Early Regional Myocardial Dysfunction in Young Patients With Duchenne Muscular Dystrophy

Mertens, Luc; Gáname, Javier; Claus, Piet; Goemans, Nathalie; Thijs, Daisy; Eyskens, Bénédicte; Laere, David Van; Bijnens, Bart; D'hooge, Jan; Sutherland, George R.; Buyse, Gunnar

doi:10.1016/j.echo.2008.03.001

Cited by 113 publications

(96 citation statements)

References 25 publications

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“…17 Echocardiography can be helpful to detect early dysfunction in young Duchenne patients. Done properly, echocardiography is viewed as the gold standard to evaluate and quantify systolic and diastolic LV dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

Bockel¹,

Lind²,

Zijlstra³

et al. 2009

NHJL

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Background. Duchenne muscular dystrophy (DMD) patients used to die mainly from pulmonary problems. However, as advances in respiratory care increase life expectancy, mortality due to cardiomyopathy rises. Echocardiography remains the standard diagnostic modality for cardiomyopathy in DMD patients, but is hampered by scoliosis and poor echocardiographic acoustic windows in adult DMD patients. Multigated cardiac radionuclide ventriculography (MUGA) does not suffer from these limitations. N-terminal proBNP (NTproBNP) has shown to be a diagnostic factor for heart failure. We present our initial experience with plasma NT-proBNP measurement in the routine screening and diagnosis of cardiomyopathy in adult mechanically ventilated DMD patients.Methods. Retrospective study, 13 patients. Echocardiography classified left ventricular (LV) function as preserved or depressed. NT-proBNP was determined using immunoassay. LV ejection fraction (LVEF) was determined using MUGA.Results. Median (range) NT-proBNP was 73 (25 to 463) ng/l. Six patients had an NT-proBNP >125 ng/l. Seven patients showed an LVEF <45% on MUGA. DMD patients with depressed LV function (n=4) as assessed by echocardiography had significantly higher median NT-proBNP than those (n=9) with preserved LV function: 346 (266 to 463) ng/l versus 69 (25 to 257) ng/l (p=0.003). NT-proBNP significantly correlated with depressed LV function on echocardiogram and with LVEF determined by MUGA.Conclusion. Although image quality of MUGA is superior to echocardiography, the combination of echocardiography and NT-proBNP achieves similar results in the evaluation of left ventricular function and is less time consuming and burdensome for our patients. We advise to add NT-proBNP to echocardiography in the routine cardiac assessment of DMD patients. (Neth Heart J 2009;17:232-7.).

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Section: Discussionmentioning

confidence: 99%

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

Bockel¹,

Lind²,

Zijlstra³

et al. 2009

NHJL

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“…Two-dimensional strain assessment was chosen as at rest is more sensitive to picking up regional myocardial dysfunction in children than traditional parameters such as ejection fraction as, for example, shown in children with Duchenne muscular dystrophy (30). This in combination with exercise imaging, which also is more sensitive to mild dysfunction, could help make 2-D strain become a useful clinical tool.…”

Section: Methodology Limitationsmentioning

confidence: 99%

The relationship between biventricular myocardial performance and metabolic parameters during incremental exercise and recovery in healthy adolescents

Pieles¹,

Gowing

Forsey

et al. 2015

American Journal of Physiology-Heart and Circulatory Physiology

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Pieles GE, Gowing L, Forsey J, Ramanujam P, Miller F, Stuart AG, Williams CA. The relationship between biventricular myocardial performance and metabolic parameters during incremental exercise and recovery in healthy adolescents. Am J Physiol Heart Circ Physiol 309: H2067-H2076, 2015. First published October 23, 2015 doi:10.1152/ajpheart.00627.2015 and right ventricular (RV) myocardial reserve during exercise in adolescents has not been directly characterized. The aim of this study was to quantify myocardial performance response to exercise by using two-dimensional (2-D) speckle tracking echocardiography and describe the relationship between myocardial reserve, respiratory, and metabolic exercise parameters. A total of 23 healthy boys and girls (mean age 13.2 Ϯ 2.7 yr; stature 159.1 Ϯ 16.4 cm; body mass 49.5 Ϯ 16.6 kg; BSA 1.47 Ϯ 0.33 m 2 ) completed an incremental cardiopulmonary exercise test (25 W·3 min increments) with simultaneous acquisition of 2-D transthoracic echocardiography at rest, each exercise stage up to 100 W, and in recovery at 2 min and 10 min. Two-dimensional LV (LV Sl) and RV (RV Sl) longitudinal strain and LV circumferential strain (LV Sc) were analyzed to define the relationship between myocardial performance reserve and metabolic exercise parameters. Participants achieved a peak oxygen uptake (V O2peak) of 40.6 Ϯ 8.9 ml·kg Ϫ1 ·min Ϫ1 and a work rate of 154 Ϯ 42 W. LV Sl and LV Sc and RV Sl increased significantly across work rates (P Ͻ 0.05). LV Sl during exercise was significantly correlated to resting strain, V O2peak, oxygen pulse, and work rate (0.530 Յ r Յ 0.784, P Ͻ 0.05). This study identifies a positive and moderate relationship between LV and RV myocardial performance and metabolic parameters during exercise by using a novel methodology. Relationships detected present novel data directly describing myocardial adaptation at different stages of exercise and recovery that in the future can help directly assess cardiac reserve in patients with cardiac pathology. CARDIOVASCULAR EXERCISE RESPONSE is a complex interplay between respiratory, metabolic, cardiac, and muscular adaptations and is traditionally assessed in children by using cardiopulmonary exercise testing (CPET) (1, 34). CPET has been used in diagnosis and risk stratification in children with heart disease (38) as it can impose physiological stress on the cardiovascular system to determine submaximal and maximal capacity, while numerous studies in adults with cardiovascular disease have shown CPET is a strong predictor of clinical outcome (2). Two methodological limitations of CPET are, however, the inability to provide data on myocardial function in response to the imposed exercise stress, the dominant process being to increase cardiac output to enhance oxygen delivery (3), and its limited and indirect inferences of cardiac reserve.Over the last decade these methodological constraints have led to several pilot studies directly investigating cardiac function during exercise in the healthy pediatric population by using echoca...

