Truncus arteriosus

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Treatments and drugs

By Mayo Clinic staff

Surgery can often repair the heart of an infant with truncus arteriosus. Sometimes multiple procedures or surgeries will be necessary. Before your baby undergoes surgery, he or she may receive more frequent feedings to ensure proper nutrition and medications to improve heart and lung function.

Medications
Medications that may be prescribed prior to surgery may include:

  • Digoxin (Lanoxin). This drug, also referred to as digitalis, increases the strength of heart muscle contractions.
  • Diuretics. Often called water pills, diuretics increase the frequency and volume of urination, preventing fluid from collecting in the body — a common effect of heart failure.

Surgical procedures
Most infants with truncus arteriosus have surgery within the first two months of life. The exact procedure will depend on your baby's condition. Most commonly your baby's surgeon will:

  • Close the hole between the two ventricles, often with a patch
  • Separate the upper portion of the pulmonary artery from the single large vessel
  • Implant a tube (conduit) and valve to connect the right ventricle with the upper portion of the pulmonary artery — creating a new, complete pulmonary artery
  • Reconstruct the single large vessel and aorta to create a new, complete aorta
  • Implant a new valve separating the left ventricle and aorta if necessary

After corrective surgery, your child will need lifelong follow-up care with a cardiologist to monitor his or her heart health. The cardiologist may recommend that your child limit physical activity, particularly intense competitive sports. Your child will need to take antibiotics before dental procedures and other surgical procedures to prevent infections.

Because the conduit does not grow with your child, one or more follow-up surgeries to replace the conduit are usually necessary as he or she gets older. Newer surgical procedures that use a cardiac catheter inserted into a blood vessel in the leg that are then threaded slowly up to the heart are being developed to replace the conduit without the need for traditional heart surgery. In addition, cardiac catheterization with an inflatable balloon tip can be used to open up an obstructed or narrowed artery, which may delay the need for follow-up surgery.

Pregnancy
Women who've had surgery to repair truncus arteriosus in infancy need to be evaluated by a cardiologist with expertise in adult congenital heart defects and an obstetrician specializing in high-risk pregnancies before attempting to become pregnant. Depending on the level of lung damage that occurred before surgery, pregnancy may or may not be recommended. In addition, some drugs taken for heart problems can be harmful to a fetus.

References
  1. Hirsh JC, et al. Congenital heart disease. In: Doherty GM. Current Diagnosis and Treatment: Surgery. 13th ed. New York, N.Y.: McGraw-Hill; 2010. http://www.accessmedicine.com/content.aspx?aID=5215009. Accessed April 16, 2010.
  2. Specific cardiac defects. In: Libby P, et al. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 8th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/book/player/book.do?method=display&type=bookPage&decorator=header&eid=4-u1.0-B978-1-4160-4106-1..50064-9--cesec197&displayedEid=4-u1.0-B978-1-4160-4106-1..50064-9--cesec319&uniq=195744492&isbn=978-1-4160-4106-1&sid=984783871. Accessed April 16, 2010.
  3. Persistent truncus arteriosus. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/print/sec19/ch287/ch287k.html. Accessed April 16, 2010.
  4. Sondheimer HM, et al. Cardiovascular diseases. In: Hay WW, et al. Current Diagnosis and Treatment: Pediatrics. 19th ed. New York, N.Y.: McGraw-Hill Medical; 2009. http://www.accessmedicine.com/content.aspx?aID=3402846. Accessed April 16, 2010.
  5. Truncus arteriosus. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=11073. Accessed April 16, 2010.
  6. Congenital heart defects. March of Dimes. http://www.marchofdimes.com/professionals/14332_1212.asp. Accessed April 16, 2010.
  7. Koenig PR, et al. Congenital coronary artery abnormalities. http://www.uptodate.com/home/index.html. Accessed April 6, 2010.
  8. Sullivan KE. Chromosome 22q11.2 deletion syndrome: DiGeorge syndrome/velocardiofacial syndrome. Immunology and Allergy Clinics of North America. 2008;28:353.
  9. Cyanotic congenital heart disease: Lesions associated with increased pulmonary blood flow. In: Kliegman MD, et al. Nelson's Textbook of Pediatrics. 18th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/195744492-3/0/1608/1010.html?tocnode=54483014&fromURL=1010.html#4-u1.0-B978-1-4160-2450-7..50433-3_8277. Accessed April 16, 2010.
  10. Verhaert D, et al. Truncus arteriosus with aortic arch interruption: Cardiovascular magnetic resonance findings in the unrepaired adult. Journal of Cardiovascular Magnetic Resonance. 2010;12:16.
DS00746 July 13, 2010

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