How the heart works

To understand congenital heart defects, it's helpful to know how the normal heart works. Your child's heart is a muscle about the size of his or her fist. It works like a pump and beats 100,000 times a day. The heart has two sides, separated by an inner wall called the septum. The right side of the heart pumps blood to the lungs to pick up oxygen. Then, oxygen-rich blood returns from the lungs to the left side of the heart, and the left side pumps it to the body. The heart has four chambers and four valves and is connected to various blood vessels. Veins are the blood vessels that carry blood from the body to the heart. Arteries are the blood vessels that carry blood away from the heart to the body. The illustration shows a cross-section of a healthy heart and its inside structures. The blue arrow shows the direction in which oxygen-poor blood flows from the body to the lungs. The red arrow shows the direction in which oxygen-rich blood flows from the lungs to the rest of the body.

Heart Chambers The heart has four chambers or "rooms."

The atria (AY-tree-uh) are the two upper chambers that collect blood as it comes into the heart. The ventricles (VEN-trih-kuls) are the two lower chambers that pump blood out of the heart to the lungs or other parts of the body.

Heart Valves Four valves control the flow of blood from the atria to the ventricles and from the ventricles into the two large arteries connected to the heart.

The tricuspid (tri-CUSS-pid) valve is in the right side of the heart, between the right atrium and the right ventricle. The pulmonary (PULL-mun-ary) valve is in the right side of the heart, between the right ventricle and the entrance to the pulmonary artery, which carries blood to the lungs. The mitral (MI-trul) valve is in the left side of the heart, between the left atrium and the left ventricle. The aortic (ay-OR-tik) valve is in the left side of the heart, between the left ventricle and the entrance to the aorta, the artery that carries blood to the body.

Valves are like doors that open and close. They open to allow blood to flow through to the next chamber or to one of the arteries, and then they shut to keep blood from flowing backward. When the heart's valves open and close, they make a "lub-DUB" sound that a doctor can hear using a stethoscope.

The first sound - the "lub" - is made by the mitral and tricuspid valves closing at the beginning of systole (SIS-toe-lee). Systole is when the ventricles contract, or squeeze, and pump blood out of the heart. The second sound - the "DUB" - is made by the aortic and pulmonary valves closing at beginning of diastole (di-AS-toe-lee). Diastole is when the ventricles relax and fill with blood pumped into them by the atria.

Arteries The arteries are major blood vessels connected to your heart.

The pulmonary artery carries blood pumped from the right side of the heart to the lungs to pick up a fresh supply of oxygen. The aorta is the main artery that carries oxygen-rich blood pumped from the left side of the heart out to the body. The coronary arteries are the other important arteries attached to the heart. They carry oxygen-rich blood from the aorta to the heart muscle, which must have its own blood supply to function.

Veins The veins are also major blood vessels connected to your heart.

The pulmonary veins carry oxygen-rich blood from the lungs to the left side of the heart so it can be pumped out to the body. The vena cava is a large vein that carries oxygen-poor blood from the body back to the heart.

Atrial septal defect (ASD). An ASD is a hole in the part of the septum that separates the atria - the upper chambers of the heart. This heart defect allows oxygen-rich blood from the left atrium to flow into the right atrium instead of flowing to the left ventricle as it should. Many children who have ASDs have few, if any, symptoms. igure A shows the normal structure and blood flow in the interior of the heart. Figure B shows a heart with an atrial septal defect, which allows oxygen-rich blood from the left atrium to mix with oxygen-poor blood from the right atrium. An ASD can be small or large. Small ASDs allow only a little blood to leak from one atrium to the other. Very small ASDs don't affect the way the heart works and therefore don't need any special treatment. Many small ASDs close on their own as the heart grows during childhood. Medium to large ASDs allow more blood to leak from one atrium to the other, and they're less likely to close on their own. Half of all ASDs close on their own or are so small that no treatment is needed. Medium to large ASDs that need treatment can usually be repaired using a catheter procedure. (See "How Are Congenital Heart Defects Treated?")

Ventricular septal defect (VSD). A VSD is a hole in the part of the septum that separates the ventricles - the lower chambers of the heart. The hole allows oxygen rich blood to flow from the left ventricle into the right ventricle instead of flowing into the aorta and out to the body as it should. Normal Heart and Heart With Ventricular Septal Defect

How do VSDs cause problems?