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“…Recently, new developments in echocardiographic imaging technology have enabled the quantification of myocardial deformation based on tissue Doppler imaging (TDI) or 2D speckle tracking (2D-strain) which provide regional information on myocardial contractility and interaction with neighboring segments and global circulation that cannot be obtained from conventional Doppler techniques [13]. Myocardial deformation, measured as strain and strain rate, better assesses intrinsic properties of the cardiac fibers and myocytes [13] and has been demonstrated to be a very sensitive marker of cardiac dysfunction in adulthood [14,15,16,17]. These techniques also permit evaluation of the different and partially independent components of ventricular contraction and motion (longitudinal, radial, or circumferential) and can assess regional changes [13].…”

Section: Introductionmentioning

confidence: 99%

Feasibility and Reproducibility of a Standard Protocol for 2D Speckle Tracking and Tissue Doppler-Based Strain and Strain Rate Analysis of the Fetal Heart

Crispi¹,

Sepulveda-Swatson²,

Cruz‐Lemini³

et al. 2012

Fetal Diagn Ther

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Purpose: Assessment of cardiac function in the fetal heart is challenging because of its small size and high heart rate, restricted physical access to the fetus, and impossibility of fetal ECG recording. We aimed to standardize the acquisition and postprocessing of fetal echocardiography for deformation analysis and to assess its feasibility, reproducibility, and correlation for longitudinal strain and strain rate measurements by tissue Doppler imaging (TDI) and 2D speckle tracking (2D-strain) during pregnancy. Methods: Echocardiography was performed in 56 fetuses. 2D and color TDI in apical or basal four-chamber views were recorded for subsequent analysis. Caution was taken to achieve a frame rate >70 Hz for speckle tracking and >150 Hz for TDI analysis. For each acquisition, 7.5 s of noncompressed data were stored in cine loop format and analyzed offline. Since fetal ECG information is by definition not available, aortic valve closure was marked from aortic flow and the onset of each cardiac cycle was manually indicated in the 2D images. Sample volume length was standardized at the minimum size. Two observers measured the left and right ventricular peak systolic longitudinal strain and strain-rate. Results: Strain and strain rate measurements were feasible in 93% of the TDI and 2D-strain acquisitions. The mean time spent on analyzing TDI images was 18 min, with an intraclass agreement coefficient of 0.86 (95% CI 0.77–0.92), 0.83 (95% CI 0.72–0.90), 0.96 (95% CI 0.93–0.98), and 0.86 (95% CI 0.76–0.92) for basal left and right free wall peak systolic strain and strain rate, respectively. Agreement between observers using tissue Doppler also showed high reliability. The mean time spent for 2D-strain analysis was 15 min, with an intraclass agreement coefficient of 0.97 (95% CI 0.95–0.98), 0.94 (95% CI 0.89–0.96), 0.96 (95% CI 0.93–0.98), and 0.84 (95% CI 0.73–0.90) for basal left and right free wall peak systolic strain and strain rate, respectively. Agreement between observers also showed a high reliability that was similar for TDI and 2D-strain. There was a weak correlation between TDI and 2D-strain measurements. Conclusions: A standard protocol with fixed acquisition and processing settings, including manual indication of the timing events of the cardiac cycle to correct for the lack of ECG, was feasible and reproducible for the evaluation of longitudinal ventricular strain and strain rate of the fetal heart by TDI as well as 2D-strain analysis. However, both techniques are not interchangeable as the correlation between them is relatively poor.

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Early Regional Myocardial Dysfunction in Young Patients With Duchenne Muscular Dystrophy

Cited by 113 publications

References 25 publications

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

The relationship between biventricular myocardial performance and metabolic parameters during incremental exercise and recovery in healthy adolescents

Feasibility and Reproducibility of a Standard Protocol for 2D Speckle Tracking and Tissue Doppler-Based Strain and Strain Rate Analysis of the Fetal Heart

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