TY - JOUR
T1 - Circumferential Strain Analysis Identifies Strata of Cardiomyopathy in Duchenne Muscular Dystrophy. A Cardiac Magnetic Resonance Tagging Study
AU - Hor, Kan N.
AU - Wansapura, Janaka
AU - Markham, Larry W.
AU - Mazur, Wojciech
AU - Cripe, Linda H.
AU - Fleck, Robert
AU - Benson, D. Woodrow
AU - Gottliebson, William M.
N1 - Funding Information:
This work was supported in part by the Children's Heart Association of Cincinnati (to Dr. Gottliebson) and the National Institutes of Health grant HL069712 (to Dr. Benson), Bethesda, Maryland.
PY - 2009/4/7
Y1 - 2009/4/7
N2 - Objectives: This study sought to evaluate the natural history of occult cardiac dysfunction in Duchenne muscular dystrophy (DMD). Background: Duchenne muscular dystrophy is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that left ventricular myocardial peak circumferential strain (εcc) would decrease in DMD before global systolic functional abnormalities regardless of age or ventricular ejection fraction (EF). Methods: We evaluated cardiac magnetic resonance image (MRI) data from 70 DMD patients and 16 aged-matched control subjects. Standard imaging data included steady-state free precession short-axis cine stack images, cine myocardial tagged images, and myocardial delayed enhancement (MDE) (an indicator of myocardial fibrosis) sequences. Analysis was performed with QMASS (Medis Medical Imaging Systems, Leiden, the Netherlands) and HARP (Diagnosoft, Palo Alto, California) software. The DMD patient data were subdivided by age (<10 or >10 years), EF (>55% or <55%), and the presence or absence of MDE. Results: The DMD patients with normal EF had reduced εcc at an early age (<10 years) compared with control subjects (p < 0.01). The DMD patients age >10 years with normal EF had further decline in εcc compared with younger DMD patients (p < 0.01). There was further decline in εcc with age in patients with reduced EF (p < 0.01) without MDE. The oldest patients, with both reduced EF and positive MDE, exhibited the lowest εcc. None of the patients had ventricular hypertrophy. Conclusions: Myocardial strain abnormalities are prevalent in young DMD patients despite normal EF, and these strain values continue to decline with advancing age. Strain analysis in combination with standard MRI and MDE imaging provides a means to stratify DMD cardiomyopathy.
AB - Objectives: This study sought to evaluate the natural history of occult cardiac dysfunction in Duchenne muscular dystrophy (DMD). Background: Duchenne muscular dystrophy is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that left ventricular myocardial peak circumferential strain (εcc) would decrease in DMD before global systolic functional abnormalities regardless of age or ventricular ejection fraction (EF). Methods: We evaluated cardiac magnetic resonance image (MRI) data from 70 DMD patients and 16 aged-matched control subjects. Standard imaging data included steady-state free precession short-axis cine stack images, cine myocardial tagged images, and myocardial delayed enhancement (MDE) (an indicator of myocardial fibrosis) sequences. Analysis was performed with QMASS (Medis Medical Imaging Systems, Leiden, the Netherlands) and HARP (Diagnosoft, Palo Alto, California) software. The DMD patient data were subdivided by age (<10 or >10 years), EF (>55% or <55%), and the presence or absence of MDE. Results: The DMD patients with normal EF had reduced εcc at an early age (<10 years) compared with control subjects (p < 0.01). The DMD patients age >10 years with normal EF had further decline in εcc compared with younger DMD patients (p < 0.01). There was further decline in εcc with age in patients with reduced EF (p < 0.01) without MDE. The oldest patients, with both reduced EF and positive MDE, exhibited the lowest εcc. None of the patients had ventricular hypertrophy. Conclusions: Myocardial strain abnormalities are prevalent in young DMD patients despite normal EF, and these strain values continue to decline with advancing age. Strain analysis in combination with standard MRI and MDE imaging provides a means to stratify DMD cardiomyopathy.
KW - Duchenne muscular dystrophy
KW - cardiac magnetic resonance imaging
KW - circumferential strain
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U2 - 10.1016/j.jacc.2008.12.032
DO - 10.1016/j.jacc.2008.12.032
M3 - Article
C2 - 19341862
AN - SCOPUS:63049083149
SN - 0735-1097
VL - 53
SP - 1204
EP - 1210
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 14
ER -