home
***
CD-ROM
|
disk
|
FTP
|
other
***
search
/
CD-ROM Today 1996 January
/
CD-ROM Today 1996 January.iso
/
dp
/
0385
/
03854.txt
< prev
next >
Wrap
Text File
|
1994-01-17
|
4KB
|
107 lines
$Unique_ID{BRK03854}
$Pretitle{}
$Title{Hypoplastic Left Heart Syndrome}
$Subject{Hypoplastic Left Heart Syndrome Aortic Atresia }
$Volume{}
$Log{}
Copyright (C) 1986, 1993 National Organization for Rare Disorders, Inc.
178:
Hypoplastic Left Heart Syndrome
** IMPORTANT **
It is possible the main title of the article (Hypoplastic Left Heart
Syndrome) is not the name you expected. Please check the SYNONYMS listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
Aortic Atresia
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.
Hypoplastic left heart syndrome is the result of a rare congenital heart
defect characterized by the failure of the left side of the heart to develop
sufficiently. The left atrium and ventricle are small, the valves connecting
the chambers to each other and to the aorta are narrowed, and the aorta is
abnormally constructed.
Symptoms
The normal heart is divided into four chambers. The blood flows from the
systemic circulation (i.e., all the tissues except the lungs) into the right
atrium, and from there into the right ventricle which pumps the blood to the
lungs. In the lungs, the blood absorbs the oxygen it will carry to the body
tissues. It then flows to the left atrium, and past the mitral valve into
the left ventricle. The strong muscle in the walls of the left ventricle
then force the blood past the aortic valve into the aorta, which delivers the
blood to the systemic arteries.
In Hypoplastic Left Heart Syndrome, the left side of the heart is not
developed normally at birth, and the heart cannot pump enough blood from the
lungs to the systemic circulation. Blood accumulates in the lungs, and
insufficient oxygen reaches the organs and tissues of the body. Persistent
heart failure results in difficulty breathing and blue colored skin
(cyanosis).
Causes
The arrest or abnormality in embryonic development that causes congenital
heart defects such as left heart hypoplasia syndrome may result from various
conditions including maternal rubella (measles), excessive alcohol intake, or
diabetes. Generally, the causes of these disorders are unknown. Rarely,
hereditary factors play a role.
Therapies: Standard
Treatment for Hypoplastic Left Heart Syndrome may consist of surgical
intervention. A neonatal cardiologist is best qualified to give parents an
opinion as to the type of treatment which best suits their child. Heart
transplantation is another type of therapy used in the treatment of
Hypoplastic Left Heart Syndrome.
Therapies: Investigational
This disease entry is based upon medical information available through March
1993. 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 Hypoplastic Left Heart Syndrome, 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 (NHLBI)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4236
References
THE CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B Wyngaarden and Lloyd H.
Smith, Jr., Eds; W.B. Saunders Co. 1988. P. 303.
THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987. Pp. 420-22.