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Heart disease, congenital

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Introduction


Congenital heart disease is a general term for a range of birth defects that affect the normal workings of the heart. Congenital means that a condition is present at birth.

Congenital heart disease is one of the most common types of birth defect, and affects an estimated 6 in every 1,000 babies born in England.

Factors that are known to increase the risk of congenital heart disease include:

  • Down's syndrome - a genetic disorder that affects a baby's normal physical development and causes mild to moderate learning difficulties
  • the mother having certain infections, such as rubella, during pregnancy
  • the mother having poorly controlled type 1 diabetes or type 2 diabetes

However, in most cases, no obvious cause of congenital heart disease can be found. In these cases, it doesn't appear that anything the mother was exposed to during pregnancy significantly increased the risk.

Find out more about the causes of congenital heart disease.

Symptoms of congenital heart disease can range from mild to life threatening and include:

  • chest pain
  • shortness of breath
  • blue-coloured skin, particularly on the fingers, toes and lips (the medical name for this is cyanosis)
  • getting tired easily, particularly after exercise

Find out more about symptoms of congenital heart disease, including the most common types of congenital heart disease and how they affect the body.

Serious heart defects are usually apparent soon after birth. However, less serious heart defects may not be noticed until later childhood or even adulthood.

Types of congenital heart disease

There are over 30 different types of congenital heart disease which fall into three main categories:

  • Septal defects - there is a hole between two of the heart's chambers (septal defects are commonly referred to as "hole in the heart"), which can place extra strain on the heart, causing symptoms such as fatigue and shortness of breath.
  • Obstruction defects - there is a partial or sometimes total blockage of the flow of blood through the various chambers of the heart, which can cause symptoms such as chest pain and dizziness.
  • Cyanotic heart disease - one or more defects within the heart leads to a lack of oxygen being pumped around the body, which can trigger symptoms such as cyanosis (see above), breathing difficulties and persistent tiredness.

Outlook

The outlook for congenital heart disease varies depending on the type and severity of the heart defect. However, in most cases, the outlook is reasonably good because surgery can often restore most or all of the heart's normal function.

Find out more about treating congenital heart disease.

Because of advances in heart surgery, 85% of children with congenital heart disease will survive into adulthood. However, adults with congenital heart disease have a range of often complex needs, from physical to psychological and practical needs, such as getting life insurance.

For more information, see complications of congenital heart disease.

Artery

Arteries are blood vessels that carry blood from the heart to the rest of the body.

Blood

Blood supplies oxygen to the body and removes carbon dioxide. It is pumped around the body by the heart.

Heart valves

Heart valves are four sets of flaps that control the direction that blood pumps around the heart.

Lungs

Lungs are a pair of organs in the chest that control breathing. They remove carbon dioxide from the blood and replace it with oxygen.

Ultrasound scans

Ultrasound scans are a way of producing pictures of inside the body using soundwaves.


Symptoms of congenital heart disease


Septal defects

The most common types of septal defects and their symptoms are described below.

Ventricular septal defect

A ventricular septal defect is one the most common types of congenital heart disease, affecting around 200 in every 100,000 babies. It occurs when there is a hole between the two lower pumping chambers of the heart (the left and right ventricle).

As blood pressure is higher on the left side of the heart, some blood that should be pumped around the body is pushed through the hole and into the right ventricle. The heart has to work harder to pump the excess blood out of the right ventricle, which can sometimes put a strain on the heart.

Symptoms of a ventricular septal defect depend on the size of the hole. Small holes do not usually cause any symptoms and often close by themselves by the time a child reaches the age of two.

Moderate-sized holes usually cause noticeable symptoms that develop soon after birth, such as:

  • excessive sweating, particularly during feeding
  • the baby appearing unusually tired
  • the baby not growing at the expected rate
  • repeated infections of the chest or airways
  • rapid breathing
  • rapid heartbeat

Larger holes cause the same symptoms, but they are usually more severe.

A small number of children with a ventricular septal defect may develop cyanosis during exercise. This is where the skin turns blue, particularly around the fingers, toes and lips.

Atrial septal defects

Atrial septal defects are another common type of congenital heart disease. Like ventricular septal defects, they affect about 200 in every 100,000 babies.

In most cases, atrial septal defects do not cause any symptoms during childhood and are usually not recognised until they're diagnosed during a test for an unrelated condition.

