Congenital Cardiovascular Defects :
What is a congenital cardiovascular defect?
Congenital means inborn or existing at birth. Among the terms you may hear are congenital heart defect, congenital heart disease and congenital cardiovascular disease. The word "defect" is more accurate than "disease." A congenital cardiovascular defect occurs when the heart or blood vessels near the heart don't develop normally before birth.
What causes congenital cardiovascular defects?
Congenital cardiovascular defects are present in about 1 percent of live births. They're the most common congenital malformations in newborns. In most cases scientists don't know why they occur. Sometimes a viral infection causes serious problems. German measles (rubella) is an example. If a woman contracts German measles while pregnant, it can interfere with how her baby's heart develops or produce other malformations. Other viral diseases also may cause congenital defects.
Heredity sometimes plays a role in congenital cardiovascular defects. More than one child in a family may have a congenital cardiovascular defect, but this rarely occurs. Certain conditions affecting multiple organs, such as Down's syndrome, can involve the heart, too. Some prescription drugs and over-the-counter medicines, as well as alcohol and "street" drugs, may increase the risk of having a baby with a heart defect. Researchers are studying other factors.
What are the types of congenital defects?
Most heart defects either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an abnormal pattern. Rarely defects occur in which only one ventricle (single ventricle) is present, or both the pulmonary artery and aorta arise from the same ventricle (double outlet ventricle). A third rare defect occurs when the right or left side of the heart is incompletely formed — hypoplastic heart.
Patent ductus arteriosus (PDA)
Pulmonary stenosis (PS)
Aortic stenosis (AS)
Coarctation of the aorta ("Coarct")
Bicuspid aortic valve
Atrial septal defect (ASD)
Ventricular septal defect (VSD)
Atrioventricular (A-V) canal defect
Tetralogy of Fallot
Transposition of the great arteries
Total anomalous pulmonary venous (P-V) connection
Hypoplastic left heart syndrome
Patent ductus arteriosus (PDA)
This defect (PA'tent DUK'tus ar-te"re-O'sis) allows blood to mix between the pulmonary artery and the aorta. Before birth an open passageway (the ductus arteriosus) exists between these two blood vessels. Normally this closes within a few hours of birth. When this doesn't happen, some blood that should flow through the aorta and on to nourish the body returns to the lungs. A ductus that doesn't close is quite common in premature infants but rather rare in full-term babies.
If the ductus arteriosus is large, a child may tire quickly, grow slowly, catch pneumonia easily and breathe rapidly. In some children symptoms may not occur until after the first weeks or months of life. If the ductus arteriosus is small, the child seems well. If surgery is needed, the surgeon can close the ductus arteriosus by tying it, without opening the heart. If there's no other defect, this restores the circulation to normal.
An obstruction is a narrowing that partly or completely blocks the flow of blood. Obstructions called stenoses (sten-O'seez) can occur in heart valves, arteries or veins.
The three most common forms are pulmonary stenosis, aortic stenosis and coarctation of the aorta. Related but less common forms include bicuspid aortic valve, subaortic stenosis and Ebstein's anomaly.
- Pulmonary stenosis (PUL'mo-nair-e sten-O'sis) (PS) —
The pulmonary or pulmonic valve is between the right ventricle and the pulmonary artery. It opens to allow blood to flow from the right ventricle to the lungs. A defective pulmonary valve that doesn't open properly is called stenotic (sten-OT'ik). This forces the right ventricle to pump harder than normal to overcome the obstruction.
If the stenosis is severe, especially in babies, some cyanosis (si"ah-NO'sis) (blueness) may occur. Older children usually have no symptoms. Treatment is needed when the pressure in the right ventricle is higher than normal. In most children the obstruction can be relieved by a procedure called balloon valvuloplasty (VAL'vu-lo-plas-te). Others may need open-heart surgery. Surgery usually opens the valve satisfactorily. The outlook after balloon valvuloplasty or surgery is favorable, but follow-up is required to determine if heart function returns to normal.
