Abstract
Background
Cardiac involvement in myotonic dystrophy type 1 (MD1) includes conduction disease, arrhythmias, and left-ventricular (LV) systolic dysfunction leading to an increased sudden cardiac death risk. An understanding of the interplay between electrical and structural myocardial changes could improve the prediction of adverse cardiac events. We aimed to explore the relationship between signs of cardiomyopathy by conventional and advanced cardiovascular magnetic resonance (CMR), and electrical abnormalities in MD1.
Methods
Fifty-seven MD1 patients (43 ± 13 years, 46% male) and 15 matched controls (41 ± 7 years, 53% male) underwent CMR including cine-imaging with feature-tracking strain analysis, late gadolinium enhancement (LGE), and native/post-contrast T1-mapping with extracellular volume calculation. Standard 12-lead and long-term ECG monitoring were performed as screening for rhythm and/or conduction abnormalities.
Results
Abnormal ECGs were recorded in 40% of MD1; a pathologic CMR was found in 44%: 21% had an impaired LV–EF and 32% showed non-ischemic LGE. When looking at MD1 patients with available long-term ECG monitoring (n = 39), those with atrial fibrillation (Afib)/flutter(Afl) episodes had lower LV–EF (52 ± 7 vs. 60 ± 5%, p = 0.002), lower global longitudinal strain (− 17 ± 3 vs. − 20 ± 3%, p = 0.034), a trend to lower left atrial emptying fraction (LA–EF) (44 ± 14 vs. 55 ± 8%, p = 0.08), and higher prevalence of LGE (88% vs. 23%, p = 0.001) with an intramural (75% vs. 23%, p = 0.01) and septal (63% vs. 13%, p = 0.009) pattern. In a model including LV–EF (OR 0.8, 95% CI 0.7–1.0, p = NS) and LGE presence (OR 14.8, 95% CI 1.4–159.0, p = 0.026), only LGE was independently associated with the occurrence of Afib/Afl episodes.
Conclusion
Myocardial abnormalities depicted by non-ischemic LGE-CMR were the only independent predictor for the occurrence of Afib/Afl on ECG monitoring, previously shown to predict adverse cardiac events in MD1.
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References
Pelargonio G, Dello Russo A, Sanna T, Martino G de, Bellocci F (2002) Myotonic dystrophy and the heart. Heart 88:665–670
Lund M, Diaz LJ, Ranthe MF, Petri H, Duno M, Juncker I et al (2014) Cardiac involvement in myotonic dystrophy: a nationwide cohort study. Eur Heart J 35:2158–2164
Petri H, Ahtarovski KA, Vejlstrup N, Vissing J, Witting N, Køber L, Bundgaard H (2014) Myocardial fibrosis in patients with myotonic dystrophy type 1: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 16:59
Groh WJ, Groh MR, Saha C, Kincaid JC, Simmons Z, Ciafaloni E et al (2008) Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. N Engl J Med 358:2688–2697
Petri H, Vissing J, Witting N, Bundgaard H, Køber L (2012) Cardiac manifestations of myotonic dystrophy type 1. Int J Cardiol 160:82–88
Petri H, Witting N, Ersbøll MK, Sajadieh A, Dunø M, Helweg-Larsen S et al (2014) High prevalence of cardiac involvement in patients with myotonic dystrophy type 1: a cross-sectional study. Int J Cardiol 174:31–36
Wahbi K, Babuty D, Probst V, Wissocque L, Labombarda F, Porcher R et al (2017) Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1. Eur Heart J 38:751–758
Brignole M, Auricchio A, Baron-Esquivias G, Bordachar P, Boriani G, Breithardt O-A et al (2013) 2013 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: the Task Force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Eur Heart J 34:2281–2329
Valaperta R, Gaeta M, Cardani R, Lombardi F, Rampoldi B, Siena C de et al (2016) High-sensitive cardiac troponin T (hs-cTnT) assay as serum biomarker to predict cardiac risk in myotonic dystrophy: a case-control study. Clin Chim Acta 463:122–128
Bhakta D, Groh MR, Shen C, Pascuzzi RM, Groh WJ (2010) Increased mortality with left ventricular systolic dysfunction and heart failure in adults with myotonic dystrophy type 1. Am Heart J 160:1137–1141, 1141.e1
Bhakta D, Lowe MR, Groh WJ (2004) Prevalence of structural cardiac abnormalities in patients with myotonic dystrophy type I. Am Heart J 147:224–227
Nguyen HH, Wolfe JT, Holmes DR, Edwards WD (1988) Pathology of the cardiac conduction system in myotonic dystrophy: a study of 12 cases. J Am Coll Cardiol 11:662–671
Rakocević-Stojanović V, Pavlović S, Seferović P, Vasiljević J, Lavrnić D, Marinković Z, Apostolski S (1999) Pathohistological changes in endomyocardial biopsy specimens in patients with myotonic dystrophy. Panminerva Med 41:27–30
Choudhary P, Nandakumar R, Greig H, Broadhurst P, Dean J, Puranik R et al (2016) Structural and electrical cardiac abnormalities are prevalent in asymptomatic adults with myotonic dystrophy. Heart 102:1472–1478
Hermans MCE, Faber CG, Bekkers SCAM, Die-Smulders CEM de, Gerrits MM, Merkies ISJ et al (2012) Structural and functional cardiac changes in myotonic dystrophy type 1: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 14:48
Motwani M, Kidambi A, Greenwood JP, Plein S (2014) Advances in cardiovascular magnetic resonance in ischaemic heart disease and non-ischaemic cardiomyopathies. Heart 100:1722–1733