If symptoms are present, they are usually mild to moderate and include:

  • shortness of breath during physical activity
  • poor weight gain and limited physical growth
  • repeated infections of the chest or airways

If an atrial septal defect is not diagnosed or treated until adulthood, it can often lead to complications such as:

  • heart failure - where the heart has difficulty pumping the required amount of blood around the body, leading to symptoms such as breathlessness and tiredness
  • pulmonary hypertension - where there is unusually high blood pressure inside the blood vessels and around the lungs, leading to symptoms such as dizziness and shortness of breath

Obstruction defects

The most common types of obstruction defects are described below.

Pulmonary stenosis

Pulmonary stenosis is a defect where the flow of blood out of the heart towards the lungs is partially blocked because the pulmonary valve is narrower than it should be. Pulmonary stenosis accounts for around 10% of cases of congenital heart disease.

Mild to moderate pulmonary stenosis does not usually cause noticeable symptoms. In severe cases, the heart has to work harder to pump blood into the lungs. This can place a strain on the heart and cause symptoms such as:

  • shortness of breath during physical activity
  • extreme tiredness

Aortic stenosis

Aortic stenosis is a less common and more serious type of obstruction defect. It accounts for around 5% of cases of congenital heart disease.

In aortic stenosis, the flow of oxygen-rich blood away from the heart towards the rest of the body becomes partially blocked because the aortic valve is narrowed.

The heart can often compensate for this during childhood by working harder. However, this can place strain on the heart that usually becomes apparent during the teenage years or adulthood. It can lead to symptoms such as:

  • an unusually strong or quick heartbeat (palpitations)
  • extreme tiredness
  • chest pain - the pain can feel tight, dull or heavy and usually passes after a few minutes
  • shortness of breath during physical activity, which can sometimes cause you to faint

The symptoms usually develop slowly and gradually get worse over time. Without treatment, many people with aortic stenosis will go on to develop heart failure.

Coarctation of the aorta

Coarctation of the aorta is where the main blood vessel that takes blood out of heart (the aorta) is narrower than it should be.

Coarctation accounts for around 10% of cases of congenital heart disease. It can occur by itself or in combination with other types of heart defects, most commonly a ventricular septal defect or a type of defect known as a patent ductus arteriosus (see below).

In around half of all cases, the narrowing is so severe that symptoms usually develop in the first few days after birth and include:

  • poor feeding
  • rapid heartbeat
  • rapid breathing
  • unusual drowsiness and a lack of energy
  • a blue tinge to the skin (cyanosis)

In the remaining half of cases, coarctation of the aorta does not cause any noticeable signs and symptoms until later childhood or, in some cases, adulthood. The two most common symptoms are:

  • high blood pressure - most people are unaware that they have high blood pressure, so this symptom is usually only uncovered during a blood pressure test
  • a heart murmur - a kind of swishing noise that can be heard as the heart beats

Other symptoms can include:

  • headache
  • chest pain
  • extreme tiredness

Cyanotic heart disease

The most common types of cyanotic heart diseases are described below.

Tetralogy of Fallot (TOF)

Tetralogy of Fallot (TOF) is not one heart defect but a combination of four different heart defects. Tetralogy is a Greek word that means "four-fold", and Fallot is the name of the doctor who first identified the condition.

TOF is one of the most common types of cyanotic heart diseases, although it is still rare in general. Only about 30 in every 100,000 babies will be born with TOF.

The four defects that affect the heart in TOF are:

  • ventricular septal defect - a hole between the left and right ventricle
  • pulmonary stenosis - narrowing of the pulmonary valve
  • right ventricular hypertrophy - where the muscles of the right ventricle are unusually thick
  • displaced aorta - where the aortic valve is in the wrong place

Due to this complex set of heart defects, oxygen-rich blood and oxygen-low blood become mixed. This leads to blood with a lower-than-normal oxygen content being pumped around the body.

The symptoms of TOF may develop shortly after birth and will almost certainly be present by two years of age.

Symptoms include:

  • low birth weight
  • poor feeding
  • a blue tinge to the skin (cyanosis), particularly when feeding
  • rapid breathing
  • fingernails that appear abnormally round and large, known as clubbing
  • delayed physical growth - including delayed puberty

You may also notice that your child prefers to sit and sometimes sleep with their knees drawn up to their chest while resting their head and arms on their knees. This is known as squatting and is common in children with cyanotic heart disease because it can help improve the flow of oxygen around the body.

Transposition of the great arteries (TGA)

Transposition of the great arteries (TGA) is another common cause of cyanotic heart disease. It accounts for around 5% of cases of congenital heart disease.

