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Research progress of magnetic resonance imaging in Duchenne muscular dystrophy cardiomyopathy
XU Ke  GUO Yingkun  XU Huayan 

Cite this article as: XU K, GUO Y K, XU H Y. Research progress of magnetic resonance imaging in Duchenne muscular dystrophy cardiomyopathy[J]. Chin J Magn Reson Imaging, 2023, 14(4): 154-159. DOI:10.12015/issn.1674-8034.2023.04.027.

[Abstract] Cardiomyopathy is the leading cause of death in patients with Duchenne muscular dystrophy (DMD) to date. Cardiac magnetic resonance (CMR) provides an accurate and highly reproducible technique for assessing cardiac structure and function, and late gadolinium enhanced (LGE) is playing an increasingly important role in the diagnosis and clinical treatment of DMD cardiomyopathy. Meanwhile, new technologies such as T1 mapping, T2 mapping, and feature tracking have also been widely used in DMD cardiomyopathy. Currently, the CMR studies of DMD population analysed the patterns of cardiac involvement, but have not confirmed the evolution of cardiac disease over time and established the added value of CMR to echocardiography and the prognostic significance of CMR findings. Thus, the authors reviewed the pathophysiology of DMD cardiomyopathy, commonly used imaging methods and the application progress of CMR in the diagnosis and follow-up of DMD cardiomyopathy, in order to serve as a reference for the early diagnosis, quantitative evaluation and risk stratification of cardiac injury in DMD patients.
[Keywords] cardiomyopathy;Duchenne muscular dystrophy;magnetic resonance imaging;T1 mapping;T2 mapping;late gadolinium enhanced;feature tracking;early diagnosis

XU Ke   GUO Yingkun   XU Huayan*  

Department of Radiology, West China Second University Hospital, Sichuan University, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Chengdu 610041, China

Corresponding author: Xu YH, E-mail:

Conflicts of interest   None.

ACKNOWLEDGMENTS National Natural Science Foundation of Chian (No. 82271981, 81901712).
Received  2022-11-29
Accepted  2023-04-07
DOI: 10.12015/issn.1674-8034.2023.04.027
Cite this article as: XU K, GUO Y K, XU H Y. Research progress of magnetic resonance imaging in Duchenne muscular dystrophy cardiomyopathy[J]. Chin J Magn Reson Imaging, 2023, 14(4): 154-159. DOI:10.12015/issn.1674-8034.2023.04.027.

