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- $Unique_ID{BRK04294}
- $Pretitle{}
- $Title{Truncus Arteriosus, Persistent}
- $Subject{Truncus Arteriosus, Persistent}
- $Volume{}
- $Log{}
-
- Copyright (C) 1986 National Organization for Rare Disorders, Inc.
-
- 180:
- Truncus Arteriosus, Persistent
-
- General Discussion
-
- ** REMINDER **
- The information contained in the Rare Disease Database is provided for
- educational purposes only. It should not be used for diagnostic or treatment
- purposes. If you wish to obtain more information about this disorder, please
- contact your personal physician and/or the agencies listed in the "Resources"
- section of this report.
-
-
- The truncus arteriosus is a fetal structure which gives rise to the two
- large arteries emerging from the heart, the aorta and the pulmonary artery.
- When the truncus arteriosus persists beyond the fetal stage, blood from both
- ventricles mixes and enters the pulmonary, coronary (serving the heart
- muscle), and systemic (serving all the organs except the lungs and heart)
- circulation. Because not all the blood flowing to the body has passed through
- the lungs to absorb oxygen, the tissues receive less oxygen than they should.
- In addition, the pulmonary vasculature is eventually damaged by the
- abnormally high blood pressures in the lungs. Persistent Truncus Arteriosus
- is a serious congenital heart defect, and is always accompanied by a
- ventricular septal defect (i.e., a hole in the wall separating the right and
- left ventricles). The condition is often fatal during infancy.
-
- Symptoms
-
- The symptoms of Persistent Truncus Arteriosus resemble those of a severe
- ventricular septal defect. They consist of congestive heart failure,
- cyanosis (a bluish tint to the flesh due to insufficient oxygen supply), an
- enlarged heart, and gradual destruction of the blood vessels of the lungs due
- to high pulmonary blood pressure. Infants feed poorly and fail to grow and
- develop normally.
-
- Characteristic heart sounds, electrocardiographic findings, and blood
- pressure abnormalities help in making the diagnosis.
-
- Causes
-
- The arrest or abnormality in embryonic development leading to congenital
- heart defects such as Persistent Truncus Arteriosus may result from various
- factors. These may include maternal rubella (measles), excessive alcohol
- consumption, or diabetes; heredity may also play a role in some cases.
-
- Related Disorders
-
- Various congenital heart defects are discussed in the Rare Disease Database.
- Please note that Pseudotruncus Arteriosus is a synonym for Tetralogy of
- Fallot, a different heart defect. (For more information on this disorder,
- choose "Tetralogy of Fallot" as your search term in the Rare Disease
- Database.)
-
- Therapies: Standard
-
- Medical measures to avert heart failure are fairly standard, and are of
- limited value in Persistent Truncus Arteriosus. Surgery may be possible in
- some cases.
-
- Therapies: Investigational
-
- This disease entry is based upon medical information available through May
- 1990 . Since NORD's resources are limited, it is not possible to keep every
- entry in the Rare Disease Database completely current and accurate. Please
- check with the agencies listed in the Resources section for the most current
- information about this disorder.
-
- Resources
-
- For more information on Persistent Truncus Arteriosus, please contact:
-
- National Organization for Rare Disorders (NORD)
- P.O. Box 8923
- New Fairfield, CT 06812-1783
- (203) 746-6518
-
- American Heart Association
- 7320 Greenville Ave.
- Dallas, TX 75231
- (214) 750-5300
-
- NIH/National Heart, Lung and Blood Institute
- 9000 Rockville Pike
- Bethesda, MD 20892
- (301) 496-4236
-
- For information on genetics and genetic counseling referrals, please
- contact:
-
- March of Dimes Birth Defects Foundation
- 1275 Mamaroneck Avenue
- White Plains, NY 10605
- (914) 428-7100
-
- Alliance of Genetic Support Groups
- 35 Wisconsin Circle, Suite 440
- Chevy Chase, MD 20815
- (800) 336-GENE
- (301) 652-5553
-
- References
-
- THE CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden and Lloyd H.
- Smith, Jr., Eds.; W.B. Saunders Co., 1988. Pp. 308.
-
-