Congenital Defects

WHAT IS CONGENITAL HEART DISEASE / 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 IS BLUE BABY DEFECT?

A blue baby is an infant born with a type of Congenital Heart Disease that causes the infant’s skin to have a bluish tint (cynosis).

Central cyanosis is a condition in which there is too much oxygen–poor blood traveling through the arteries, which normally carry only oxygen–rich blood to nourish the body. In technical terms, cyanosis is diagnosed when the level of reduced (desaturated) hemoglobin exceeds 3  grams per deciliter (g/dl) of blood in the capilliary bed – the network of tiny arteries where oxygen exchange takes place throughout the body.

Not all cyanosis is caused by congenital heart defects.
Peripheral cyanosis, or a slight bluish tinge in the extremities, may occur in healthy, fair–skinned babies. This type of cyanosis is caused when the degree of oxygen saturation in the blood is normal, but there is increased extraction of oxygen from the blood because the blood is moving too slowly through the capillaries. This causes some tissues to receive too little oxygen. Causes of peripheral cyanosis include constriction of blood vessels due to cold, obstructions in the veins, high blood pressure in the veins and other factors.

Cyanosis may also be caused by lung abnormalities and blood conditions that somehow impede the delivery of oxygen to the tissues and organs of the body

WHAT ARE THE SIGNS AND SYMPTOMS OF CONGENITAL HEART DEFECT?

Aside from the bluish tint of the skin, nails, lips, tongue or other parts of the body, signs and symptoms of a blue baby include:

1. Easily fatigued, especially during crying spells and at feeding time
2. Low tolerance for exercise or extra exertion
3. Shortness of breath (dyspnea) and/or rapid breathing
4. Difficulty eating, breathing or sucking
5. Poor weight gain
6. Heart murmur, as detected by a physician

Some of the cyanotic heart defects will create what’s known as differential cyanosis. This condition results when one half of the body, either the upper or lower half, is healthy and pink and the other half is bluish. This can help the physician narrow down the possible causes of cyanosis, if the causes aren’t known already.

WHAT ARE THE CAUSES OF CONGENITAL CARDIOVASULAR DISEASE?

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. Some of the so far known causes are:

1. 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.
2. 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.
3. Certain conditions affecting multiple organs, such as Down’s syndrome, can involve the heart, too.
4.  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 DISEASES?

In most of the cases blood flow in the heart or vessels near is obstructed or blood flow is routed in an abnormal fashion. Other defects could be:

1. Presence of only one (single) ventricle
2. Pulmonary artery and Aorta arising from the same ventricle (double outlet ventricle)
3. Incompletely formed left or right side of the heart-hypoplastic heart.

WHAT ARE DEFECTS CAUSED BY OBSTRUCTION IN BLOOD FLOW?

An obstruction is a narrowing that partly or completely blocks the flow of blood.  Obstructions called stenoses 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(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. This forces the right ventricle to pump harder than normal to overcome the obstruction.

If the stenosis is severe, especially in babies, some cyanosis (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. 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Aortic Stenosis(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 valve may have only one cusp (unicuspid) or two cusps (bicuspid), which are thick and stiff.

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 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Coarctation of the aorta— 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Bicuspid aortic 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 (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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Subaortic stenosis — 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 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Ebstein’s anomaly 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 (or right ventricle). It’s usually associated with an atrial septal defect.

People with Ebstein’s anomaly, before and after treatment, are at risk for getting an infection within the heart valve (endocarditis). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

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.

• Atrial septal 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 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 a ventricular septal defect are at risk for getting an infection of the heart’s walls or valves (endocarditis). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures. After a VSD has been successfully fixed with surgery, antibiotics should no longer be needed.

• 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. 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Atrioventricular(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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

Cyanotic defects

Another type of heart defect is congenital cyanotic 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 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical 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 the ventricular septal defect. Another may be a vessel connecting the pulmonary artery with the aorta, called patent ductus arteriosus. Most babies with transposition of the great arteries are extremely blue (cyanotic) soon after birth because these connections are inadequate.

To improve the body’s oxygen supply, a special procedure called balloon atrial septostomy 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Tricuspid Atresia— 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 septal defect. Because the circulation is abnormal, the blood can’t get enough oxygen, and the child looks blue (cyanotic).
• 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Pulmonary Atresia— 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) because there’s less oxygen in the blood circulating through the arteries. The only source of lung blood flow is the patent ductus arteriosus (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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Truncus Arteriosus— 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 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). To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

• Total anomalous pulmonary venous(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).

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.

Hypoplastic left heart syndrome

In hypoplastic 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.

