Congenital heart defects due to birth defects are the most common heart disease in children. It may be, for example, holes in the partitions of the heart or constrictions of the flaps in the heart or large vessels. About one-third of the congenital injuries are treated during the first months of life. Most children are treated with good results, but often the child needs to be followed up for a long time.
Open-standing ductus arteriosus
Open ductus arteriosus means that a connection between the body and pulmonary arteries remains from the fetal period.
During the fetal period, this connection carries a large part of the blood from the pulmonary artery directly to the body pulmonary artery, since the baby does not breathe and the lungs do not need as much blood flow. Ductus usually closes during the first week after birth.
Sometimes it remains open and is then a kind of deformity. Then blood flows from the body pulmonary artery to the pulmonary artery. The blood flow through the lungs increases, there is greater reflux to the left heart and thus increases the load on the left ventricle.
If the connection is large, the load on the left ventricle increases, which can lead to heart failure. If the connection is less, the child usually has no symptoms. If the connection is very large, the pressure in the pulmonary artery is increased. It can in the long run damage the function of the pulmonary vessels.
Usually, the connection, ductus arteriosus, can be closed in conjunction with cardiac catheterization. Then a plug or so-called coil (synthetic) of synthetic material is inserted into the ductus by means of a small plastic tube through a blood vessel in the groin.
In premature infants and children with symptoms during the first months of life, the ductus needs to be closed instead of surgery.
If the child has heart failure and elevated pressure in the pulmonary artery, it is operated early in the newborn period. Then the connection between the body and pulmonary arteries is divided.
When the ductus arteriosus is closed, the baby often becomes completely healthy.
Ovarian ductus arteriosus in preterm infants
In premature infants, a normal ductus arteriosus can also cause problems. This is especially true of premature babies with respiratory problems who often need respiratory care. The ductus is then usually very large and remains open longer than normal. This leads to increased blood flow from the body to the pulmonary artery, which strains the heart and causes heart failure. It is often possible to close the connection by giving the child a drug that pulls the ductus together. If this fails, the child is operated on.
AV septal defects
AV septum defects are malformations with holes in the lower part of the partition between the atrium and holes in the upper part of the chamber partition. The flaps between the atrium and the chamber are also malformed.
The most complicated type of malformation is called AV-commune. There is then a large, common hole between the two atoms and the two chambers. In addition, the child has a large flap instead of two separate flaps between the atrium and the chambers. The malformation may also consist of only a hole between the atrium and a malformed flap between the left atrium and left chamber, so-called atrial septum defect of the primate type.
Blood from the left half of the heart goes to the right side. Then the blood flow through the lungs increases and the pressure in the pulmonary artery is usually increased, which in the long run can damage the function of the pulmonary vessels. In addition, the malformed flap leaks between the chambers and atrium, further straining the heart.
A commune is most common in children with Down syndrome.
Because the heart is strained, the children develop early heart failure. Blood oxygen levels may decrease slightly as a small amount of blood can go from the right heart to the left.
Heart failure is treated with drugs. The child needs to undergo an operation when the holes in the partitions are closed and the malformed flaps are repaired. The child is often operated on during the first months of life. The child will often have a slight leakage in the flap between the left atrium and chamber. The child needs to be checked regularly for many years after the operation.
Chamber septum defect means that there is a hole in the partition between the chambers. A ventricular septal defect is also called a ventricular septal defect, VSD.
The hole causes blood from the left ventricle to the right ventricle and increases the blood flow to the lungs. If the child has a large hole between the chambers, the blood pressure in the right ventricle and pulmonary artery will be elevated. In the long run, it can cause the smallest blood vessels in the lungs to function worse.
About one-third of all heart defects are ventricular septal defects.
At small holes in the partition, the child has no symptoms. Large holes allow the left ventricle to pump much more blood than normal, leading to heart failure during the first months of life. The hole can eventually decrease in size and even close itself. However, this does not apply to large holes.
If the child has heart failure, it is treated with medication and surgery.
If there is a large hole that increases the pressure in the pulmonary artery, the child is operated on during the first months of life. Large holes are usually closed with a patch of some synthetic material.
If the hole is medium in size and if the blood pressure in the pulmonary artery is normal, it may be possible to wait a few years to see if the hole decreases in size by itself. If not, the child is operated on when it is preschool age.
Small ventricular septal defects need not be operated.
After the operation, the child is checked regularly for a few years. If the child has had elevated blood pressure in the pulmonary artery, it is followed for an extended period of time.
Atrial septal defect
Atrial septal defect means that there is a hole in the septum between the two atoms of the heart. Blood from the left atrium goes to the right side. This increases the load on the right ventricle and the blood flow to the lungs increases. Children usually do not have any symptoms but later in life there is a risk of disruption of the heart’s rhythm and heart failure with impaired ability to pump blood. An atrial septal defect is also called an atrial septal defect, ASD.
The heart defect can often be treated by closing the hole in the partition with a so-called device made of artificial material. It is inserted into the heart through a thin tube, catheter, which is inserted into the body via a blood vessel in the groin. Thus, the child does not need surgery. Those children who have a hole that is not suitable for the above treatment are usually operated between two to four years of age. The hole is then sewn together or covered with a patch from the child’s own heartbeat or a patch of artificial material.
