Prevalence and distribution of regional scar in dysfunctional myocardial segments in Duchenne muscular dystrophyReport as inadecuate

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Journal of Cardiovascular Magnetic Resonance

, 13:20

First Online: 11 March 2011Received: 30 November 2010Accepted: 11 March 2011


BackgroundThe segmental relationship between cardiovascular magnetic resonance CMR peak circumferential strain Ecc and myocardial scar has not been well characterized in Duchenne muscular dystrophy DMD, and it is unknown whether echocardiography accurately measures Ecc in DMD. We assessed segmental Ecc and scar using CMR with myocardial tissue tagging and late gadolinium enhancement LGE in patients with DMD, then compared CMR with echocardiographic velocity vector imaging VVI for regional Ecc based on independent observer assessments.

ResultsParticipants enrolled n = 16; age 8-23 had median left ventricular LV ejection fraction of 0.52 range 0.28-0.69, and 156 basal and mid-cavity myocardial segments from the 13 patients completing the LGE protocol were analyzed for strain and scar. Segmental CMR Ecc in the most negative quartile quartile 4 ruled out scar in that segment, but scar was present in 46% of segments in the least negative most dysfunctional Ecc quartile 1, 33% of Ecc quartile 2 segments, and 15% of Ecc quartile 3 segments. Overall scar prevalence in inferior, inferolateral, and anterolateral segments was eight times higher than in inferoseptal, anteroseptal, and anterior segments p < 0.001. This increased proportion of scar in lateral versus septal segments was consistent across CMR Ecc quartiles quartile 1: 76% versus 11%, p = 0.001; quartile 2: 65% versus 9%, p < 0.001; quartile 3: 38% versus 0%, p < 0.001. Echocardiographic analysis could be performed in 12 of 14 patients with CMR exams and had to be limited to mid-cavity slices. Echo segmental Ecc in the most negative quartile made scar by CMR in that segment highly unlikely, but the correlation in segmental Ecc between CMR and echo was limited r = 0.27; p = 0.02.

ConclusionsThe relationship between scar and Ecc in DMD is complex. Among myocardial segments with depressed Ecc, scar prevalence was much higher in inferior, inferolateral, and anterolateral segments, indicating a regionally dependent association between abnormal Ecc and scar, with free wall segments commonly developing dysfunction with scar and septal segments developing dysfunction without scar. Although normal echocardiographic Ecc predicted absence of scar, regional echocardiographic Ecc by VVI has only a limited association with CMR Ecc in DMD.

AbbreviationsCMRcardiovascular magnetic resonance

DMDDuchenne muscular dystrophy

Ecccircumferential strain


HARPharmonic phase

IQRinterquartile range

LGElate gadolinium enhancement

LVleft ventricle

LVEDVleft ventricular end-diastolic volume

LVESVleft ventricular end-systolic volume

MTTmyocardial tissue tagging.

Electronic supplementary materialThe online version of this article doi:10.1186-1532-429X-13-20 contains supplementary material, which is available to authorized users.

Daniel Drachman and W Reid Thompson contributed equally to this work.

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Author: Kenneth C Bilchick - Michael Salerno - David Plitt - Yoav Dori - Thomas O Crawford - Daniel Drachman - W Reid Thompson


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