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Causes
By Mayo Clinic staff
Truncus arteriosus occurs during fetal growth when your baby's heart is developing and is, therefore, present at birth (congenital). In most cases the cause is unknown. An overview of typical heart structure and function is helpful in understanding the defects of truncus arteriosus.
The heart
Your heart has four pumping chambers that circulate your blood. The "doors" of the chambers (valves) control the flow of blood, opening and closing to ensure that blood flows in a single direction.
The heart's four chambers are:
- The right atrium, the upper right chamber, receives oxygen-poor blood from your body and delivers it into the right ventricle.
- The right ventricle, the lower right chamber, pumps the blood through a large vessel called the pulmonary artery and into the lungs, where the blood is resupplied with oxygen.
- The left atrium, the upper left chamber, receives the oxygen-rich blood from the lungs and delivers it into the left ventricle.
- The left ventricle, the lower left chamber, pumps the oxygen-rich blood through a large vessel called the aorta and on to the rest of the body.
Normal heart development
The formation of the fetal heart is a complex process. At a certain point, all fetuses have a single large vessel (truncus arteriosus) exiting the heart. During normal development of the heart, however, this very large single vessel divides into two parts. One part becomes the lower portion of the aorta, which is attached to the left ventricle. The other part becomes the lower portion of the pulmonary artery, which is attached to the right ventricle.
Also during this process, the ventricles develop into two chambers separated by a wall (septum).
Truncus arteriosus in newborns
In babies born with truncus arteriosus, the single large vessel never finished dividing into two separate vessels. And the wall separating the two ventricles never closed completely, resulting in a large hole between the two chambers (ventricular septal defect).
Truncus arteriosus in a newborn is also called persistent truncus arteriosus, because an early stage of heart development persisted until birth.
In addition to the primary defects of truncus arteriosus, the valve controlling blood flow from the ventricles to the singe large vessel (truncal valve) is often defective, allowing blood to flow backward into the heart.
- 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.
- 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.
- 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.
- 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.
- Truncus arteriosus. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=11073. Accessed April 16, 2010.
- Congenital heart defects. March of Dimes. http://www.marchofdimes.com/professionals/14332_1212.asp. Accessed April 16, 2010.
- Koenig PR, et al. Congenital coronary artery abnormalities. http://www.uptodate.com/home/index.html. Accessed April 6, 2010.
- Sullivan KE. Chromosome 22q11.2 deletion syndrome: DiGeorge syndrome/velocardiofacial syndrome. Immunology and Allergy Clinics of North America. 2008;28:353.
- 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.
- 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.
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