Cooperative international neuromuscular research group duchenne natural history study demonstrates insufficient diagnosis and treatment of cardiomyopathy in duchenne muscular dystrophy

Christopher Spurney, Reiko Shimizu, Lauren P. Morgenroth, Hanna Kolski, Heather Gordish-Dressman, Paula R. Clemens, Michelle Cregan, Erica Goude, Merete Glick, Linda Johnson, Jay Han, Nanette Joyce, David Kilmer, Alina Nicorici, C. Chidambaranathan, Suresh Kumar, Laila Eliasoph, Elizabeth Hosaki, Angela Gonzales, Vivienne HarrisAngela Chiu, Jennifer Thannhauser, Lori Walker, Caitlin Wright, Mehrnaz Yousefi, Anne Christine Alhander, Lisa Wahlgren, Anne Berit Ekstrom, Anna Karin Kroksmark, Ulrika Sterky, Marissa Birkmeier, Tina Duong, Sarah Kaminski, Kate Carroll, Katy DeValle, Rachel Kennedy, Andrew Kornberg, Victoria Rodriguez, Dani Villano, Adina Bar Leve, Elana Wisband, Debbie Yaffe, Luz Andreone, Jose Corderi, Lilia Mesa, Lorena Levi, Krista Coleman-Wood, Ann Hoffman, Wendy Korn-Petersen, Nancy Kuntz, Duygu Selcen, Hoda Abdel-Hamid, Christopher Bise, Ann Craig, Casey Nguyen, Jason Weimer, M. Al-Lozi, Julaine Florence, Paul Golumbak, Glenn Lopate, Justin Malane, Betsy Malkus, Renee Renna, Jeanine Schierbacker, Catherine Seiner, Charlie Wulf, Susan Blair, Barbara Grillo, Karen Jones, Eugenio Monasterio, Meegan Barrett-Adair, Judy Clift, Cassandra Feliciano, Rachel Young, Kristy Rose, Richard Webster, Stephanie Wicks, Lucia Chen, Cameron Kennedy, Brenda Deliz, Sheila Espada, Pura Fuste, Carlos Luciano, Maria Beneggi, Luca Capone, Alessandro Molteni, Valentina Morettini, Anjali Gupta, Robert McNeil, Amy Erickson, Marcia Margolis, Cameron Naughton, Gareth J Parry, David Walk, Adrienne Arrieta, Naomi Bartley, Paola Canelos, Robert Casper, Avital Cnaan, Tina Duong, Fengming Hu, Zoë Sund, Angela Zimmerman

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Introduction: Cardiomyopathy is a common cause of morbidity and death in patients with Duchenne muscular dystrophy (DMD). Methods: This investigation was a cross-sectional cross-sectional analysis of clinical data from the multi-institutional Cooperative International Neuromuscular Research Group (CINRG) DMD Natural History Study of 340 DMD patients aged 2-28 years. Cardiomyopathy was defined as shortening fraction (SF) <28% or ejection fraction (EF) <55%. Results: Two hundred thirty-one participants reported a prior clinical echocardiogram study, and 174 had data for SF or EF. The prevalence of cardiomyopathy was 27% (47 of 174), and it was associated significantly with age and clinical stage. The association of cardiomyopathy with age and clinical stage was not changed by glucocorticoid use as a covariate (P>0.68). In patients with cardiomyopathy, 57% (27 of 47) reported not taking any cardiac medications. Cardiac medications were used in 12% (15 of 127) of patients without cardiomyopathy. Conclusions: We found that echocardiograms were underutilized, and cardiomyopathy was undertreated in this DMD natural history cohort.

Original languageEnglish (US)
Pages (from-to)250-256
Number of pages7
JournalMuscle and Nerve
Issue number2
StatePublished - Aug 1 2014


  • Cardiomyopathy
  • Duchenne muscular dystrophy
  • Echocardiogram
  • Glucocorticoid
  • Natural history


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