Dilated Cardiomyopathy

Dilated cardiomyopathy in children

Dilated cardiomyopathy (DCM) occurs when heart muscle cells are abnormal or damaged. Overall, it is quite rare in children. 

The normal heart is a four-chamber pump whose beat is controlled by the heart’s electrical system. The heart walls are made of muscle cells that respond to the heart’s electrical impulse by briskly contracting (shortening). When the cells all contract together, the blood is pumped forward. In cardiomyopathy, the abnormal heart muscle cells prevent the heart from pumping or filling normally. The abnormal cells can also be a source of abnormal heart rhythms, called arrhythmias (see below).

Causes of dilated cardiomyopathy in children

In many cases, DCM is genetic, that is, passed down from parent to child. Other less frequent causes include:

  • infection or inflammation of the heart (myocarditis)
  • endocrine (gland) problems
  • metabolic diseases
  • some chemotherapy drugs
  • muscular dystrophy 
  • electrical conduction abnormalities

In many children, the exact cause cannot be identified.

How does dilated cardiomyopathy affect my child's health?

The health effects of DCM vary widely. In mild cases, there may be no symptoms or symptoms only with exercise. In more severe cases, symptoms may be present even at rest. DCM can also be associated with abnormal heart rhythms (atrial flutter, atrial fibrillation, heart block, or ventricular tachycardia) and a risk for sudden cardiac arrest.

The course of the disease varies greatly. Most DCM patients can see stabilization or improvement in heart function with medications while some patients’ condition may progress and require more intensive medical treatment, a mechanical heart pump, and heart transplantation.

How is this problem diagnosed?

Symptoms: Possible symptoms of heart failure due to DCM include:

  • abdominal pain
  • poor appetite
  • nausea
  • vomiting
  • difficulty breathing
  • low energy levels
  • activity intolerance
  • weight loss or poor weight gain
  • heart palpitations (heart racing or "skipping" heart beats)
  • fainting

Older children may develop swelling around the eyes, ankles/feet and abdomen.

Physical findings: The exam depends on the severity of DCM. If mild, the exam may be normal. If there is congestive heart failure, the heart rate and breathing rate are often fast. There may be a heart murmur (made by abnormal backward flow through the heart valves) or other extra heart sounds and the liver is often enlarged.

Medical tests: An echocardiogram is the main test used to make the diagnosis and to assess the severity of the problem. Blood work is often done. Other heart tests include an electrocardiogram (ECG) and Holter monitor to assess for arrhythmias. On chest x-ray, the heart is usually enlarged. Sometimes a heart catheterization is performed to measure pressures within the heart and to guide therapies.

How is the problem treated?

In most cases, several different forms of anti-heart failure medications that can help the heart function improve will be prescribed. 

Diuretic medicines may be prescribed if there are significant symptoms. These medicines help your body get rid of extra fluid. Blood thinners may also be used to prevent clots from forming within the heart. Rapid heart rhythms are treated by heart medicines and, if life-threatening, an implanted cardioverter-defibrillator (ICD) may be considered. 

If symptoms worsen or become severe, your child will be referred for evaluation by a heart failure specialist and advanced therapies such as intravenous medications, mechanical heart pumps (ventricular assist devices), and heart transplantation may be considered. 

What are the long-term health issues for my child?

If diagnosed with DCM, your child will require lifelong follow-up and cardiology care. The long-term health effects vary and depend on the degree of heart damage and the rate of disease progression. Fortunately, improving medical management of heart failure has resulted in better outcomes for these children. With rapidly advancing therapies, children with the severest forms of DCM can be well-supported and have a high likelihood of survival. 

Medication compliance: It is critical for your child to take anti-heart failure medications as prescribed. Patients that stop taking their medications may relapse and have worsened heart function. 

Nutrition: Maintaining a healthy diet is very important in DCM. Our specialized dietician will work closely with your family to ensure adequate nutrition and growth. 

Exercise guidelines: An active lifestyle is associated with better outcomes in DCM. An individualized exercise guideline for your child will be developed and discussed. Children with significant DCM are often not allowed to play competitive sports. However, children are usually encouraged to participate in recreational sports and gym class but should be allowed to self-limit their level of exertion.

Why choose C.S. Mott Children’s Hospital

The Congenital Heart Center at University of Michigan Health C.S. Mott Children’s Hospital has developed a team-based approach to care for patients and families affected by DCM and other inherited cardiomyopathies. The team includes cardiologists who specialize in cardiomyopathy, heart failure and rhythm disorders among other subspecialties. The team also includes a nurse practitioner, genetic counselor, social worker and child life specialist. Counselors familiar with the challenges of an DCM diagnosis are also available for support.

childrens cardiomyopathy foundation logo

Mott is a Children’s Cardiomyopathy Foundation (CCF) accredited center of care. CCF recognizes centers that manage a high volume of pediatric cardiomyopathy patients, offer a variety of pediatric patient services, specialize in the treatment and management of cardiomyopathy in children, and are affiliated with an academic research institution. We participate in a variety of research projects aimed toward improving the health outcomes and quality of life for children and families with cardiomyopathy.

Transition to adult care

DCM is a progressive condition that is more common in adults. For this reason, we work very closely with our colleagues in adult cardiology at the Frankel Cardiovascular Center’s Inherited Cardiomyopathy Program to create consistency for patients and families as patients transition from childhood to adulthood. We meet regularly to discuss patient care issues, to update research activities and to identify opportunities to enhance or improve care practices. In addition, both the adult and pediatric teams are assisted by the same genetic counselor who provides care for the whole family, coordinates family screenings and facilitates follow-up.

Appointment & information

To make an appointment with our team, or to learn more about the Congenital Heart Center, please call 734-764-5176.