PMID- 27788681 OWN - NLM STAT- MEDLINE DCOM- 20171212 LR - 20240117 IS - 1532-429X (Electronic) IS - 1097-6647 (Print) IS - 1097-6647 (Linking) VI - 18 IP - 1 DP - 2016 Oct 28 TI - Native T1 values identify myocardial changes and stratify disease severity in patients with Duchenne muscular dystrophy. PG - 72 LID - 72 AB - BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked, inherited disorder causing dilated cardiomyopathy with variable onset and progression. Currently we lack objective markers of the effect of therapies targeted towards preventing progression of subclinical cardiac disease. Thus, our aim was to compare the ability of native T1 and extracellular volume (ECV) measurements to differentiate risk of myocardial disease in DMD and controls. METHODS: Twenty boys with DMD and 16 age/gender-matched controls without history predisposing to cardiac fibrosis, but with a clinical indication for cardiovascular magnetic resonance (CMR) evaluation, underwent CMR with contrast. Data points collected include left ventricular ejection fraction (LVEF), left ventricular mass, and presence of late gadolinium enhancement (LGE). Native T1, and ECV regional mapping were obtained using both a modified Look-Locker (MOLLI) and saturation recovery single shot sequence (SASHA) on a 1.5T scanner. Using ordinal logistic regression models, controlling for age and LVEF, LGE-free septal we evaluated the ability native T1 and ECV assessments to differentiate levels of cardiomyopathy. RESULTS: Twenty DMD subjects aged 14.4 +/- 4 years had an LVEF of 56.3 +/- 7.4 %; 12/20 had LGE, all confined to the lateral wall. Sixteen controls aged 16.1 +/- 2.2 years had an LVEF 60.4 +/- 5.1 % and no LGE. Native T1 and ECV values were significantly higher in the DMD group (p < 0.05) with both MOLLI and SASHA imaging techniques. Native T1 demonstrated a 50 % increase in the ability to predict disease state (control, DMD without fibrosis, DMD with fibrosis). ECV demonstrated only the ability to predict presence of LGE, but could not distinguish between controls and DMD without fibrosis. CONCLUSIONS: LGE-spared regions of boys with DMD have significantly different native T1 and ECV values compared to controls. Native T1 measurements can identify early changes in DMD patients without the presence of LGE and help predict disease severity more effectively than ECV. Native T1 may be a novel outcome measure for early cardiac therapies in DMD and other cardiomyopathies. FAU - Olivieri, Laura J AU - Olivieri LJ AD - Division of Cardiology, Children's National Health System, 111 Michigan Avenue NW, W3-200, Washington, DC, 20010, USA. lolivier@childrensnational.org. AD - National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, 20892, USA. lolivier@childrensnational.org. FAU - Kellman, Peter AU - Kellman P AD - National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, 20892, USA. FAU - McCarter, Robert J AU - McCarter RJ AD - Children's National Health System, Clinical and Translational Science Institute, 111 Michigan Ave NW, Washington, DC, 20010, USA. FAU - Cross, Russell R AU - Cross RR AD - Division of Cardiology, Children's National Health System, 111 Michigan Avenue NW, W3-200, Washington, DC, 20010, USA. FAU - Hansen, Michael S AU - Hansen MS AD - National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, 20892, USA. FAU - Spurney, Christopher F AU - Spurney CF AD - Division of Cardiology, Children's National Health System, 111 Michigan Avenue NW, W3-200, Washington, DC, 20010, USA. AD - Children's National Health System, Center for Genetic Medicine Research, 111 Michigan Ave NW, Washington, DC, 20010, USA. LA - eng PT - Comparative Study PT - Journal Article DEP - 20161028 PL - England TA - J Cardiovasc Magn Reson JT - Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance JID - 9815616 RN - 0 (Contrast Media) RN - 0 (Organometallic Compounds) RN - 1BJ477IO2L (gadobutrol) SB - IM MH - Adolescent MH - Cardiomyopathy, Dilated/*diagnostic imaging/etiology/physiopathology MH - Case-Control Studies MH - Contrast Media/administration & dosage MH - Diagnosis, Differential MH - Equipment Design MH - Humans MH - Image Interpretation, Computer-Assisted MH - Logistic Models MH - Magnetic Resonance Imaging/instrumentation/*methods MH - Male MH - Muscular Dystrophy, Duchenne/*complications/diagnosis MH - Organometallic Compounds/administration & dosage MH - Predictive Value of Tests MH - Severity of Illness Index MH - Stroke Volume MH - Ventricular Function, Left PMC - PMC5084339 OTO - NOTNLM OT - Cardiomyopathy OT - Cardiovascular magnetic resonance OT - Duchenne muscular dystrophy OT - Pediatrics OT - T1 mapping EDAT- 2016/10/30 06:00 MHDA- 2017/12/13 06:00 PMCR- 2016/10/28 CRDT- 2016/10/30 06:00 PHST- 2016/06/28 00:00 [received] PHST- 2016/10/05 00:00 [accepted] PHST- 2016/10/30 06:00 [pubmed] PHST- 2017/12/13 06:00 [medline] PHST- 2016/10/30 06:00 [entrez] PHST- 2016/10/28 00:00 [pmc-release] AID - S1097-6647(23)01013-X [pii] AID - 292 [pii] AID - 10.1186/s12968-016-0292-8 [doi] PST - epublish SO - J Cardiovasc Magn Reson. 2016 Oct 28;18(1):72. doi: 10.1186/s12968-016-0292-8.