Conditions + Treatments

Cardiomyopathy | Treatments

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Contact the Cardiomyopathy Program

Boston Children's Hospital cardiologists, cardiac surgeons and nurses, cardiac imaging professionals and other clinicians have years of expertise in treating not only the multiple forms of cardiomyopathy in children, but also the various stages of the disease.

Your child's exact course of treatment will be determined by the type of cardiomyopathy she has, her specific symptoms and your family's preferences.

A child with virtually no symptoms might be able to continue without medication or further procedures for the time being, checking in with the cardiologist at least every six months for monitoring and to gauge the likely progression of the disease.

A child with more serious symptoms may need additional tests to give the treatment team more detailed information about how the cardiomyopathy is affecting the heart.

Many children with dilated cardiomyopathy do very well with medication alone. Others need surgery to improve the function of valves that may leak as a result of dilated cardiomyopathy, and in the most severe cases, some children need a heart transplant.

Medication for cardiomyopathy

  • ACE inhibitors– drugs that dilate blood vessels in the body, fighting the constricting effect caused by heart failure. ACE stands for “angiotestin converting enzyme.” Common examples of ACE inhibitors include:
    • benazepril (brand name: Lotensin)
    • captopril (brand name Capoten)
    • enalapril (brand name: Vasotec)
    • moexipril (brand name: Univasc)
  • antiarrhythmic medications– drugs that combat the abnormal heart rhythms caused by irregular electrical activity within the heart. Common examples are:
    • flecainide (brand name: Tambocor)
    • procainamide (brand name: Procanbid)
    • amiodarone (brand name: Codarone)
    • sotalol (brand name: Betapace)
  • beta blockers– drugs that block certain chemicals from binding to nerve receptors in the heart. This slows the heart rate and lowers blood pressure. Common examples are:
    • propranolol (brand name: Inderal)
    • atenolol (brand name: Tenormin)
    • metoprolol (brand names: Lopressor, Toprol XL)
    • carvedilol (brand name: Coreg)
  • blood thinners, or anticoagulants– drugs that help prevent the formation of blood clots, especially in children with the dilated form of cardiomyopathy. Common examples are:
    • aspirin
    • Coumadin
    • Warfarin
  • diuretics– drugs that cause increased urination, preventing the buildup of fluid in the body. Diuretics aid breathing by reducing fluid in the lungs, and may also be helpful in treating scar tissue on the heart. Common examples include:
    • bumetanide (Bumex)
    • furosemide (Lasix)

Surgical treatments for cardiomyopathy

  • the implantation of a pacemaker
  • removal of part of the enlarged heart muscle
  • heart transplant

Defibrillators

For some children with cardiomyopathy—particularly the hypertrophic form—an implantable cardioverter-defibrillator (ICD) can be a life-saving implement. This tiny instrument, only about the size of a pager, is implanted in the chest, where it monitors the child's heartbeat. In the event of an arrhythmia, the defibrillator will administer a precise electrical pulse to restore normal heart rhythm.

Pacemakers

Pacemaker implantation can be a helpful, minimally invasive option for some children with cardiomyopathy. The pacemaker—a small electronic device—is inserted directly under the skin, where it sends electric signals to the child's heart, controlling and monitoring the heart rate. The procedure can be performed under local anesthesia in a matter of hours.

Radiofrequency ablation

Radiofrequency ablation is another type of minimally invasive treatment that can be very effective for some children with the arrhythmogenic right ventricular form of cardiomyopathy. A small, needle-like probe is inserted into the scarred tissue of the heart muscle, sending out radiofrequency waves that burn away the scarring.

Surgical removal of some heart muscle

In serious cases of hypertrophic cardiomyopathy, the treatment team may elect to perform a surgery called a septal myectomy or septal myomectomy. This procedure involves the removal of a portion of the thickened muscle in the heart, widening the channel in the heart's ventricle that leads to the aortic valve. The procedure has a very good success rate, and most children experience improved blood flow throughout the heart and body.

Heart transplant

Children with the most severe cases of cardiomyopathy—in any of the forms of the condition—may need a heart transplant if other methods prove insufficient in managing their symptoms. Given the shortage of donor organs across the country, your child may have to wait for weeks or months for an available heart.

During the waiting period, a ventricular-assist device (VAD) may be used to support the heart and allow the patient to get stronger.  In many cases, patients can return to school and other activities.

Learn more about Boston Children's Heart Transplant Program.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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