People with pulmonary stenosis, before and after treatment, are at risk for getting an infection of the valve (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Aortic stenosis (a-OR'tik sten-O'sis) (AS) —
The aortic valve, between the left ventricle and the aorta, is narrowed. The heart has difficulty pumping blood to the body. Aortic stenosis occurs when the aortic valve didn't form properly. A normal valve has three leaflets (cusps) but a stenotic (sten-OT'ik) valve may have only one cusp (unicuspid) or two cusps (bicuspid), which are thick and stiff. (See bicuspid aortic valve below.)
Sometimes stenosis is severe and symptoms occur in infancy. Otherwise, most children with aortic stenosis have no symptoms. Some children may have chest pain, unusual tiring, dizziness or fainting. The need for surgery depends on how bad the stenosis is. In children, a surgeon may be able to enlarge the valve opening. Surgery may improve the stenosis, but the valve remains deformed. Eventually the valve may need to be replaced with an artificial one.
Balloon valvuloplasty (VAL'vu-lo-plas-te) has been used in some children with aortic stenosis. The long-term results of this procedure are still being studied. Children with aortic stenosis need lifelong medical follow-up. Even mild stenosis may worsen over time, and surgical relief of a blockage is sometimes incomplete. Check with your pediatric cardiologist about limiting some kinds of exercise.
People with aortic stenosis, before and after treatment, are at risk for getting an infection of the valve (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Coarctation (ko"ark-TA'shun) of the aorta ("Coarct") —
The aorta is pinched or constricted. This obstructs blood flow to the lower body and increases blood pressure above the constriction. Usually there are no symptoms at birth, but they can develop as early as a baby's first week. A baby may develop congestive heart failure or high blood pressure that requires early surgery. Otherwise, surgery usually can be delayed. A child with a severe coarctation should have surgery in early childhood. This prevents problems such as developing high blood pressure as an adult.
The outlook after surgery is favorable, but long-term follow-up is required. Rarely, coarctation of the aorta may recur. Some of these cases can be treated by balloon angioplasty. The long-term results are still being studied. Also, blood pressure may stay high even when the aorta's narrowing has been repaired.
People with coarctation of the aorta, before and after treatment, are at risk for getting an infection within the aorta or the heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Bicuspid aortic (bi-KUS'pid a-OR'tik) valve —
The normal aortic valve has three flaps (cusps) that open and close. A bicuspid valve has only two flaps. There may be no symptoms in childhood, but by adulthood (often middle age or older), the valve can become stenotic (sten-OT'ik) (narrowed), making it harder for blood to pass through it, or regurgitant (allowing blood to leak backward through it). Treatment depends on how well the valve works.
People with bicuspid aortic valve, before and after treatment, are at risk for getting an infection within the aorta or the heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Subaortic stenosis (sub"a-OR'tik sten-O'sis) —
Stenosis means constriction or narrowing. Subaortic means below the aorta. Subaortic stenosis refers to a narrowing of the left ventricle just below the aortic valve, which blood passes through to go into the aorta. This stenosis limits the flow of blood out of the left ventricle. This condition may be congenital or may be due to a particular form of cardiomyopathy (kar"de-o-mi-OP'ah-the) known as "idiopathic hypertrophic (hi"per-TRO'fik) subaortic stenosis" (IHSS). Treatment depends on the cause and the severity of the narrowing. It can include drugs or surgery.
People with subaortic stenosis, before and after treatment, are at risk for getting an infection within the aorta or the heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Ebstein's anomaly (ah-NOM'ah-lee) —
This is a congenital downward displacement of the tricuspid valve (located between the heart's upper and lower chambers on the right side) into the heart's right bottom chamber (right ventricle). It's usually associated with an atrial septal defect (see below).
People with Ebstein's anomaly, before and after treatment, are at risk for getting an infection within the heart valve (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
Some congenital cardiovascular defects let blood flow between the heart's right and left chambers. This happens when a baby is born with an opening between the wall (septum) that separates the right and left sides of the heart. This defect is sometimes called "a hole in the heart."
The two most common types of this defect are atrial septal defect and ventricular septal defect. Two variations are Eisenmenger's complex and atrioventricular canal defect.
- Atrial septal (A'tre-al SEP'tal) defect (ASD) —
An opening exists between the heart's two upper chambers. This lets some blood from the left atrium (blood that's already been to the lungs) return via the hole to the right atrium instead of flowing through the left ventricle, out the aorta and to the body. Many children with ASD have few, if any, symptoms. Closing the atrial defect by open-heart surgery in childhood can prevent serious problems later in life.