In TGA, the pulmonary and aortic valves and the arteries they are connected to have swapped positions. This leads to oxygen-low blood, which should be pumped into the lungs, being pumped around the body.

The skin turning blue (cyanosis) is a common symptom of TGA and is usually noticeable at birth. Other symptoms develop three to six weeks after birth and include:

  • rapid breathing
  • rapid heartbeat
  • delayed weight gain
  • excessive sweating

Tricuspid atresia

Tricuspid atresia is an uncommon type of cyanotic heart disease where the tricuspid heart valve is either missing or is abnormally developed. This disrupts the normal flow of blood through the heart, which means that not enough blood is sent to the lungs to receive oxygen.

It is estimated that 10 babies in every 100,000 are affected by tricuspid atresia.

The symptoms of tricuspid atresia include:

  • a blue tinge to the skin (cyanosis), which is usually present at birth
  • shortness of breath
  • fatigue
  • feeding difficulties
  • excessive sweating
  • failure to grow at the expected rate
  • repeated respiratory infections

Total anomalous pulmonary venous connection (TAPVC)

Total anomalous pulmonary venous connection (TAPVC) occurs when the veins that bring oxygen-rich blood back from the lungs to the heart are located in the wrong place. They are connected to the right atrium when they should be connected to the left atrium. In some cases of TAPVC, there is also a blockage in the main vein that connects the lungs and the heart (the pulmonary vein).

TAVC is an uncommon type of congenital heart disease that affects around 7 in every 100,000 babies. The pattern of symptoms will depend on whether the pulmonary vein is blocked.

If the vein is blocked, the symptoms of TAPVC usually develop within 24 to 36 hours after birth and include:

  • a blue tinge to the skin (cyanosis)
  • rapid breathing
  • rapid heartbeat
  • breathing difficulties that get more severe over time

Without treatment, TAPVC associated with blockage will inevitably be fatal within a month after birth.

If the vein is not blocked, the symptoms of TAPVC are much less serious and include:

  • shortness of breath during physical activity
  • poor weight gain and limited physical growth
  • repeated respiratory infections

Truncus arteriosus

Truncus arteriosus is another uncommon type of cyanotic heart disease that affects around 5 in every 100,000 babies.

A blood vessel that should split into two smaller vessels as the baby's heart develops during pregnancy remains as a single vessel. This results in too much blood being sent to the lungs which, over time, can cause breathing difficulties and damage the blood vessels inside the lungs.

The initial symptoms of truncus arteriosus include:

  • a blue tinge to the skin (cyanosis)
  • poor feeding
  • rapid heartbeat
  • excessive sweating

If truncus arteriosus is not treated, heart failure will develop. This usually leads to death within a year of birth.

Patent ductus arteriosus (PDA)

Patent ductus arteriosus (PDA) is a rare type of congenital heart disease that does not fit into the categories of septal, obstructive or cyanotic heart disease. It is estimated that around 5 in every 100,000 babies are affected by PDA.

The ductus arteriosus is a duct or passage in the heart that is meant to close shortly after birth. When an unborn baby is in the womb, there is no need for the baby's heart to send blood to the lungs because the baby gets all the oxygen it needs from the mother. Therefore, the ductus arteriosus acts as a bypass between the aortic and pulmonary valves, allowing the blood to flow back into the heart without entering the lungs.

Shortly after birth, the duct should close as the baby begins to breathe normally. However, in PDA, the duct does not close completely. This means that some oxygen-rich blood that is meant to be pumped away from the lungs leaks back through the duct into the pulmonary valve and into the lungs.

Small PDAs do not usually cause any symptoms. However, larger PDAs can cause symptoms such as:

  • poor feeding
  • rapid breathing
  • rapid heartbeat
  • sweating while feeding
  • poor weight gain and limited physical growth

Older adults with untreated PDA have an increased risk of heart failure.


Causes of congenital heart disease


Congenital heart disease is caused when something disrupts the normal development of the heart.

The development of the heart

The development of the heart is a very complex process with several stages. Anything that disrupts this process could cause a type of congenital heart disease to develop.

The main stages of the heart's development are:

  • stage 1 - types of specialised cells come together to form a horseshoe-shaped tube of tissue (this occurs around day 15 of pregnancy)
  • stage 2 - the tube grows in size and begins beating (this occurs around day 20 of pregnancy)
  • stage 3 - the heart begins to loop and fold in on itself, creating the chambers of the heart. At the same time, blood vessels that connect the heart to the rest of the body are created (this occurs between day 28 and day 32 of pregnancy)

It is thought that most cases of congenital heart disease occur when something disrupts the crucial third stage of the heart's development.

