Left ventricular longitudinal strain impairment predicts cardiovascular events in asymptomatic type 1 myotonic dystrophy

Garcia, Rodrigue; Rehman, Michaela B.; Goujeau, Cyril; Degand, Bruno; Gal, François Le; Stordeur, B.; Labarre, Q.; Christiaens, Luc; Bouleti, Claire

doi:10.1016/j.ijcard.2017.05.061

Cited by 11 publications

(11 citation statements)

References 24 publications

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“…This agrees with previous pathological studies conducted on DM1 patients, which found the septum to be more prone to fibrosis than other left ventricular zones (9). Furthermore, a study by Garcia et al (15) states that impaired strain obtained by echocardiography may be a predictor of adverse events in DM1 patients. We may thus hypothesize the same to be true for CMR-derived strain, since a strong correlation between CMR and echocardiographic strain values has already been proven (30).…”

Section: Discussionsupporting

confidence: 91%

“…Moreover, CMR allows for the calculation of functional parameters such as cardiac strain, an indicator of cardiac contractility, which can be impaired when the myocardium is fibrotic or degenerated (12). Cardiac strain can also be obtained through echocardiography (13,14) and serve as a predictor of cardiovascular events in asymptomatic DM1 patients (15). However, a study by Khan et al (16) showed that strain parameters obtained at CMR are more reliable and reproducible than those obtained at echocardiography due to high dependence on the operator and lower acoustic window quality of the latter technique compared to the former.…”

Section: Introductionmentioning

confidence: 99%

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Rare Disease: Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1

et al. 2020

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Introduction: To evaluate myocardial strain and extracellular volume in myotonic dystrophy type 1 (DM1) patients as potential imaging biomarkers of subclinical cardiac pathology. Materials and methods:We retrospectively analyzed 9 DM1 patients without apparent cardiac disease who had undergone cardiac magnetic resonance at our center. Patients were age-and sex-matched with healthy controls. The Mann-Whitney U test was used to compare cardiac strain between the two groups. The t-test was used to compare the extracellular volume obtained in DM1 patients with that in healthy subject. Spearman's ρ was used for studying the associations among imaging parameters.Results: Global cardiac strain (median −19.1%; IQR −20.5%, −16.5%) in DM1 patients was lower (p = 0.011) than that in controls (median−21.7%; IQR−22.7%,-21.3%). Global extracellular volume in DM1 patients (median 32.3%; IQR 29.3%,36.8%) was significantly (p = 0.008) higher than that reported in literature in healthy subjects (median 25.6%; IQR 19.9%,31.9%). Global cardiac strain showed a strong, positive correlation with septal strain (ρ = 0.767, p = 0.016) and with both global (ρ = 0.733 p = 0.025) and septal extracellular volume (ρ = 0.767, p = 0.016). Discussion:The increase in cardiac extracellular volume and decrease in strain are signs of early cardiac pathology in DM1. Physicians dealing with DM1 may take into consideration cardiac magnetic resonance as a screening tool to identify early cardiac involvement in this condition.

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Section: Discussionsupporting

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Rare Disease: Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1

et al. 2020

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“…25 GLS was similar to or slightly higher than in previous reports using different methodology. 26,27 Particularly in apical wall regions of the LV, we detected slightly decreased myocardial strain in DM patients, which did not significantly affect global longitudinal systolic strain (GLS) or LV EF, similar to previous findings. 28 Non-ischaemic LGE is a cMR marker of regional interstitial fibrosis.…”

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confidence: 91%

Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies

et al. 2020

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Aims Cardiac involvement in myopathies that primarily affect the skeletal muscle is variable and may be subtle, necessitating sensitive diagnostic approaches. Here, we describe the prevalence of cardiac abnormalities in a cohort of patients with skeletal muscle disease presenting at a tertiary care neuromuscular centre. Methods and results We systematically investigated patients with skeletal myopathies and comprehensively analysed their cardiac phenotype including 24 h electrocardiogram, echocardiography with strain analyses, contrast-enhanced cardiac magnetic resonance imaging, and, if at increased risk of coronary artery disease, computed tomography coronary angiography. We prospectively screened 91 patients with diverse skeletal myopathies and enrolled 73 patients. The most pronounced cardiac involvement was present in patients with dystrophic myopathies (cardiac abnormalities in 59% of patients). We analysed myotonic dystrophies (n = 29) in more detail and found prolonged QRS (99.4 ± 15.6 vs. 91.5 ± 10.3 ms; P = 0.027) and QTc times (441.1 ± 28.1 vs. 413.0 ± 23.3 ms; P < 0.001) and increased left atrial size (27.28 ± 3.9 vs. 25.0 ± 3.2 mm/m 2 ; P = 0.021) when compared with healthy controls. Left ventricular systolic function was reduced (ejection fraction < 55%) in 31% of myotonic dystrophies, while only 4% had an ejection fraction < 50%. Apical peak systolic longitudinal strain was slightly reduced (P = 0.023). Conclusions Screening for cardiac involvement in the skeletal muscle disease seems prudent particularly in patients with dystrophic myopathies. In the subset of myotonic dystrophy patients, QRS and QTc times as well as myocardial strain may be useful parameters. Their potential for predicting cardiac adverse events needs further evaluation.

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“…Despite more and more studies in the literature, cardiac management of asymptomatic DM1 is not codified and identification of tools enabling to predict these complications is crucial (Lau et al, 2015). Several parameters have been described as predictors of cardiovascular events: age, left bundle branch block, syncope, atrial fibrillation, longitudinal strain, left ventricular ejection fraction (Bhakta et al, 2010;Garcia, Labarre, et al, 2017;Garcia, Rehman, et al, 2017;Lau et al, 2015;Wahbi et al, 2017). Nevertheless, the relevancy of 24-hour holter ECG, which is currently recommended, has never been assessed (Lau et al, 2015).…”

Section: Discussionmentioning

confidence: 99%

“…Despite the fact that 30% of deaths are caused by arrhythmias, most of the studies seem to agree on the difficulty of predicting cardiac disorders in DM1 patients (Pelargonio et al, 2002). Several predictors of cardiovascular events have been described but DM1 patient follow-up is not standardized (Bhakta, Groh, Shen, Pascuzzi, & Groh, 2010;Garcia, Labarre, et al, 2017;Garcia, Rehman, et al, 2017;Lau, Sy, Corbett, & Kritharides, 2015;Wahbi et al, 2017).…”

Section: Backg Rou N Dmentioning

confidence: 99%

Twenty‐four‐hour ambulatory ECG monitoring relevancy in myotonic dystrophy type 1 follow‐up: Prognostic value and heart rate variability evolution

Gamet

Degand

Gal

et al. 2018

Noninvasive Electrocardiol

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Left ventricular longitudinal strain impairment predicts cardiovascular events in asymptomatic type 1 myotonic dystrophy

Cited by 11 publications

References 24 publications

Rare Disease: Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1

Rare Disease: Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1

Cardiac involvement in a cross‐sectional cohort of myotonic dystrophies and other skeletal myopathies

Twenty‐four‐hour ambulatory ECG monitoring relevancy in myotonic dystrophy type 1 follow‐up: Prognostic value and heart rate variability evolution

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