Transposition of the Great Arteries

Transposition of the great arteries is a reversal of the aorta and pulmonary artery, the two major blood vessels leaving from the heart. There are two types of transpositions, a D-type and an L-type.

In D-type transposition of the great arteries (D-TGA), the two main arteries (aorta and pulmonary artery) are connected to the wrong chambers of the heart. When the vessels are switched, the aorta is on the wrong side of the heart and gets oxygen-poor blood from the body instead of oxygen-rich blood fresh from the lungs. The oxygen-poor blood continues to recirculate to the body. At the same time, the pulmonary artery keeps recirculating oxygen-rich blood to the lungs instead of sending it out to the body. This heart defect will require surgery.

In L-type transposition of the great arteries (L-TGA), not only are the two great arteries reversed, but the two ventricles also are in abnormal positions. Oxygen-poor blood travels from the right atrium to the left ventricle, then out to the pulmonary artery and lungs. Oxygen-rich blood travels from the left atrium to the right ventricle then out the aorta to the body. Overall, blood flow is normal and the condition does not require an operation, however there are many heart defects associated with L-TGA that may require intervention. In addition, the conduction system of the heart can be abnormal and the right ventricle has to do the work of the left ventricle, which makes it apt to fail over time.

The cardiothoracic surgeons at Norton Children’s Heart Institute — the leading providers of pediatric heart care in Louisville and Southern Indiana — have been repairing D-type transposition of the great vessels with the arterial switch surgery for decades.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute, affiliated with the University of Louisville, have the skill and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

D-Type Transposition of the Great Arteries Symptoms

D-type transposition of the great vessels (D-TGA) almost always is diagnosed in the first hours or days of life. Babies born with D-TGA often appear blue — a condition called cyanosis — caused by low oxygen levels in the bloodstream. They may have trouble feeding and breathing.

Babies with D-TGA need a connection between the right and left circulations. This can occur via a patent ductus arteriosus (PDA), an atrial septal defect or patent foramen ovale (PFO), or a ventricular septal defect. These connections may allow enough mixing of the blood to prevent severe cyanosis in the first hours or days of life. A PDA or PFO typically closes within a few days after birth, which can make the cyanosis more severe.

As the child experiences low oxygen levels, rapid breathing occurs. The infants may become more blue or cyanotic in color, not move around as much, and not want to feed.

Transposition of the Great Arteries Diagnosis

Transposition of the great arteries can be diagnosed by an ultrasound test during pregnancy, although it can be missed during a routine fetal ultrasound. A fetal echocardiogram performed by heart specialists may be needed to find a transposition of the great arteries during pregnancy. The echocardiogram will show the child’s heart and allow specialists to diagnose the condition.

Babies with cyanosis often are placed on oxygen. If a baby has an issue with the lungs, the oxygen therapy will improve the baby’s oxygen levels. In cases of a heart issue, oxygen therapy will not improve the child’s oxygen levels. When this happens, it is a sign that the baby may have a heart condition such as transposition of the great arteries. Doctors will then order an echocardiogram (echo), which will help them diagnose transposition of the great arteries.

If left untreated, over 50% of infants with transposition of the great arteries will die in the first month of life. About 90% will die in the first year.

D-Type Transposition of the Great Arteries Treatment

When a baby is first suspected of having D-type transposition of the great arteries (D-TGA), the first focus is to help the child maintain safe oxygen levels as well as safe heart and lung function.

After a diagnosis is suspected or confirmed, doctors may:

  • Give a steady dose of prostaglandins, a medicine that helps keep the ductus arteriosus open. This allows oxygen-rich blood to mix with some of the oxygen-poor blood.

Once the diagnosis is confirmed, doctors may:

  • Perform a balloon atrial septostomy. With this procedure, a balloon-tipped catheter is inserted into the heart to stretch or enlarge the foramen ovale, a natural connection that babies have between the right atrium and left atrium. The catheter is placed by threading it through a blood vessel in the navel or groin. Making the foramen ovale bigger creates an atrial septal defect, a hole that allows oxygen-rich blood to mix with oxygen-poor blood. This way, the baby’s oxygen levels can stay in a safe range, often around 80%. After a balloon atrial septostomy, the baby can stop receiving prostaglandins. The balloon placement helps keep the baby stable before the D-TGA is corrected with surgery. Corrective surgery usually is performed during the first week of life.
  • Perform an arterial switch. This operation is performed on most cases of D-TGA. Surgeons cut the aorta and pulmonary arteries just above the point where they leave the heart, then reconnect them to the proper ventricles. The valves stay attached to the ventricles, so what was once the pulmonary valve becomes the aortic valve and vice versa. The coronary arteries must be taken off the area above the valve and replanted separately above the new aortic valve. During this surgery, any ventricular septal defects (if present) and atrial septal defects also are repaired. In some cases, an arterial switch may not be the best answer. Your heart team will work with you and your child to decide the best course of treatment.