The pressure generated during contraction by the left ventricle is higher than that generated by the simultaneous contraction of the right ventricle. Blood will thus be pushed through the VSD (also called "shunted") from the left ventricle to the right ventricle. The right ventricle has to do extra work to handle the additional blood volume. It may have trouble keeping up with the load and enlarge, affecting its ability to pump efficiently. In addition, the lungs receive too much blood under too much pressure. The arterioles (small arteries) in the lungs thicken in response to the excess blood under excess pressure. If this extra pressure persists, permanent damage can be done to the lungs. It makes a considerable difference whether the size of the VSD is small or large.

How is a VSD diagnosed? What are the symptoms of a VSD?

The diagnosis of a VSD is usually suspected clinically by hearing a characteristic heart murmur. A murmur is a sound generated by abnormally turbulent flow of blood through the heart. This murmur is the result of blood being shunted through the VSD from the higher-pressure left ventricle into the lower-pressure right ventricle. At birth, this pressure imbalance is minimal and does not usually develop until later in the first week of life. As such, it is rare for a doctor to hear the murmur of a VSD until the baby is a few days of age.

The evaluation of a child with a possible VSD is designed to confirm the diagnosis but also to check for other anatomical defects in the heart and to estimate the size of the shunt of blood from the left to right ventricle. Such an evaluation usually begins with an electrocardiogram (EKG, sometimes also abbreviated ECG) and possibly a chest X-ray. A soundwave test of the heart (echocardiogram) is used to define the anatomy and evaluate the characteristics (amount and pressures) of the shunted blood. With the advent of superb echocardiography, the previously required cardiac catheterization is rarely necessary.

What if the VSD is small?

Small defects (less than 0.5 square cm) are common. With a small VSD, there is minimal shunting of blood and the pressure in the right ventricle remains normal. Since the right ventricular pressure is normal, there is no damage to the lung arterioles. The heart functions normally. A prominent murmur heard through a stethoscope is usually the only sign that brings the VSD to attention. This murmur is commonly noted during the first week of life.

How is a small VSD treated?

One-third to one-half of all small VSDs close spontaneously (on their own). This seemingly miraculous event occurs most often before the baby is 1 year old, almost always before age 4 (75% by 2 years of age). The closure is due to the small VSD being located between heart fibers that increase in size in time, thus encroaching upon the opening in the ventricular septum. Even if a small VSD does not close spontaneously, surgical repair is usually not recommended. However, long-term follow-up is required.

What if the VSD is large?

With a large VSD (usually one greater than 1 cm2), there is significant shunting of blood from the left ventricle into the right ventricle. Thus extra blood volume puts a strain on the right ventricle and causes an increase in the blood pressure of the lungs called "pulmonary hypertension." The child may have labored breathing, difficulty feeding, grow poorly, and have pallor.

How is a large VSD treated?

Ultimately, the patient with a large VSD will need surgery to "patch the hole" in the ventricular septum. The timing of the surgery is an individualized decision based upon several factors. These include 1. The extent and duration of increased pulmonary artery pressure. Chronic pulmonary arteriolar pressure may become irreversible and put a strain on the

right ventricle. These side effects may be treated with medications until surgery is appropriate.

2. A child with a large VSD often will not grow as robustly as his peers. The work of increased metabolic demands often requires additional calories when compared to children without such a cardiac defect. High-calorie dense supplements may be added to formula. Some infants may require nighttime continuous feedings using a tube that is passed through the nose to the stomach (nasogastic tube) to maximize growth. It is very rare to restrict fluid volumes in these children.

3. Infants with iron-deficiency anemia should receive iron supplements to maximize the oxygen carrying capacity of their blood. Blood transfusions to address such an anemia are rare.

What types of surgery are available to correct a VSD?

There are two types of surgery available to correct a VSD: the intra-cardiac technique and the trans-catheter technique. Which surgical technique is chosen is an individualized decision based upon the nature of the VSD and associated side effects on the patient's heart and lungs. The intra-cardiac approach is the most common technique and is done while the patient is under cardiopulmonary bypass (a "heartlung machine") and is an open-heart operation. This is the procedure of choice for most children and at most pediatric surgical centers. Another technique uses surgical instruments that are passed through catheters placed in the patient's large blood vessels into the heart. This "trans-catheter approach" is generally more difficult and should only be considered on select patients and at pediatric centers that have expertise in such a procedure.

What is pulmonary hypertension?

The right ventricle pumps blood returning from the body into the pulmonary arteries to the lungs to receive oxygen. The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation. When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary hypertension, pulmonary artery hypertension, or PAH. Pulmonary hypertension results from constriction, or stiffening, of the pulmonary arteries that supply blood to the lungs. Consequently, it becomes more difficult for the heart to pump blood forward through the lungs. This stress on the heart leads to enlargement of the right heart and eventually fluid can build up in the liver and other tissues, such as the in the legs.

