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| Restrictive Cardiomyopathy |
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| Restrictive cardiomyopathy is rare in the United States and
most other industrial nations. In this disease, the walls
of the ventricles stiffen and lose their flexibility due to
infiltration by abnormal tissue. As a result, the heart
cannot fill adequately with blood and eventually loses its
ability to pump properly.
Restrictive cardiomyopathy typically results from another
disease, which occurs elsewhere in the body. In the United
States, restrictive cardiomyopathy is most commonly related
to the following: amyloidosis, in which abnormal protein
fibers (amyloid) accumulate in the heart's walls; sarcoidosis, an inflammatory disease that causes the formation of small lumps in organs; and hemochromatosis, an iron overload of the body, usually due to a genetic disease.
In general, restrictive cardiomyopathy does not appear to be
inherited; however, some of the diseases that lead to the
condition are genetically transmitted.
Typical signs of the condition include symptoms of
congestive heart failure: weakness, fatigue, and
breathlessness. Swelling of the legs, caused by fluid
retention, occurs in a significant number of patients.
Other symptoms include nausea, bloating, and poor appetite,
probably because of the retention of fluid around the liver,
stomach, and intestines. |
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| Course of Disease |
| The condition is similar to dilated cardiomyopathy and tends
to worsen with time. Only about 30 percent of patients
survive more than 5 years after diagnosis. |
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| Treatment Regiment |
| Restrictive cadiomyopathy has no specific treatment. The underlying disease that leads to the heart problem also may not be treatable.
In general, the use of traditional heart drugs has been limited in this cardiomyopathy, although diuretics may help control fluid accumulation.
In rare cases, surgery is sometimes used to try to improve blood flow into the heart. |
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| Future Directions for Cardiomyopathy |
| Future advances in the diagnosis and treatment of
cardiomyopathy depend on a better understanding of the
disease process and why heart muscle is damaged. A lot of
research is under way to identify these processes and
whether they can be halted or even reversed. Much of the
research is conducted at or supported by the NHLBI.
Promising clues came from a team of NHLBI investigators that
discovered some of the genes responsible for hypertrophic
cardiomyopathy. Their work represents an important first
step in understanding how the disease is transmitted and how
it progresses.
Researchers also are trying to determine the best use of
currently available treatments, especially drug therapies.
Drugs useful for other conditions may help treat
cardiomyopathy. For example, drugs effective in treating
high blood pressure also help manage heart failure and
irregular heartbeats.
Additionally, much work has been--and continues to be--done
on identifying factors that increase or decrease a person's
risk of death in cardiomyopathy. Knowing which patients are
at the greatest risk is very important in determining the
best approach to the evaluation and treatment of their
condition.
The development of improved treatments for cardiomyopathy,
however, awaits still more research and a better
understanding of the disease process.
Source: National Institutes of Health |
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