Due to the multisystemic nature of myotonic dystrophy type 1 (DM1), it has been a challenge to conduct the studies and collect the rigorous evidence required to create an evidence-based guideline for the cardiac clinical care of patients with myotonic dystrophy type 1. In order to improve the quality of life and standardize care for people living with DM1, Myotonic organized and supported 10 leading DM1 cardiologists in Canada, Japan, Western Europe, the United Kingdom, and the United States to create the Consensus-based Care Recommendations for Cardiologists Treating Adults with Myotonic Dystrophy Type 1.
To promote the development of cardiac care guidelines for myotonic dystrophy, Myotonic solicited the input of care experts and organized the drafting of these recommendations. The 10 leading DM1 cardiologists created the Consensus-based Care Recommendations for Cardiologists Treating Adults with Myotonic Dystrophy Type 1 through an iterative drafting process. All authors contributed content to create the first draft and leading author Elizabeth McNally, M.D., Ph.D. was in charge of managing further editing cycles. The process began in 2017 and concluded in February 2020, with the article published on the Journal of the American Heart Association website on 2/6/20 and in print on 2/18/20 in Volume 9, Issue 4.
Tell Your Cardiologist
The guidelines review cardiac management of myotonic dystrophy, including surveillance for arrhythmias and left ventricular dysfunction, both of which occur in progressive manner and contribute to morbidity and mortality. Other topics reviewed in the guidelines include heart imaging, cardiomyopathy treatment and stroke risk, concluding in 15 summary recommendations. The recommendations represent expert consensus opinion from those with experience in the management of myotonic dystrophy, in part supported by literature‐based evidence where available.
The guidelines are intended for use by cardiologists but individuals diagnosed with DM1 are encouraged to provide a copy of the recommendations to their cardiologists. The guidelines represent an exciting step in the cardiac treatment and management of DM1 and Myotonic is thrilled to support this vital educational effort to standardize care.
Digitally access the full article on the Journal of the American Heart Association website.
You can download the full Consensus-based Care Recommendations for Cardiologists Treating Adults with Myotonic Dystrophy Type 1 as well as other critical care resources to share with your doctor on our Toolkits & Publications page.
If you would like a hard copy of the article or guidelines mailed to you, please email Myotonic's Program Director, Leah Hellerstein, LCSW, MPH at Leah.Hellerstein@myotonic.org.
Clinical Care Recommendations for Cardiologists Treating Adults With Myotonic Dystrophy
McNally EM, Mann DL, Pinto Y, Bhakta D, Tomaselli G, Nazarian S, Groh WJ, Tamura T, Duboc D, Itoh H, Hellerstein L, and Mammen PPA
JAHA. 2020 Feb 6. doi 10.1161/JAJA.119.014006 [Epub ahead of print]