While some genetic and environmental factors are known to increase the risk of congenital heart disease, in most cases no obvious cause can be found.

Risk factors

Genetic conditions

Several genetic health conditions, which a baby inherits from one or both parents, can cause congenital heart disease.

Down's syndrome is the most widely known genetic condition that can cause congenital heart disease. Children with Down's syndrome are born with a range of disabilities as the result of a genetic abnormality.

About half of all children with Down's syndrome have congenital heart disease. In 90% of cases, this is a type of septal defect.

Other genetic conditions associated with congenital heart disease include:

  • Turner syndrome - a genetic condition that only affects females. It is estimated that 25% of children with Turner syndrome will be born with congenital heart disease, which is usually a type of obstruction defect.
  • Noonan syndrome - a genetic disorder that can cause a wide range of potential symptoms. Around 80% of children with Noonan syndrome will be born with congenital heart disease. In about half of all cases, this will be pulmonary stenosis.

Maternal diabetes

Women with diabetes are five times more likely to give birth to a baby with congenital heart disease than women who do not have diabetes. It is estimated that 3-6% of women with diabetes who become pregnant will give birth to a baby with a heart defect, most commonly transposition of the great arteries.

This increased risk only applies to type 1 diabetes and type 2 diabetes. It does not apply to gestational diabetes, which can develop during pregnancy and usually goes away once the baby is born.

The increased risk is thought to be caused by the high levels of the hormone insulin in the blood, which may interfere with the normal development of a foetus.

Foetal alcohol syndrome

Foetal alcohol syndrome refers to a range of birth defects caused by a child's mother drinking too much alcohol during pregnancy. Alcohol can have a poisonous effect on the tissue of the foetus.

It is estimated that as many as half of all children with foetal alcohol syndrome will have congenital heart disease, most commonly an atrial septal defect.

The Department of Health recommends that pregnant women should not drink alcohol. If you choose to drink, you should not drink more than one or two units of alcohol once or twice a week, to minimise the risk to your unborn baby.

See Can I drink alcohol if I'm pregnant? for more information about alcohol and pregnancy.

Rubella

Rubella (German measles) is an infectious condition caused by a virus. Rubella is not usually a serious infection for adults or children. However, it can have a devastating effect on an unborn baby if a mother develops a rubella infection during the first 8-10 weeks of pregnancy.

A rubella infection can cause multiple birth defects, including congenital heart disease. All women of child-bearing age should be vaccinated against rubella. The vaccine is now given as part of the routine childhood vaccination schedule. Contact your GP for advice if you're not sure whether you have been vaccinated for rubella.

Flu

Women who get flu during the first trimester (three months) of pregnancy are twice as likely to give birth to a baby with congenital heart disease than the general population. The reasons for this are unclear.

Vaccination for seasonal flu and swine flu is recommended for all pregnant women.

Ibuprofen

Women who take the painkiller ibuprofen during the first trimester of their pregnancy are twice as likely to give birth to a baby with congenital heart disease than the general population.

Paracetamol is a safer alternative, although ideally you should avoid taking any medicines while you are pregnant, particularly during the first three months of pregnancy.

See Can I take ibuprofen when I'm pregnant? and Can I take paracetamol during pregnancy? for more information and advice.

Phenylketonuria (PKU)

Phenylketonuria (PKU) is a rare genetic condition that is present from birth. In PKU, the body cannot break down a chemical called phenylalanine which builds up in the blood and brain. This can cause symptoms such as learning and behavioural difficulties.

PKU can usually be controlled by sticking to a low protein diet. Pregnant mothers who do not do this are six times more likely to give birth to a baby with congenital heart disease than the general population.

Organ solvents

Women who are exposed to organic solvents are three times more likely to give birth to a baby with congenital heart disease than the general population. Organic solvents are chemicals found in a wide range of products and substances, such as:

  • paint
  • nail polish
  • glue

Diagnosing congenital heart disease


Diagnosis before the birth

More and more cases of congenital heart disease are diagnosed before a baby is born. This is done using a procedure called foetal echocardiography.

Foetal echocardiography uses a specially designed ultrasound scanner to build up a picture of the inside of the chambers of the heart. The procedure should be carried out during routine antenatal examinations, usually some time between week 18 and week 20 of pregnancy.

However, it is not always possible to detect heart defects, particularly mild ones, using foetal echocardiography.

