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Transposition of the great arteries

d-TGA; Congenital heart defect - transposition; Cyanotic heart disease - transposition; Birth defect - transposition; Transposition of the great vessels; TGV

Transposition of the great arteries (TGA) is a heart defect that occurs from birth (congenital). The two major arteries that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched (transposed).

Causes

The cause of TGA is unknown. It is not associated with any one common genetic abnormality. It rarely occurs in other family members.

TGA is a cyanotic heart defect. This means there is decreased oxygen in the blood that is pumped from the heart to the rest of the body.

In normal hearts, blood that returns from the body goes through the right side of the heart and pulmonary artery to the lungs to get oxygen. The blood then comes back to the left side of the heart and travels out the aorta to the body.

In TGA, venous blood returns normally to the heart through the right atrium. But, instead of going to the lungs to absorb oxygen, this blood is pumped out through the aorta and back to the body. This blood has not been recharged with oxygen and leads to cyanosis.

Symptoms appear at birth or very soon afterward. How bad the symptoms are depends on the type and size of additional heart defects (such as atrial septal defect, ventricular septal defect, or patent ductus arteriosus) and how much the blood can mix between the two abnormal circulations.

Symptoms

Symptoms may include:

Exams and Tests

The health care provider may detect a heart murmur while listening to the chest with a stethoscope. The baby's mouth and skin will be a blue color.

Tests often include the following:

Treatment

The initial step in treatment is to allow oxygen-rich blood to mix with poorly oxygenated blood. The baby will immediately receive a medicine called prostaglandin through an IV (intravenous line). This medicine helps keep a blood vessel called the ductus arteriosus open, allowing some mixing of the two blood circulations. In some cases, an opening between the right and left atrium can be created with procedure using a balloon catheter. This allows blood to mix. This procedure is known as balloon atrial septostomy.

Permanent treatment involves heart surgery during which the great arteries are cut and stitched back to their correct position. This is called an arterial switch operation (ASO). Prior to the development of this surgery, a surgery called an atrial switch (or Mustard procedure or Senning procedure) was used.

Outlook (Prognosis)

The child's symptoms will improve after surgery to correct the defect. Most infants who undergo arterial switch do not have symptoms after surgery and live normal lives. If corrective surgery is not performed, the life expectancy is only months.

Possible Complications

Complications may include:

  • Coronary artery problems
  • Heart valve problems
  • Irregular heart rhythms (arrhythmias)

When to Contact a Medical Professional

This condition can be diagnosed before birth using a fetal echocardiogram. If not, it is most often diagnosed soon after a baby is born.

Go to the emergency room or call the local emergency number (such as 911) if your baby's skin develops a bluish color, especially in the face or trunk.

Call your provider if your baby has this condition and new symptoms develop, get worse, or continue after treatment.

Prevention

Women who plan to become pregnant should be immunized against rubella if they are not already immune. Eating well, avoiding alcohol, and controlling diabetes both before and during pregnancy may be helpful.

References

Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice. 20th ed. Philadelphia, PA: Elsevier; 2017:chap 58.

Kliegman RM, Stanton BF, St. Geme JW, Schor NF. Cyanotic congenital heart lesions: lesions associated with decreased pulmonary blood flow. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 430.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Mann DL, Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 62.

Text only

  • Heart, section through the middle

    Heart, section through the middle - illustration

    The interior of the heart is composed of valves, chambers, and associated vessels.

    Heart, section through the middle

    illustration

  • Heart, front view

    Heart, front view - illustration

    The external structures of the heart include the ventricles, atria, arteries and veins. Arteries carry blood away from the heart while veins carry blood into the heart. The vessels colored blue indicate the transport of blood with relatively low content of oxygen and high content of carbon dioxide. The vessels colored red indicate the transport of blood with relatively high content of oxygen and low content of carbon dioxide.

    Heart, front view

    illustration

  • Transposition of the great vessels

    Transposition of the great vessels - illustration

    Transposition of the great vessels is a congenital heart defect in which the position of the two major vessels that carry blood away from the heart, the aorta and the pulmonary artery, is switched (transposed). This defect is classified as a cyanotic heart defect because the condition results in insufficiently oxygenated blood pumped to the body which leads to cyanosis (a bluish-purple coloration to the skin) and shortness of breath.

    Transposition of the great vessels

    illustration

    • Heart, section through the middle

      Heart, section through the middle - illustration

      The interior of the heart is composed of valves, chambers, and associated vessels.

      Heart, section through the middle

      illustration

    • Heart, front view

      Heart, front view - illustration

      The external structures of the heart include the ventricles, atria, arteries and veins. Arteries carry blood away from the heart while veins carry blood into the heart. The vessels colored blue indicate the transport of blood with relatively low content of oxygen and high content of carbon dioxide. The vessels colored red indicate the transport of blood with relatively high content of oxygen and low content of carbon dioxide.

      Heart, front view

      illustration

    • Transposition of the great vessels

      Transposition of the great vessels - illustration

      Transposition of the great vessels is a congenital heart defect in which the position of the two major vessels that carry blood away from the heart, the aorta and the pulmonary artery, is switched (transposed). This defect is classified as a cyanotic heart defect because the condition results in insufficiently oxygenated blood pumped to the body which leads to cyanosis (a bluish-purple coloration to the skin) and shortness of breath.

      Transposition of the great vessels

      illustration

     

    Review Date: 12/15/2017

    Reviewed By: Steven Kang, MD, Director, Cardiac Electrophysiology, Alta Bates Summit Medical Center, Stanford Healthcare, Oakland, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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