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What is Restrictive Cardiomyopathy?

Restrictive cardiomyopathy refers to a rare form of cardiomyopathy where the lower chambers of the heart do not fill up with blood properly due to abnormal stiffness of the walls. This condition may affect one or both ventricles or lower chambers of the heart. While the pumping action of the heart (systolic action) is normal, the diastolic action of filling the chambers with blood is compromised to some extent. The heart cannot relax during heartbeats and this abnormal condition can affect the functioning of the lungs, liver, and other important organs of the body.

In time, the heart may not be able to pump blood as well and the condition may result in heart failure that could be fatal.

Symptoms

Symptoms of heart problems tend to develop over a period of time and may not be evident until much later. In some cases however, symptoms could occur suddenly and cause severe reactions in the body. Some of the common signs and symptoms of restrictive cardiomyopathy include:

• Chronic cough
• Breathing difficulties that are aggravated when lying flat, during the night or after exertion
• Exhaustion and fatigue
• Inability to exercise for long periods of time or at all
• Loss of appetite
• Bloating and nausea
• Swollen abdomen
• Swollen ankles and feet
• Palpitations or fluttering in the chest caused by abnormal heart rhythms
• Low frequency of urination
• Excessive need to urinate at night (in adults)
• Dramatic decrease in blood pressure (shock)
• Weight gain
• Enlarged liver
• Bulging veins in the neck
• Children may have feeding difficulties, pale skin, poor growth, and a weak pulse
• Less common symptoms include fainting and pressure or pain in the chest area

Causes

The exact causes of restrictive cardiomyopathy remain largely unknown.

One fact that is confirmed is that the condition is definitely not inherited. The possible causes of restrictive cardiomyopathy are:

• Amyloidosis (build up of abnormal proteins in the heart muscle)
• Scarring of the heart (often for no known reason)
• Heart transplant
• Carcinoid heart disease
• Hemochromatosis or iron overload
• Radiation fibrosis
• Chemotherapy
• Endomyocardial fibrosis and other diseases of the heart lining
• Sarcoidosis
• Scleroderma
• Heart tumor

Treatment

Once the cause of restrictive cardiomyopathy is diagnosed, necessary treatment for the same can be prescribed. However, there is no known cure for restrictive cardiomyopathy and most treatments are aimed at controlling symptoms and improving the overall quality of a patient’s life. The following treatments may be prescribed:

• Blood thinning medications such as aspirin or warfarin
• Medications such as beta-blockers or ACE inhibitors
• Diuretics to prevent water retention and improve other symptoms such as breathing difficulties
• If symptoms continue to occur, digoxin and aldosterone inhibitors may be added
• Steroids
• Medications for irregular heart rhythms
• In some cases, chemotherapy may be required
• Therapy may also be given to treat underlying causes such as sarcoidosis, amyloidosis, and hemochromatosis
• If the functioning of the heart is very poor, a heart transplant may be considered.

The following lifestyle changes are also recommended:

• Reduction of sodium to 2,000 milligrams to 3,000 milligrams per day to relieve symptoms such as shortness of breath or exhaustion
• Exercise is normally good for the heart. However, your doctor will advise you what type of exercises you should do to prevent fatigue and shortness of breath. Walking or swimming are safe exercise options but be sure to start off slowly. It is recommended that you take short breaks often while exercising and build up your endurance slowly. Lifting heavy weights is not advised.

References

• http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001241/