Diagnosis after the birth

If a baby is born with cyanotic heart disease, a diagnosis can usually be made quickly and confidently due to the distinctive blue colour of their skin (called cyanosis).

If your baby is born with a septal defect or an obstruction defect, their symptoms may not be noticeable for several months or sometimes many years after they are born.

Possible signs that your child has cyanotic heart disease include:

  • not developing at the expected rate
  • shortness of breath during physical activity
  • complaining of feeling tired most or all of the time
  • poor feeding

See your GP if your child has any of these symptoms. Further testing can usually help confirm or disprove a diagnosis of congenital heart disease.

Further testing

Further tests that your child may have are described below.

Echocardiography

Echocardiography may be used to check the inside of your child's heart. Sometimes, problems with the heart that were missed during foetal echocardiography can be detected as a child develops.

Electrocardiogram

An electrocardiogram is a test that measures the electrical activity of the heart. Electrodes are placed on the skin around the heart and are connected to a computer. The computer analyses the electrical signals that are produced by the heart to assess how well it is beating.

Chest X-ray

A chest X-ray of the heart and lungs can be used to check whether there is an excess amount of blood in the lungs or whether the heart is larger than normal. Both may be signs of heart disease.

Pulse oximetry

Pulse oximetry is a test that measures the amount of oxygen present in your child's blood.

The test involves placing a special sensor on your child's fingertip, ear or toe which sends out light waves. A computer is connected to the sensor and measures how the light waves are absorbed.

Oxygen can affect how the light waves are absorbed, so by analysing the results the computer can quickly determine how much oxygen is present in your child's blood.

Cardiac catheterisation

Cardiac catheterisation is a useful way of obtaining more information about exactly how the blood is being pumped through your child's heart. The procedure is performed under a local anaesthetic and so is painless.

A small flexible tube, called a catheter, is inserted into one of your child's blood vessels, usually in the groin or arm. The tube is moved up into the heart, guided by X-rays or MRI scanners.

The end of the tube contains a tiny blood pressure monitor, which can be used to take blood pressure readings in different parts of the heart. A coloured dye that shows up on X-rays can also be injected into the tube. The dye can be studied as it moves through the heart, enabling medical staff to see how well each chamber of the heart is working.


Treating congenital heart disease


Specific treatments for the most common types of congenital heart disease are described below.

Septal defects

Ventricular and atrial septal defects are treated in much the same way. The recommended treatment depends on the size of the defect and whether your child has any symptoms.

No treatment will be required if your child has a small septal defect that does not cause any symptoms. These types of septal defects have an excellent outcome and do not pose a serious threat to your child's health.

If your child has a larger septal defect that causes symptoms such as extreme tiredness and failure to grow, a combination of medications is usually recommended. These may include:

  • diuretics - medication that removes fluid from the body, making breathing easier
  • angiotensin-converting enzyme (ACE) inhibitors - these widen the walls of the blood vessels, which should help reduce the strain on your child's heart and improve their circulation
  • digoxin - medication that slows down the speed of your child's heartbeat and increases the strength at which the heart pumps, which should help relieve symptoms

Your child may also be given high-calorie food supplements to help stimulate their physical growth.

If medication does not control your child's symptoms, surgery will usually be required.

Two main types of surgery can be used:

  • open heart surgery - the surgeon makes a cut in the chest to access the heart and closes the septal defect, sealing it with a synthetic patch
  • transcatheter repair - a small tube called a catheter is guided to the heart through blood vessels, and special tools are passed down it and used to close the defect

Unlike open heart surgery, transcatheter repair does not cause any scarring and is associated with less pain after the operation and a quicker recovery.

However, transcatheter repair is a specialised technique that is only available at units that specialise in treating heart disease in children. Therefore, waiting times for this type of surgery may be longer than for open heart surgery.

Obstruction defects

Pulmonary stenosis

Mild pulmonary stenosis does not require treatment. This is because it does not cause any symptoms and is unlikely to pose a serious threat to your child's health. However, in most cases, an annual check-up is likely to be recommended.

Moderate to severe cases of pulmonary stenosis usually require treatment, even if they cause few or no symptoms. Without treatment, pulmonary stenosis could lead to heart failure in later life. In these circumstances, a surgical technique known as a balloon pulmonary valvuloplasty is usually the preferred surgical option.

A balloon pulmonary valvuloplasty involves guiding a small tube (catheter) through the blood vessels to the site of the narrowed pulmonary artery. A balloon attached to the catheter is inflated, which helps to widen the pulmonary artery and relieve any blockage in blood flow.

