise and those with breathing difficulties. Parents should talk to their cardiologists if they are concerned that one of these contraindications may be true for their child.

In addition to flu vaccinations, vaccinations against RSV (bronchiolitis) may also be offered to your child, but you should ask your child’s cardiologist for more advice on this. This is usually given monthly from October to February or March using a vaccine called Palivizumab and may require special arrangements with your GP or local hospital because of its expense.


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What is it for?

DiphtheriaDiphtheria is a highly infectious disease caused by bacteria and is spread through people who are already infected when they sneeze or cough. In the early stage, the symptoms include a sore throat, swollen neck glands and a fever, but in the later stages diphtheria can cause damage to the lungs, heart and nervous system.

TetanusThe germs that cause tetanus are found in soil and can be passed into your body through cuts and burns. They affect the muscles, causing stiff muscles, and interfere with breathing.

Whooping CoughThis is a very exhausting and painful disease for children who contract it, as it lasts several weeks and can cause vomiting and difficulties with breathing after long periods of coughing.

Hib (Haemophilus influenzae type B)Despite the misleading name, this is a bacterial infection that is not actually related to influenza. It tends to affect mainly babies and children up to the age of four, and can lead to many serious illnesses, including meningitis, blood poisoning and pneumonia.

Meningitis CMeningitis C is one of the bacterial causes of meningitis or blood poisoning (septicaemia). Children can catch the infection through droplets from the sneezes and/or coughs of an infected person. Not all forms of meningitis can be prevented by immunisations.

PolioThis is a virus which is passed on from human faeces, sewage or saliva of someone with the disease. Although immunisation means that it is no longer a problem in the UK, polio does still exist in developing countries. It affects the nervous system, leading to symptoms such as headaches, upset stomach and stiffness of back and neck. It can sometimes cause permanent muscle paralysis. The current vaccine is inactivated so that it can’t cause the disease - something that used to occur in rare cases with the polio drops.


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RotavirusRotavirus causes gastroenteritis with diarrhoea and vomiting. Before the vaccine came out this illness was so common that by the time children were five years old just about every child had had the infection. Children with heart problems have enough difficulties without losing a few days to this unpleasant illness so joining in with the rest of the population in having this vaccine is advisable. This vaccine is contraindicated after six months of age because there is a small risk of a serious bowel disorder.

MMRMeasles, mumps and rubella (German measles) are diseases that are all caused by viruses. Measles causes a rash and rarely severe pneumonia and encephalitis, while mumps causes swollen glands. Rubella is the mildest of all three but if contracted by a pregnant woman, it can harm her unborn baby.

Pneumococcal InfectionThe pneumococcus is a bacterium that can cause serious chest infections, ear infections and meningitis. It is spread by people coughing and sneezing. This is a relatively recent addition to the routine vaccination schedule. Where your cardiologist considers your child to be at increased risk of this infection you will be offered an additional vaccination at two years which protects against a wider range of these bacteria.

RSV (Bronchiolitis)This is a virus that causes a wheezy chest infection in children under one year old. It is spread by coughing, sneezing and on the hands of carers. The immunisation is short-lasting so it is given monthly over the winter if it is needed.

HPVThe HPV vaccine protects against two of the most serious human papilloma viruses that cause cervical cancer. The inoculation is given in three doses spread over six months to girls in secondary schools. This inoculation will not replace the need to have cervical screening later in life.


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Some questions and answers on immunisationsWhy does my child need an injection when no one has the disease any more?These illnesses still affect many millions of children across the world and are just a short plane journey away. If children were not vaccinated against the disease the illnesses would soon come back into the country: for example, whooping cough has increased because some parents decided not to immunise their child.

Should my child have all the vaccinations?Yes, and it is important not to delay during the first year of your child’s life when they are often more prone to infections. By vaccinating them against serious life-threatening diseases you offer them some protection from unnecessary illness even though they may still get simple colds and coughs.

Most doctors now agree that immunisations should be given regardless of mild illnesses or planned surgery as these are the children who most need to be protected. Some vaccines do cause a mild reaction, including a temperature, so should not be given later than 48 hours prior to surgery. The MMR immunisation should not be given later than ten days prior to surgery. Immunisations close to surgery should be confirmed by your cardiologist prior to administration.

Are there any children who should not have immunisations?If your child has problems in fighting infections because they have a reduced immunity it is extremely important to offer them immunisations to help to protect them, but extra care may be needed as to which form of injection is used.

Should my child have the immunisations if they’ve had a reaction to one before?Research shows that the vast majority of children who have a reaction to a vaccination can be safely given further vaccinations. If the reaction was particularly severe you should discuss this with your health visitor, your GP and your cardiologist.

Will the immunisations react with any of my child’s medications?Most routinely given cardiac medications will not react with immunisations. However, where children are on blood-thinning treatment such as Warfarin, then the vaccine may need to be given subcutaneously (just under the skin)


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rather than into the muscle. Talk to the doctor who prescribes the Warfarin before the injection is given.

