Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?
BACKGROUND
Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function.


METHODS
The cardiology databases at Primary Children's Medical Center and Cincinnati Children's Hospital Medical Center were reviewed to identify patients with DMD who had undergone a CMRI within the last 2 years. Age, LV ejection fraction, LV mass, presence and location of LGE were documented. Volumes were measured using MASS (Medis, Inc.) to calculate ejection fraction and mass. LGE images were acquired and when positive, customized computer assisted sizing of the areas of late gadolinium enhancement were performed on all slices. Normal function was defined as LV ejection fraction >54%.


RESULTS
A total of 74 patients with DMD had complete data sets (median age 13.7 years, range 7.7-26.4). Twenty-four patients (32%) had LGE involving the posterobasal region of the LV in a sub-epicardial distribution. Those patients with more involvement had spread to the inferior and left lateral free wall with progressive transmural fibrous replacement. There was relative sparing of the interventricular septum and right ventricle. Patients with LGE were significantly older than those without (mean age 16.4 vs 12.9 years, P < 0.001). LGE was positively associated with BSA-adjusted LV mass, LV end-diastolic volume, LV end-systolic volume, and RV end-systolic volume but inversely correlated with ejection fraction of the LV (P < 0.001) and RV (P = 0.004).


CONCLUSIONS
LGE by CMRI is able to detect fibrosis in selective regions of myocardium in patients with DMD. Unfavorable LV remodeling, with a corresponding decreased ejection fraction, is associated with the presence of LGE. Serial studies are warranted to determine if LGE precedes a decrease in function, and if early medical management is useful in preventing progression once LGE is documented.
Puchalski
Michael D
MD
Department of Pediatrics, University of Utah and Primary Children's Medical Center, 100 N. Medical Drive, Salt Lake City, UT 84113, USA. michael.puchalski@imail.org
Williams
Richard V
RV
Askovich
Bojana
B
Sower
C Todd
CT
Hor
Kan H
KH
Su
Jason T
JT
Pack
Nathan
N
Dibella
Edward
E
Gottliebson
William M
WM
D000293:Adolescent; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000209:etiology*; D002648:Child; D003287:Contrast Media; D005260:Female; D005682:Gadolinium; D006801:Humans; D007089:Image Enhancement / Q000379:methods; D007090:Image Interpretation, Computer-Assisted; D016014:Linear Models; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D055815:Young Adult
D003287:Contrast Media; D005682:Gadolinium