Clinical Care Recommendations for Cardiologists Treating Adults With Myotonic Dystrophy

Elizabeth M. McNally, Douglas L. Mann, Yigal Pinto, Deepak Bhakta, Gordon Tomaselli, Saman Nazarian, William J. Groh, Takuhisa Tamura, Denis Duboc, Hideki Itoh, Leah Hellerstein, Pradeep P.A. Mammen

Research output: Contribution to journalArticle

Abstract

Abstract: Myotonic dystrophy is an inherited systemic disorder affecting skeletal muscle and the heart. Genetic testing for myotonic dystrophy is diagnostic and identifies those at risk for cardiac complications. The 2 major genetic forms of myotonic dystrophy, type 1 and type 2, differ in genetic etiology yet share clinical features. The cardiac management of myotonic dystrophy should include surveillance for arrhythmias and left ventricular dysfunction, both of which occur in progressive manner and contribute to morbidity and mortality. To promote the development of care guidelines for myotonic dystrophy, the Myotonic Foundation solicited the input of care experts and organized the drafting of these recommendations. As a rare disorder, large scale clinical trial data to guide the management of myotonic dystrophy are largely lacking. The following recommendations represent expert consensus opinion from those with experience in the management of myotonic dystrophy, in part supported by literature-based evidence where available.

Original languageEnglish (US)
Article numbere014006
JournalJournal of the American Heart Association
Volume9
Issue number4
DOIs
StatePublished - Feb 18 2020

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Keywords

  • arrhythmias
  • conduction system disease
  • heart failure
  • management
  • myotonic dystrophy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

McNally, E. M., Mann, D. L., Pinto, Y., Bhakta, D., Tomaselli, G., Nazarian, S., Groh, W. J., Tamura, T., Duboc, D., Itoh, H., Hellerstein, L., & Mammen, P. P. A. (2020). Clinical Care Recommendations for Cardiologists Treating Adults With Myotonic Dystrophy. Journal of the American Heart Association, 9(4), [e014006]. https://doi.org/10.1161/JAHA.119.014006