Aortic stenosis

Treatment for aortic stenosis is usually delayed until your child develops symptoms, even if they are diagnosed shortly after birth. However, they will probably be referred for check-ups every six months.

Initial surgery for aortic stenosis is a balloon valvuloplasty, as described above. If this does not improve your child's symptoms, it is usually necessary to remove and replace the valve in open heart surgery.

Replacement valves can be obtained from human donors, made from artificial materials, such as titanium, or taken from pigs before being modified for human use. Pigs are used because, biologically, their heart is closest to the human heart.

An alternative technique has recently become available that involves using a catheter to replace the aortic valve. The technique is known as a catheter insertion of a new aortic valve.

During the procedure, a catheter is passed through the groin and up into the heart. A replacement valve is then passed through the catheter and into position.

As catheter insertion of a new aortic valve is a new procedure, there is limited evidence about its long-term safety and effectiveness. This type of treatment is usually only recommended for people who are too ill or too weak to cope with the effects of open heart surgery.

Coarctation of the aorta

If your child has the more serious form of coarctation of the aorta that develops shortly after birth, they will be given medication, such as diuretics and digoxin, to help stabilise them. Surgery to restore the flow of blood through the aorta is usually recommended.

Several surgical techniques can be used, including:

  • removing the narrowed section of the aorta and reconnecting the two remaining ends
  • inserting a catheter into the aorta and widening it with a balloon or metal tube (stent)
  • removing sections of blood vessels from other parts of your child's body and using them to create a bypass around the site of the blockage (this is similar to a coronary artery bypass graft which is used to treat heart disease)

If your child develops the less serious form of coarctation of the aorta in later life, the main goal of treatment will be to control their high blood pressure. This will usually involve using a combination of diet, exercise and, in some cases, medication. Find out more about treatment for high blood pressure.

Cyanotic heart diseases

Tetralogy of Fallot (TOF)

Tetralogy of Fallot (TOF) is usually treated using surgery. If your baby is born with severe symptoms, surgery may be recommended soon after birth. If the symptoms are less severe, surgery will usually be carried out when your child is three to six months old.

Open heart surgery is usually used to treat TOF. During the procedure, the surgeon will seal the ventricular septal defect and widen the pulmonary valve.

Transposition of the great arteries (TGA)

Shortly after birth, your baby will be given an injection of medication called prostaglandin. This will prevent the ductus arteriosus closing after birth. The ductus arteriosus is the passage between the aortic and pulmonary valve.

Keeping the ductus arteriosus open means that oxygen-rich blood is able to mix with oxygen-poor blood, which should help relieve your baby's symptoms. In some cases, it may also be necessary to use a catheter to create a temporary hole in the atrial septum (the wall that separates the two upper chambers of the heart) to further encourage the mixing of blood.

Once your baby's health has stabilised, it is likely that surgery will be recommended. This should ideally be carried out during the first month of the baby's life. A surgical technique called arterial switch is used. This involves detaching the arteries and reattaching them in the correct position.

Tricuspid atresia

As with TGA, if your baby has tricuspid atresia they will be given prostaglandin shortly after being born to encourage the mixing of oxygen-rich blood with oxygen-poor blood. The condition will then need to be treated using a three-stage procedure.

The first stage is usually performed during the first few days of life. An artificial passage, known as a shunt, is created between the heart and the lungs so that blood can enter the lungs. However, not all babies will need to have a shunt.

The second stage will be performed when your child is four to six months old. The surgeon will connect veins from the upper part of the body, which carry oxygen-poor blood, directly to your child's pulmonary valve. This will allow blood to be pumped into the lungs, where it can be filled with oxygen.

The final stage is usually performed when your child is 18 to 36 months old. It involves connecting the remaining veins to the pulmonary valve. After this final stage, your child should be completely or mostly free of symptoms.

An effective alternative to this three-stage procedure would be to carry out a heart transplant, where a healthy heart is taken from a donor and surgically implanted into your baby. However, heart transplant surgery is rarely carried out in these circumstances because of a shortage of donated hearts that are a suitable size for a young baby.

Total anomalous pulmonary venous connection (TAPVC)

Total anomalous pulmonary venous connection (TAPVC) is also treated with surgery. During the procedure, the surgeon will disconnect the veins that are incorrectly connected to the right atrium, before reconnecting them to the left atrium.

