Summary
Generally
A congenital heart defect means that there are malformations in the heart or in the large blood vessels. Most heart defects cause no or mild discomfort, but sometimes it can be more serious. Then the child often gets symptoms during his first weeks of life and may need surgery.
Usually, it is unclear what a malformation of the heart is due to.
Most children who have been operated on for heart failure can live an active life like other children but need to go on checks for many years to come.
Symptoms
Most serious malformations cause symptoms during the child’s first weeks of life. Common symptoms are that
- the baby’s skin and lips have a bluish tone, called cyanosis
- the child is difficult to breathe
- the baby is too tired to eat enough.
Anxiety in older children can sometimes appear as fatigue and lethargy, but it is common that they have no problems at all. Instead, the child may have a heart defect in connection with other investigations.
Treatment
Most children with early symptoms need surgery early. Sometimes several operations are needed.
When to seek care?
If a child is often tired and powerless, you can contact a health care center or pediatric clinic.
One should seek care directly at a child ward or emergency ward if
- the baby’s skin and lips have a bluish tone without being chilled
- the child seems to have a hard time breathing, for example having quick and superficial or deep and sighing breaths
- the child is too tired to eat or gets stressed, sweaty or short of breath during breastfeeding or bottle feeding.
You can always call the medical advice for advice.
What are congenital heart defects?
Congenital heart defects mean malformations of the heart or malformations of the large blood vessels. For example, there may be holes between the atrium or chambers, so-called atrial septum defect or ventricular septal defect. Then the blood flow from one half of the heart to the other becomes too large, which increases the load on the heart. It can also be too tight at the heart valves, which causes the heart to work more for the blood to pass.
For example, more complicated cardiac malformations may be the absence of a chamber or flap, which causes the child to experience severe symptoms.
About one-third of all heart malformations are so mild that they do not cause any problems and do not need to be treated. One third requires treatment at some point in life. The remaining third of the malformations are more severe, cause symptoms early and therefore need to be treated during the first months of life.
The cause is usually unclear
Usually, it is not possible to find any cause of heart malformations. The birth defects occur during the earliest part of fetal development, usually within the first ten weeks of pregnancy. The fetus is usually not affected, but sometimes it can be severely affected. Many heart malformations can be detected by ultrasound examination during pregnancy.
Symptoms of congenital heart defects in children
Symptoms of congenital heart defects appear especially during the first year of life. Most serious malformations cause symptoms during the baby’s first week of life when blood circulation is switched from the fetal’s special blood circulation to the circulation needed in life outside the uterus.
Common symptoms of Congenital heart defects in children are that
- the baby’s skin and lips have a bluish tone
- the child has a hard time breathing
- the baby is too tired to eat enough.
The symptoms are often due to heart failure or lower blood oxygen levels, cyanosis. Severe heart malformations can cause the child to suddenly have severe symptoms.
Heart failure
Heart failure can occur in conjunction with certain heart defects and means that the heart does not have the capacity to pump around the blood sufficiently to provide the various organs in the body with sufficient blood flow. This causes, among other things, that the body gets too little oxygen.
When the heart is not pumping effectively, the blood is congested in the lungs. This affects the child’s breathing which becomes faster and more strained. You can see retractions between the ribs at each breath. Such symptoms may also be present in lung diseases.
In the event of heart failure, the baby’s heart beats faster than usual. The child can also be tired and powerless. In infants, it may turn out that the baby is too tired to eat enough. Therefore, it does not gain weight properly.
Cyanosis
If the baby’s skin, lips, and mucous membranes become bluish, it may be because the blood oxygen level is lowered. This, in turn, is mainly because oxygen-poor blood from the right heart does not go to the lungs to oxygenate properly. Instead, the blood transitions directly to the left heart and further into the bloodstream. It is called cyanosis and occurs in various deformities of the heart. Cyanosis is not the same as when a completely healthy child turns blue about the lips when it is cooled down, for example when bathing for a long time.
Heart failure without symptoms
Some congenital heart malformations cause no symptoms. This may include mild heart defects but also malformations that may cause symptoms later on and need to be treated later. Some of these heart malformations are detected during the first months of life. In many BB divisions, the baby’s oxygen content is measured in the blood prior to discharge, in order for severe heart failure to be detected as soon as possible.
