Restrictive Cardiomyopathy

Treatment Overview

In most cases, restrictive cardiomyopathy is a progressive disease in which the cause of the disease is not known (idiopathic restrictive cardiomyopathy) and so cannot be directly treated. Typically, the heart muscle continues to stiffen and lose function and strength, and heart failure (inability of the heart to pump enough blood) develops. In these cases, treatment involves trying to decrease the heart's workload.

In some cases the cause of restrictive cardiomyopathy can be identified, such as in carcinoid syndrome, sarcoidosis, and amyloidosis, although the treatment is generally ineffective. Corticosteroids may offer limited benefit in treating sarcoidosis and amyloidosis. In most of these cases, the condition is progressive, and treatment involves trying to manage heart failure symptoms.

But in other cases where the cause is diagnosed early and can be treated, further heart damage may be prevented. For example, in restrictive cardiomyopathy caused by hemochromatosis (a buildup of iron in the heart muscle), treatment may involve the use of drugs that help eliminate excess iron (chelating agents) and/or phlebotomy (withdrawing blood). In another example, an inherited problem called Fabry's disease can be treated to prevent restrictive cardiomyopathy from happening.

If heart failure has developed, your doctor will prescribe medicines to manage its symptoms and complications. They may include:

• Diuretics, which help eliminate fluid buildup in the lungs and elsewhere in the body.
• Angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs) to improve blood flow and reduce the heart's workload. ARBs may be used when a person cannot tolerate ACE inhibitors or when ACE inhibitors are not controlling symptoms.
• Beta-blockers, which slow the heart rate and reduce blood pressure.
• Anticoagulants, to help prevent blood clots from forming in the heart. People with restrictive cardiomyopathy, especially those with atrial fibrillation, are at risk for developing clots, which can travel through the bloodstream to other places in the body and cause a heart attack, pulmonary embolism, or stroke.
• Digoxin, which can help increase the strength of heart contractions. These medicines are sometimes used, although with caution in those people who have amyloidosis, because they can lead to serious arrhythmias.
• Calcium channel blockers, which slow your heart rate and lower blood pressure. These medicines may be used to treat diastolic heart failure, when the heart has problems filling with blood.

Surgery

An artificial pacemaker may be surgically placed in the chest. Scar tissue that develops in restrictive cardiomyopathy may block electrical impulses traveling through the heart and result in abnormal heartbeats, called arrhythmias. Pacemakers stimulate the heart muscle to beat regularly when the electrical signals from the top of the heart are blocked.

An implantable cardioverter-defibrillator (ICD) is another small device that may be surgically placed in the chest. It is used to lower the risk of sudden death from life-threatening irregular heart rhythms (arrhythmias). An ICD continuously monitors your heart. If it detects a life-threatening rapid heart rhythm, it sends an electric shock to your heart to restore a normal rhythm. You may need an ICD if you have had a serious episode of life-threatening irregular heart rhythm or are at high risk for having one.

A heart transplant is available to a small number of people who have severe end-stage restrictive cardiomyopathy and who meet specific criteria for transplantation. The diseased heart is removed and replaced with a healthy heart donated by a person who has recently died. There are limited donor hearts available.

Stem cell transplantation may be used for amyloidosis (a buildup of protein), although the long-term benefits are not known.

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Author:	Robin Parks, MS	Last Updated: August 26, 2008
Medical Review:	Caroline S. Rhoads, MD - Internal Medicine Stephen Fort, MD, MRCP, FRCPC - Interventional Cardiology
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