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Pacemakers are very small battery operated devices that are implanted into the chest. They contain wires or Leads that are thread into the cardiac veins and into the heart chambers.

The purpose of the pacemaker is to replace the hearts natural electrical signal. The electrical signal is what makes the heart pump or beat and send your blood supply throughout the body.

There are single chamber and dual chamber pacemakers. Which one your doctor uses depends on the condition of your cardiac issues.

The advancement in the design and functionality of pacemakers is growing with advances in technology. So as the field grows there will be more options for patients requiring one.

For EDMD patients the need for the pacemaker usually occurs in the early teens. There are cases of patients who are into their 30’s and have yet to be deemed in need of one.

However when the patient is required to have one it will be as a result of heart block starting or coming on suddenly in a more advanced stage. Pacemakers can also contain internal defibrillators to control abnormal cardiac rhythms in the heart.

The Pacemaker surgery itself is a relatively low risk surgery in the sense that it is not a new procedure. With the advancement of the technology of pacemakers the surgeries may change. But the assumption is that the surgeries would become safer and more advanced.

If you or your child are going to be going in for pacemaker surgery it is vitally important to make the hospital, surgeons and staff aware of the anesthesia warnings for EDMD patients. You must inform them that EDMD patients are at a high risk of Malignant Hyperthermia and Volatile Anesthetics, such as gas form anesthesia cannot be used. The specific warning relates to Succinylcholine which is a common form of anesthesia so you need to discuss anesthesia options with the medical staff.

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The image shown here is of a Dual-Chamber Pacemaker. The wires are shown in both the upper chamber (the Atrium) and the lower chamber (The Ventricle) on the right side of the heart. The pacemaker will generate a signal in the atrium lead which will be received by the lead in the ventricle making the heart pump. The leads and pacemaker unit replace the function of the bridge between the Right Atrium and the Right Ventricle. The Bridge is the natural transmitter of this signal and when there is a block in the heart this bridge ceases to function normally by failing to transmit the signal from the Atrium.

Photo Credit:

There are also Rate Responsive Pacemakers which will generate a faster heart rhythm to keep up with the activity of the patient throughout the day.

When a doctor wants to determine if a pacemaker is needed in a patient they will commonly order an event monitor. The event monitor is different from the Holter Monitor in that the patient will wear it for a longer period of time.

The Holter Monitor is typically worn for up to 24 hours and will continuously record the activity of the heart. The patient will usually be asked to keep a diary of the activities performed throughout the day as well as push a button on the device to mark periods where they feel an abnormal heart rhythm.

Usually an event monitor works the same way except that it can be worn for a week or more.

The device itself is connected to the patient using EKG wires and stickers. The patient will place these stickers and connect the device themselves (usually in the case of an event monitor) or the hospital will place it for you (usually in the case of a Holter Monitor) and the return to the hospital when it is time to remove the device.

The doctor will analyze the readout from the monitor looking for periods where the heart rhythm was slow. If the heart rhythm is slow usually the call for a Pacemaker will be made.