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

doi:10.1016/j.ijcard.2017.05.061

International Journal of Cardiology

Volume 243, 15 September 2017, Pages 424-430

https://doi.org/10.1016/j.ijcard.2017.05.061 Get rights and content

Abstract

Background

Type 1 myotonic dystrophy (DM1) patients' prognosis is very poor. Up until now, only a few prognostic factors for cardiovascular events have been identified, and they are predictive of end-stage disease. The aim was to assess the prognostic value of global longitudinal strain (GLS) for cardiovascular events in asymptomatic DM1 patients.

Methods

DM1 patients were included between 2011 and 2015 and followed up until January 2016. Patients underwent a transthoracic echocardiography at inclusion. The primary endpoint was a composite of all-cause mortality, type 2 Mobitz 2 and type 3 atrioventricular block, symptomatic sino-atrial block, HV interval ≥ 70 ms at invasive electrophysiology exploration, left ventricular ejection fraction (LVEF) ≤ 45% and newly developed atrial fibrillation.

Results

Forty-six patients (25 males, mean age 40 years old) were included. The primary outcome was reached in 14 patients with a mean follow-up of 38 months. GLS of patients who reached the primary endpoint was significantly impaired as compared to those who did not (− 15.1 [− 16.7; − 12.7] vs. − 18.2 [− 19.2; − 16.7] respectively; P = 0.001). According to ROC curve analysis, probability of primary outcome occurrence was significantly greater in patients with GLS values ≥ − 17.2% (P = 0.001). On multivariate analysis, PR electrocardiogram interval and GLS remained significantly and independently associated with the primary endpoint [hazard ratio (HR) 1.03, 95% confidence interval (CI) 1.01–1.04, P = 0.006 for PR interval; HR 1.4, 95% CI 1.1–1.7, P = 0.002 for GLS] while LVEF alone was not.

Conclusion

Left ventricular GLS is a powerful marker to predict cardiovascular events in asymptomatic DM1 patients, independently of LVEF.

Introduction

Type 1 myotonic dystrophy (DM1) is the most frequent myotonia in adults [1]. Prognosis is very poor as only 18% of patients reach the age of 65 years [2] and its evolution is marked by cardiac and respiratory complications.

Prognostic value of left ventricular systolic function has already been demonstrated in DM1. After a follow-up of 9.2 years, DM1 patients with left ventricular ejection fraction (LVEF) < 50% had a 5.7 fold increased risk of cardiac death compared to patients with LVEF ≥ 50% [3]. New tools have been developed for myocardial systolic evaluation. Among them, left ventricular strain could be more reliable than LVEF assessment and would allow earlier screening of deformation abnormalities [4], [5], [6], [7], [8], [9]. Longitudinal strain impairment has already been demonstrated in DM1 patients compared to healthy controls [10], [11], [12]. Nevertheless, the mid-term prognostic value of longitudinal strain has never been evaluated in DM1 patients.

The aim of this study was to determine whether global longitudinal strain (GLS) impairment could predict cardiovascular events in asymptomatic DM1 patients. It would therefore identify patients at high cardiac risk, before serious myocardial impairment, as encountered in impaired LVEF.

Section snippets

Patients

All consecutive DM1 patients referred to the Cardiology department of Poitiers University Hospital were screened for eligibility between January 2011 and January 2015 in this prospective study. Patients were excluded in case of age < 18 years, poor echogenicity, history of coronary artery disease, significant valvulopathy, left ventricular segmental kinetic abnormality at trans-thoracic echocardiography, type 2 Mobitz 2 and type 3 atrioventricular block, symptomatic sino-atrial block, clinical

Population characteristics

Out of the 52 consecutive patients eligible to participate in study, 6 were not included. The reasons for non-inclusion were: insufficient echogenicity (n = 1), history of atrial fibrillation (n = 1), presence of a pacemaker (n = 2) and LVEF < 45% (n = 2) (Fig. 1). Forty-six patients (25 males, mean age 40 [29; 49] years old) were finally included in the study. Baseline characteristics are summarized in Table 1. Five (11%) had dyslipidemia, 1 (2%) had type 2 diabetes, 13 (28%) were current smokers and 9

Discussion

While longitudinal strain impairment has previously been highlighted in the clinical evolution of DM1 patients, its prognostic value has never been assessed. Our data suggest that left ventricular longitudinal strain, particularly GLS, is a powerful marker to predict cardiovascular events in asymptomatic DM1 patients. Even in the type 1 atrioventricular block subgroup, these results were confirmed. After adjustment for potential confounding factors, only a longer PR electrocardiogram interval

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Conflict of interest

None.

Acknowledgments

We thank Jeffrey Arsham for proof-reading this article.

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Cited by (9)