ARAUJO A P Q C, NARDES F, FORTES C P D D, et al. Brazilian consensus on Duchenne muscular dystrophy. Part 2: rehabilitation and systemic care[J]. Arq Neuropsiquiatr, 2018, 76(7): 481-489. DOI: 10.1590/0004-282X20180062.
DUAN D S, GOEMANS N, TAKEDA S, et al. Duchenne muscular dystrophy[J/OL]. Nat Rev Dis Primers, 2021, 7(1): 13 [2023-03-30]. DOI: 10.1038/s41572-021-00248-3.
SCHULTZ T I, RAUCCI F J, SALLOUM F N. Cardiovascular disease in Duchenne muscular dystrophy: overview and insight into novel therapeutic targets[J]. JACC Basic Transl Sci, 2022, 7(6): 608-625. DOI: 10.1016/j.jacbts.2021.11.004.
BIRNKRANT D J, BUSHBY K, BANN C M, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management[J]. Lancet Neurol, 2018, 17(4): 347-361. DOI: 10.1016/S1474-4422(18)30025-5.
WILTON-CLARK H, YOKOTA T. Recent trends in antisense therapies for Duchenne muscular dystrophy[J/OL]. Pharmaceutics, 2023, 15(3): 778 [2023-03-30]. DOI: 10.3390/pharmaceutics15030778.
ADORISIO R, MENCARELLI E, CANTARUTTI N, et al. Duchenne dilated cardiomyopathy: cardiac management from prevention to advanced cardiovascular therapies[J/OL]. J Clin Med, 2020, 9(10): 3186 [2023-03-30]. DOI: 10.3390/jcm9103186.
CHRZANOWSKI S M, DARRAS B T, RUTKOVE S B. The value of imaging and composition-based biomarkers in Duchenne muscular dystrophy clinical trials[J]. Neurotherapeutics, 2020, 17(1): 142-152. DOI: 10.1007/s13311-019-00825-1.
CONNUCK D M, SLEEPER L A, COLAN S D, et al. Characteristics and outcomes of cardiomyopathy in children with Duchenne or Becker muscular dystrophy: a comparative study from the Pediatric Cardiomyopathy Registry[J]. Am Heart J, 2008, 155(6): 998-1005. DOI: 10.1016/j.ahj.2008.01.018.
LEE S, LEE M, HOR K N. The role of imaging in characterizing the cardiac natural history of Duchenne muscular dystrophy[J]. Pediatr Pulmonol, 2021, 56(4): 766-781. DOI: 10.1002/ppul.25227.
FOX H, MILLINGTON L, MAHABEER I, et al. Duchenne muscular dystrophy[J/OL]. BMJ, 2020, 368: l7012 [2023-03-30]. DOI: 10.1136/bmj.l7012.
BUSHBY K, FINKEL R, BIRNKRANT D J, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care[J]. Lancet Neurol, 2010, 9(2): 177-189. DOI: 10.1016/S1474-4422(09)70272-8.
POONJA S, POWER A, MAH J K, et al. Current cardiac imaging approaches in Duchenne muscular dystrophy[J]. J Clin Neuromuscul Dis, 2018, 20(2): 85-93. DOI: 10.1097/CND.0000000000000204.
GRIGORATOS C, AIMO A, BARISON A, et al. Cardiac magnetic resonance in patients with muscular dystrophies[J]. Eur J Prev Cardiol, 2021, 28(14): 1526-1535. DOI: 10.1177/2047487320923052.
BUDDHE S, LEWIN M, OLSON A, et al. Comparison of left ventricular function assessment between echocardiography and MRI in Duchenne muscular dystrophy[J]. Pediatr Radiol, 2016, 46(10): 1399-1408. DOI: 10.1007/s00247-016-3622-y.
MAFORO N G, MAGRATH P, MOULIN K, et al. T1-Mapping and extracellular volume estimates in pediatric subjects with Duchenne muscular dystrophy and healthy controls at 3T[J/OL]. J Cardiovasc Magn Reson, 2020, 22(1): 85 [2023-03-30]. DOI: 10.1186/s12968-020-00687-z.
PANOVSKÝ R, PEŠL M, HOLEČEK T, et al. Cardiac profile of the Czech population of Duchenne muscular dystrophy patients: a cardiovascular magnetic resonance study with T1 mapping[J/OL]. Orphanet J Rare Dis, 2019, 14(1): 10 [2023-03-30]. DOI: 10.1186/s13023-018-0986-0.
DUAL S A, MAFORO N G, MCELHINNEY D B, et al. Right ventricular function and T1-mapping in boys with Duchenne muscular dystrophy[J]. J Magn Reson Imaging, 2021, 54(5): 1503-1513. DOI: 10.1002/jmri.27729.
MEHMOOD M, AMBACH S A, TAYLOR M D, et al. Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy [J]. Pediatr Cardiol, 2016, 37(5): 878-883. DOI: 10.1007/s00246-016-1362-2.
PRAKASH N, SUTHAR R, SIHAG B K, et al. Cardiac MRI and echocardiography for early diagnosis of cardiomyopathy among boys with Duchenne muscular dystrophy: a cross-sectional study[J/OL]. Front Pediatr, 2022, 10: 818608 [2023-03-30]. DOI: 10.3389/fped.2022.818608.
