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Transposition of the great vessels

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

 

Transposition of the great vessels is a heart defect that occurs from birth (congenital). The 2 major vessels that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched (transposed).

Causes

 

The cause of most congenital heart defects is unknown.

Factors in the mother that may increase the risk for this condition include:

  • Age over 40
  • Alcoholism
  • Diabetes
  • Poor nutrition during pregnancy (prenatal nutrition)
  • Rubella or other viral illness during pregnancy

Transposition of the great vessels 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 transposition of the great vessels, the blood goes to the lungs, picks up oxygen, returns to the heart, and then flows right back to the lungs without ever going to the body. Blood from the body returns to the heart and goes back to the body without ever picking up oxygen in the lungs.

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 2 abnormal circulations.

 

Symptoms

 

Symptoms may include:

  • Blueness of the skin
  • Clubbing of the fingers or toes
  • Poor feeding
  • Shortness of breath

 

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:

  • Cardiac catheterization
  • Chest x-ray
  • ECG
  • Echocardiogram (if done before birth, it is called a fetal echocardiogram)
  • Pulse oximetry (to check blood oxygen level)

 

Treatment

 

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 2 blood circulations.

A procedure using a long, thin flexible tube (balloon atrial septostomy) may be needed to create a large hole in the atrial septum to allow blood to mix.

A surgery called an arterial switch procedure is used to permanently correct the problem within the baby's first week of life. This surgery switches the great arteries back to the normal position and keeps the coronary arteries attached to the aorta. Prior to the development of this surgery, a surgery called an atrial switch (or Mustard procedure or Senning procedure) was used. There are still many people alive who had this surgery.

 

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, Carberry KE. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 19th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 59.

Kliegman RM, Stanton BF, St Geme JW, Schor NF. Cyanotic congenital heart lesions. 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.

 
  • 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

    A Closer Look

     

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        Tests for Transposition of the great vessels

         

           

          Review Date: 10/22/2015

          Reviewed By: Larry A. Weinrauch, MD, Assistant Professor of Medicine, Harvard Medical School, Cardiovascular Disease and Clinical Outcomes Research, Watertown, MA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

          The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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