Site Normal
[4]

pressure range in mmHg ()38 systolic 1530 diastolic 38 systolic 1530 diastolic 412 215

Central venous pressure Right ventricular pressure Pulmonary artery pressure Pulmonary vein/ Pulmonary capillary wedge pressure

Left ventricular pressure

systolic 100140 diastolic 3-12

Narrowed Valves Simple congenital heart defects also can involve the heart's valves, which control the flow of blood from the atria to the ventricles and from the ventricles into the two large arteries connected to the heart (the aorta and the pulmonary artery). Valves can have the following types of defects:

Stenosis. This is when the valve doesn't open completely, and the heart has to work harder to pump the blood through the valve.

Atresia. This is when the valve doesn't form correctly, so there is no opening for blood to pass through. Regurgitation (re-GUR-ji-TA-shun). This is when the valve doesn't close completely, so blood leaks back through the valve.

The most common valve defect is called pulmonary valve stenosis, which is a narrowing of the pulmonary valve. This valve allows blood to flow from the right ventricle into the pulmonary arteries and out to the lungs to pick up oxygen. Pulmonary valve stenosis can range from mild to severe. Most children with this defect have no signs or symptoms other than a heart murmur. Treatment isn't needed if the stenosis is mild. In a baby with severe pulmonary valve stenosis, the right ventricle can get very overworked trying to pump blood to the pulmonary arteries. Oxygen-poor blood can back up from the right side of the heart into the left side, causing cyanosis. Cyanosis is a bluish tint to the skin, lips, and fingernails. It occurs because the oxygen level in the blood leaving the heart is below normal. Older children with severe pulmonary valve stenosis may have symptoms such as fatigue (tiredness) when exercising. Severe pulmonary valve stenosis is treated with a catheter procedure. Example of a Complex Congenital Heart Defect Complex congenital heart defects need to be repaired with surgery. Because of advances in diagnosis and treatment, doctors can now successfully repair even very complex congenital heart defects. The most common complex heart defect is

tetralogy of Fallot (tehTRALL-o-gee of fall-O), a combination of four defects:

Pulmonary valve stenosis. A large VSD. An overriding aorta. The aorta sits above both the left and right ventricles over the VSD, rather than just over the left ventricle. As a result, oxygen poor blood from the right ventricle can flow directly into the aorta instead of into the pulmonary artery to the lungs. Right ventricular hypertrophy. The muscle of the right ventricle is thicker than usual because of having to work harder than normal.

These defects prevent enough blood from flowing to the lungs to get oxygen, while oxygen-poor blood flows directly out to the body. Normal Heart and Heart With Tetralogy of Fallot

Figure A shows the normal structure and blood flow in the interior of the heart. Figure B shows a heart with the four defects of tetralogy of Fallot. Babies and children with tetralogy of Fallot have episodes of cyanosis, which can sometimes be severe. In the past, when this condition wasn't treated in infancy, older children would get very tired during exercise and could have fainting spells. Tetralogy of Fallot is now repaired in infancy to prevent these types of symptoms. Tetralogy of Fallot must be repaired with open heart surgery, either soon after birth or later in infancy, depending on how severely the pulmonary artery is narrowed. Children who have had this heart defect repaired need lifelong medical care from a specialist to make sure they stay as healthy as possible.

What are other names for congenital heart defects?

Congenital heart disease Cyanotic heart disease Heart defects Congenital cardiovascular malformations

What causes congenital heart defects?

If you have a child with a congenital heart defect, you may think you did something wrong during your pregnancy to cause the problem. However, most of the time doctors don't know why congenital heart defects develop.

Heredity may play a role in some heart defects. For example, a parent who has a congenital heart defect may be more likely than other people to have a child with the condition. In rare cases, more than one child in a family is born with a heart defect. Children with genetic defects often have congenital heart defects. An example of this is Down syndrome - half of all babies with Down syndrome have congenital heart defects. Scientists continue to search for the causes of congenital heart defects.