D-Type Transposition of the Great Arteries Complications and Follow-up After Surgery

Before surgeons developed the arterial switch procedure a generation ago, half the babies born with this defect died within a month. Up to 90% of babies didn’t live to see their first birthday. Now, some of the first babies to have this surgery to correct transposition of the great vessels are young adults and living full, active lives.

Here is what you can expect after surgery, including complications children may experience.

Since the arterial switch surgery reconstructs the heart to work the way it should, long-term heart function should be excellent. Patients usually have normal ventricular function and no heart rhythm issues, such as murmurs.

However, a small percentage of children can experience:

  • A narrowing (stenosis) where the aorta and pulmonary artery are reattached to the correct ventricles, which can happen months or years after surgery. It may require a repair, including cardiac catheterization with balloon dilation of the narrowed area or another operation, depending on the characteristics of the narrowing.
  • The aorta enlarging where it arises from the left ventricle
  • Issues with flow through the coronary arteries

As a child grows after an arterial switch, they can have good quality of life, including participating in physical activity and sports without difficulty or restrictions. Repeat surgeries usually are not expected, but may be needed in later years if narrowing of the great arteries or enlargement of the aorta occurs.

A child is expected to have long-term follow-up with a cardiologist to watch for any issues as they arise. Follow-up will include regular heart exams, scheduled echocardiograms and perhaps exercise stress tests. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist to provide care.

Why Choose Norton Children’s Heart Institute

No other congenital heart surgery program in Kentucky, Ohio or Southern Indiana is rated higher by the Society of Thoracic Surgeons than the Norton Children’s Heart Institute Pediatric Cardiothoracic Surgery Program.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • Our board-certified and fellowship-trained pediatric cardiovascular surgeons are leaders in the field as clinicians and researchers.
  • More than 5,000 children a year visit Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, for advanced heart care.
  • Norton Children’s Heart Institute successfully performs more than 17,500 procedures a year.
  • The Society of Thoracic Surgeons rated Norton Children’s Heart Institute among the best in the region after studying years of our patients’ outcomes and our ability to treat a range of pediatric heart conditions, including the most severe.
  • Norton Children’s Heart Institute has satellite outpatient offices in Ashland, Bowling Green, Campbellsville, Elizabethtown, Frankfort, London, Madisonville, Murray, Owensboro, Paducah and Shepherdsville in Kentucky; as well as Corydon, Jasper, Madison and Scottsburg in Indiana; 28 tele-echocardiography locations in Kentucky and Southern Indiana; and six fetal echocardiography locations across Kentucky.
  • The American Board of Thoracic Surgery has awarded the cardiothoracic surgeons at Norton Children’s Hospital with subspecialty certification in congenital heart surgery.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
  • Our multidisciplinary approach to pediatric heart surgery brings together our specialists in cardiothoracic surgery, cardiology, anesthesiology, cardiac critical care and other areas to create a complete care plan tailored for your child.
Heart – 2929

Norton Children’s Heart Institute

Call for an appointment

(502) 629-2929

2 years after emergency heart surgery when he was born, Trent is happy and energetic

Trent Robinson was rushed to Norton Children’s Hospital by ambulance from a nearby hospital just after being delivered by emergency cesarean section in March 2018. “He was turning blue. I got to see him for […]

Read Full Story

Bowling Green family becomes a ‘Brave Heart family’

The Miller family of Bowling Green, Kentucky, has a reason to celebrate Heart Month every day. Their daughter, Abigail, recently celebrated her first birthday — her “heartiversary” — with family and friends. A year ago, […]

Read Full Story

After arterial switch heart operation, the baby is a man on the move

Logan Carroll is a young man on the move. A junior at Western Kentucky University, Carroll spent last summer biking 3,600 miles across the United States to raise money for Alzheimer’s research with his fraternity, […]

Read Full Story
Related Stories

2 years after emergency heart surgery when he was born, Trent is happy and energetic

Trent Robinson was rushed to Norton Children’s Hospital by ambulance from a nearby hospital just after being delivered by emergency cesarean section in March 2018. “He was turning blue. I got to see him for […]

Read Full Story

Bowling Green family becomes a ‘Brave Heart family’

The Miller family of Bowling Green, Kentucky, has a reason to celebrate Heart Month every day. Their daughter, Abigail, recently celebrated her first birthday — her “heartiversary” — with family and friends. A year ago, […]

Read Full Story

After arterial switch heart operation, the baby is a man on the move

Logan Carroll is a young man on the move. A junior at Western Kentucky University, Carroll spent last summer biking 3,600 miles across the United States to raise money for Alzheimer’s research with his fraternity, […]

Read Full Story

Search our entire site.