International Foundation for Emery-
We are not doctors. This is not medical advice. You need to verify all information provided with your doctors. The information on this site was provided by medical professionals. If your doctors need to verify any of the information on this site please contact us or have your doctors contact us so we my connect them or you with the doctors themselves.
The normal human heart works by sending and receiving electrical signals which tell the heart to pump blood through the body. This electrical signal begins in the brain, sending neurological signals to the atrium of the heart (the upper chambers), and is passed down to the ventricles of the heart (the lower chambers) through a bridge that connects the two halves. This bridge can become altered or blocked creating the heart block associated with EDMD. This is known as Heart Block. EDMD always affects the heart. There are many different ways in which the heart can be effected and they are not all muscle related. This disease can be fatal if the heart condition is undiagnosed, untreated or misdiagnosed. EDMD with the deficiency of Emerin caused the heart to function improperly.
You can also be diagnosed with thinning heart (Cardiomyopathy) walls as a result of this disease making the annual procedure of Echo-
Heart Block will usually begin at “first degree” and can progress all the way to atrial standstill which means the bridge will stop working completely. Which is why a Pacemaker is required.
Heart Block is diagnosed using an EKG which is done by a Cardiologist or Electro-
Cardiac Issues in EDMD
The atrium (upper portion) of the heart can produce more issues that EDMD patients will need to deal with. The atrium of the heart works by producing an electrical signal that the bottom of the heart receives. These electrical signals tell the heart when to pump blood throughout the body. The problem with EDMD is that somewhere in the heart there can be “extra” signals produced which is referred to an Atrial Flutter, Atrial Tachycardia and /or Supraventricular Tachycardia.
Superventricular Tachycardia in EDMD is a random signal. The signal can be produced anywhere in the atrium even changing its origin within the atrium. It is unpredictable and best left treated only with beta-
Atrial Flutter is known as a condition where the atrium of the heart produces a fast heart rhythm. It is often temporary causing palpitations.
Atrial Tachycardia presents itself with a fast rhythm in the atrium, usually being produced from a single or multiple points within the atrium.
Superventricular Tachycardia in EDMD is often a random signal. The signal can be produced anywhere in the atrium even changing its origin within the atrium. It is unpredictable and best left treated only with beta-
Ectopic Atrial Tachycardia
Ectopic Atrial Tachycardia is the most common form of super atrial tachycardia in children. It is the most resistant to cardio-
The heart will stop producing any signal in the atrium. This event will require a pacemaker in order to keep the heart beating. Blood thinners will be required to prevent blood clots which can form in the heart resulting in one or more strokes. The risk of blood clots is very real in EDMD and if your doctor prescribes a blood thinner it is very important to adhere to those instructions.
Cardiomyopathy is a weakening of the heart wall, and can occur in EDMD. Those with severe cases may eventually require a heart transplant.
A great resource for learning the function of the heart, including an interactive heart diagram, can be found at http://www.bostonscientific.com/lifebeat-