If the holes in the partition are very small, no treatment is needed.
After treatment, the child is usually checked regularly for a period of time. If the heart and circulation work properly, the baby can be explained well after a couple of years.
Aortic stenosis means that the flap between the left ventricle and the large coronary artery, the aortic valve, is too narrow. If the constriction is severe, the load on the left chamber increases.
When the heart’s ability to pump blood is impaired, it can cause heart failure in infants. Larger children usually have no symptoms, but may have reduced energy and become more easily tired with exertion.
If the child has a problem or if it is found during examinations that the left wall muscle wall is thickened due to the increased load, the child is usually operated on. During surgery, the narrow opening in the aortic valve is widened. In some cases, the flap may be widened with a catheter technique instead.
Sometimes the baby needs surgery again because the narrowing can come back or the flap can become leaky after surgery. The leakage causes some blood to flow back from the carotid artery to the left ventricle, rather than being pumped further, which increases the load on the left ventricle. In adulthood, the flap may need to be replaced with an artificial flap.
The pulmonary artery transports the oxygen-poor blood from the body via the right atrium and chamber to the lungs. Pulmonary stenosis means that it is crowded at the flap between the right ventricle of the heart and the pulmonary artery.
If the constriction is very severe, the child may have a reduced ability to pump out blood, leading to heart failure. The child can also get reduced oxygen levels in the blood with bluish skin, lips and mucous membranes, so-called cyanosis.
If the child has symptoms and the constriction is severe, the valve is usually widened with so-called catheter treatment or operated on. In catheter treatment, a balloon is inserted into the heart and pulmonary artery via a thin plastic tube, catheter, and the flap is dilated.
The child needs to undergo regular checkups for many years as there may be a slight constriction or leakage in the flap.
Congestion of large body pulmonary artery, aortic arching
Coarctation means that there is a narrowing of the large arterial artery, aorta.
A severe constriction results in an increased load of the left ventricle, leading to heart failure during the newborn period. Older children rarely have symptoms, but they have high blood pressure in their arms.
The child usually needs surgery. During the operation, the narrow area is widened. If the child has symptoms, it is operated during the newborn period, otherwise during the first years of life. If the child is operated on during the newborn period, there is a certain risk that the narrowing will return. A new constriction can often be treated in conjunction with cardiac catheterization. The constriction is then widened with the help of a balloon.
The child needs to undergo regular checkups for many years as the constriction may recur and the child may have persistently elevated blood pressure.
Fallot’s deformity means that there are holes in the septum between the heart chambers and that the outflow to the pulmonary artery is too narrow. Sometimes the vessels that originate from the pulmonary artery are also narrower than normal.
Poorer oxygenated blood goes from the right ventricle to the left ventricle and into the coronary artery. This means that the oxygen content in the blood vessels of the body artery becomes lower and the skin, lips and mucous membranes can become blue-toned, so-called cyanosis. Often the child gets symptoms during the first few weeks, but sometimes it is discovered later. Sometimes the cyanosis can come as sudden attacks, so-called blue spells.
The child needs surgery. The hole between the chambers is covered with a patch of synthetic material and the outflow to the pulmonary artery dilates. The operation is done during the first year of life. If the child has severe cyanosis early, it is sometimes operated in two stages. The first operation is then a so-called shunt operation) which improves the child’s condition in the short term. A few months later, the complete operation is then done as described above.
The child needs to be checked regularly throughout life as it may continue to be a little crowded in the outflow from the right chamber. There may also be a leak in the valve which allows blood to flow back to the chamber.
Pulmonary ulcer resection with a ventricular septal defect
In pulmonary resection, blood cannot pass from the right ventricle to the pulmonary artery because the blood vessel is blocked. There are holes in the partition between the chambers. The blood goes to the pulmonary artery from the coronary artery, aorta through a compound called ductus arteriosus.
Ductus arteriosus is a fetal compound that should close during the first week of life. With pulmonary artery resection, the low oxygen level in the blood makes the connection often open longer than usual, but it closes later. The child then gets lower blood oxygen levels.
The child gets early blood oxygen levels with blue-tinted skin, lips and mucous membranes, so-called cyanosis. The oxygen content drops further as the ductus arteriosus begins to close.
If the ductus arteriosus begins to close during the newborn period, the baby receives via drip a body-specific substance, prostaglandin, which keeps the connection open. Then the child needs surgery. In the first operation, a connection is made from the body pulse vein to the pulsing vein using a small plastic tube, called a shunt.
The final operation involves closing the hole in the partition and creating a connection, called a conduit, between the right ventricle and the pulmonary artery. For example, the conductor may consist of an artificial tube with flaps. The final surgery is usually done at the age of one year. The child then has to go on checks regularly for many years because the inoperative connection, the conduit, can have a deteriorating function and then needs to be replaced.