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).  To help prevent this, they’ll need to take antibiotics before certain dental and surgical procedures.

Good dental hygiene also lowers the risk of endocarditis.

HOW ARE CONGENITAL DEFECTS / BLUE BABY SYNDROME DIAGNOSED?

If an infant exhibits cyanosis, physicians will order many tests to determine the cause of the condition. Theses tests will vary according to the type, nature and severity of symptoms. Some of these tests wll be:

1. Chest X-Ray- This provides an image of the heart’s chambers, vessels and muscles.  This will also allow the physician to evaluate the lungs to see if there are abnormalities in lung structure or function, which can affect blood oxygenation.
2. Electrocardiogram (ECG) to gauge the electrical activity of the heart.
3. Echocardiogram to define the relationships, flows, function, dimensions, and dynamics of all valves, vessels, chambers, and septa.  This is one of the definitive tests to evaluate a patient for possible heart disease. There is no recognized risk, radiation, or side-effect to an echocardiogram.

This test could be fetal (performed during pregnancy) and during infancy. Fetal echocardiography (e.g., ultrasounds) is a routine test and can help diagnose potentially cyanotic heart conditions before birth. This important advance allows both physicians and parents to plan for treatment immediately after the birth. Some congenital heart defects are and should be treated as soon as possible, instead of waiting for the child to grow.

1. Angiography to determine if valve damage or abnormalities are present. During this procedure a special dye (contrast medium) is injected into the blood vessels and chambers to view the activity, relationships, and size of vessel walls, valves and the heart muscle.
2. Detailed blood tests to assess for oxygen levels and other indicators of illness or medical conditions

WHAT ARE THE TREATMENT OPTIONS FOR CONGENITAL HEART DISEASE?

Diagnosing congenital disease at an early stage itself can be helpful. In many cases, the blue baby syndrome may be apparent shortly after birth but the condition may not become apparent until later. Because this is often the result of an underlying and serious heart defect, newborns who exhibit the cyanosis must be taken to an appropriate pediatric cardiac facility as quickly as possible. There, physicians will attempt to diagnose the defect and develop a plan of action.
The treatment could vary as per the magnitude of the disease. It could be:

• medications to manage the symptoms,
• interventional cardiac catheterization procedures or temporary surgeries (palliative) to give the child time to grow until the defect can be fixed,
• Corrective surgeries to fix the underlying defect.

Medications may be used to ease the symptoms of by reducing the workload of the muscles, vessels or chambers of the heart.

If medications are not helpful, then a more invasive treatment option may be advised. During this procedure, the physician can employ various strategies to improve valve, vessel and chamber problems.

Surgery may be undertaken during pregnancy itself or soon after birth. These surgeries include:

• Arterial Switch Operation. A procedure in which the two reversed major arteries (pulmonary artery and aorta) are switched to their normal positions.
• Shunting procedure. This is usually a temporary way to relieve the symptoms of a defect (palliative surgery), and the defect may be fully repaired after the patient has had some time to grow. In one such procedure, a Blalock Taussing shunt is used to form a new passageway between the aorta and the pulmonary artery. Thus, additional blood can travel from the aorta to the lungs through the pulmonary artery, increasing the flow and quality of blood flow to the lungs and restoring the patient’s natural pinkish color.
• Damus-Kaye- Stansel Procedure; This is surgery performed to repair transposition of the great arteries with subaortic obstruction, in which the pulmonary artery is cut into two segments. The first segment is already connected to the lungs at one end, and the surgeon connects the other end to the right ventricle. Thus, oxygen–poor blood can travel from the right ventricle through the first segment of the pulmonary artery and to the lungs. The second segment is already connected to the left ventricle at one end, and the surgeon connects the other end to the aorta. Thus, oxygen–rich blood can travel from the left ventricle through the second segment of the pulmonary artery and out the aorta, to the rest of the body.

• Fontan procedure. This is a multiple-stage procedure that corrects such cyanotic conditions as pulmonary atresia, tricuspid atresia and hypoplastic left heart syndrome. It is performed by connecting the veins returning from the body to the pulmonary artery, thus allowing blood to travel directly from the body to the lungs, via the pulmonary artery, bypassing the right ventricle. In patients with HLHS, the right ventricle will also be converted into the main pumping chamber for the body, to compensate for the underdeveloped left ventricle.

Many of these patients, both before and after surgery, are at risk of developing an inflammation or infection of the lining of the heart. Patients should discuss with their physician ways to avoid this.

While the long–term prognosis depends on the nature and severity of the specific condition(s) and on the appropriate treatment options, chances are better than ever before that properly treated blue babies will grow up to live happy, healthy lives.