Current state of cardiac troponin testing in Duchenne muscular dystrophy cardiomyopathy: review and recommendations from the Parent Project Muscular Dystrophy expert panel
Christopher F Spurney,
Lawrence Charnas,
Linda Cripe,
Kan Hor,
Nicholas King,
Kathi Kinnett,
Elizabeth M McNally,
John-Michael Sauer,
Lee Sweeney,
Chet Villa,
Larry W Markham
Affiliations
Christopher F Spurney
Children`s National Heart Institute, Children`s National Hospital, Washington, District of Columbia, USA
Lawrence Charnas
Clinical Research Rare Neurology Disease, Pfizer, Cambridge, Massachusetts, USA
Linda Cripe
The Heart Center, Nationwide Children`s Hospital, Columbus, Ohio, USA
Kan Hor
The Heart Center, Nationwide Children`s Hospital, Columbus, Ohio, USA
Nicholas King
Critical Path Institute, Tucson, Arizona, USA
Kathi Kinnett
Parent Project Muscular Dystrophy, Hackensack, New Jersey, USA
Elizabeth M McNally
Center for Genetic Medicine, Northwestern Medicine, Chicago, Illinois, USA
John-Michael Sauer
Critical Path Institute, Tucson, Arizona, USA
Lee Sweeney
Department of Pharmacology and Therapeutics, University of Florida, Gainesville, Florida, USA
Chet Villa
Cardiology, Cincinnati Children`s Hospital Medical Center, Cincinnati, Ohio, USA
Larry W Markham
Pediatrics, Division of Pediatric Cardiology, Riley Hospital for Children at Indiana University Health, Indianapolis, Indiana, USA
Cardiac disease is now the leading cause of death in Duchenne muscular dystrophy (DMD). Clinical evaluations over time have demonstrated asymptomatic cardiac troponin elevations and acute elevations are associated with symptoms and cardiac dysfunction in DMD. Clinicians require a better understanding of the relationship of symptoms, troponin levels and progression of cardiac disease in DMD. As clinical trials begin to assess novel cardiac therapeutics in DMD, troponin levels in DMD are important for safety monitoring and outcome measures. The Parent Project Muscular Dystrophy convened an expert panel of cardiologists, scientists, and regulatory and industry specialists on 16 December 2019 in Silver Spring, Maryland and reviewed published and unpublished data from their institutions. The panel recommended retrospective troponin data analyses, prospective longitudinal troponin collection using high-sensitivity cardiac troponin I assays, inclusion of troponin in future clinical trial outcomes and future development of clinical guidelines for monitoring and treating troponin elevations in DMD.