The goal of treatment is to reduce the frequency and severity of the arrhythmias, manage any signs or symptoms of heart failure, and to prevent sudden cardiac death. Currently, the primary treatment for ACM is to use appropriate medications alone or together with an implantable cardioverter defibrillator (ICD) to correct life threatening heart rhythms.
What are ICDs?
Implantable Cardiac Defibrillators (ICDs) are commonly used to treat individuals who experience potentially dangerous ventricular arrhythmias. These devices monitor the heartbeat continuously and automatically deliver an electrical shock to the heart to return it to normal rhythm if a sustained rapid heart rhythm occurs. The electrical shock has been described by patients as being “kicked in the chest,” and may cause momentary discomfort. ICDs can also function as pacemakers and treat slow heart rhythms. If for some reason a decision is made NOT to implant an ICD, very close monitoring and frequent checkups are needed for those individuals experiencing symptoms of ACM and who would clearly benefit from an ICD. It’s interesting to note that implanting ICDs for the primary prevention of sudden death is a more common treatment in the United States than in Europe.
ICD implantation surgery
The ICD is surgically implanted by making a 2-inch incision beneath the collarbone (and sometimes beneath the breast muscle to help hide the incision) and creating a “pocket” for the device. Wires, called leads, are inserted into veins that lead to the heart and attached to the device. The doctor tests the ICD by triggering an abnormal heart rhythm during the procedure to be sure that the ICD will detect and stop the abnormal heart rhythm. Depending on your condition and age, the entire surgery may be done as an outpatient surgery, or may require an overnight stay in the hospital. For a few days to weeks after surgery, you may experience pain, swelling, and/or tenderness in the area where the ICD was implanted. Your doctor will help you decide how soon you can resume your normal activities and what pain medications to take. Most people return to their normal activities within a few days of their surgery. Many patients find that they gradually return to the lifestyle they enjoyed before surgery with added assurance that the ICD is watching over their heart rhythms. In general, work, hobbies, non-strenuous exercise and travel are possible after ICD surgery. ICDs should be checked every 3-6 months and usually replaced every 4-10 years, or if an unforeseen complication arises. It’s extremely important to follow your doctor’s instructions after surgery. Since each person’s medical case is different, it’s best to discuss with your doctor if an ICD is the best treatment for you.
MRI’s for ACM patients with ICDs
A MRI, or Magnetic Resonance Imaging, can be used to detect many diseases such as cancer, orthopedic injuries, and more. Until recently, patients with ICDs were unable to have a MRI because of the risk of the MRI’s heat, vibrations and magnetic field causing the ICD to malfunction. Precautions can now be made by programming the ICD into a “safe” mode for the MRI. The ICD is monitored during the procedure in case any problems occur. After the MRI, the ICD is checked to make sure it’s working properly again. Many people can undergo the MRI safely, but more data is needed to make sure it would be safe for everyone.
MRI’s are being performed in patients with ICDs in academic centers at present. Guidelines are being written so that non-academic centers will be able to conform in the future.
ACM medications
Many people with ACM take medications, called antiarrhythmic agents, to help lessen the frequency and severity of their arrhythmia. These medications change the electrical properties of the heart directly or indirectly. Drugs used in ACM treatment such as beta-blockers and antiarrhythmogenic agents (sotalol and amioadrone) are commonly used to reduce arrhythmias. Beta-blockers are sometimes used to slow the heart rate, blood pressure, and counter the effects of adrenaline, which seem to play a part in causing abnormal heart rhythms. Sotalol appears to be the most effective antiarrhythmic drug to prevent ventricular tachycardia in patients with ACM. Amiodarone alone or in combination with beta-blocking drugs may be an alternative for those patients not responding to other drug therapies, but frequent side effects of amiodarone during long-term therapy in young patients limit this particular treatment. Evidence to support their use is not conclusive. Beta-blockers and ACE Inhibitors are also used due to their proven benefit in slowing disease progression in other heart diseases, but evidence of their effectiveness with ACM is lacking. All medications may cause side effects, and new medications are being developed every year. A close working relationship with your doctor is necessary to monitor the effectiveness of your medications in controlling your arrhythmias.
Additional ACM treatments
Sometimes an electrophysiology study (EP study) can locate what area(s) of the heart are causing the abnormal rhythm, and these areas can be eliminated. This procedure is called catheter ablation and consists of locating the site of the abnormal rhythm and burning it out by sending an electrical current to the tip of a catheter that is directed to the site of the abnormal rhythm. It is done in an EP lab. Catheter ablation is another option for treatment of patients with ACM who have ventricular tachycardia. It’s clear that catheter ablation reduces the frequency of ventricular tachycardia episodes and appropriate ICD therapies in patients with ACM. This results in improved quality of life. It’s important to remember, however, that catheter ablation is not a cure and patients are still at risk for ventricular tachycardia recurrence or sudden death. Catheter ablation should not be viewed as an alternative to ICD implantation. Catheter ablation is usually considered when patients with an ICD have recurrent ventricular tachycardia and have failed to respond to drug therapy.
Whether catheter ablation should be done prior to or after failure of antiarrhythmic drug treatment including sotalol or amiodarone is a decision that must be discussed fully between patients and their physicians so an informed decision can be made. If patients have many areas of electrical abnormality, catheter ablation can’t be performed. Ablations may need to be repeated.
Also, Dr. Jared Churko, Director of the University of Arizona iPSC Core and Assistant Professor in the Department of Cellular and Molecular Medicine, is currently focusing his research on the possibility that iPSC (stem cell) research may identify the basic molecular mechanisms that cause ACM. This is very exciting and promising research.
For more information about Dr. Jared Churko and his work, please see under “About Sarver Heart Center” on our Home page.
Treatment failure
A small minority of patients with ACM may need a heart transplant due to progressive loss of heart muscle resulting in heart failure, or when arrhythmias can’t be controlled by an ICD and/or antiarrhythmic drugs and no other treatment is successful. With limited hearts available for transplantation, an area of active research at the University of Arizona Sarver Heart Center is ” bridge-to-regeneration” trials in which one’s own stem cells are injected into a patient’s failing heart to offer hope for increased regeneration and recovery.