Effects of steroids and angiotensin converting enzyme inhibition on circumferential strain in boys with Duchenne muscular dystrophy: a cross-sectional and longitudinal study utilizing cardiovascular magnetic resonance.
BACKGROUND
Steroid use has prolonged ambulation in Duchenne muscular dystrophy (DMD) and combined with advances in respiratory care overall management has improved such that cardiac manifestations have become the major cause of death. Unfortunately, there is no consensus for DMD-associated cardiac disease management. Our purpose was to assess effects of steroid use alone or in combination with angiotensin converting enzyme inhibitors (ACEI) or angiotension receptor blocker (ARB) on cardiovascular magnetic resonance (CMR) derived circumferential strain (εcc).


METHODS
We used CMR to assess effects of corticosteroids alone (Group A) or in combination with ACEI or ARB (Group B) on heart rate (HR), left ventricular ejection fraction (LVEF), mass (LVM), end diastolic volume (LVEDV) and circumferential strain (εcc) in a cohort of 171 DMD patients >5 years of age. Treatment decisions were made independently by physicians at both our institution and referral centers and not based on CMR results.


RESULTS
Patients in Group A (114 studies) were younger than those in Group B (92 studies)(10 ± 2.4 vs. 12.4 ± 3.2 years, p < 0.0001), but HR, LVEF, LVEDV and LVM were not different. Although εcc magnitude was lower in Group B than Group A (-13.8 ± 1.9 vs. -12.8 ± 2.0, p = 0.0004), age correction using covariance analysis eliminated this effect. In a subset of patients who underwent serial CMR exams with an inter-study time of ~15 months, εcc worsened regardless of treatment group.


CONCLUSIONS
These results support the need for prospective clinical trials to identify more effective treatment regimens for DMD associated cardiac disease.
The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. kan.hor@cchmc.org
Kan N
KN
Hor
Wojciech
W
Mazur
Michael D
MD
Taylor
Hussein R
HR
Al-Khalidi
Linda H
LH
Cripe
John L
JL
Jefferies
Subha V
SV
Raman
Eugene S
ES
Chung
Kathi J
KJ
Kinnett
Katelyn
K
Williams
William M
WM
Gottliebson
D Woodrow
DW
Benson
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000009:adverse effects*; D000704:Analysis of Variance; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D009202:Cardiomyopathies / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D002648:Child; D003430:Cross-Sectional Studies; D018450:Disease Progression; D006339:Heart Rate / Q000187:drug effects; D006801:Humans; D008137:Longitudinal Studies; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000188:drug therapy*; D009200:Myocardial Contraction / Q000187:drug effects; D009820:Ohio; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D013318:Stroke Volume / Q000187:drug effects; D013997:Time Factors; D016896:Treatment Outcome; D016277:Ventricular Function, Left / Q000187:drug effects
D000305:Adrenal Cortex Hormones; D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors