Transposition of the Great Arteries

An introduction to the Symptoms, Diagnosis and Treatment for Transposition of the Great Arteries

Overview

Transposition of the great arteries is a serious but rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). The condition is also called dextro-transposition of the great arteries. A rarer type of this condition is called levo-transposition of the great arteries.

Transposition of the great arteries changes the way blood circulates through the body, leaving a shortage of oxygen in blood flowing from the heart to the rest of the body. Without an adequate supply of oxygen-rich blood, the body can’t function properly and your child faces serious complications or death without treatment.

Transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life.

Corrective surgery soon after birth is the usual treatment for transposition of the great arteries, and with proper treatment, the outlook is promising.

Even when this condition is diagnosed and treated early in childhood, there is a potential to develop complications later in life. All people with Transposition of the Great Arteries should have lifelong follow up with a congenital heart specialist. Our experts at the Irish Congenital Heart Centre are trained in congenital cardiology and have vast experience in treating people with Transposition of the Great Arteries.

Transposition of the great arteries

Symptoms

In infants, Transposition of the great arteries symptoms include:

  • Blue colour of the skin (cyanosis)
  • Shortness of breath
  • Lack of appetite
  • Poor weight gain

In adults who have had previous Transposition of the Great arteries repaired, they may still experience:

  • Shortness of breath with exercise/exertion
  • Palpitations
  • Dizziness/fainting episodes
  • Chest pain

Diagnosis

Although it’s possible your baby’s transposition of the great arteries may be diagnosed before birth, it can be difficult to diagnose. Prenatal tests for transposition of the great arteries aren’t usually done unless your doctor suspects your baby may have congenital heart disease.

A physical exam alone isn’t enough to accurately diagnose transposition of the great arteries. One or more of the following tests are necessary for an accurate diagnosis:

  • Echocardiogram: An echocardiogram is an ultrasound of the heart — it uses sound waves that bounce off your baby’s heart and produce moving images that can be viewed on a video screen. Doctors use this test to diagnose transposition of the great arteries by looking at the position of the aorta and the pulmonary artery. Echocardiograms can also identify associated heart defects, such as a ventricular septal defect, atrial septal defect or patent ductus arteriosus.
  • Cardiac catheterisation: This procedure is typically done only when other tests, such as an echocardiogram, don’t show enough information to make a diagnosis. During a cardiac catheterisation, the doctor inserts a thin flexible tube (catheter) into an artery or vein in your baby’s groin and directs it up to his or her heart. A dye is injected through the catheter to make your baby’s heart structures visible on X-ray pictures. The catheter also measures pressure in the chambers of your baby’s heart and in the blood vessels and can measure the amount of oxygen in the blood. Cardiac catheterisation may be done urgently to perform a temporary treatment for transposition of the great arteries (balloon atrial septostomy).
  • Chest X-ray: Although a chest X-ray doesn’t provide a definitive diagnosis of transposition of the great arteries, it does allow the doctor to see your baby’s heart size and the position of the aorta and pulmonary artery.
  • Electrocardiogram: An electrocardiogram records the electrical activity in the heart each time it contracts. During this procedure, patches with wires (electrodes) are placed on your baby’s chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper.

Treatment

All infants with transposition of the great arteries need surgery to correct the defect.

Before surgery

Your baby’s doctor may recommend several options to help manage the condition before corrective surgery. They include:

  • Medication: The medication called prostaglandin E1 helps keep the connection between the aorta and pulmonary artery open (ductus arteriosus), increasing blood flow and improving mixing of oxygen-poor and oxygen-rich blood until surgery can be performed.
  • Atrial septostomy: This procedure — usually done using cardiac catheterisation rather than surgery — enlarges a natural connection between the heart’s upper chambers (atria). It allows for the oxygen-rich and oxygen-poor blood to mix and results in improved oxygen delivery to your baby’s body.

Surgery

Surgical options include:

  • Arterial switch operation: This is the most common surgery used to correct transposition of the great arteries. Surgeons usually perform this surgery within the first month of life. During an arterial switch operation, the pulmonary artery and the aorta are moved to their normal positions: The pulmonary artery is connected to the right ventricle, and the aorta is connected to the left ventricle. The coronary arteries also are reattached to the aorta. If your baby has a ventricular septal defect or an atrial septal defect, those holes usually are closed during surgery. In some cases, however, the doctor may leave small ventricular septal defects to close on their own.
  • Atrial switch operation: In this surgery, the surgeon makes a tunnel (baffle) between the heart’s two upper chambers (atria). This diverts the oxygen-poor blood to the left ventricle and the pulmonary artery and the oxygen-rich blood to the right ventricle and the aorta. With this procedure, the right ventricle must pump blood to the body, instead of just to the lungs as it would do in a normal heart. Possible complications of the atrial switch operation include irregular heartbeats, baffle obstructions or leaks, and heart failure due to long-term problems with right ventricle function.

Follow-up Care

You or your child will need to be monitored and treated throughout your life.

Many people who undergo the arterial switch operation don’t need additional surgery. However, some complications, such as abnormal heart rhythms, heart valve leaks or problems with the heart’s pumping, may require additional treatment.

Those who have had an atrial switch surgery may develop complications related to the baffle material (leak or narrowing). They may also experience heart rhythm abnormalities or poor pumping function of the heart.

Here at the Irish Congenital Heart Centre, our experts routinely assess for any possible complications at your follow up appointments. They will advise you about your heart health, and if necessary, discuss available treatment options.

Conditions We Treat

Pulmonary Valve Stenosis

Pulmonary valve stenosis is a condition in which a deformity on or near your pulmonary valve narrows the pulmonary valve opening and slows the blood flow. The pulmonary valve is located between the lower right heart chamber (right ventricle) and the pulmonary arteries. Adults occasionally have pulmonary valve stenosis as a complication of another illness, but mostly, pulmonary valve stenosis develops before birth as a congenital heart defect. Pulmonary valve stenosis ranges from mild to severe. Mild pulmonary stenosis doesn't usually worsen over time, but moderate and severe cases may worsen and require surgery. Fortunately, treatment is generally highly successful, and most people with pulmonary valve stenosis can expect to lead normal lives. Find Out More >>

Patent Foramen Ovale

A patent foramen ovale (PFO) is a hole in the heart that didn't close the way it should have after birth. During fetal development, a small flap-like opening — the foramen ovale — is present in the wall between the right and left upper chambers of the heart (atria). It normally closes during infancy. When the foramen ovale doesn't close, it's called a patent foramen ovale. Patent foramen ovale occurs in about 25% of the normal population, but most people with the condition never know they have it. Learning that you have a patent foramen ovale is understandably concerning, but most people never need treatment for this condition. A patent foramen ovale is often discovered during tests for other problems. In certain conditions, such as unexplained strokes (cryptogenic stroke) in young people, closure might be recommended by your doctor. Find Out More >>

Tetralogy of Fallot

Tetralogy of Fallot is a rare condition caused by a combination of four heart defects that are present at birth (congenital). These defects, which affect the structure of the heart, cause oxygen-poor blood to flow out of the heart and to the rest of the body. Tetralogy of Fallot is usually diagnosed during infancy or soon after. However, it might not be detected until later in life in some adults, depending on the severity of the defects and symptoms. With early diagnosis followed by appropriate surgical treatment, most people who have Tetralogy of Fallot live relatively normal lives. Many adults with repaired Tetralogy of Fallot will require another procedure or intervention during their lifetimes. It is important to have regular follow-up with a cardiologist trained in congenital heart disease who can evaluate you and determine the appropriate timing of another intervention or procedure. At the Irish Congenital Heart Centre, our specialists have trained in congenital heart disease and are expert at caring for people with Tetralogy of Fallot. Find Out More >>

Partial Anomalous Pulmonary Venous Return

Partial anomalous pulmonary venous return, sometimes called partial anomalous pulmonary venous connection, is a heart defect present at birth (congenital) in which some of the pulmonary veins carrying blood from the lungs to the heart flow into other blood vessels or into the heart's upper right chamber (right atrium), instead of correctly entering the heart's upper left chamber (left atrium). This causes some oxygen-rich blood from the lungs to mix with oxygen-poor blood before entering the right atrium. Some people with this defect also have a hole between the upper heart chambers (atrial septal defect), which allows blood to flow between the upper heart chambers (atria). People with this condition may also have other congenital heart defects. Find Out More >>

Ebstein Anomaly

Ebstein anomaly is a rare heart defect that's present at birth (congenital). In Ebstein anomaly, your tricuspid valve — the valve between the two right heart chambers (right atrium and right ventricle) — doesn't work properly. The tricuspid valve sits lower than normal in the right ventricle, and the tricuspid valve's leaflets are abnormally formed. Blood may leak back through the valve, making your heart work less efficiently. Ebstein anomaly may also lead to enlargement of the heart or heart failure. A significant proportion of people with Ebstein anomaly will also have a communication between the top two chambers of the heart - an atrial septal defect (ASD). Abnormal electrical activity of the heart, leading to abnormal heartbeats (arrhythmia) is also a common problem for people with Ebstein anomaly. If you have no signs or symptoms associated with Ebstein anomaly, careful monitoring of your heart may be all that's necessary. If signs and symptoms bother you, or if the heart is enlarging or becoming weaker, treatment for Ebstein anomaly may be necessary. Treatment options include medications and surgery. Find Out More >>