The timing of surgery will usually depend on whether your child's pulmonary vein (the vein that connects the lungs and heart) is also obstructed. If the pulmonary vein is obstructed, surgery will be performed after birth. If the vein is not obstructed, surgery can often be postponed until your child is a few weeks or months old.

Truncus arteriosus

Once a diagnosis of truncus arteriosus has been confirmed, it is likely that your baby will be given diuretics to help relieve their symptoms and stabilise their condition. Diuretics are a type of medication that removes fluid from the body.

Surgery will then be performed, usually within a few weeks after birth. The abnormal blood vessel will be split in two to create two new blood vessels, and each one will be reconnected in the correct position.

Patent ductus arteriosus (PDA)

Many cases of patent ductus arteriosus (PDA) can be treated with medication shortly after birth. Two types of medication have been shown to effectively stimulate the closure of the duct that is responsible for PDA. These are:

  • indomethacin
  • ibuprofen

If PDA does not respond to medication, a catheter can be used to seal the duct with a metal coil or plug.


Complications of congenital heart disease


Developmental delays

Many children with congenital heart disease experience delays in their development. For example, they may take longer to start walking or talking. They may also have lifelong problems with physical co-ordination.

Around half of all children with cyanotic heart disease also have learning difficulties, which are thought to be caused by a poor oxygen supply during early life that affects the development of the brain.

Natural intelligence is usually unaffected, but these children often perform well below the academic level they would be expected to reach.

This is due to problems such as:

  • impaired memory
  • problems expressing themselves using language
  • problems understanding the language of others
  • low attention span and difficulty concentrating
  • poor planning abilities
  • poor impulse control - acting rashly without thinking about the possible consequences

Recent research has found that children who have had surgery for transposition of the great arteries have significant problems understanding a concept known as theory of mind (TOM)

TOM is the ability to understand other people's mental state and recognise that they may differ from your own. In other words, to recognise that everyone has their own set of desires, intentions, beliefs, emotions, perspective, likes and dislikes. In simple terms, TOM is the ability to see the world through another person's eyes.

An inability to recognise other people's mental states can lead to problems with social interaction and behaviour in later life.

Endocarditis

Both children and adults with congenital heart disease have an increased risk of developing endocarditis. Endocarditis is an infection of the lining of the heart. If it is not treated, it can be life threatening because it damages the heart's valves.

Symptoms of endocarditis include:

  • a high temperature (fever) of 38C (100.4F) or above
  • chills
  • loss of appetite
  • unexplained weight loss
  • headache
  • muscle and joint pain
  • night sweats
  • shortness of breath
  • persistent cough
  • heart murmurs - where your heart makes a whooshing or swishing noise in between beats

Endocarditis will need to be treated in hospital with injections of antibiotics.

The condition usually develops when an infection in another part of the body, such as on the skin or the gums, spreads through the blood into the heart.

Because gum disease can potentially lead to endocarditis, if you have congenital heart disease, it is very important to maintain excellent oral hygiene.

You are also usually recommended not to have any cosmetic procedure that involves piercing the skin, such tattoos or body piercings.

Complications in adults

The treatment of adults with congenital heart disease is relatively new. This is because in the past, most children with these types of heart conditions died before reaching adulthood.

The development of effective treatments for adults with congenital heart disease is very important. This is because there is growing evidence that they have an increased risk of several complications.

You may consider congenital heart disease to be something you had as a child. If you were treated for it when you were a baby, you may have no memory of it at all. However, it is important not to ignore the fact that you have a history of congenital heart disease, and to find out the following information:

  • the type of congenital heart disease you had or still have
  • the type of treatment you received
  • any recommendations that were made about check-ups, exercise or the use of medication
  • whether you have an increased risk of developing certain conditions or complications in later life, such as high blood pressure, arrhythmias or heart failure (see below)

Ask your GP for advice if you're not sure about any of these points. They can access your medical records to confirm any details that need clarifying.

Some of the main complications that can affect adults with congenital heart disease are described in more detail below.

Arrhythmia

An arrhythmia is an abnormal heartbeat. It is a relatively common complication in adults with a history of some types of congenital heart disease.

It is thought to be caused by damage to the heart muscles that occurs during heart surgery. The damaged muscles disrupt the electrical signals that the body uses to control the heart.

One of the most common types of arrhythmia to affect adults with a history of congenital heart disease is called atrial flutter. This is thought to affect up to half of all people who have surgery to treat cyanotic heart disease.

