Unfortunately, there is no cure for cardiomyopathy. However, current treatment options allow children with cardiomyopathy to live a relatively normal life.
Most treatments are designed to minimize symptoms and improve heart function by decreasing the workload on the heart and allowing it to deliver oxygen and blood to the body more efficiently. Another treatment priority is to control dangerous arrhythmias that could lead to sudden cardiac death.
Common treatment options are medications and implantable cardiac devices (pacemaker, defibrillator). Surgery (myectomy or mitral valve repair) may be recommended for older children with hypertrophic cardiomyopathy when symptoms cannot be controlled by medication.
If the disease progresses to heart failure, more aggressive interventions may be necessary, such as mechanical ventilatory support (intubation), intravenous medication (dobutamine, dopamine) and mechanical cardiac assist devices (ECMO, LVAD, BIVAD). Children with advanced and progressive cardiomyopathy may require a heart transplant if they have persistent symptoms of congestive heart failure and no longer respond to standard treatment.
The overall goal of treatment is to restore a child to the best possible health with the least amount of intervention. The challenge is there are no established standards to guide treatment, and cardiomyopathy is highly variable in its presentation, causes and outcome. As a result, each child requires an individualized treatment plan to address short and long-term medical issues.
Factors that determine a child’s treatment plan include the child's age, overall health, medical history, type of cardiomyopathy, stage of the disease, and tolerance for specific medications, procedures or therapies. Each form of form of cardiomyopathy has different treatment goals.
Follow-up visits are important to monitor for side effects to medications, changes to the heart, treatment effectiveness and new complications. During examination, the cardiologist will try to determine a child’s risk for abnormal heart rhythm and sudden cardiac arrest, and predisposition to progressive heart failure.
A multidisciplinary team approach to care is key to getting the best treatment possible. Ideally, a child’s medical care should be provided collaboratively in consultation with a pediatric cardiologist, geneticist, and possibly a cardiothoracic surgeon and electrophysiologist. Other healthcare professionals may also be involved, including the cardiac nurse, genetic counselor, child life specialist, social worker, neurologist, psychiatrist, gastroenterologist, nutritionist, and occupational or physical therapist.