- Ventricular septal (ven-TRIK'u-ler SEP'tal) defect (VSD) —
An opening exists between the heart's two lower chambers. Some blood that's returned from the lungs and been pumped into the left ventricle flows to the right ventricle through the hole instead of being pumped into the aorta. Because the heart has to pump extra blood and is overworked, it may enlarge.
If the opening is small, it doesn't strain the heart. In that case, the only abnormal finding is a loud murmur. But if the opening is large, open-heart surgery is recommended to close the hole and prevent serious problems. Some babies with a large ventricular septal defect don't grow normally and may become undernourished. Babies with VSD may develop severe symptoms or high blood pressure in their lungs. Repairing a ventricular septal defect with surgery usually restores normal blood circulation. The long-term outlook is good, but long-term follow-up is required.
People with unrepaired ventricular septal defects are at risk for getting an infection of the heart's walls or valves (endocarditis). After a VSD has been successfully fixed with surgery, antibiotics should no longer be needed. If surgery or other procedures do not completely repair the VSD, the person is still at risk for developing endocarditis. Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Eisenmenger's complex
is a ventricular septal defect coupled with pulmonary high blood pressure, the passage of blood from the right side of the heart to the left (right to left shunt), an enlarged right ventricle and a latent or clearly visible bluish discoloration of the skin called cyanosis (si"ah-NO'sis). It may also include a malpositioned aorta that receives ejected blood from both the right and left ventricles (an overriding aorta).
People with Eisenmenger's complex, before and after treatment, are at risk for getting an infection within the aorta or the heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Atrioventricular (A'tre-o-ven-TRIK'u-ler) (A-V) canal defect (also called endocardial cushion defect or atrioventricular septal defect) —
A large hole in the center of the heart exists where the wall between the upper chambers joins the wall between the lower chambers. Also, the tricuspid and mitral valves that normally separate the heart's upper and lower chambers aren't formed as individual valves. Instead, a single large valve forms that crosses the defect. The large opening in the center of the heart lets oxygen-rich (red) blood from the heart's left side – blood that's just gone through the lungs – pass into the heart's right side. There, the oxygen-rich blood, along with venous (bluish) blood from the body, is sent back to the lungs. The heart must pump an extra amount of blood and may enlarge. Most babies with an atrioventricular canal don't grow normally and may become undernourished. Because of the large amount of blood flowing to the lungs, high blood pressure may occur there and damage the blood vessels.
In some babies the common valve between the upper and lower chambers doesn't close properly. This lets blood leak backward from the heart's lower chambers to the upper ones. This leak, called regurgitation or insufficiency, can occur on the right side, left side or both sides of the heart. With a valve leak, the heart pumps an extra amount of blood, becomes overworked and enlarges.
In babies with severe symptoms or high blood pressure in the lungs, surgery usually must be done in infancy. The surgeon closes the large hole with one or two patches and divides the single valve between the heart's upper and lower chambers to make two separate valves. Surgical repair of an atrioventricular canal usually restores the blood circulation to normal. However, the reconstructed valve may not work normally.
Rarely, the defect may be too complex to repair in infancy. In this case, the surgeon may do a procedure called pulmonary artery banding to reduce the blood flow and high pressure in the lungs. When a child is older, the band is removed and corrective surgery is done. More medical or surgical treatment is sometimes needed.
People with atrioventricular canal defect, before and after treatment, are at risk for getting an infection within the heart's walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
Another type of heart defect is congenital cyanotic (si"ah-NOT'ik) heart defects. In these defects, blood pumped to the body contains less oxygen than normal. This causes a condition called cyanosis (si"ah-NO'sis), a blue discoloration of the skin. Infants with cyanosis are often called "blue babies."
Examples of cyanotic defects are tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, pulmonary atresia, truncus arteriosus and total anomalous pulmonary venous connection.
- Tetralogy of Fallot (TE'TRAL'o-je of fal-O')
has four components. The two major ones are a large hole, or ventricular septal defect, that lets blood pass from the right to the left ventricle without going through the lungs; and a narrowing (stenosis) at or just beneath the pulmonary valve. This narrowing partially blocks the blood flow from the heart's right side to the lungs. The other two components are: the right ventricle is more muscular than normal; and the aorta lies directly over the ventricular septal defect.