Atrial Septal Defect

An atrial septal defect (ASD) is a hole in the wall between the two upper chambers of your heart (atria). The condition is present at birth (congenital). Small defects may never cause a problem and may be found incidentally. It's also possible that small atrial septal defects may close on their own during infancy or early childhood. Large and long-standing atrial septal defects can damage your heart and lungs. An adult who has had an undetected atrial septal defect for decades may have a shortened life span from heart failure or high blood pressure that affects the arteries in the lungs (pulmonary hypertension). Surgery may be necessary to repair atrial septal defects to prevent complications. Find Out More >>

Atrioventricular Canal Defect

Atrioventricular canal defect is a combination of heart problems resulting in a defect in the centre of the heart. The condition occurs when there's a hole between the heart's chambers and problems with the valves that regulate blood flow in the heart. Sometimes called endocardial cushion defect or atrioventricular septal defect, atrioventricular canal defect is present at birth (congenital). The condition is often associated with Down Syndrome. Atrioventricular canal defect allows extra blood to flow to the lungs. The extra blood forces the heart to overwork, causing the heart muscle to enlarge. Untreated, atrioventricular canal defect can cause heart failure and high blood pressure in the lungs. Doctors generally recommend surgery during the first year of life to close the hole in the heart and to reconstruct the valves. Find Out More >>

Bicuspid Aortic Valve

Bicuspid aortic valve is the commonest form of congenital heart disease. Approximately 1% of the population are born with this condition. A bicuspid aortic valve is an aortic valve that has only two leafelts (bicuspid) instead of the normal three leaflets (tricuspid) . Some people may also be born with one (unicuspid) or four (quadricuspid) leaflets, but these are rare. A bicuspid aortic valve may cause the heart's aortic valve to narrow (aortic valve stenosis). This narrowing prevents the valve from opening fully, which reduces or blocks blood flow from the heart to the body. In some cases, the bicuspid aortic valve doesn't close efficiently, causing blood to leak backward into the left ventricle (aortic valve regurgitation). Some people with a bicuspid aortic valve may develop an enlarged aorta — the main blood vessel leading from the heart (see also  our Aortopathy section) Most people with a bicuspid aortic valve aren't affected by valve problems until they are adults, and some may not be affected until they are older adults. However if you are known to have a biscupid aortic valve, you should be followed up by a cardiologist at regular intervals. Find Out More >>

Coarctation of the Aorta

Coarctation of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrowed part of your aorta. Coarctation of the aorta is generally present at birth (congenital). The condition can range from mild to severe, and might not be detected until adulthood, depending on how narrowed the aorta is. Coarctation of the aorta often occurs along with other heart defects. While treatment is usually successful, the condition requires careful lifelong follow-up. Find Out More >>

Ventricular Septal Defect

A ventricular septal defect (VSD) is a type of hole in the heart. It is present at birth (congenital). The hole (defect) occurs in the wall (septum) that separates the heart's lower chambers (ventricles) and allows blood to pass from the left to the right side of the heart. This means that oxygen-rich blood then gets pumped back to the lungs instead of out to the body, causing the heart to work harder. A small ventricular septal defect may cause no problems, and many small VSDs close on their own. Medium or larger VSDs may need surgical repair early in life to prevent complications. Find Out More >>

Transposition of the Great Arteries

Transposition of the great arteries is a serious but rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). The condition is also called dextro-transposition of the great arteries. A rarer type of this condition is called levo-transposition of the great arteries. Transposition of the great arteries changes the way blood circulates through the body, leaving a shortage of oxygen in blood flowing from the heart to the rest of the body. Without an adequate supply of oxygen-rich blood, the body can't function properly and your child faces serious complications or death without treatment. Transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life. Corrective surgery soon after birth is the usual treatment for transposition of the great arteries, and with proper treatment, the outlook is promising. Even when this condition is diagnosed and treated early in childhood, there is a potential to develop complications later in life. All people with Transposition of the Great Arteries should have lifelong follow up with a congenital heart specialist. Our experts at the Irish Congenital Heart Centre are trained in congenital cardiology and have vast experience in treating people with Transposition of the Great Arteries. Find Out More >>

Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). The left side of the heart is critically underdeveloped in hypoplastic left heart syndrome. If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body. Instead, the right side of the heart must pump blood to the lungs and to the rest of the body. Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome. With advances in care, the outlook for babies born with hypoplastic left heart syndrome is better now than in the past. All people who are born with hypoplastic left heart syndrome will have undergone multiple complex cardiac surgeries and require lifelong follow up by a specialist congenital heart expert. Find Out More >>

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