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What is
Restrictive Cardiomyopathy?
Restrictive cardiomyopathy is the rarest of the cardiomyopathies. It is characterised by increased stiffness of the heart muscle, usually due
to scar tissue, which prevents adequate filling of the chambers of the heart.
The actual pumping action of the heart is usually normal.
The cause of scar tissue is unknown in the majority of cases. In some cases
it may be due to very rare conditions which cause infiltration of the heart,
infection and drugs. Hypertrophic cardiomyopathy may also behave in a similar
fashion.
In advanced cases, reduced filling of the left heart chamber causes a build up
of pressure and occasionally fluid in the lungs. Reduced filling of the right
heart causes a build up of pressure and fluid in the abdomen and legs.
Conditions that can cause
Restrictive Cardiomyopathy
-
Endomyocardial Fibrosis
- Infiltrative disorders: Amyloid
Sarcoid
Haemochromatosis
- Carcinoid Syndrome
- Systemic Sclerosis
- Radiotherapy to the chest
Symptoms
The main symptoms of restrictive
cardiomyopathy are breathlessness and ankle
swelling. In some cases the diseased heart may beat rapidly or with an abnormal
pattern resulting in palpitations. Occasionally the rhythm disturbance is severe
enough to cause "blackouts". Sudden death is a recognised feature.
Diagnosis
The diagnosis is confirmed with a combination of
ECG and Echocardiography. In
some cases the patient will undergo an invasive cardiac catheter study to
measure the pressures within various chambers of the heart.
Genetics
A familial form of the disease is recognised but it is not know how common
this is.
Treatment
There is no cure for restrictive
cardiomyopathy but a variety of drugs are
available to treat symptoms. Treatment is with diuretics and drugs which control disturbances of heart rhythm. Patients with severe heart failure, despite
medication, are assessed for heart transplantation.
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