Open AccessRisk Factors for Cardiac and Non-cardiac Causes of Death in Males with Duchenne Muscular Dystrophy
Author(s) -
Carol A. Wittlieb-Weber,
Kenneth R. Knecht,
Chet Villa,
Chentel Cunningham,
Jennifer Conway,
M.J. Bock,
Katheryn Gambetta,
Ashwin K. Lal,
K. Schumacher,
Sabrina Law,
Shriprasad Deshpande,
Shawn C. West,
Joshua M. FriedlandLittle,
Irene D. Lytrivi,
Michael A. McCulloch,
Ryan J. Butts,
David R. Weber,
Jonathan N. Johnson
Publication year - 2020
Publication title -
pediatric cardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.646
H-Index - 71
eISSN - 1432-1971
pISSN - 0172-0643
DOI - 10.1007/s00246-020-02309-y
Subject(s) - medicine , duchenne muscular dystrophy , cardiac surgery , cardiac function curve , ejection fraction , cardiology , retrospective cohort study , cause of death , cohort , cohort study , heart failure , disease
As survival and neuromuscular function in Duchenne muscular dystrophy (DMD) have improved with glucocorticoid (GC) therapy and ventilatory support, cardiac deaths are increasing. Little is known about risk factors for cardiac and non-cardiac causes of death in DMD. A multi-center retrospective cohort study of 408 males with DMD, followed from January 1, 2005 to December 31, 2015, was conducted to identify risk factors for death. Those dying of cardiac causes were compared to those dying of non-cardiac causes and to those alive at study end. There were 29 (7.1%) deaths at a median age of 19.5 (IQR: 16.9-24.6) years; 8 (27.6%) cardiac, and 21 non-cardiac. Those living were younger [14.9 (IQR: 11.0-19.1) years] than those dying of cardiac [18 (IQR 15.5-24) years, p = 0.03] and non-cardiac [19 (IQR: 16.5-23) years, p = 0.002] causes. GC use was lower for those dying of cardiac causes compared to those living [2/8 (25%) vs. 304/378 (80.4%), p = 0.001]. Last ejection fraction prior to death/study end was lower for those dying of cardiac causes compared to those living (37.5% ± 12.8 vs. 54.5% ± 10.8, p = 0.01) but not compared to those dying of non-cardiac causes (37.5% ± 12.8 vs. 41.2% ± 19.3, p = 0.58). In a large DMD cohort, approximately 30% of deaths were cardiac. Lack of GC use was associated with cardiac causes of death, while systolic dysfunction was associated with death from any cause. Further work is needed to ensure guideline adherence and to define optimal management of systolic dysfunction in males with DMD with hopes of extending survival.