If your GP or health visitor is concerned about giving any vaccination, they can ring your congenital cardiac specialist nurse or cardiologist for advice.

Are these immunisations safe?Parents always worry about the risks of giving vaccinations. It would be wrong to say that vaccinations are 100% risk-free, but the risks of the vaccination are far lower than the risks if you had the disease. Always spend time making sure that you understand why an immunisation is being given before giving consent for the injection. Health visitors and GPs as well as paediatricians and cardiologists should be able to answer your questions.

If I want more information about immunisations, where can I find it?All health centres now have written information on current immunisations and your health visitor and GP will be able to tell you more. You can also find more information on immunisations at www.nhs.uk/conditions/vaccinations/pages/vaccination-schedule-age-checklist.aspx. If you still have unanswered questions talk to the cardiac specialist nurse or outreach nurse attached to your hospital.


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Dental Care

Good dental hygiene is extremely important for children with single ventricle heart conditions. The reason it is even more important for these children is that they are at an increased risk of something called endocarditis (a very serious infection of the inner lining of the heart), which is explained below. Always tell your dentist about your child’s heart condition and any medications he or she takes, especially anticoagulants such as Warfarin or Aspirin.

What is Endocarditis?We all have small bugs that live in our mouths and on our skin; they cause us no harm at all until we get gum disease (gingivitis/periodontitis) or a bad or broken tooth, or a skin injury which becomes infected. For most of us, this would mean a course of antibiotics and we would then be fine, but if you have congenital heart disease it can be more serious.

The bugs that infect our mouths can get into the blood stream and attach to the areas in the heart where surgery has been performed. The bugs then grow and cause the heart to become infected; this is called infective endocarditis. If this should happen, the only way to cure the infection is a four- to six-week course of intravenous antibiotics. If this goes untreated your child would become sicker and sicker. Skin infections would need to be treated equally swiftly.

How should I look after my child’s teeth?Ask your dentist if you would like any extra advice about looking after your child’s teeth. The ideas below should help, as the best approach is to try to prevent any infection from happening.

Dental HygieneYAll heart patients should visit the dentist as often as is suggested by them. These routine check-ups should continue even when another specialist such as an orthodontist is involved.

YFrom the first signs of tooth growth, children should brush their teeth twice a day, using an age-appropriate toothbrush and toothpaste containing fluoride. Children up to the age of eight may need adult help with brushing.


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YUse a small smear of a fluoride toothpaste. Ask your dentist whether fluoride supplements (e.g. drops) or a high-fluoride toothpaste would be beneficial for your child in the area where you live, or whether fluoride varnish (Duraphat) can be applied to the baby and new adult teeth regularly.

YSpecial sealants called fissure sealants can sometimes be applied to the biting surfaces of the back adult teeth (six-year molars onwards). Your dentist will advise whether this is suitable for your child. It can help keep the molars clean and free from decay, but it is not a substitute for following good dental hygiene advice.

DietYSnacks should be healthy: cheese, milk and raw vegetables. Children with single ventricle hearts often need extra calories in their diet, so nutritious snacks should be planned as part of your child’s day, e.g. three meals plus two or three snacks, instead of constant ‘grazing’ which is bad for teeth.

YSweet drinks (including squash and fruit juices), sweets and fruit should be limited to mealtimes or immediately after meals. Brushing the teeth well afterwards may help.

YWater and plain milk are the best drinks for teeth. However, many children with single ventricle hearts will have calorie supplements in milk or juice (or high-calorie milk) at some point as babies or children. It is important that your child does not feed continuously.

YRequest sugar-free medicines on your child’s repeat prescriptions - this is sometimes abbreviated to ‘SF’. If your child can manage tablets instead of liquid medicines, this may be helpful from a dental point of view.

LifestyleYOral piercings should be avoided at all costs. They are very likely to cause nasty infections even in otherwise healthy people. Piercings of all types can lead to cardiac infections.

Dental Treatment, Antibiotics and EndocarditisIn 2008, new guidelines were published on the prescribing of antibiotics for dental treatment (and reviewed in 2011).

NICE, the government organisation that rationalises medical treatments, has researched the use of antibiotics during dental treatment. Its recommendations are that no one needs antibiotics as a preventive measure (sometimes referred to as ‘antibiotic prophylaxis’ by doctors and dentists) during dental and skin treatment.


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These new guidelines are very different from the previous guidance. Dentists must follow these recommendations so are unable to prescribe antibiotics themselves, but there are still a few cardiologists who would prefer their patients to have antibiotics when they have dental treatment or if they choose to have piercings. Next time you have an outpatient appointment ask your cardiologist what they would like for your child. If they do want your child to have antibiotics, then it is their responsibility to prescribe and arrange administration of them. They should not ask your dentist or GP to do this.

Baby teeth naturally falling out should not pose any risk to your child.