Atrial flutter is where the heart beats very quickly, sometimes up to 350 beats a minute. At rest, a normal heart rate is between 60 and 100 beats a minute.

Symptoms of atrial flutter include:

  • palpitations
  • extreme tiredness
  • shortness of breath
  • chest pain
  • dizziness
  • fainting

Due to the way the heart beats, there is also a risk of a blood clot forming inside the heart and travelling up to the lungs or the brain. This can lead to a:

  • pulmonary embolism - where the blood supply to the lungs is blocked
  • stroke - where the blood supply to the brain is blocked

Several treatment options are available for atrial flutter:

  • medication to stabilise the heartbeat, such as beta-blockers
  • medication to prevent blood clots, such as warfarin
  • catheter ablation - a thin, flexible tube called a catheter is guided through a vein to the heart and a pulse of energy is used to destroy the area of muscle that is disrupting your heart's electrical signals
  • pacemaker - an electrical device is implanted in your body and is used to regulate your heartbeat

Sudden cardiac death

Sudden cardiac death is a small but serious risk in people with a history of congenital heart disease who are affected by arrhythmia. This is where the heart suddenly goes into spasm and is unable to pump blood around the body, resulting in death. The medical term for this is ventricular arrhythmia which leads to sudden cardiac death.

It is estimated that 1 in every 1,000 adults with congenital heart disease will experience sudden cardiac death in any given year.

Tests, such as an electrocardiogram (ECG), are moderately effective in determining which people are most at risk of sudden cardiac death. An ECG records the rhythm and electrical activity of the heart.

If testing shows that you are at risk, it may be recommended that you have a device called an implantable cardioverter-defibrillator implanted in your body. This device is similar to a pacemaker and can deliver a pulse of electricity when required, which should stop your heart going into spasm.

Pulmonary hypertension

Some types of congenital heart disease, such as coarctation of the aorta and truncus arteriosus, can cause the blood pressure inside the arteries that connect the heart and lungs to be much higher than it should be. This is known as pulmonary hypertension.

Symptoms of pulmonary hypertension include:

  • shortness of breath
  • extreme tiredness
  • dizziness
  • feeling faint
  • chest pain
  • a rapid heartbeat

A range of medications can be used to treat pulmonary hypertension. Find out more about treatment for pulmonary hypertension.

Heart failure

Heart failure is where the heart cannot pump enough blood around the body to meet the body's needs. It can occur when a previously mild and untreated type of congenital heart disease, such as a minor atrial septal defect or pulmonary stenosis, gets worse in later life. Heart failure can also occur when something goes wrong with a previous surgical repair of the heart.

Symptoms of heart failure include:

  • breathlessness when you're active or sometimes resting
  • extreme tiredness and weakness
  • swelling in the legs, ankles and feet

Heart failure can be treated using a combination of lifestyle changes, such as giving up smoking and medication.

Find out more about treatment for heart failure.


Preventing congenital heart disease


As so little is known about what causes congenital heart disease, there is no guaranteed way of avoiding having a baby with the condition. However, if you are pregnant, you can reduce the risk:

  • Ensure that you are vaccinated against rubella and flu.
  • Take 400 micrograms of folic acid supplement a day during your pregnancy - research has found that this lowers your risk of giving birth to a child with congenital heart disease, as well as several other types of birth defect.
  • Check with your GP or pharmacist before you take any medication during pregnancy, including herbal remedies and medication that is available over the counter.
  • Avoid contact with people who are known to have an infection.
  • Avoid exposure to organic solvents, such as those used in dry cleaning, paint thinners and nail polish remover.

See what the doctor sees with Map of Medicine


The Map of Medicine is used by doctors throughout the NHS to determine the best treatment options for their patients. NHS Choices offers everyone in England exclusive and free access to this cutting-edge internet resource, which lets you see exactly what your doctor sees.

The information in the Map has been approved by the UK's leading clinical experts, is based on the best available clinical evidence, and is continually updated. To take advantage of this unique resource go to:

Map of Medicine: valvular heart disease


The materials in this website are provided by Medicine Chest and NHS Choices.  Neither Co-operative Group Limited or Co-operative Healthcare Limited (trading as The Co-operative Pharmacy or otherwise) shall be in any way responsible or liable for its content.

The materials in this website are in no way intended to replace the professional medical care, advice, diagnosis or treatment of a doctor.  The website does not have answers to all problems and answers to specific problems may not apply to everyone.  If you notice medical symptoms or feel unwell, you should consult your doctor.  For further information, consult the terms and conditions.


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