Myotonic Dystrophy Foundation

The article, titled “Electrocardiographic Abnormalities and Sudden Death in Myotonic Dystrophy Type 1” concluded that patients with adult myotonic dystrophy type 1 (DM1) are at high risk for arrhythmias and sudden death caused by cardiac arrest.  A severe abnormality on the EKG and a diagnosis of an atrial tachyarrhythmia predict sudden death.  While this study was limited to DM1 patients, DM2 patients have also been cautioned that in the absence of contrary studies, individuals with DM2 should also follow the same guidelines.

In a newsletter to participants, the authors clarify the difference between heart attack and cardiac arrest:

1) Heart attack – results from clogged arteries. People may have symptoms (such as heart pain) for several hours before death occurs.

2) Cardiac arrest – results from a problem with the electrical system of your heart. Death results within minutes (a person just loses consciousness).

The authors contend that a yearly EKG can provide information which will indicate if a person is at risk for a heart rhythm abnormality, and measures can be taken to try to prevent cardiac arrest.

Read a synopsis of this study.