Cardiomyopathy

Cardiomyopathy is a form of heart disease in which the heart is abnormally enlarged, thickened and/or stiffened.

This impairs the heart's ability to pump blood throughout the body effectively and can sometimes lead to heart failure, irregular heartbeats called arrhythmias, and blood clots. In children, cardiomyopathy is most common in infants under 1 year old.

There are two general types of cardiomyopathy: ischemic cardiomyopathy, which results from coronary artery disease, and nonischemic cardiomyopathy.

All cases of cardiomyopathy in children are considered nonischemic, of which there are four main types:

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  • Dilated Cardiomyopathy (DCM) — Also know as congestive cardiomyopathy, this is the most common form of the disease. It occurs when heart muscle tissue is stretched and enlarged, making it difficult for the heart to function and often causing congestive heart failure. For the most part, the cause of DCM in children remains unknown. It is sometimes caused by viruses such as Coxsackie B viruses, certain diseases, severe anemia or nutritional deficiencies, and rare pregnancy complications. Other cases may be genetic.
  • Hypertrophic Cardiomyopathy (HCM) — This is the second most common type of cardiomyopathy and results in excessive thickening of the heart walls. Blood flow is restricted as the heart chambers become smaller and stiffer. Most HCM is due to genetic factors.
  • Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) — This is a very rare condition, especially in children, where fatty tissue replaces heart muscle. ARVC is thought to be inherited and is usually found in teenagers or young adults.
  • Restrictive Cardiomyopathy (RCM) — This is the least common form of cardiomyopathy and is especially rare in children. RCM affects the ventricles — the lower chambers of the heart — restricting blood flow to the heart. The cause of RCM in children is not known.

Acquired Cardiomyopathy

Although many factors are associated with cardiomyopathy, the exact cause of the disease remains unknown. Many cases are genetic in nature, where the condition is inherited, while others are acquired.

The most common cause of acquired cardiomyopathy is a viral infection called myocarditis, which weakens the heart muscle. Other causes include:

  • Infectious diseases
  • Diseases that affect the immune system, such as HIV
  • Nutritional deficiencies or obesity
  • Exposure to toxins such as drugs, alcohol and radiation
  • Pregnancy-related complications

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Symptoms of cardiomyopathy vary. This is further complicated by the fact that infants and young children are often unable to express how they feel. That said, there are a number of symptoms associated with the different forms of cardiomyopathy:

  • Dilated Cardiomyopathy (DCM) — Symptoms in infants include difficulty breathing, poor appetite and slow weight gain. Older children may also have trouble being physically active and become excessively fatigued when exercising.
  • Hypertrophic Cardiomyopathy (HCM) — Infants with HCM often have trouble breathing, may sweat excessively and have a poor appetite. Older children may have shortness of breath, dizziness and chest pain. They also may faint or have trouble being physically active.
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In some cases, cardiomyopathy is diagnosed when a heart murmur is detected at a routine doctor visit, although children with cardiomyopathy do not always have a heart murmur. Another way of diagnosing cardiomyopathy is through special genetic screening, which may be performed if a family member has the condition. Unfortunately, since cardiomyopathy is hard to detect, many children are not diagnosed until the disease has progressed to the point that they have signs of heart failure.

A number of tests may be used to aid the diagnosis of cardiomyopathy as well as to determine the type of the disease. These include:

  • Echocardiogram — This test, which helps doctors determine the type of cardiomyopathy a child has as well as the level of heart dysfunction, uses sound waves to measure the heart's size and shape and creates an image of the heart.
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Children diagnosed with cardiomyopathy often require aggressive treatment specific to the type of cardiomyopathy they have and the extent of the damage to their hearts. The goal of treatment is to control or prevent congestive heart failure, improve heart function and prevent complications, such as arrhythmias and blood clots.

Treatment options include:

  • Drug Therapy — Medications may be used to treat the symptoms of cardiomyopathy. The specific drugs vary by the type of cardiomyopathy.
  • Implantation of a Pacemaker or Defibrillator — If drug therapy fails to alleviate heart obstructions or if your child has dangerous arrhythmias, a pacemaker or defibrillator may be inserted under the skin of the chest with wires that go through veins into the heart. The implantation is considered minor surgery requiring a short hospital stay. The pacemaker or defibrillator helps monitor and stabilize the heart.
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Reviewed by health care specialists at UCSF Benioff Children's Hospital.

Related Information

UCSF Clinics & Centers

Heart Center

Cardiac Intensive Care Unit
1975 Fourth St., Fourth Floor
San Francisco, CA 94158
Phone: (415) 353-1955
Fax: (415) 353-9144

Cardiothoracic Surgery Program
1825 Fourth St., Sixth Floor
San Francisco, CA 94158
Phone: (415) 476-3501
Fax: (415) 353-4144

Pediatric Heart Center Clinic at Mission Bay
1825 Fourth St., Sixth Floor
San Francisco, CA 94158
Phone: (415) 353-2008
Fax: (415) 353-4144
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