MAVROGENI S, GIANNAKOPOULOU A, PAPAVASILIOU A, et al. Cardiac profile of asymptomatic children with Becker and Duchenne muscular dystrophy under treatment with steroids and with/without perindopril[J/OL]. BMC Cardiovasc Disord, 2017, 17(1): 197 [2023-03-30]. DOI: 10.1186/s12872-017-0627-x.
XIE L J, XU R, XU Z Y, et al. Myocardial motion-corrected phase-sensitive inversion recovery late gadolinium enhancement in free breathing paediatric patients: a comparison with single-shot coherent gradient echo ("TrueFISP") phase-sensitive inversion recovery[J/OL]. Clin Radiol, 2021, 76(6): 471.e17-471.e25 [2023-03-30]. DOI: 10.1016/j.crad.2021.01.018.
YILMAZ A, GDYNIA H J, BACCOUCHE H, et al. Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach[J/OL]. J Cardiovasc Magn Reson, 2008, 10(1): 50 [2023-03-30]. DOI: 10.1186/1532-429X-10-50.
SANCHEZ F, WEITZ C, GUTIERREZ J M, et al. Cardiac MR imaging of muscular dystrophies[J]. Curr Probl Diagn Radiol, 2022, 51(2): 225-234. DOI: 10.1067/j.cpradiol.2020.12.010.
ALMOGHEER B, ANTONOPOULOS A S, AZZU A, et al. Diagnostic and prognostic value of cardiovascular magnetic resonance in neuromuscular cardiomyopathies[J]. Pediatr Cardiol, 2022, 43(1): 27-38. DOI: 10.1007/s00246-021-02686-y.
FLORIAN A, LUDWIG A, ENGELEN M, et al. Left ventricular systolic function and the pattern of late-gadolinium-enhancement independently and additively predict adverse cardiac events in muscular dystrophy patients[J/OL]. J Cardiovasc Magn Reson, 2014, 16(1): 81 [2023-03-30]. DOI: 10.1186/s12968-014-0081-1.
HOR K N, TAYLOR M D, AL-KHALIDI H R, et al. Prevalence and distribution of late gadolinium enhancement in a large population of patients with Duchenne muscular dystrophy: effect of age and left ventricular systolic function[J/OL]. J Cardiovasc Magn Reson, 2013, 15(1): 107 [2023-03-30]. DOI: 10.1186/1532-429X-15-107.
TANDON A, VILLA C R, HOR K N, et al. Myocardial fibrosis burden predicts left ventricular ejection fraction and is associated with age and steroid treatment duration in Duchenne muscular dystrophy[J/OL]. J Am Heart Assoc, 2015, 4(4): e001338 [2023-03-30]. DOI: 10.1161/JAHA.114.001338.
HENSON S E, LANG S M, KHOURY P R, et al. The effect of adiposity on cardiovascular function and myocardial fibrosis in patients with Duchenne muscular dystrophy[J/OL]. J Am Heart Assoc, 2021, 10(19): e021037 [2023-03-30]. DOI: 10.1161/JAHA.121.021037.
JAMES L, MENTEER J, MOSS L C, et al. Early-onset late gadolinium enhancement is a prognostic factor for Duchenne cardiomyopathy[J]. Pediatr Cardiol, 2023, 44(2): 433-440. DOI: 10.1007/s00246-022-02989-8.
FOGEL M A, ANWAR S, BROBERG C, et al. Society for Cardiovascular Magnetic Resonance/European Society of Cardiovascular Imaging/American Society of Echocardiography/Society for Pediatric Radiology/North American Society for Cardiovascular Imaging Guidelines for the use of cardiovascular magnetic resonance in pediatric congenital and acquired heart disease: endorsed by The American Heart Association[J/OL]. J Cardiovasc Magn Reson, 2022, 24(1): 37 [2023-03-30]. DOI: 10.1186/s12968-022-00843-7.
MOTOKI T, SHIMIZU-MOTOHASHI Y, SAITO I, et al. Renal dysfunction can occur in advanced-stage Duchenne muscular dystrophy[J]. Muscle Nerve, 2020, 61(2): 192-197. DOI: 10.1002/mus.26757.
XU K, XU H Y, XU R, et al. Global, segmental and layer specific analysis of myocardial involvement in Duchenne muscular dystrophy by cardiovascular magnetic resonance native T1 mapping[J/OL]. J Cardiovasc Magn Reson, 2021, 23(1): 110 [2023-03-30]. DOI: 10.1186/s12968-021-00802-8.
VOLETI S, OLIVIERI L, HAMANN K, et al. Troponin I levels correlate with cardiac MR LGE and native T1 values in Duchenne muscular dystrophy cardiomyopathy and identify early disease progression[J]. Pediatr Cardiol, 2020, 41(6): 1173-1179. DOI: 10.1007/s00246-020-02372-5.
