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Cardiomyopathy
What is
cardiomyopathy?
Cardiomyopathy is a
serious disease in which the heart muscle becomes
inflamed and doesn't work as well as it should. There
may be multiple causes including viral infections.
Cardiomyopathy can be
classified as primary or secondary. Primary
cardiomyopathy can't be attributed to a specific cause,
such as high blood pressure, heart valve disease, artery
diseases or congenital heart defects. Secondary
cardiomyopathy is due to specific causes. It's often
associated with diseases involving other organs as well
as the heart.
There are three main
types of cardiomyopathy: dilated, hypertrophic and
restrictive.
What is dilated (congestive)
cardiomyopathy?
This
is the most common form. In it, the heart cavity is
enlarged and stretched (cardiac dilation). The heart is
weak and doesn't pump normally, and most patients
develop heart failure. Abnormal heart rhythms called
arrhythmias and disturbances in the heart's electrical
conduction also may occur.
Blood flows more slowly
through an enlarged heart, so blood clots may form.
A blood clot that forms in an artery or the
heart is called a thrombus. A clot that breaks
free, circulates in the bloodstream and blocks a small
blood vessel is called an embolus.
- Clots that stick to the inner lining of the heart
are called mural thrombi.
- If the clot breaks off the right ventricle
(pumping chamber), it can be carried into the
pulmonary circulation in the lung, forming pulmonary
emboli.
- Blood clots that form in the heart's left
side may be dislodged and carried into the body's
circulation to form cerebral emboli in the brain,
renal emboli in the kidney, peripheral emboli or even
coronary artery emboli.
A condition known as Barth
syndrome, a rare and relatively unknown genetically
linked cardiac disease, can cause dilated
cardiomyopathy. This syndrome affects male children,
usually during their first year of life. It can also be
diagnosed later. (For more
information on Barth syndrome, visit the Barth Syndrome
Foundation at http://www.barthsyndrome.org.
This link is provided for convenience only,
and is not an endorsement or assurance of the entity or
any product or service.)
In these young patients the
heart condition is often associated with changes in the
skeletal muscles, short stature and an increased
likelihood of catching bacterial infections. They also
have neutropenia, which is a decrease in the number
of white blood cells known as neutrophils. There are
clinical signs of the cardiomyopathy in the newborn
child or within the first months of life. These
children also have metabolic and mitochondrial
abnormalities.
How is dilated (congestive) cardiomyopathy
treated?
A person
with cardiomyopathy may suffer an embolus before any
other symptom of cardiomyopathy appears. That's
why anti-clotting (anticoagulant) drug therapy may
be needed. Arrhythmias may require antiarrhythmic
drugs. Therapy for dilated cardiomyopathy is often
aimed at treating the underlying cause, however. If the
person is young and otherwise healthy, and if the
disease gets worse, a heart transplant may be
considered.
When cardiomyopathy results
in a significantly enlarged heart, the mitral and
tricuspid valves may not be able to close properly,
resulting in murmurs. Blood pressure may increase
because of increased sympathetic nerve activity. These
nerves can also cause arteries to narrow. This mimics
hypertensive heart disease (high blood pressure). That's
why some people have high blood pressure readings.
Because the blood pressure determines the heart's
workload and oxygen needs, one treatment approach is to
use vasodilators (drugs that "relax" the arteries). They
lower blood pressure and thus the left ventricle's
workload.
What is hypertrophic
cardiomyopathy?
In
this condition, the muscle mass of the left ventricle
enlarges or "hypertrophies."
In one form of the disease,
the wall (septum) between the two
ventricles (pumping chamber) becomes enlarged and
obstructs the blood flow from the left ventricle. The
syndrome is known as hypertrophic obstructive
cardiomyopathy (H.O.C.M.) or asymmetric septal
hypertrophy (A.S.H.). It's also called idiopathic
hypertrophic subaortic stenosis (I.H.S.S.).
Besides obstructing blood
flow, the thickened wall sometimes distorts one leaflet
of the mitral valve, causing it to leak. Hypertrophic
cardiomyopathy is the most common inherited heart
defect, occurring in one of 500
individuals. Close blood relatives (parents,
children or siblings) of such persons often have
enlarged septums, although they may have no symptoms.
This disease is most common in young adults.
In the other form of the
disease, non-obstructive hypertrophic cardiomyopathy,
the enlarged muscle doesn't obstruct blood flow.
The symptoms of hypertrophic
cardiomyopathy include shortness of breath on exertion,
dizziness, fainting and angina pectoris. (Angina
is chest pain or discomfort caused by reduced blood
supply to the heart muscle.) Some people have cardiac
arrhythmias. These are abnormal heart rhythms that
in some cases can lead to sudden death. Often an
implanted cardioverter defibrillator (ICD) is needed to
shock the heart to restart a normal heart rhythm and
prevent sudden dealth. The obstruction to blood flow
from the left ventricle increases the ventricle's work,
and a heart murmur may be heard.
How is hypertrophic cardiomyopathy
treated?
The usual
treatment involves taking a drug known as a beta
blocker (such as propranolol) or a calcium channel
blocker. If a person has an arrhythmia, an
antiarrhythmic drug may also be used. Surgical treatment
of the obstructive form is possible in some cases if the
drug treatment fails.
Alcohol ablation is a type
of nonsurgical treatment for hypertrophic obstructive
cardiomyopathy. It involves injecting alcohol down a
small branch of one of the heart arteries to deaden the
extra heart muscle. This allows the extra
heart muscle to thin out without having to cut it
out surgically.
What is restrictive
cardiomyopathy?
This
is the least common type in the United States. The
myocardium (heart muscle) of the ventricles becomes
excessively "rigid," so it's harder for the ventricles
to fill with blood between heartbeats. A person with
restrictive cardiomyopathy often complains of being
tired, may have swollen hands and feet, and may have
difficulty breathing on exertion. This type of
cardiomyopathy is usually seen in the elderly and may be
due to another disease process.

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