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Heart Problems in Myotonic Dystrophy Brochure

People that have either myotonic dystrophy or the congenital form are at a high risk of developing life threatening heart problems. There is a correlation between the number of repeats for the diseases and the severity of the cardiac problems. Eleven percent (11%) of patients in a recent study died suddenly from what is believed to be cardiac problems. Another 28% had cardiac problems of one type or another.

Personal Story about Cardiac Problems:

Recent Cardiac Studies: Correlation between CTG triplet repeat length and the extent of multi-systemic disorders in myotonic dystrophy

Pacemakers and Defibrillators: Pacemakers are generally used to manage a heartbeat that is too slow or irregular, caused by disorders that disrupt the heart's normal electrical conduction system. This condition known as bradicardia can cause inadequate blood flow through the body creating symptoms such as fatigue, dizziness, and fainting.

ECK Recommendations for Patients with DM from Scottish Workgroup

All individuals with myotonic dystrophy should receive a baseline ECG
Patients with significant muscle disease and/or abnormal ECG should receive an annual ECG (Grade C)
Patients with mild symptoms and a normal or minimal ECG should have their ECG repeated every 2 years (Grade C)
Asymtomatic gene carriers should be offered an ECG at 5 year intervals
Patients whose ECG indicate significant (second degree or greater) heart block should be referred to Cardiology
Children with symptomatic myotonic dystrophy should have regular pediatric review including an annual ECG

Study: When would a pacemaker be suitable for patient with DM?: Rollin J. Hawley, MD, Neurology Service, VA Medical Center, Neurology Department, Georgetown University Medical School, Washington, DC; John A. Colleran, DO, Ross Fletcher, MD, Ralph J. Verdino, MD, Medical Service, VA Medical Center, and the Department of Medicine, Georgetown University Medical School; Peter Kokkinos, PhD, Ellen Pinnow, PhD, Biostatistics, Washington Hospital Center, and George Washington University Medical School, Washington, DC

*This information was copied from the evidence based clinical guidelines developed by the Collaborative Project Byall Scottish Clinical Genetics Services.