Transposition of the big vessels
Transposition of the large vessels means that the body and pulmonary arteries have changed place and are leaving the wrong chambers. The badly oxygenated blood goes back into the body. A small hole in the atrial septum allows some oxygenated blood to still enter the body. In addition, the ductus arteriosus is usually open initially.
During the fetal period, blood circulation works well because there is then an open connection between the body pulmonary artery and pulmonary artery, so-called open-standing ductus arteriosus. When the connection closes during the baby’s first day after birth, oxygen levels in the blood drop sharply.
Low oxygen levels in the blood cause the child to get blue-tinted skin, lips, and mucous membranes, so-called cyanosis, during the first days of life. The low oxygen content is harmful and the child needs early treatment.
The child can, via drip, receive a body substance, prostaglandin, which keeps the ductus open. In this way, the blood is mixed, which improves the oxygen content.
In anticipation of surgery, the blood oxygen level can be improved by inserting a thin plastic tube, catheter, into the heart. A small balloon in the catheter is inflated and pulled between the left and right atrium and widen the opening there.
The heart defect needs to be operated as soon as possible. The child is often operated on during the first two weeks of life. During the operation, the body and pulmonary arteries are split above the flaps and they can be changed. The coronary artery of the heart must also be moved from the old to the new coronary artery. The hole between the atoms is closed.
The child needs to undergo regular check-ups for many years, especially to monitor what happens to the coronary vessels that were moved during the operation.
The single-chamber heart is a collective term for several different complicated cardiac malformations, which means that a chamber receives the inflow of blood from both atoms and performs pump function. Acid-rich blood is mixed with oxygen-poor blood in the chamber. The blood that goes out into the large body pulse veins will, therefore, have lowered oxygen levels.
The symptoms vary greatly because the malformation can look so different. When the heart’s ability to pump out blood is impaired, it can cause heart failure. The child also has decreased oxygen levels in the blood with bluish skin, lips and mucous membranes, so-called cyanosis.
The single-chamber heart is treated with a number of operations that take place at different ages. An alternative to surgery is heart transplantation, which is not very common as the availability of the heart in that size is limited. During the child’s first months of life, a so-called shunt operation or bandaging of the pulmonary artery is often performed. The child then has to undergo a more complicated operation, which means that the blood is led from the hollow veins to the pulmonary artery. This is often done in two steps. The chamber then only pumps the blood to the body’s artery.
In the first stage, the upper pericardium is connected to the pulmonary artery after the pulmonary artery is divided (Glenn’s operation).
The second step means that the lower penis is also connected to the pulmonary artery. It is called total Cavo-pulmonary connection, TCPC. The blood from the lower vein is led through a tunnel up to the pulmonary artery. The tunnel connection can either go through the right atrium as shown in the picture below or be placed outside the right atrium.
Sometimes both steps of this operation can be done simultaneously. The first operation is usually done after three months of age and the second operation usually before the age of three.
The child needs to be checked regularly for the rest of his life after the operation.
Hypoplastic left ventricle
Hypoplastic left ventricle means that the left ventricle and often also the coronary artery are very underdeveloped. Blood circulation is maintained by the right ventricle, which, via a fetal compound called the ductus arteriosus, also supplies the body’s pulmonary artery with blood.
Circulation works well during the fetal period and the first hours after birth, since the compound, ductus arteriosus, is then open. When it closes, as it does in all children, the blood flow to the carotid artery becomes insufficient. There is also an opening between the atrium that allows oxygenated blood from the lungs to flow from the left atrium to the right atrium. Then the blood flows to the right ventricle and is pumped out to the pulmonary circulation. Part of the blood goes through the ductus arteriosus from the pulmonary artery to the body pulmonary artery.
When the ductus joins, it leads to an insufficient flow of oxygenated blood to the body’s pulmonary artery, low blood pressure, and insufficient blood supply to all organs of the body. The child needs surgery early in order to survive.
Most children with hypoplastic left ventricles can be operated on. In anticipation of surgery, the ductus arteriosus can be kept open with the help of drugs. The operation takes place in several stages during the first years of life.
An alternative to surgery is heart transplantation, which is not very common as the availability of the heart in that size is limited. The child needs to be checked regularly for the rest of his life after the operation.
In normal cases, the right ventricle pumps the blood to the lungs for oxygenation via the pulmonary artery. The left ventricle pumps the oxygenated blood out to the body via the large coronary artery, which is also called the aorta. In the truncus arteriosus, only a common, wide blood vessel leaves the heart, which first divides into the body and pulmonary arteries. There are holes in the partition between the chambers.
Blood from both the left and right chambers passes into the arterial artery, and the oxygen content in the blood is therefore lower.
The child develops heart failure with impaired ability to pump blood. Blood oxygen levels drop slightly. and the child may have bluish skin, lips, and mucous membranes, called cyanosis.
The child needs surgery. The large common blood vessel becomes a coronary artery and the hole in the septum closes. A connection, called a conduit, is created between the right ventricle and the pulmonary artery. For example, the conductor may consist of an artificial tube with flaps.
The surgery is done during the first weeks of life.
The child needs to undergo regular check-ups for many years as the implanted connection can deteriorate in its function and need to be replaced. The conductor may need to be changed several times during life.