This results in cyanosis (blueness), which may appear soon after birth, in infancy or later in childhood. These "blue babies" may have sudden episodes of severe cyanosis with rapid breathing. They may even become unconscious. During exercise, older children may become short of breath and faint. These symptoms occur because not enough blood flows to the lungs to supply the child's body with oxygen.
Some infants with severe tetralogy of Fallot may need an operation to give temporary relief by increasing blood flow to the lungs with a shunt. This is done by making a connection between the aorta and the pulmonary artery. Then some blood from the aorta flows into the lungs to get more oxygen. This reduces the cyanosis and allows the child to grow and develop until the problem can be fixed when they are older.
Most children with tetralogy of Fallot have open-heart surgery before school age. The operation involves closing the ventricular septal defect and removing the obstructing muscle. After surgery the long-term outlook varies, depending largely on how severe the defects were before surgery. Lifelong medical follow-up is needed.
People with tetralogy of Fallot, before and after treatment, are at risk for getting an infection within the aorta or the heart valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Transposition of the great arteries —
The positions of the pulmonary artery and the aorta are reversed. The aorta is connected to the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs. The pulmonary artery is connected to the left ventricle, so most of the blood returning from the lungs goes back to the lungs again.
Infants born with transposition survive only if they have one or more connections that let oxygen-rich blood reach the body. One such connection may be a hole between the two atria, called atrial septal defect, or between the two ventricles, called ventricular (ven-TRIK'u-ler) septal defect. Another may be a vessel connecting the pulmonary artery with the aorta, called patent ductus arteriosus (PA'tent DUK'tus ar-te"re-O'sis). Most babies with transposition of the great arteries are extremely blue (cyanotic) (si"ah-NOT'ik) soon after birth because these connections are inadequate.
To improve the body's oxygen supply, a special procedure called balloon atrial septostomy (sep-TOS'to-me) is used. Two general types of surgery may be used to help fix the transposition. One is a venous switch or intra-atrial baffle procedure that creates a tunnel inside the atria. Another is an arterial switch. After surgery, the long-term outlook varies quite a bit. It depends largely on how severe the defects were before surgery. Lifelong follow-up is needed.
People with transposition of the great arteries, before and after treatment, are at risk for getting an infection on the heart's walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Tricuspid atresia (tri-KUS'pid ah-TRE'zhuh) —
In this condition, there's no tricuspid valve. That means no blood can flow from the right atrium to the right ventricle. As a result, the right ventricle is small and not fully developed. The child's survival depends on there being an opening in the wall between the atria called an atrial septal defect and usually an opening in the wall between the two ventricles called a ventricular (ven-TRIK'u-ler) septal defect. Because the circulation is abnormal, the blood can't get enough oxygen, and the child looks blue (cyanotic) (si"ah-NOT'ik).
Often a surgical shunting procedure is needed to increase blood flow to the lungs. This reduces the cyanosis. Some children with tricuspid atresia have too much blood flowing to the lungs. They may need a procedure (pulmonary artery banding) to reduce blood flow to the lungs. Other children with tricuspid atresia may have a more functional repair (Fontan procedure). Children with tricuspid atresia require lifelong follow-up by a cardiologist.
People with tricuspid atresia, before and after treatment, are at risk for getting an infection of the valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Pulmonary atresia (PUL'mo-nair-e ah-TRE'zhuh) —
No pulmonary valve exists, so blood can't flow from the right ventricle into the pulmonary artery and on to the lungs. The right ventricle acts as a blind pouch that may stay small and not well developed. The tricuspid valve is often poorly developed, too.
An opening in the atrial septum lets blood exit the right atrium, so venous (bluish) blood mixes with the oxygen-rich (red) blood in the left atrium. The left ventricle pumps this mixture of oxygen-poor blood into the aorta and out to the body. The baby appears blue (cyanotic) (si"ah-NOT'ik) because there's less oxygen in the blood circulating through the arteries. The only source of lung blood flow is the patent ductus arteriosus (PA'tent DUK'tus ar-te"re-O'sis) (PDA), an open passageway between the pulmonary artery and the aorta. If the PDA narrows or closes, the lung blood flow is reduced to critically low levels. This can cause very severe cyanosis.
