PMID- 26795569 OWN - NLM STAT- MEDLINE DCOM- 20161114 LR - 20240117 IS - 1532-429X (Electronic) IS - 1097-6647 (Print) IS - 1097-6647 (Linking) VI - 18 DP - 2016 Jan 21 TI - Increased myocardial native T1 and extracellular volume in patients with Duchenne muscular dystrophy. PG - 5 LID - 10.1186/s12968-016-0224-7 [doi] LID - 5 AB - BACKGROUND: Duchenne muscular dystrophy (DMD) cardiomyopathy is a progressive disease for which there is no cure. Disease-specific therapies are needed that can be initiated before irreversible myocardial damage ensues. In order to evaluate therapeutic efficacy, surrogate endpoints other than ejection fraction must be found. The hypothesis of this study is that T1 and extracellular volume fraction (ECV) mapping using cardiovascular magnetic resonance (CMR) can detect diffuse extracellular matrix expansion in DMD patients with normal left ventricular ejection fraction (LVEF) and without myocardial late gadolinium enhancement (LGE). METHODS: Thirty-one DMD and 11 healthy control participants were prospectively enrolled. CMR using a modified Look-Locker (MOLLI) sequence was performed in all participants before and after contrast administration. T1 and ECV maps of the mid left ventricular myocardium were generated and regions of interest were contoured using the standard 6-segment AHA model. Global and segmental values were compared between DMD and controls using a Wilcoxon rank-sum test. RESULTS: The DMD participants had significantly higher mean native T1 compared with controls (1045 ms vs. 988 ms, p = 0.001). DMD participants with normal LVEF and without evidence of LGE also demonstrated elevated mean native T1 (1039 ms vs. 988 ms, p = 0.002, and 1038 ms vs. 988 ms, p = 0.011). DMD participants had a significantly greater mean ECV than controls (0.31 vs. 0.24, p < 0.001), even in the settings of normal LVEF (0.28 vs. 0.24, p < 0.001) and negative LGE (0.29 vs. 0.24, p = 0.001). CONCLUSIONS: DMD participants have elevated LV myocardial native T1 and ECV, even in the setting of normal LVEF and in the absence of LGE. T1 and ECV mapping in DMD have potential to serve as surrogate cardiomyopathy outcome measures for clinical trials. FAU - Soslow, Jonathan H AU - Soslow JH AD - Thomas P Graham Jr. Division of Pediatric Cardiology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital, Vanderbilt University Medical Center, 2200 Children's Way Suite 5230, Doctors' Office Tower, Nashville, TN, 37232-9119, USA. Jonathan.h.soslow@vanderbilt.edu. FAU - Damon, Stephen M AU - Damon SM AD - Department of Electrical Engineering and Computer Sciences, Vanderbilt University, Nashville, TN, USA. Stephen.m.damon@vanderbilt.edu. FAU - Crum, Kimberly AU - Crum K AD - Thomas P Graham Jr. Division of Pediatric Cardiology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital, Vanderbilt University Medical Center, 2200 Children's Way Suite 5230, Doctors' Office Tower, Nashville, TN, 37232-9119, USA. Kimberly.crum@vanderbilt.edu. FAU - Lawson, Mark A AU - Lawson MA AD - Division of Cardiovascular Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. Mark.lawson@vanderbilt.edu. FAU - Slaughter, James C AU - Slaughter JC AD - Department of Biostatistics, Vanderbilt University Medical Center, Nashville, TN, USA. James.c.slaughter@vanderbilt.edu. FAU - Xu, Meng AU - Xu M AD - Department of Biostatistics, Vanderbilt University Medical Center, Nashville, TN, USA. Meng.xu@vanderbilt.edu. FAU - Arai, Andrew E AU - Arai AE AD - National Heart, Lung and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, MD, USA. araia@nih.gov. FAU - Sawyer, Douglas B AU - Sawyer DB AD - Department of Cardiac Services, Maine Medical Center, Portland, ME, USA. Dsawyer@mmc.org. FAU - Parra, David A AU - Parra DA AD - Thomas P Graham Jr. Division of Pediatric Cardiology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital, Vanderbilt University Medical Center, 2200 Children's Way Suite 5230, Doctors' Office Tower, Nashville, TN, 37232-9119, USA. David.parra@vanderbilt.edu. FAU - Damon, Bruce M AU - Damon BM AD - Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, Nashville, TN, USA. Bruce.damon@vanderbilt.edu. AD - Department of Molecular Physiology and Biophysics, Vanderbilt University, Nashville, TN, USA. Bruce.damon@vanderbilt.edu. AD - Department of Biomedical Engineering, Vanderbilt University, Nashville, TN, USA. Bruce.damon@vanderbilt.edu. FAU - Markham, Larry W AU - Markham LW AD - Thomas P Graham Jr. Division of Pediatric Cardiology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital, Vanderbilt University Medical Center, 2200 Children's Way Suite 5230, Doctors' Office Tower, Nashville, TN, 37232-9119, USA. Larry.markham@vanderbilt.edu. LA - eng GR - UL1 TR000445/TR/NCATS NIH HHS/United States GR - 2 UL1 TR000445-06/TR/NCATS NIH HHS/United States GR - K23HL123938/HL/NHLBI NIH HHS/United States GR - UL1 TR001425/TR/NCATS NIH HHS/United States GR - UL1 RR024975/RR/NCRR NIH HHS/United States GR - K23 HL123938/HL/NHLBI NIH HHS/United States GR - UL1 RR024975-01/RR/NCRR NIH HHS/United States PT - Journal Article PT - Observational Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20160121 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 - K2I13DR72L (Gadolinium DTPA) SB - IM MH - Adolescent MH - Adult MH - Cardiomyopathies/*etiology/*pathology/physiopathology MH - Case-Control Studies MH - Child MH - Contrast Media MH - Extracellular Matrix/*pathology MH - Female MH - Gadolinium DTPA MH - Humans MH - *Magnetic Resonance Imaging MH - Male MH - Muscular Dystrophy, Duchenne/*complications/diagnosis MH - Myocardium/*pathology MH - Predictive Value of Tests MH - Prospective Studies MH - Stroke Volume MH - Ventricular Function, Left MH - Young Adult PMC - PMC4722665 EDAT- 2016/01/23 06:00 MHDA- 2016/11/15 06:00 PMCR- 2016/01/21 CRDT- 2016/01/23 06:00 PHST- 2015/09/10 00:00 [received] PHST- 2016/01/11 00:00 [accepted] PHST- 2016/01/23 06:00 [entrez] PHST- 2016/01/23 06:00 [pubmed] PHST- 2016/11/15 06:00 [medline] PHST- 2016/01/21 00:00 [pmc-release] AID - S1097-6647(23)00946-8 [pii] AID - 224 [pii] AID - 10.1186/s12968-016-0224-7 [doi] PST - epublish SO - J Cardiovasc Magn Reson. 2016 Jan 21;18:5. doi: 10.1186/s12968-016-0224-7.