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Congenital Cardiovascular Defects :
Congenital Heart Surgery, Congenital Heart India, India Heart Defect, Congenital, Cardiothoracic
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
Subaortic stenosis
Ebstein's anomaly
Atrial septal defect (ASD)
Ventricular septal defect (VSD)
Eisenmenger's complex
Atrioventricular (A-V) canal defect
Tetralogy of Fallot
Transposition of the great arteries
Tricuspid atresia
Pulmonary atresia
Truncus arteriosus
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.

Obstruction defects

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.

Septal defects

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.

Cyanotic defects

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.

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.

This includes:

  1. People with a prosthetic cardiac valve

  2. People who have previously had endocarditis

  3. People with certain types of congenital heart defects, including

    1. Unrepaired cyanotic congenital heart defects, including palliative shunts and conduits

    2. 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)

    3. 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)

  4. 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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