Miller CA, Naish JH, Bishop P, Coutts G, Clark D, Zhao S et al (2013) Comprehensive validation of cardiovascular magnetic resonance techniques for the assessment of myocardial extracellular volume. Circ Cardiovasc Imaging 6:373–383
Taylor RJ, Moody WE, Umar F, Edwards NC, Taylor TJ, Stegemann B et al (2015) Myocardial strain measurement with feature-tracking cardiovascular magnetic resonance: normal values. Eur Heart J Cardiovasc Imaging 16:871–881
Florian A, Ludwig A, Engelen M, Waltenberger J, Rösch S, Sechtem U, Yilmaz A (2014) Left ventricular systolic function and the pattern of late-gadolinium-enhancement independently and additively predict adverse cardiac events in muscular dystrophy patients. J Cardiovasc Magn Reson 16:81
Hudsmith LE, Petersen SE, Francis JM, Robson MD, Neubauer S (2005) Normal human left and right ventricular and left atrial dimensions using steady state free precession magnetic resonance imaging. J Cardiovasc Magn Reson 7:775–782
Blume GG, Mcleod CJ, Barnes ME, Seward JB, Pellikka PA, Bastiansen PM, Tsang TSM (2011) Left atrial function: physiology, assessment, and clinical implications. Eur J Echocardiogr 12:421–430
Kawel-Boehm N, Maceira A, Valsangiacomo-Buechel ER, Vogel-Claussen J, Turkbey EB, Williams R et al (2015) Normal values for cardiovascular magnetic resonance in adults and children. J Cardiovasc Magn Reson 17:29
Habibi M, Samiei S, Venkatesh BA, Opdahl A, Helle-Valle TM, Zareian M et al (2016) CMR-measured left atrial volume and function and incident atrial fibrillation: results from the multi-ethnic study of atherosclerosis (MESA). Circ Cardiovasc Imaging. https://doi.org/10.1161/CIRCIMAGING.115.004299
Habibi M, Chahal H, Opdahl A, Gjesdal O, Helle-Valle TM, Heckbert SR et al (2014) Association of CMR-measured LA function with heart failure development: results from the MESA study. JACC Cardiovasc Imaging 7:570–579
Groh WJ, Lowe MR, Zipes DP (2002) Severity of cardiac conduction involvement and arrhythmias in myotonic dystrophy type 1 correlates with age and CTG repeat length. J Cardiovasc Electrophysiol 13:444–448
Florian A, Ludwig A, Rösch S, Yildiz H, Sechtem U, Yilmaz A (2014) Myocardial fibrosis imaging based on T1-mapping and extracellular volume fraction (ECV) measurement in muscular dystrophy patients: diagnostic value compared with conventional late gadolinium enhancement (LGE) imaging. Eur Heart J Cardiovasc Imaging 15:1004–1012
Garcia R, Rehman M, Goujeau C, Degand B, Le Gal F, Stordeur B et al (2017) Left ventricular longitudinal strain impairment predicts cardiovascular events in asymptomatic type 1 myotonic dystrophy. Int J Cardiol 243:424–430
Sado DM, White SK, Piechnik SK, Banypersad SM, Treibel T, Captur G et al (2013) Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping. Circ Cardiovasc Imaging 6:392–398
Turkbey EB, Gai N, Lima JAC, van der Geest RJ, Wagner KR, Tomaselli GF et al (2012) Assessment of cardiac involvement in myotonic muscular dystrophy by T1 mapping on magnetic resonance imaging. Heart Rhythm 9:1691–1697
Sommerville RB, Vincenti MG, Winborn K, Casey A, Stitziel NO, Connolly AM, Mann DL (2017) Diagnosis and management of adult hereditary cardio-neuromuscular disorders: a model for the multidisciplinary care of complex genetic disorders. Trends Cardiovasc Med 27:51–58
Merlevede K, Vermander D, Theys P, Legius E, Ector H, Robberecht W (2002) Cardiac involvement and CTG expansion in myotonic dystrophy. J Neurol 249:693–698
Russo V, Rago A, Ciardiello C, Russo MG, Calabrò P, Politano L, Nigro G (2016) The role of the atrial electromechanical delay in predicting atrial fibrillation in myotonic dystrophy type 1 patients. J Cardiovasc Electrophysiol 27:65–72
Haeusler KG, Gröschel K, Köhrmann M, Anker SD, Brachmann J, Böhm M et al (2018) Expert opinion paper on atrial fibrillation detection after ischemic stroke. Clin Res Cardiol 107:871–880
Jobs A, Schwind J, Katalinic A, Babaev V, Tilz RR, Rausch S et al (2018) Prognostic significance of atrial fibrillation in acute decompensated heart failure with reduced versus preserved ejection fraction. Clin Res Cardiol. https://doi.org/10.1007/s00392-018-1321-4
Bettin M, Dechering D, Kochhäuser S, Bode N, Eckardt L, Frommeyer G, Reinke F (2018) Extended ECG monitoring with an implantable loop recorder in patients with cryptogenic stroke: time schedule, reasons for explantation and incidental findings (results from the TRACK-AF trial). Clin Res Cardiol. https://doi.org/10.1007/s00392-018-1358-4
Puntmann VO, Peker E, Chandrashekhar Y, Nagel E (2016) T1 mapping in characterizing myocardial disease: a comprehensive review. Circ Res 119:277–299
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Chmielewski, L., Bietenbeck, M., Patrascu, A. et al. Non-invasive evaluation of the relationship between electrical and structural cardiac abnormalities in patients with myotonic dystrophy type 1. Clin Res Cardiol 108, 857–867 (2019). https://doi.org/10.1007/s00392-019-01414-0
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DOI: https://doi.org/10.1007/s00392-019-01414-0