Do cardiac medications have any impact on dental care?Most children with single ventricle heart conditions take anticoagulants such as Warfarin or Aspirin. As these medications make it more difficult for blood to clot, some dental procedures, such as having a tooth removed, could cause more bleeding than in another child.

Remind your dentist about these medications before any dental treatment begins. He or she might ask for your child to have an extra blood test (INR) the day before the procedure. If you have a home testing kit (CoaguChek machine), it would be really helpful to your dentist if you can take an INR reading on the morning of the procedure.

Planning dental procedures early in the morning is sensible, so that you can easily get help if any complications such as unexpected bleeding happen later in the day.

The NHS National Patient Safety Agency states that an INR of four or less can be safely managed in a standard dental setting. Your dentist will know what extra precautions are needed (e.g. special packing or extra stitches if a tooth is taken out).

Your dentist will advise you how to care for your child’s mouth after any treatment. The advice will cover areas such as resting, being very careful as the blood clot forms, and what to do if bleeding starts again. Regular Paracetamol may be advised as a form of pain relief. Unless you are told otherwise, avoid taking medicines like Ibuprofen or Aspirin at the same time as Warfarin.

Further InformationPlease talk to your dentist, cardiologist or cardiac specialist nurse if you have any queries at all about caring for your child’s teeth.

Little Hearts Matter can also help with queries and get advice from our dental adviser; call us on 0121 455 8982 or email [email protected].


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Other Support and Information AvailableThe following publications are available from Little Hearts Matter, free of charge to members.

Antenatal Information Booklet and DVDA guide for parents following an antenatal diagnosis of a single ventricle heart condition during a routine pregnancy scan.

Feeding at Home - a guide for parentsThis booklet covers many aspects of feeding a child with a complex heart condition, and it includes medical input and parents’ experiences.

Preparation for HospitalThis booklet has been designed to help parents to prepare their children for a hospital visit. It also includes some practical tips which other parents have found useful in the hospital environment.

Benefits - a guide for parentsA guide which describes some of the sources of funding which may be available to help families.

Education Booklet - Early Years and Infant SchoolThis booklet is part of a series to help children with a single ventricle heart condition make the most of their education and covers early years and infant school.

Education Booklet - Junior SchoolThis booklet helps families positively face the challenges that single ventricle heart disease creates for children in school. It concentrates on the middle years in school and looks at the transition between Key Stage 2 and Key Stage 3.

Education Booklet - Transition to Secondary SchoolThis booklet has been created to help children and parents to plan the move into secondary school and to build a partnership between school and home life.

Statutory Assessment and Statementing BookletThis explains the different levels of support possible, how statutory assessment works and who is involved.


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Living with AnticoagulationThis booklet aims to equip parents and children with an understanding of why anticoagulation is needed, how to manage it on a day-to-day basis and what the associated risks are.

The FontanThis provides information on the operation, what you can expect to happen when you go into hospital and the changes you may notice afterwards.

Sports and Exercise BookletWritten primarily for parents, but it will be useful for young people, this booklet aims to give overall guidance for school sports and exercise and includes a chart which can be used to give an up-to-date personalised picture.

Travel and TripsA booklet which aims to guide you through the planning of your holiday, weekend or even the fi rst time your child goes away without you.

MCT Diet - Information booklet for parentsDesigned to be used in conjunction with information from your dietician following a prescription of this specialised diet, this booklet provides recipe ideas, useful food ideas and practical information to help families.

Disability Living Allowance - a guideA booklet designed to guide families through the complex application for Disability Living Allowance; a form with sample answers and points to consider is also included for babies and children.

From Us to You bookThis book has been written by bereaved families who want to share their journey through grief.

Preparation for Hospital PackA pack which contains toys and books to help prepare your child for a hospital visit. It is available from the LHM offi ce to borrow for a period of three weeks.

0121 455 89829am to 10pm

seven days a week.


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Acknowledgements

Written by: Suzie Hutchinson RGN, RSCN Chief Executive, Little Hearts Matter

Dr Stephen Williams, MBChB, FRCPCH, MSc Comm Child Health Paediatrician

Dr Jonathan J Carter, BDS (Wales) Dentist

Medically Edited by: Justine Kidd, RGN, RSCN Cardiac Nurse Specialist

Verified by: Dr Oliver Stümper, MD, PhD Consultant Paediatric Cardiologist

Edited by: Isabel Baumber Parent Trustee

Designed by: Deb Rahman Design and Information Administrator Little Hearts Matter

Illustrations by: Jane Fallows

Disclosure: Dr Oliver Stümper has no conflict of interest.

Funded by: The James Tudor Foundation.

References are available from the LHM office on request.


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Documents

LHM Filofax June2014RedesignDec2014 · visit the LHM website at or email us on [email protected]. uk. Verified by Dr Oliver Stümper October 2014 0121 455 8982. Little Hearts Matter is