What are the signs and symptoms and signs of congenital heart defects?
Many congenital heart defects have few or no symptoms. A doctor may not even detect signs of a heart defect during a physical exam. Some heart defects do have symptoms. These depend on the number and type of defects and how severe the defects are. Severe defects can cause symptoms, usually in newborn babies. These symptoms can include:

Rapid breathing Cyanosis (a bluish tint to the skin, lips, and fingernails) Fatigue (tiredness) Poor blood circulation

Congenital heart defects don't cause chest pain or other painful symptoms. Abnormal blood flow through the heart caused by a heart defect will make a certain sound. Your doctor can hear this sound, called a heart murmur, with a stethoscope. However, not all murmurs are a sign of a congenital heart defect. Many healthy children have heart murmurs. Normal growth and development depend on a normal workload for the heart and normal flow of oxygen-rich blood to all parts of the body. Babies with congenital heart defects may have cyanosis or tire easily when feeding. Sometimes they have both problems. As a result, they may not gain weight or grow as they should. Older children may get tired easily or short of breath during exercise or activity. Many types of congenital heart defects cause the heart to work harder than it should. In severe defects, this can lead to heart failure, a condition in which the heart can't pump blood strongly throughout the body. Symptoms of heart failure include:

Fatigue with exercise Shortness of breath A buildup of blood and fluid in the lungs A buildup of fluid in the feet, ankles, and legs

Patent Foramen Ovale (PFO)

Patent Foramen Ovale

Patent Foramen Ovale (PFO) How is a Patent Foramen Ovale (PFO) Closed Using a Catheter-based Procedure

What is a PFO?
A patent foramen ovale (PFO) is a defect in the septum (wall) between the two upper (atrial) chambers of the heart. Specifically, the defect is an incomplete closure of the atrial septum that results in the creation of a flap or a valve-like opening in the atrial septal wall (see illustration). A PFO is present in everyone before birth but seals shut in about 80% of people. With each heart beat or when a person with this defect creates pressure inside his or her chest - such as when coughing, sneezing, or straining during a bowel movement the flap can open, and blood can flow in either direction directly between the right and left atrium. When blood moves directly from the right atrium to the left atrium, this blood bypasses the filtering system of the lungs (the lungs actually do dissolve tiny blood clots). If debris is present in the blood, such as small blood clots, it now passes through the left atrium and can lodge in the brain, causing a stroke, or another organ, such as the heart, eyes, or kidneys.

What are the symptoms of a PFO?

Although present in about one in five adults, PFOs usually cause no symptoms at all. Far less than 1% has a stroke or other outcome that results in the need to have the PFO closed.

What causes a PFO?

A PFO is congenital, meaning it is a defect that is inborn or exists at birth. Stated another way, the defect is an abnormality, not a disease. The septum between the two atria of the heart developed normally before birth, but the flap did not seal completely after birth.

Heart defects in general

Sometimes a viral infection can cause heart defects to develop, other causes include genetic factors, certain other medical conditions (Down syndrome, for example), some prescription and nonprescription drugs, but 95% of the time a cause cannot be identified.

How is a PFO diagnosed?

Frequently a PFO is not diagnosed until a child or adult with this defect has a transient ischemic attack (TIA) - symptoms of a stroke that last for less than 24 hours - or a stroke. Symptoms of a TIA or stroke include any of the following:

Sudden numbness or weakness in the face, arm or leg (especially on one side of the body) Difficulty speaking or understanding words or simple sentences Sudden blurred vision or decreased vision in one or both eyes Difficulty swallowing Dizziness, loss of balance or coordination Brief loss of consciousness Sudden inability to move part of the body (paralysis)

PFO can only be detected by a specialized test. It does not cause a heart murmur. If a PFO is suspected, your doctor may order tests that can include:

An electrocardiogram (ECG or EKG) - a test that records the electrical changes that occur during a heartbeat; reveals abnormal heart rhythms (arrhythmias) and detects heart muscle stress A chest X-ray - a test to show the size and shape of the heart and lungs An echocardiogram - a test that uses sound waves to create a moving picture of the hearts internal structures. Doppler ultrasound - a test that uses sound waves to measure blood flow; often combined with echocardiogram to evaluate both the internal structure of the heart and blood flow across the hearts valves Transesophageal echocardiography - an ultrasound test used to visualize the heart and defect, where an imaging probe with a camera is placed into the esophagus Cardiac magnetic resonance imaging (MRI) - a test that uses three-dimensional imaging to reveal how blood flows through the heart and how the heart is working Cardiac catheterization - a procedure that involves inserting a thin tube (a catheter) into a vein or artery and passing it into the heart to sample the level of oxygen, measure pressure changes, and make X-ray movies of the heart and its internal structures. Angiography - a dye-enhanced X-ray of the hearts internal structures

Additional tests may be ordered as necessary.