THOMAS K E, FOTAKI A, BOTNAR R M, et al. Imaging methods: magnetic resonance imaging[J/OL]. Circ Cardiovasc Imaging, 2023, 16(1): e014068 [2023-03-30]. DOI: 10.1161/CIRCIMAGING.122.014068.
OLIVIERI L J, KELLMAN P, MCCARTER R J, et al. Native T1 values identify myocardial changes and stratify disease severity in patients with Duchenne muscular dystrophy[J/OL]. J Cardiovasc Magn Reson, 2016, 18(1): 72 [2023-03-30]. DOI: 10.1186/s12968-016-0292-8.
MAVROGENI S, TZELEPIS G E, ATHANASOPOULOS G, et al. Cardiac and sternocleidomastoid muscle involvement in Duchenne muscular dystrophy: an MRI study[J]. Chest, 2005, 127(1): 143-148. DOI: 10.1378/chest.127.1.143.
MAVROGENI S, PAPAVASILIOU A, SPARGIAS K, et al. Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study[J/OL]. BMC Neurol, 2010, 10: 33 [2023-03-30]. DOI: 10.1186/1471-2377-10-33.
HAGENBUCH S C, GOTTLIEBSON W M, WANSAPURA J, et al. Detection of progressive cardiac dysfunction by serial evaluation of circumferential strain in patients with Duchenne muscular dystrophy[J]. Am J Cardiol, 2010, 105(10): 1451-1455. DOI: 10.1016/j.amjcard.2009.12.070.
GAUR L, HANNA A, BANDETTINI W P, et al. Upper arm and cardiac magnetic resonance imaging in Duchenne muscular dystrophy[J]. Ann Clin Transl Neurol, 2016, 3(12): 948-955. DOI: 10.1002/acn3.367.
EARL C C, SOSLOW J H, MARKHAM L W, et al. Myocardial strain imaging in Duchenne muscular dystrophy[J/OL]. Front Cardiovasc Med, 2022, 9: 1031205 [2023-03-30]. DOI: 10.3389/fcvm.2022.1031205.
LANGE T, SCHUSTER A. Quantification of myocardial deformation applying CMR-feature-tracking-all about the left ventricle?[J]. Curr Heart Fail Rep, 2021, 18(4): 225-239. DOI: 10.1007/s11897-021-00515-0.
EARL C C, PYLE V I, CLARK S Q, et al. Localized strain characterization of cardiomyopathy in Duchenne muscular dystrophy using novel 4D kinematic analysis of cine cardiovascular magnetic resonance[J/OL]. J Cardiovasc Magn Reson, 2023, 25(1): 14 [2023-03-30]. DOI: 10.1186/s12968-023-00922-3.
PANOVSKÝ R, PEŠL M, MÁCHAL J, et al. Quantitative assessment of left ventricular longitudinal function and myocardial deformation in Duchenne muscular dystrophy patients[J/OL]. Orphanet J Rare Dis, 2021, 16(1): 57 [2023-03-30]. DOI: 10.1186/s13023-021-01704-9.
AZZU A, ANTONOPOULOS A S, KRUPICKOVA S, et al. Myocardial strain analysis by cardiac magnetic resonance 3D feature-tracking identifies subclinical abnormalities in patients with neuromuscular disease and no overt cardiac involvement[J]. Eur Heart J Cardiovasc Imaging, 2023, 24(4): 503-511. DOI: 10.1093/ehjci/jeac129.
SIEGEL B, OLIVIERI L, GORDISH-DRESSMAN H, et al. Myocardial strain using cardiac MR feature tracking and speckle tracking echocardiography in Duchenne muscular dystrophy patients[J]. Pediatr Cardiol, 2018, 39(3): 478-483. DOI: 10.1007/s00246-017-1777-4.
HOR K N, MAZUR W, TAYLOR M D, et al. Effects of steroids and angiotensin converting enzyme inhibition on circumferential strain in boys with Duchenne muscular dystrophy: a cross-sectional and longitudinal study utilizing cardiovascular magnetic resonance[J/OL]. J Cardiovasc Magn Reson, 2011, 13(1): 60 [2023-03-30]. DOI: 10.1186/1532-429X-13-60.
RAUCCI F J, XU M, GEORGE-DURRETT K, et al. Non-contrast cardiovascular magnetic resonance detection of myocardial fibrosis in Duchenne muscular dystrophy[J/OL]. J Cardiovasc Magn Reson, 2021, 23(1): 48 [2023-03-30]. DOI: 10.1186/s12968-021-00736-1.
SIDDIQUI S, ALSAIED T, HENSON S E, et al. Left ventricular magnetic resonance imaging strain predicts the onset of Duchenne muscular dystrophy-associated cardiomyopathy[J/OL]. Circ Cardiovasc Imaging, 2020, 13(11): e011526 [2023-03-30]. DOI: 10.1161/CIRCIMAGING.120.011526.

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