After the infant year, it is uncommon for children to have symptoms that cause heart failure to be detected. However, heart failure is sometimes detected when the doctor listens to the heart during a regular health examination. Many of these heart malformations are mild and usually do not need treatment. However, regular checks and any treatment later are often needed. Among children who have heart defects without symptoms, there are some heart defects that can have long-term negative effects if not corrected. For example, there may be an atrial septal defect, which may need to be treated during childhood with surgery or catheter treatment.
Investigations
For example, if the child has symptoms or if the doctor, during a routine examination, detects an abnormal bleeding sound in the heart, the child is further referred for an examination at a pediatric clinic. A doctor in charge of examining, monitoring and supervising the child.
Further investigation is being done at the regional hospital. There are specialists in heart disease in children, pediatric cardiologists. There, the follow-up is also done if the child has a more complicated heart failure. As a parent, you can always be with the child in the hospital.
In the first investigation of a suspected congenital heart defect, the child is usually examined with ECG and ultrasound of the heart and sometimes X-rays of the heart and lungs.
Treatment for congenital heart defects in children
A child with heart failure is usually treated with liquid-driving medication, called diuretics, which relieve the heart. Often, the child also receives other medicines that increase the pumping capacity of the heart and reduce the load on the heart. For example, it can be called ACE inhibitors, beta-blockers or digitalis. The baby may also need extra oxygen.
It is common for infants with heart failure to be too tired to eat. Then the baby may need to be fed through a thin tube that goes through the nose down to the stomach, a so-called probe.
Operation
Children with heart defects are operated at two hospitals in the country, in Lund and in Gothenburg. There are special pediatric cardiac centers that specialize in cardiac surgery and cardiac care for children. Pediatric cardiologists and other healthcare professionals there are trained for the special conditions that apply to surgery and intensive care of children with cardiac malformations.
When it is appropriate that the child is operated on depends on the symptoms and what heart defect the child has. Sometimes it may be good for the child to be treated with drugs for a period before surgery. Some operations need to be done in several steps. In most cardiac operations, the baby is connected to a so-called cardio-pulmonary machine that oxygenates and pumps blood into the body during surgery. In all cardiac operations, the child is anesthetized in anesthesia.
Some children need surgery again later in life.
Preparatory operations
In some congenital heart defects in children, a first preparatory operation needs to be done to alleviate various symptoms while waiting for the actual surgery. Sometimes the child can be cared for at home while waiting for the preparatory operation.
Strapping
Banding means that a band is placed around the pulmonary artery to reduce flow to the lungs and lower the pressure in the pulmonary artery. See the green ring in the picture. The amount of blood then reaches the lungs in a more reasonable amount. Banding is done to reduce the symptoms of heart failure. After a few months, the tape is removed and the child undergoes permanent heart surgery.
Shunting
In some heart defects, the blood flow to the lungs is too small. It is possible to increase the blood flow to the lungs by creating a connection from the body pulse vein to the pulmonary vein using a small plastic tube. See the green ring in the picture.
A shunt operation is done on children with complicated heart defects if the definitive surgery needs to be postponed until the child is slightly larger. The purpose is to increase the blood supply to the lungs so that the oxygen content in the blood becomes better.
Catheter Treatment
Instead of surgery, some heart defects can be treated in conjunction with a so-called cardiac catheterization when a small plastic tube, catheter, is inserted into the heart via a blood vessel. It is gentler for the child than a major surgical procedure.
For example, if the child has a narrowing of the flap to the pulmonary artery, so-called pulmonary stenosis, the narrowing can be widened with the help of a balloon on a catheter. The catheter is inserted into the heart via a blood vessel in the groin and up through the narrowed flap. The balloon that sits near the tip of the catheter is then filled with contrast fluid so that the narrow flap expands and the constriction disappears. The balloon shrinks when the contrast fluid is sucked back and the catheter can then be pulled out again.
If there is a gap between the two heart halves that should not be there, they can be closed with cardiac catheterization. With the help of the catheter, you then insert a small patch of synthetic material that can seal the hole.
When the child becomes an adult
When the teenager has reached the age of 18, he or she should begin to go into adult care checks. Then a joint reception visit is often arranged where the parent together with the teenager meets both the child’s former child cardiologist and the adult cardiologist who takes over responsibility for the teen’s treatment and follow-up.
