Restrictive Cardiomyopathy

Treatment Overview

In most cases, restrictive cardiomyopathy is a progressive disease in which the underlying cause of the disease is not known (idiopathic restrictive cardiomyopathy) and, therefore, 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 underlying 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.² In most of these cases, the condition is progressive, and treatment involves trying to manage heart failure symptoms.

However, in other cases where the underlying 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).

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.
• Vasodilators, to open, or dilate, the blood vessels. However, sometimes they can make symptoms worse.
• 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.²
• Digoxin, medicine that increase the strength of heart contractions. This medicine is sometimes used, although with caution in those people who have amyloidosis, because it can lead to serious arrhythmias.³

Surgery

An artificial pacemaker or a device called an implantable cardioverter defibrillator (ICD) may be surgically placed in the chest. Scar tissue that develops in restrictive cardiomyopathy may block electrical impulses travelling 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. ICDs are used to control life-threatening rapid heart rhythms. 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.

A heart transplant is available to a small number of people who have severe end-stage restrictive cardiomyopathy and 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 transplant may be used for amyloidosis (a buildup of protein), although the long-term benefits are not known.²

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Author:	Douglas Dana Robin Parks, MS	Last Updated: October 19, 2006
Medical Review:	Adam Husney, MD - Family Medicine Kathleen Romito, MD - Family Medicine Stephen Fort, MD, MRCP, FRCPC - Interventional Cardiology
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