How are PFOs treated?

If you or your child is diagnosed with a PFO, your primary care doctor will recommend that you meet with a congenital heart specialist (a doctor who has the training and equipment to determine the heart problem) who will order the necessary special tests, medical care and follow-up checkups. A careful assessment of the patients stroke (if he or she has already experienced one) by a neurologist will first need to be done to determine the best course of action for the PFO. The usual care for a patient who has had a stroke is the use of blood-thinning medications, such as aspirin or the prescription drugs warfarin (Coumadin) or clopidrogrel (Plavix). These drugs keep the blood cells from sticking together, reducing the risk of blood clot development that could lead to new TIAs or stroke. However, there are risks and inconveniences associated with the long-term use of blood-thinning medications including:

Development of ulcers Internal bleeding Bleeding in the brain Blood in the urine Bleeding in the rectal tissue The need to avoid activities that could result in injury, which could trigger internal or external bleeding

An alternative for patients unable to take blood-thinning drugs or those who have a second stroke while on blood thinning drugs is nonsurgical (catheter based) closure of the hole. To learn more about this procedure, please see "How is a PFO Closed Using a Catheter-Based Procedure?".

Common AV Canal (Atrio-ventricular septal defect, endocardial cushion defect, atrial ventricular canal defect)
Background:

AV canal is a complex congenital heart defect that results in a large hole in the center portion of the heart. This defect may involve several abnormalities of structures inside the heart:
Atrial

septal defect (ASD), which is a hole between the two upper heart chambers. Ventricular septal defect (VSD), which is a hole between the two lower heart chambers. Maldevelopment of the tricuspid and mitral valves. Most patients have a single (common) large and leaky AV valve instead.

The end result of these heart defects is volume overload. The heart is working harder trying to compensate for the excessive amount of blood being retained inside the heart plus a large amount of blood passing through the heart and lungs.

The primary defect in this malformation is failure of formation of the part of the heart that arises from an embryonic structure called the endocardial cushions. These cushions are responsible for separating the central parts of the heart at the level of the tricuspid and mitral valves. The structures that are supposed to develop from this endocardial cushions include the lower part of the atrial septum (the wall that divides the two upper heart chambers) and the ventricular septum (the middle wall that divides the two lower heart chambers). These cushions also complete the separation of the mitral and tricuspid valve by dividing the embryonic single AV valve into two. In the complete form of AV canal there is failure of formation of the mitral and tricuspid valves and a great part of the middle walls of the heart.

Etiology:

The AV canal may be associated with Down syndrome, other chromosomal abnormalities and heterotaxia (splenic syndromes). Other conditions may be associated with development of AV canal.

Many times patients with AV canal have other congenital heart defects, some of them quite complex in themselves

Symptoms:

The doctor will usually hear a heart murmur or may refer an asymptomatic child who has Down syndrome. These patients may present with no murmur at all. Symptoms may occur at any time from birth to a few months of age. Most infants with AV canal do not grow normally and they become undernourished because of the large amount of blood flowing through the heart and lungs. Heart failure (CHF) and pulmonary hypertension (high blood pressure in the lungs) may develop during early infancy. In addition, if left untreated this heart defect can cause irreversible lung damage. The symptoms depend on the size of the holes and leakage of the common AV valve as well as the amount of volume overload (increased amount of blood passing through the heart and lungs) and if there are associated heart defects. Symptoms may include:
Poor Poor

feeding or tiring out weight gain Fatigue Excessive sweating Rapid or heavy breathing Congested breathing Rapid heart rate Pale, cold or dusky skin Blue baby

Tests:

Electrocardiogram, Echocardiogram, chest x-ray, blood tests and, in some cases, cardiac catheterization. The echocardiogram is an essential part of the cardiovascular evaluation as it helps determine how much the common AV valve is leaking, the amount of holes and how large the holes are. It also helps in identifying any associated heart defects and any underdeveloped heart chambers.

Treatment:

Most patients with AV canal will require cardiac medications, which may include diuretics (water pill), Digoxin and ACE-inhibitors (Enalapril, Lisinopril). The above treatment helps alleviate symptoms and allows the baby to get big enough to undergo surgical repair with lower risks. Most patients have heart surgery by mid-infancy. Children with a transitional or partial AV canal may undergo surgery between 6 to 24 months of age.

Surgery involves closure of the upper and lower holes with a patch or patches, and reconstruction of the leaky valve. Surgery is considered a palliative procedure (it is not a cure or fix). Many patients will remain with leaky valves or obstruction (mitral stenosis). In addition, the patch may have a residual leak.

