Cardiomyopathy | Diagnosis and Treatment

LIke ThisLIke ThisLIke ThisLIke ThisLIke This

Contact the Cardiomyopathy Program

How is cardiomyopathy diagnosed?

Because the symptoms of cardiomyopathy can be diverse, it is often misdiagnosed as asthma, an infection or a gastrointestinal problem.

In children who have no symptoms, cardiomyopathy is sometimes diagnosed when the child has a chest x-ray that shows an enlarged heart or an echocardiogram for another reason.

If your child’s doctor suspects cardiomyopathy, he or she may order one or more of the following tests:

Erin, who was born with cardiomyopathy celebrates her 20 years since her heart transplant.

What are the treatment options for cardiomyopathy?

Your child's treatment options will be determined by the type of cardiomyopathy he or she has, as well as the specific symptoms. A child with no symptoms might not need medication or other treatment right away. Instead, the cardiologist will monitor your child to gauge the 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 and the rest of the body. .

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

Medication for cardiomyopathy

There are several different types of medications for cardiomyopathy, depending on which type your child has and the symptoms.

  • Angiotestin converting enzyme (ACE) inhibitors are drugs that dilate blood vessels in the body, fighting the constricting effect caused by heart failure.
  • Antiarrhythmic medications combat the abnormal heart rhythms caused by irregular electrical activity within the heart. 
  • Beta blockers block certain chemicals from binding to nerve receptors in the heart, slowing the heart rate and lowering blood pressure.  
  • Blood thinners or anticoagulants help prevent the formation of blood clots, especially in children with the dilated form of cardiomyopathy. 
  • Diuretics prevent the buildup of fluid in the body and can help breathing by reducing fluid in the lungs. These drugs may also be helpful in treating scar tissue on the heart.

Surgical treatments for cardiomyopathy

There are several options for treating cardiomyopathy using surgery or minimally-invasive procedures.

Defibrillators

For some children with cardiomyopathy — particularly HCM — an implantable cardioverter-defibrillator (ICD) can be a life-saving option. This tiny instrument, about the size of a deck of cards, is placed in the chest to monitor the child's heartbeat. If the child has an arrhythmia, the defibrillator will administer a precise electrical pulse to restore normal heart rhythm.

Pacemakers

Pacemaker implantation is 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 cardiomyopathy due to arrhythmia. A small, needle-like probe is inserted into the scarred tissue of the heart muscle, sending out radiofrequency waves that burns away the scar tissue and the arrhythmia.

Surgical removal of some heart muscle

In serious cases of hypertrophic cardiomyopathy, the treatment team may 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 have improved blood flow throughout the heart and body.

Ventricular assist device

In certain types of cardiomyopathy, a ventricular-assist device (VAD) can be used to help recover the heart and normalize the heart’s function. After the heart’s function has returned to normal, the VAD will be removed and the child will be monitored for any further heart problems. This approach using the VAD is called a “bridge to recovery.”

Heart transplant

Children with the most severe cases of cardiomyopathy may need a heart transplant if other methods don’t manage symptoms. While your child is waiting for an available heart, a VAD may be used to support the heart. In many cases, children can return to school and other activities while waiting for a transplant.

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

Boston Children's Hospital
300 Longwood Avenue, Boston, MA 02115
For Patients: 617-355-6000
For Referring Providers: 844-BCH-PEDS | 844-224-7337

Close