Early treatment often includes using a drug to keep the PDA from closing. A surgeon can create a shunt between the aorta and the pulmonary artery to help increase blood flow to the lungs. A more complete repair depends on the size of the pulmonary artery and right ventricle. If they're very small, it may not be possible to correct the defect with surgery. In cases where the pulmonary artery and right ventricle are a more normal size, open-heart surgery may produce a good improvement in how the heart works.
If the right ventricle stays too small to be a good pumping chamber, the surgeon can compensate by connecting the right atrium directly to the pulmonary artery. The atrial defect also can be closed to relieve the cyanosis. This is called a Fontan procedure. Children with tricuspid atresia require lifelong follow-up by a cardiologist.
People with pulmonary atresia, before and after treatment, are at risk for getting an infection on the heart's walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Truncus arteriosus (TRUN'kus ar-te"re-O'sis) —
This is a complex malformation where only one artery arises from the heart and forms the aorta and pulmonary artery. Surgery for this condition usually is required early in life. It includes closing a large ventricular (ven-TRIK'u-ler) septal defect within the heart, detaching the pulmonary arteries from the large common artery, and connecting the pulmonary arteries to the right ventricle with a tube graft. Children with truncus arteriosus need lifelong follow-up to see how well the heart is working.
People with truncus arteriosus, before and after treatment, are at risk for getting an infection on the heart's walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
- Total anomalous pulmonary venous (ah-NOM'ah-lus PUL'mo-nair-e VE'nus) (P-V) connection —
The pulmonary veins that bring oxygen-rich (red) blood from the lungs back to the heart aren't connected to the left atrium. Instead, the pulmonary veins drain through abnormal connections to the right atrium.
In the right atrium, oxygen-rich (red) blood from the pulmonary veins mixes with venous (bluish) blood from the body. Part of this mixture passes through the atrial septum (atrial septal defect) into the left atrium. From there it goes into the left ventricle, to the aorta and out to the body. The rest of the poorly oxygenated mixture flows through the right ventricle, into the pulmonary artery and on to the lungs. The blood passing through the aorta to the body doesn't have enough oxygen, which causes the child to look blue (cyanotic) (si"ah-NOT'ik).
This defect must be surgically repaired in early infancy. The pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. When surgical repair is done in early infancy, the long-term outlook is very good. Still, lifelong follow-up is needed to make sure that any remaining problems, such as an obstruction in the pulmonary veins or irregularities in heart rhythm, are treated properly. It's important to make certain that a blockage doesn't develop in the pulmonary veins or where they're attached to the left atrium. Heart rhythm irregularities (arrhythmias) also may occur at any time after surgery.
People with total anomalous pulmonary venous connection, before and after treatment, are at risk for getting an infection on the heart's walls or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
Hypoplastic left heart syndrome
In hypoplastic (hi"po-PLAS'tik) left heart syndrome, the left side of the heart is underdeveloped – including the aorta, aortic valve, left ventricle and mitral valve. Blood returning from the lungs must flow through an opening in the wall between the atria, called an atrial septal defect. The right ventricle pumps the blood into the pulmonary artery, and blood reaches the aorta through a patent ductus arteriosus (PA'tent DUK'tus ar-te"re-O'sis). (See above.)
The baby often seems normal at birth, but will come to medical attention within a few days as the ductus closes. Babies with this syndrome become ashen, have rapid and difficult breathing and have difficulty feeding. This heart defect is usually fatal within the first days or months of life without treatment.
This defect isn't correctable, but some babies can be treated with a series of operations or with a heart transplant. Until an operation is performed, the ductus is kept open by intravenous (IV) medication. Because these operations are complex and different for each patient, you need to discuss all the medical and surgical options with your child's doctor. Your doctor will help you decide which is best for your baby.
If you and your child's doctor choose surgery, it will be done in several stages. The first stage, called the Norwood procedure, allows the right ventricle to pump blood to both the lungs and the body. It must be performed soon after birth. The final stage(s) has many names including bi-directional Glenn, Fontan operation and lateral tunnel. These operations create a connection between the veins returning blue blood to the heart and the pulmonary artery. The overall goal is to allow the right ventricle to pump only oxygenated blood to the body and to prevent or reduce mixing of the red and blue blood. Some infants require several intermediate operations to achieve the final goal.