The conduction system that conducts the electrical impulse through the heart is very close where the surgical stitches are placed and there is a small chance of creating an electrical heart block, which usually requires implantation of a pacemaker. In any event, the heart surgery is considered low-risk, unless it is a complex type of AV canal with other associated heart defects. In those patients with more complex heart lesions the surgical risk would be more significant. About 10% of patients may require more than one heart surgery during their lifetime. Children with Down syndrome will benefit from special programs that enhance their mental and physical development.

Cardiac Center
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Transposition of the Great Arteries (TGA)

What is transposition of the great arteries (TGA)?

View Large Transposition of the Great Arteries IllustrationTransposition of the great arteries (TGA) is a complex congenital heart defect in which the two large arteries that carry blood out of the heart are connected to the heart abnormally:

The aorta is attached to the right-sided pumping chamber (ventricle), instead of the left. The pulmonary artery is attached to the left-sided pumping chamber (ventricle), instead of the right.

In the animation above, doctors describe the structure of a heart with transposition of the great arteries. Normally, blood flows in this pattern: body right side of heart lungs left side of heart back to body. The pulmonary artery carries blood from the right side of the heart to the lungs, and the aorta carries blood from the left side of the heart to the body. In children with TGA, the normal pattern of flow does not exist, and the body doesn't get enough oxygenated blood. BACK TO TOP

What are the symptoms of TGA?

The symptoms of TGA include:

Blue or purple tint to lips, skin and nails (cyanosis) Rapid breathing Difficulty feeding; poor appetite and poor weight gain

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How is transposition of the great arteries diagnosed?

Transposition of the great arteries may be diagnosed before birth, with fetal echocardiogram (ultrasound). Our Fetal Heart Program will prepare a plan for delivery and care immediately after birth. Sometimes TGA is diagnosed when an infant is a few hours or days old, and in some cases, infants may not have visible symptoms for weeks or months. Pediatricians from other hospitals refer newborns to the Cardiac Center when they notice symptoms or abnormal values on laboratory testing such as pulse oximetry.

Diagnosis of TGA may require some or all of these tests:

Echocardiogram (also called "echo" or ultrasound) - sound waves create an image of the heart Electrocardiogram (ECG) - a record of the electrical activity of the heart Chest X-ray Pulse oximetry - a non-invasive way to monitor the oxygen content of the blood Cardiac catheterization - a thin tube is inserted into the heart through a vein and/or artery in either the leg or through the umbilicus ("belly button") Cardiac MRI - a three-dimensional image shows the heart's abnormalities

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What are the treatment options for TGA?

The animation above shows balloon atrial septostomy. Transposition of the great arteries is unpredictable. Approximately one-third of newborns with the condition will require an urgent intervention, called a balloon atrial septostomy (BAS), within hours after birth. This procedure, which creates or enlarges a hole between the upper chambers of the heart to allow blood to mix, was developed at The Childrens Hospital of Philadelphia in the 1960s. For babies requiring a BAS procedure, the clock begins ticking at the moment of birth and access to immediate expert care is essential. At CHOP, we may recommend that these babies be delivered in our Garbose Family Special Delivery Unit, which is just steps away from our cardiac operative and catheterization facilities. All children with transposition of the great arteries will require open heart surgery to treat the defect. Without surgical repair, the overwhelming majority of patients with TGA will not survive their first year. The surgery, known as the arterial switch operation, is typically performed within a few days of birth. Surgeons reconstruct the heart so that the aorta is attached to the left ventricle and the pulmonary artery is attached to the right ventricle. After surgery your child will recover in our Tabas Cardiac Intensive Care Unit (CICU). BACK TO TOP

What is the follow-up care for TGA?

Through age 18
In the animation above, doctors describe the arterial switch operation. Children who have had surgical repair of TGA require life-long care by a cardiologist. Ongoing medication use is uncommon. More surgery may be required as the child grows.

Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physicians.

Into adulthood
We will help patients transition to an adult cardiologist. The Philadelphia Adult Congenital Heart Center, a joint program of The Children's Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects. Until approximately 25 years ago, infants with transposition of the great arteries were managed by alternative surgical procedures, sometimes referred to as the "Senning" or "Mustard" operations. As a result, we don't yet know the truly long-term effects of the arterial switch operation beyond young adulthood. It is anticipated that the overwhelming majority of children born with TGA will go on to lead healthy, productive lives. Limitations to day-to-day activities, including sports, are rare.