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Cardiomyopathy (CMP)

Background: Cardiomyopathy is a form of heart disease in which the heart is enlarged (dilated CM), thickened (hypertrophic CM) or stiffened (restrictive CM). In CM the heart’s ability to effectively pump blood to the body is impaired and can lead to heart failure. There are many gene mutations associated with CMP. Family screening is recommended as there might be other members with undiagnosed CMP.

 

Dilated Cardiomyopathy:

The most common form is dilated cardiomyopathy. In most cases the cause of dilated CM remains unknown. It may be caused by viruses, metabolic diseases, infectious diseases, AIDS, obesity, nutritional deficiency, alcohol, radiation, chemotherapy, adverse drug reactions, carnitine-deficiency, severe anemia or it may be genetic (familial type).

Hypertrophic Cardiomyopathy:

The second most common type of cardiomyopathy. In this condition the heart walls (mainly the middle wall) become excessively thickened. Blood flow through the heart is restricted. Most cases of this type of cardiomyopathy are genetic in nature.

 

Arrhythmogenic Right Ventricular Dysplasia:

This is a very rare cardiomyopathy that usually manifests later on in life. It is a progressive condition where the heart muscle is replaced with fatty tissue. Patients may present with palpitations, syncope and ventricular tachycardia. Most cases are genetic in nature.

 

Restrictive Cardiomyopathy:

This is the least common form of cardiomyopathy. Many of these patients may end up with a heart transplant.

 

Symptoms:

Many patients with cardiomyopathy may experience arrhythmia (abnormal heart beats) which can be life threatening. Other symptoms may include palpitations, fatigue, difficulty breathing (especially during exercise), poor appetite, shaking, sweating, chest pain, fainting, upset stomach, failure to grow and others.

 

Cardiovascular Tests:

Some patients may need genetic testing, cardiac catheterization, MRI, heart biopsy and EP testing. Blood and urine testing is performed in all patients.

 

 

Treatment:

Many patients will require treatment with multiple heart medications. Some patients may require implantation of a pacemaker and/or defibrillator. Cardiomyopathy is one of the two leading reasons for heart transplantation in children.

 

The goal of treatment is to control or prevent heart failure and complications such as blood clots, arrhythmias and sudden death.

 

Growth failure is present in over one-third of patients. A diet rich in fruits, vegetables, whole grains, and fish with omega-3 fatty acids (tuna, salmon, trout) is beneficial in most patients. Nutritional supplements to consider include co-enzyme Q10, L-carnitine, and taurine (an amino acid).

 

Restrictions:

Most of the patients are restricted from any strenuous exercise and competitive sports. Cardiomyopathy is the leading cause of sudden cardiac death in pediatrics and young athletes.

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