Some doctors will recommend a heart transplant to treat this problem. Although it provides the infant with a heart that has normal structure, the infant will require lifelong medications to prevent rejection. Many other problems related to transplants can develop. You should discuss these with your doctor.
Children with hypoplastic left heart syndrome require lifelong follow-up by a cardiologist for repeated checks of how their heart is working. Virtually all the children will require heart medicines.
People with hypoplastic left heart syndrome, before and after treatment, are at risk for getting an infection on the heart's inner lining or valves (endocarditis). Please see the section on “Endocarditis” below to determine whether your child will need to take antibiotics before certain dental procedures.
The American Heart Association has recently changed its guidelines for prevention of endocarditis. Some children who used to take antibiotics before going to the dentist no longer have to do so.
The AHA now recommends these routine antibiotics before dental visits for patients who are at the highest risk for adverse outcomes if they develop endocarditis.
- People with a prosthetic cardiac valve
- People who have previously had endocarditis
- People with certain types of congenital heart defects, including
- Unrepaired cyanotic congenital heart defects, including palliative shunts and conduits
- Completely repaired congenital heart defects with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first six months after the procedure (prophylaxis is recommended for first six months because endothelialization of prosthetic material occurs within six months after the procedure)
- Repaired congenital heart defect with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization)
- Cardiac transplantation recipients who develop heart valve dysfunction
Congenital heart defect corrective surgeries
Congenital heart defect corrective surgeries fix or treat heart defects that a child is born with. A baby born with heart defects has congenital heart disease. Surgery is needed if the defects are dangerous to the child’s health or well-being.
The surgeries described below are done to correct many different heart defects in children.
For more information about risks, how to prepare for surgery, and descriptions of open-heart and closed-heart surgery techniques, see: Pediatric heart surgery.
Patent ductus arteriosus (PDA) ligation
Before birth, there is a natural blood vessel between the aorta (the main artery to the body) and the pulmonary artery (the main artery to the lungs) called the ductus arteriosus. This opening usually closes shortly after birth. A PDA occurs when this opening fails to close.
Sometimes a simple surgery can be done. In this procedure, the surgeon inserts a few small tubes into an artery in the leg and passes them up to the heart. Then, a small metal coil or another device is put into the child’s arteriosus artery. The coil or other device blocks the blood flow, and this corrects the problem.
Another method is to make a small cut, or incision, on the left side of the chest. The surgeon reaches in and ties off the ductus arteriosus, or divides and cuts it. Tying off the ductus arteriosus is called ligation.
Coarctation of the aorta repair
Coarctation of the aorta occurs when a part of the aorta has a very narrow section, like in an hourglass timer. To repair this defect, an incision is usually made on the left side of the chest, between the ribs.
The most common way to repair this is to cut the narrow section and make it bigger with a patch made of Gore-tex, a man-made material.
Another way to repair this problem is to remove the narrow section of the aorta and stitch the remaining ends together. This can usually be done in older children.
A third way to repair this problem is called a subclavian flap. First, an incision is made in the narrow portion of the aorta. Then, a patch is taken from the left subclavian artery (the artery to the arm) to enlarge the narrow section of the aorta.
A fourth way to repair the problem is to connect a tube to the normal sections of the aorta, on either side of the narrow section. Blood flows through the tube and bypasses the narrow section.
Atrial septal defect (ASD) repair
The atrial septum is the wall between the left and right atria (upper chambers) of the heart. There is a natural opening before birth that usually closes on its own when a baby is born. When the flap does not close, the child has an ASD.
Sometimes ASDs can be closed without open-heart surgery. First, the surgeon makes a tiny cut in the groin. Then the surgeon inserts tubes into a blood vessel that go into the heart. Next, 2 small umbrella-shaped "clamshell" devices are placed on the right and left sides of the septum. These 2 devices are attached to each other. This closes the hole in the heart. Not all medical centers do this procedure.
Open-heart surgery may also be done to repair ASD. Using open-heart surgery, the septum can be closed using stitches, or sutures. Another way is to cover the septum with a patch made of membrane or a man-made material.
