Assessment of cardiac involvement in myotonic muscular dystrophy by T1 mapping on magnetic resonance imaging

Türkbey, Evrim; Gai, Neville; Lima, João A.C.; Geest, Rob J. van der; Wagner, Kathryn R.; Tomaselli, Gordon F.; Bluemke, David A.; Nazarian, Saman

doi:10.1016/j.hrthm.2012.06.032

Cited by 49 publications

(72 citation statements)

References 22 publications

(33 reference statements)

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“…37 In contrast to our results, there was no evidence of focal fibrosis but they found shorter Significant differences were found between healthy and LGE-positive patients (*) as well as healthy and LGE-negative patients ( †). The T2 values of our patients were slightly higher compared with the control group.…”

Section: Discussioncontrasting

confidence: 99%

Cardiac Involvement in Myotonic Dystrophy Type 2 Patients With Preserved Ejection Fraction

Schmacht

Traber

Grieben

et al. 2016

Circ: Cardiovascular Imaging

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H magnetic resonance spectroscopy were performed. Extracellular volume fraction was calculated. Conduction abnormalities were diagnosed based on Groh criteria. LGE located subepicardial basal inferolateral was detectable in 22% of the patients. Extracellular volume was increased in this region and in the adjacent medial inferolateral segment (P=0.03 compared with healthy controls). In 21% of patients with DM2, fat deposits were detectable (all women). The control group showed no abnormalities. Myocardial triglycerides were not different in LGE-positive and LGE-negative subjects (P=0.47). Six patients had indicators for conduction disease (60% of LGE-positive patients and 12.5% of LGE-negative patients). Conclusions-In DM2, subclinical myocardial injury was already detectable in preserved left ventricular ejection fraction.

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Section: Discussioncontrasting

confidence: 99%

Cardiac Involvement in Myotonic Dystrophy Type 2 Patients With Preserved Ejection Fraction

Schmacht

Traber

Grieben

et al. 2016

Circ: Cardiovascular Imaging

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“…207 CMR can be requested to noninvasively assess fatty infiltration and fibrosis in the myocardium. 212,213 An electrophysiology study may be indicated in anticipation of pacemaker/ICD implantation …”

Section: Cardiac Evaluation In Dmmentioning

confidence: 99%

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Feingold¹,

Mahle²,

Auerbach³

et al. 2017

Circulation

214

195

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For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic. In this statement, we provide background on several NMDs in which there is cardiac involvement, highlighting unique features of NMD-associated myocardial disease that require clinicians to tailor their approach to prevention and treatment of heart failure. Undoubtedly, further investigations are required to best inform future guidelines on NMD-specific cardiovascular health risks, treatments, and outcomes. N euromuscular diseases (NMDs) encompass a broad spectrum of diagnoses with overlapping but distinct phenotypes. Common to many NMDs is cardiac involvement. Although the past 3 decades have seen marked advances in our understanding of many NMDs, significant gaps in knowledge remain on how best to approach cardiac care in these patients. For example, survival in Duchenne muscular dystrophy (DMD) has been extended through the use of glucocorticoid use and respiratory support, yet cardiac complications remain a significant cause of morbidity and mortality.1-3 To achieve further gains in care, we will need to improve our understanding of the pathophysiologies driving cardiac involvement in NMDs and advance treatments aimed at preventing the progression of heart failure (HF) and sudden death in NMDs. The recently published findings of an expert working group on cardiac involvement in DMD, which outlined key gaps in knowledge and made specific recommendations aimed at improving diagnosis and management, are a step toward this goal. 4 In this statement, we include a comprehensive overview of the major categories of NMDs with cardiac involvement. For each, a brief background of the gene defect(s), common clinical manifestations (particularly cardiac findings), and current therapies is summarized. Gaps in knowledge are highlighted, and where possible, clinical treatment suggestions are made by the expert writing group appointed by the American Heart Association to review the available literature. Selection of the writing group was performed in accordance with the American Heart Association's conflict-of-interest management policy. Participants volunteered to write sections relevant to their expertise and experience. CLINICAL STATEMENTS AND GUIDELINESconducted a general search of the literature, restricted to human subjects research published between 1980 and 2016. Drafts of each section were written and sent to the chair of the writing group for incorporation into a single document, which was then edited. The edited document was discussed electron...

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“…Turkbey et al (26) showed that an increase in postcontrast T1 time was associated with an increase in signal-averaged filtered QRS duration in patients with myotonic muscular dystrophy. Shah et al (27) recently reported that patients with aortic stenosis, LV hypertrophy, and strain at ECG had increased LV diffuse fibrosis and increased LV mass compared with those without LV hypertrophy or those with LV hypertrophy without strain, when the LV hypertrophy was assessed by using the…”

Section: Electrophysiologic Implications Of LV Diffuse Fibrosismentioning

confidence: 99%

Electrocardiographic Impact of Myocardial Diffuse Fibrosis and Scar: MESA (Multi-Ethnic Study of Atherosclerosis)

et al. 2017

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Purpose:To examine the associations of myocardial diffuse fibrosis and scar with surface electrocardiographic (ECG) parameters in individuals free of prior coronary heart disease in four different ethnicities. Materials and Methods:This prospective cross-sectional study was approved by the institutional review boards, and all participants gave informed consent. A total of 1669 participants in the Multi-Ethnic Study of Atherosclerosis, or MESA, who were free of prior myocardial infarction underwent both ECG and cardiac magnetic resonance imaging. In individuals without a late gadolinium enhancement-defined myocardial scar (n = 1131), T1 mapping was used to assess left ventricular (LV) interstitial diffuse fibrosis. The associations of LV diffuse fibrosis or myocardial scar with ECG parameters (QRS voltage, QRS duration, and corrected QT interval [QTc]) were evaluated by using multivariable regression analyses adjusted for demographic data, risk factors for scar, LV end-diastolic volume, and LV mass. Results:The mean age of the 1669 participants was 67.4 years 6 8.7 (standard deviation); 49.8% were women. Lower postcontrast T1 time at 12 minutes was significantly associated with lower QRS Sokolow-Lyon voltage (b = 15.1 µV/10 msec, P = .004), lower QRS Cornell voltage (b = 9.2 µV/10 msec, P = .031), and shorter QRS duration (b = 0.16 msec/10 msec, P = .049). Greater extracellular volume (ECV) fraction was also significantly associated with lower QRS Sokolow-Lyon voltage (b = 235.2 µV/1% ECV increase, P , .001) and Cornell voltage (b = 223.7 µV/1% ECV increase, P , .001), independent of LV structural indexes. In contrast, the presence of LV scar (n = 106) was associated with longer QTc (b = 4.3 msec, P = .031). Conclusion:In older adults without prior coronary heart disease, underlying greater LV diffuse fibrosis is associated with lower QRS voltage and shorter QRS duration at surface ECG, whereas clinically unrecognized myocardial scar is associated with a longer QT interval.q RSNA, 2016

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Assessment of cardiac involvement in myotonic muscular dystrophy by T1 mapping on magnetic resonance imaging

Cited by 49 publications

References 22 publications

Cardiac Involvement in Myotonic Dystrophy Type 2 Patients With Preserved Ejection Fraction

Cardiac Involvement in Myotonic Dystrophy Type 2 Patients With Preserved Ejection Fraction

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Electrocardiographic Impact of Myocardial Diffuse Fibrosis and Scar: MESA (Multi-Ethnic Study of Atherosclerosis)

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