Occasionally, the child and adult cardiologist may have a joint meeting without any parent or teen. You are then called to a reception visit with the adult cardiologist. There is an organized collaboration between pediatric cardiologists, pediatric cardiac surgeons and the adult cardiologists responsible for the care of adults with congenital heart defects.
The older the child is, the more important it is for the child to participate in discussions and decisions. It becomes especially important during adolescence. The child gradually needs to be prepared for his or her own responsibility when it comes to discussions and decisions about his or her situation. This may include, for example, the medication the child should take and how to take it, other treatments, lifestyle and consequences of heart failure. New issues are also emerging, such as the issue of driving licenses, the future, vocational education, contraception, and family formation.
How is life affected by a congenital heart defect?
Physical activity and sport
Most children with heart failure can live a normal life and participate in the same activities as other children of the same age. Sometimes the child’s cardiologist needs to have contact with the sports teacher at school. It can make it easier for the teacher to adapt to the child’s activities to physical ability.
For some children, it may be necessary to adapt life to heart failure, such as not engaging in competitive sports. After some complicated surgeries, a change in blood circulation can make the child unable to cope as much as other children of the same age. The child is usually examined regularly during routine check-ups at the child’s cardiologist through, for example, ultrasound, work test, ECG, blood pressure and a general examination.
Trip
In most cases, having a congenital heart defect does not limit the ability to travel. Even children who have slightly lowered oxygen levels in their blood can travel by air because the aircraft has a pressure cabin and the child, therefore, gets enough oxygen anyway.
If the child has a more severe heart defect, it may be helpful to include a description of the heart defect and what medications the child may need. Information about this is usually obtained from the child’s treating physician. The description should also include the telephone number of the unit responsible for the child’s care in normal cases.
After some complicated cardiac surgery, the child may be more sensitive than normal to losing fluid in diarrhea or hot climates, for example. Then the child may need fluid replacement earlier than other children.
Some children are treated with blood thinners and then the child needs regular blood test checks. These children can travel as well, but this often requires more planning. You can get advice on the unit where the child is being treated for his or her heart failure.
Career choices
During the teenage years, the question of career choice may become relevant. For the most part, heart failure usually does not have a major impact on which profession you choose. For some physically very heavy professions, there may be restrictions that need to be discussed with career counselors at school and with the child’s physician well in advance.
Driving license
Most people with congenital heart failure can take a driver’s license. Often, a cardiologist is required to issue a special certificate of heart failure.
Dental health in children with heart failure
The teeth of children with heart defects are often more prone to holes, caries. The teeth of children with heart disease should be kept as healthy as possible for several reasons.
It is positive for the child if he does not have to undergo strenuous dental treatments. A healthy oral cavity is a good protection against infections of the heart, called endocarditis. Untreated caries can be an obstacle if the child needs surgery.
To prevent endocarditis
Endocarditis is an infection of the membrane that covers the inside of the heart muscle. The infection means that the heart valves can be attacked by bacteria and, in the worst case, damaged or destroyed. It is very uncommon in healthy people and also in children with heart failure. However, because it is a serious condition, some children with heart failure receive antibiotics for preventive purposes in connection with certain oral operations where there is a risk of bleeding and because bacteria can spread through the blood to the heart.
If the dentist does not already know about it, it is important to inform the parent that the child has a heart defect and possibly need antibiotic prophylaxis.
An early visit to the dentist
At one to one and a half years of age, all children who have a congenital heart defect are usually allowed to visit the dentist in order to obtain information about dental health as a parent. Then the dentist also makes an assessment of the risk of the child getting dental disease. The parent and the dentist agree on how the child’s continued dental care is planned.
More information
Patient version of the National Board of Health and Welfare’s national guidelines for care for congenital heart defects
The National Board of Health and Welfare has developed national guidelines for how congenital heart defects and a number of other diseases should be investigated and treated. The guidelines contain recommendations to health care about certain examinations and treatments that may be relevant to treating you with a congenital heart failure.
In the area there are also examinations and treatments that are done more routinely. They are not included in the guidelines.
Here you can read the patient version of the national guidelines for congenital heart defects.