TY - JOUR
T1 - Clinical Care Recommendations for Cardiologists Treating Adults With Myotonic Dystrophy
AU - McNally, Elizabeth M.
AU - Mann, Douglas L.
AU - Pinto, Yigal
AU - Bhakta, Deepak
AU - Tomaselli, Gordon
AU - Nazarian, Saman
AU - Groh, William J.
AU - Tamura, Takuhisa
AU - Duboc, Denis
AU - Itoh, Hideki
AU - Hellerstein, Leah
AU - Mammen, Pradeep P.A.
N1 - Funding Information:
Dr McNally is supported by National Institutes of Health AR052646. Dr Mammen is supported by National Institutes of Health HD087351.
Publisher Copyright:
© 2020 The Authors and Myotonic. Published on behalf of the American Heart Association, Inc., by Wiley.
PY - 2020/2/18
Y1 - 2020/2/18
N2 - 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.
AB - 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.
KW - arrhythmias
KW - conduction system disease
KW - heart failure
KW - management
KW - myotonic dystrophy
UR - http://www.scopus.com/inward/record.url?scp=85079676049&partnerID=8YFLogxK
U2 - 10.1161/JAHA.119.014006
DO - 10.1161/JAHA.119.014006
M3 - Article
C2 - 32067592
AN - SCOPUS:85079676049
SN - 2047-9980
VL - 9
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
IS - 4
M1 - e014006
ER -