Outcome measures frequently used to assess muscle strength in patients with myotonic dystrophy type 1: a systematic review
2022, Neuromuscular Disorders
Citation Excerpt :
Main findings of the measures used to assess cardiac muscle strength for each study can be found in Table 1 and Fig. 2A. Left ventricular ejection fraction (n = 21) [34–37,39–47,49–52,105,106,112,113], left ventricular shortening fraction (n = 4) [39,46,51,52], left ventricular global longitudinal strain (n = 3) [43,44,106], endocardial shortening fraction (n = 2) [47,48] and stroke volume (n = 1) [46] were evaluated (Table 1). The range of left ventricular ejection fraction values obtained was 56%−70% for patients with DM1 and 61%−77% for controls [34–37,39–47,49–52,105,112,113] (Table 1).
Measurement of muscle strength is fundamental for the management of patients with myotonic dystrophy type 1 (DM1). Nevertheless, guidance on this topic is somewhat limited due to heterogeneous outcome measures used. This systematic literature review aimed to summarize the most frequent outcome measures to assess muscle strength in patients with DM1. We searched on Pubmed, Web of Science and Embase databases. Observational studies using measures of muscle strength assessment in adult patients with DM1 were included. From a total of 80 included studies, 24 measured cardiac, 45 skeletal and 23 respiratory muscle strength. The most common method and outcome measures used to assess cardiac muscle strength were echocardiography and ejection fraction, for skeletal muscle strength were quantitative muscle test, manual muscle test and maximum isometric torque and medical research council and for respiratory muscle strength were manometry and maximal inspiratory and expiratory pressure. We successfully gathered the more consensual methods and measures to evaluate muscle strength in future clinical studies, particularly to test muscle strength response to treatments in patients with DM1. Future consensus on a set of measures to evaluate muscle strength (core outcome set), is important for these patients.
Three-dimensional speckle tracking echocardiography: The future is now
2018, Revista Portuguesa de Cardiologia
Importance of three-dimensional speckle tracking in the assessment of left atrial and ventricular dysfunction in patients with myotonic dystrophy type 1
2018, Revista Portuguesa de Cardiologia
Citation Excerpt :
In one study of 36 DM1 patients, serial Holter monitoring resulted in pacemaker implantation in 11 patients,26 and implantable loop recorders have been used in small studies of asymptomatic patients, leading to the detection of ventricular arrhythmias and device implantation in four out of seven patients.27,28 A recently published study demonstrated the prognostic value of LV GLS in patients with asymptomatic DM1, concluding that GLS constitutes a strong predictor of cardiovascular events.29 3D wall motion tracking uses a box template to detect speckle motion in 3D.
Myotonic dystrophy type 1 (DM1) is a multisystem disease in which cardiac involvement is common. The aim of this study was to identify early changes in left atrial (LA) mechanics and left ventricular (LV) systolic function in patients with myotonic dystrophy type 1 using three-dimensional (3D) speckle tracking echocardiography (3D-STE).
This observational study included 25 patients with DM1 and 25 healthy volunteers. We assessed LA and LV global strain parameters using 3D-STE.
Patients with DM1 showed significantly lower longitudinal LA strain (22.85%±5.06 vs. 26.82%±5.15; p=0.008 in univariate analysis and p=0.026 in multivariate analysis) and global LV longitudinal strain (-13.55%±1.82 vs. -16.11%±1.33; p<0.001 in univariate analysis and p<0.001 in multivariate analysis), which was not observed with LA area tracking (p=0.412) or LV global circumferential strain (p=0.879), global radial strain (p=0.058), area tracking (p=0.092) or twist (p=0.992).
LA and LV global longitudinal strain is significantly decreased in patients with DM1, which may be an early marker of subclinical dysfunction in these patients.
A distrofia miotónica do tipo 1 é uma doença multissistémica com o envolvimento cardíaco a ser comum. O objetivo deste estudo foi identificar alterações precoces no strain da aurícula esquerda e função sistólica do ventrículo esquerdo em doentes com distrofia miotónica tipo 1, com o uso da tecnologia 3D speckle tracking.
Estudo observacional, que incluiu 25 doentes com distrofia miotónica do tipo 1 e 25 voluntários saudáveis. Foram avaliados os parâmetros de deformação global da aurícula esquerda e do ventrículo com o uso de 3D speckle tracking.
Os doentes com distrofia miotónica do tipo 1 apresentaram strain longitudinal da aurícula esquerda (22,85%±5,06 versus 26,82%±5,15; p=0,008 na análise univariada e p=0,026 na análise multivariada) e strain global longitudinal do ventrículo esquerdo (-13,55%±1,82 versus -16,11%±1,33, p<0,001 na análise univariada e p<0,001 na análise multivariada) significativamente menores, o que não se verificou com a area tracking da aurícula esquerda (p=0,412) ou com o strain global circunferencial (p=0,879), strain global radial (p=0,058), area tracking (p=0,092) e twist (p=0,992) do ventrículo esquerdo.
O strain longitudinal global do ventrículo esquerdo e da aurícula esquerda encontra-se significativamente diminuído em doentes com distrofia miotónica do tipo 1, pode ser um marcador precoce de disfunção subclínica nesaes doentes.
Echocardiographic Features of Cardiac Involvement in Myotonic Dystrophy 1: Prevalence and Prognostic Value
2023, Journal of Clinical Medicine
Cardiac Involvement and Arrhythmias Associated with Myotonic Dystrophy
2022, Reviews in Cardiovascular Medicine
Cardiac involvement in a cross-sectional cohort of myotonic dystrophies and other skeletal myopathies
2020, ESC Heart Failure

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^☆: Acknowledgment for grant support: There was no grant support in this study.

¹: This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

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

Abstract

Background

Methods

Results

Conclusion

Introduction

Section snippets

Patients

Population characteristics

Discussion

Funding

Conflict of interest

Acknowledgments

Am. Heart J.

J. Am. Soc. Echocardiogr.

Am. Heart J.

Int. J. Cardiol.

Int. J. Cardiol.

J. Am. Soc. Echocardiogr.

Int. J. Cardiol.

J. Am. Coll. Cardiol.

Am. J. Med.

Int. J. Cardiol.

JACC Cardiovasc. Imaging

J. Am. Coll. Cardiol.

J. Am. Soc. Echocardiogr.