Ventricular septal defect (VSD) repair
The ventricular septum is the wall between the left and right ventricles (lower chambers) of the heart. A hole in the ventricular septum is called a VSD.
By age 8 for most children with this problem, small VSDs often close on their own and do not need surgery. This depends on where the hole is.
Larger VSDs, small ones in certain parts of the ventricular septum, or ones that cause heart failure or endocarditis (inflammation) need open-heart surgery. They also require placing a man-made patch over the hole to cover it.
Some septal defects can be closed using heart catheterization (passing a thin tube into the heart).
Tetralogy of Fallot repair
Tetralogy of Fallot is a congenital heart defect that usually includes 4 defects in the heart.
Open-heart surgery is needed, and it is often done when the child is between 6 months and 2 years of age.
Different types of repairs are done, depending on the defects. The ventricular septal defect is one repair, and it is described above. The pulmonary valve is opened and the thickened muscle (stenosis) is removed. A patch may be placed on the right ventricle and main pulmonary artery to improve blood flow to the lungs.
The child may have a shunt procedure done first. A shunt moves blood from one area to another. This is done if the open-heart surgery needs to be delayed. A shunt procedure requires making a cut between two of the ribs.
Transposition of the great vessels repair
In a normal heart, the aorta comes from the left side of the heart, and the pulmonary artery comes from the right side. Transposition of the great vessels is when these arteries come from the opposite sides of the heart.
Correcting transposition of the great vessels requires open-heart surgery. If possible, this surgery is done shortly after birth.
The most common repair is an arterial switch. The aorta and pulmonary artery are divided. The pulmonary artery is connected to the right ventricle, where it belongs. Then, the aorta and coronary arteries are connected to the left ventricle, where they belong.
Truncus arteriosus repair
Truncus arteriosus is a rare condition that occurs when the aorta, coronary arteries, and the pulmonary artery all come out of one common trunk. This is a very complex defect, and it requires complex open-heart surgery to repair it.
Repair is generally done in the first few days or weeks of the child’s life. The pulmonary arteries are separated from the aortic trunk, and any defects are patched. Usually, there is also a ventricular septal defect, and that is patched. A connection is then placed between the right ventricle and the pulmonary arteries.
Many children need 1 or 2 more surgeries as they grow.
Tricuspid atresia repair
The tricuspid valve is the valve between the upper and lower chambers on the right side of the heart. Tricuspid atresia occurs when this valve is missing. To get to the lungs, blood must cross an atrial septal defect (ASD), ventricular septal defect (VSD), or a patent ductus artery (PDA). (These conditions are described above.) This severely restricts blood flow to the lungs.
Other defects may exist with tricuspid atresia. A medicine called prostaglandin E may be given for a patent ductus arteriosus to maintain it as an alternate channel to the lungs until surgery can be done.
A series of shunts and surgeries may be necessary to correct this defect. The goal of this surgery is to allow blood from the body to flow into the lungs, and blood from the lungs to be pumped to the rest of the body through the left ventricle.
Total anomalous pulmonary venous return (TAPVR) correction
TAPVR occurs when the pulmonary veins bring oxygen-rich blood from the lungs back to the right side of the heart, instead of to the left side of the heart, where it should be.
This condition requires surgery to correct it. When the surgery is done will depend on how sick the baby is. The surgery may be done in the newborn period if the infant has severe symptoms. If it is not done right after birth, it is done in the first 6 months of the baby’s life.
TAPVR repair requires an open-heart surgery. The pulmonary veins are attached to the left side of the heart, where they belong, and any abnormal connections are closed.
If a PDA is present, it is tied off and divided.
Hypoplastic left heart repair
This is a very severe heart defect that results from a severely underdeveloped left heart. If it is not treated, it causes death in most babies who are born with it. Operations to treat this defect are done at specialized medical centers. Usually surgery corrects this defect. A series of 3 heart operations is usually needed.
The first operation is done in the first week of the baby’s life. This is a complicated surgery where one blood vessel is formed from the pulmonary artery and the aorta. This new vessel carries blood to the lungs and the rest of the body.
The second operation is usually done when the baby is 4 to 6 months old.
The third operation is done a year after the second operation.
A heart transplant may be done to treat this condition. But, finding a donor heart for an infant is very difficult. Infant heart transplants can be done only ata few medical centers.
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