Patient
Information
Cardiomyopathy in Children and Adolescents
Cardiomyopathy is a very general term that means only that
there is a problem with the muscle of the heart. There are many
different types of cardiomyopathy. Although most people with CM
have either dilated cardiomyopathy or hypertrophic cardiomyopathy,
there are other very rare types of CM including ischemic CM
(related to coronary artery disease and heart attacks), peripartum
CM (following childbirth), restrictive CM (caused by stiff heart
muscle making the heart difficult to fill), and arrhythmogenic
right ventricle (a very thin right pumping chamber associated with
irregular heart rhythms). For the more rare types of CM, please
check the links listed below.
Dilated cardiomyopathy (DCM) is a disorder in which the heart
muscle is weak and thin, and the heart is quite enlarged. About
half the time no cause is identified for DCM, and in most other
cases it is thought to be the result of a prior virus infection.
Some rare disorders of the metabolism can also result in DCM, such
as carnatine deficiency and muscular dystrophy. DCM does not
generally run in families. The heart can become quite enlarged in
DCM. If CM is severe enough, heart can become quite enlarged. In
advanced cases, a patient can develop a syndrome called
"congestive heart failure". Signs and symptoms of CHF
can include poor exercise tolerance, shortness of breath, poor
feeding and poor weight gain, or even weight loss. In addition,
the liver can become enlarged and there can be swelling of the
legs, feet or other areas (edema). Advanced CHF can lead to
dizziness, fainting or even death. DCM can also be associated with
abnormal rhythms of the heart, resulting in palpitations or even
sudden death. Medications are often used for patients with CHF to
relieve symptoms, and in advanced cases heart transplant may even
be necessary.
Hypertrophic cardiomyopathy (HCM) is a disorder in which the
heart muscle is very thick, and can even create tight areas that
make it hard for the heart to pump the blood out to the body
(subaortic stenosis). Other names for HCM include "idiopathic
hypertrophic subaortic stenosis (IHSS) and hypertrophic
obstructive cardiomyopathy (HOCM). Most cases of HCM are inherited
from an affected parent (autosomal dominant inheritance), although
in many cases it occurs randomly when neither parent is affected.
HCM can be treated with medications (which are very different from
those used in DCM), and there is some evidence that medical
treatment, particularly with a type of medication called
"calcium channel blockers", may slow the progression of
the disease. There are also surgical procedures to relieve the
subaortic stenosis. Patients with HCM may also develop CHF and are
at risk of sudden death, presumably because of irregular heart
rhythms. It is sometimes necessary to consider heart transplant,
or implantation of a device to protect against irregular heart
rhythms (defibrillator). Children of a parent with HCM are at risk
to develop the disease as they get older, and should be screened
about every three years. Although there is some understanding of
the genetics of HCM, there are not yet easy tests that will
consistently show whether a child is at risk of developing HCM
later in life.
Any patient who is interested in pursuing the possibility of
genetic testing for HCM could contact one of the researchers doing
work in this area. I can recommend:
Dr. Christine Seidman, MD
Department of Genetics
Harvard Medical School
Alpert Room 533
200 Longwood Avenue
Boston, MA 02115
(617) 432-7838
http://www.hhmi.org/research/investigators/seidmance.html
Please have a look as some of the
web sites below. As always with the web, consider who wrote the
material before you take it too seriously.
Information about cardiomyopathy
American Heart Association
National Institutes of Health (NIH)
Mayo Clinic
The Cardiomyopathy Association
HeliosHealth
Hypertrophic cardiomyopathy (including IHSS and HOCM)
The Cardiomyopathy Association
Hypertrophic Cardiomyopathy Association
Dilated cardiomyopathy
The Cardiomyopathy Association
Familial Dilated Cardiomyopathy Project at Oregon Health
Sciences University
Peripartum Cardiomyopathy
iVillage
Arrhythmogenic right ventricle cardiomyopathy
The Cardiomyopathy Association
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