Cardiomyopathy in Children: Symptoms, Treatment, & Mortality

Need for aggressive treatment
Most adults with cardiomyopathy have minor symptoms without serious limitations or major problems for years. By contrast, cardiomyopathy diagnosed in young children usually signifies that a serious heart condition that requires aggressive treatment — especially with concerns over how the heart muscle will respond with each additional growth spurt. With the help of medication or surgery, the condition may stabilize in some older children over time. But in severe cases, small children may experience progressive symptoms quickly leading to heart failure.

Cardiomyopathy also progresses differently in children than in adults. Children who are diagnosed at an early age, generally have a poor prognosis — but this also depends on the form of the disease and its stage. When diagnosed in young children for example, dilated cardiomyopathy, can progress quite rapidly.

Up to 40 percent of diagnosed children with dilated cardiomyopathy fail medical management within the first year of diagnosis. Those that survive many have permanently impaired heart function. Children with hypertrophic cardiomyopathy may fare better, but not always.

Sudden death accounts for 50 percent of deaths in children with hypertrophic cardiomyopathy and 28 percent of deaths in children with restrictive cardiomyopathy

Heart failure is usually a long-term or chronic illness. It may get worse over time and some people even develop severe heart failure that medicines, surgery and other treatments can no longer improve. Compared with adults who have cardiomyopathy are more like to die or need heart transplants — the rates of both death and transplants are much higher in children than in adults.

This is usually attributed to the more fragile state of infants and young children and to the advanced progression of the disease when it’s coupled with another genetic disorder. Often, this is also due to the fact that cardiomyopathy is not detected until the end stage when obvious symptoms of heart failure are apparent. In routine check-ups, cardiomyopathy is often missed when there are no obvious symptoms — like heart murmur — or when there is no reason for diagnostic testing, like when there is no family history of the disease.

Right now, there is no cure or treatment that can return the heart to normal or guarantee long-term survival. Occasionally, children with certain types of cardiomyopathy improve — but the vast majority don’t show any recovery in heart function.

If cardiomyopathy is diagnosed in the early stages, it may be controlled with long-term drug therapy and placement of a pacemaker or defibrillator. If it’s detected at an advanced stage, critically ill patients will require immediate lifesaving measures — placement of a breathing tube or mechanical ventilator and administration of medications intravenously — usually dobutamine and dopamine — to improve blood pressure and heart function. It’s only when the patient has stabilized that therapy involving oral medication, implantable devices, surgery or heart transplantation can be considered and begun.

The medical management of a child with cardiomyopathy should be done in consultation with:
• a pediatric cardiologist
• a cardiothoracic surgeon
• an electrophysiologist
• a geneticist

A comprehensive evaluation is needed to pinpoint the cause so that proper measures can be taken. Several factors will determine the required therapy:
• The child’s age
• Overall health
• Medical history
• Stage of the disease
• The child’s tolerance for specific medications, procedures or therapies.

The risk for the following should also be determined by the doctor:
• sudden death
• predisposition to progressive symptoms
• atrial fibrillations
• predisposition to end stage heart failure.

Because children grow at such rapid rates, frequent visits to the cardiologist are needed to monitor changes in the heart and prevent complications.

The conditions that cause dilated cardiomyopathy need to be treated first.

Heart failure is treated by:
• Taking medicines. Drugs are used to relieve heart failure symptoms, decrease the workload on the heart, decrease the oxygen requirements of the heart and regulate abnormal heartbeats. But patients need to be monitored for side effects and complications.
• Wearing a pacemaker to treat a slow heart rate or to help both sides of your heart beat at the same time. This device is used to monitor and stabilize slow heartbeats. In older children — and adults — with obstructive hypertrophic cardiomyopathy, dual chamber pacing has been used to decrease outflow obstruction. While this procedure isn’t recommended for infants or small children, recent advances have allowed for single chamber pacing in younger children.
• Wearing an automatic implantable cardioverter defibrillator (AICD). Often called the “emergency room in the chest,” an AICD sends an electrical pulse to stop life-threatening, abnormal heart rhythms. This device may benefit higher risk patients who have experienced serious episodes of fainting, been resuscitated from cardiac arrest, have experienced life threatening arrhythmias or are susceptible to sudden death. Defibrillators are often recommended for children diagnosed with hypertrophic cardiomyopathy, restrictive cardiomyopathy and restrictive cardiomyopathy with arrhythmias.

Both devices are implanted under the skin of the chest or abdomen with wire leads threaded through veins into the heart. This is considered minor surgery, requiring a short hospital stay. Once a pacemaker or defibrillator is inserted, it requires careful monitoring to determine when it needs to be replaced and to ensure that the electrical settings are correct.

A cardiac catheterization may also be done to see if the child needs a coronary artery bypass (CABG) surgery or an angioplasty, which can improve blood flow to the damaged or weakened heart muscle. It may also be done to measure pressures and check your heart function (called right-heart catheterization).

When children have tried all the standard treatments and still have very severe symptoms, they may need a heart transplant. Implantable artificial heart pumps have been developed recently, but few patients are able to have this advanced treatment.

The outlook depends on many different things, including:

• The cause and type of cardiomyopathy
• How severe the heart problem is
• How well you respond to treatment

But often enough, heart failure can be controlled with medicine, lifestyle changes, and by treating the condition that caused it.

Heart failure may suddenly become worse due to:

• Angina
• Eating high-salt foods
• Heart attack
• Infections or other illnesses
• Not taking your medicine correctly

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