Distribution of strain patterns in children with dilated cardiomyopathy

Boer, Susanna L. den; Sarvaas, Gideon J. du Marchie; Klitsie, Liselotte M.; Iperen, Gabriëlle G. van; Tanke, Ronald B.; Helbing, Willem A.; Backx, A.P.C.M.; Rammeloo, Lukas A. J.; Dalinghaus, Michiel; Harkel, Arend D J Ten

doi:10.1111/echo.13548

Cited by 6 publications

(1 citation statement)

References 22 publications

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“…Despite a preserved LVEF, longitudinal myocardial strain analysis speckle tracking could show different patterns indicative of early abnormal segmental strain deformation (especially septal strain compared with non-septal strain), post-systolic deformation, and mechanical dispersion (Hasselberg et al, 2014;Haugaa et al, 2015;Boer et al, 2017). (Hasselberg et al, 2014;Haugaa et al, 2015;Boer et al, 2017).…”

Section: Echocardiographic Analysis During Follow-upmentioning

confidence: 99%

Characterization of cardiac involvement in children with LMNA-related muscular dystrophy

César

Campuzano

Cruzalegui

et al. 2023

Front. Cell Dev. Biol.

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Introduction: LMNA-related muscular dystrophy is a rare entity that produce “laminopathies” such as Emery–Dreifuss muscular dystrophy (EDMD), limb–girdle muscular dystrophy type 1B (LGMD1B), and LMNA-related congenital muscular dystrophy (L-CMD). Heart failure, malignant arrhythmias, and sudden death may occur. No consensus exists on cardiovascular management in pediatric laminopathies. The aim was to perform an exhaustive cardiologic follow-up in pediatric patients diagnosed with LMNA-related muscular dystrophy.Methods: Baseline cardiac work-up consisted of clinical assessment, transthoracic Doppler echocardiography, 12-lead electrocardiogram, electrophysiological study, and implantation of a long-term implantable cardiac loop recorder (ILR).Results: We enrolled twenty-eight pediatric patients diagnosed with EDMD (13 patients), L-CMD (11 patients), LGMD1B (2 patients), and LMNA-related mild weakness (2 patients). Follow-up showed dilated cardiomyopathy (DCM) in six patients and malignant arrhythmias in five (four concomitant with DCM) detected by the ILR that required implantable cardioverter defibrillator (ICD) implantation. Malignant arrhythmias were detected in 20% of our cohort and early-onset EDMD showed worse cardiac prognosis.Discussion: Patients diagnosed with early-onset EDMD are at higher risk of DCM, while potentially life-threatening arrhythmias without DCM appear earlier in L-CMD patients. Early onset neurologic symptoms could be related with worse cardiac prognosis. Specific clinical guidelines for children are needed to prevent sudden death.

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Section: Echocardiographic Analysis During Follow-upmentioning

confidence: 99%

Characterization of cardiac involvement in children with LMNA-related muscular dystrophy

César

Campuzano

Cruzalegui

et al. 2023

Front. Cell Dev. Biol.

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Quantitative mechanical dyssynchrony in dilated cardiomyopathy measured by deformable registration algorithm

et al. 2020

Eur Radiol

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Left ventricular dyssynchrony in long-term childhood cancer survivors treated with anthracyclines: a retrospective cross-sectional study

Pourier

Dull

Weijers

et al. 2021

Int J Cardiovasc Imaging

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The purpose of this study was to investigate left ventricular contraction patterns in asymptomatic Childhood cancer survivors (CCS) using two-dimensional speckle tracking echocardiography (2DSTE). Left ventricular longitudinal and circumferential myocardial parameters were assessed using 2DSTE, in asymptomatic CCS and age matched healthy controls. Time to peak (T2P) systolic strain was quantified. Dyssynchrony index (DI) was measured by calculating the standard deviation of T2P systolic strain of six segments in each view. Difference between T2P systolic longitudinal strain of septal and lateral wall was also assessed as a parameter for dyssynchrony. We included 115 CCS with a median age of 17.2 years (range 5.6–39.5) and a median follow up of 11.3 years (range 4.9–29.5) and 119 controls. Conventional echocardiographic parameters and global longitudinal strain were significantly decreased in CCS compared to controls (p < 0.01 and p = 0.02, respectively). Dyssynchrony index did not differ between CCS and controls. There was a clinically insignificant smaller absolute difference between T2P systolic longitudinal of septal and lateral wall in CCS compared to controls. We showed no difference in longitudinal or circumferential left ventricular dyssynchrony in CCS compared to controls using 2DSTE. Future research should focus on assessing dyssynchrony in more segments and a larger CCS population, using both 2D and 3DSTE.

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Distribution of strain patterns in children with dilated cardiomyopathy

Abstract: In this study, quantitative dyssynchrony parameters were not reproducible, precluding their use in children. Qualitative pattern analysis showed predominantly reduced strain, suggesting that in children with DCM dyssynchrony may be a minor problem.

Cited by 6 publications

References 22 publications

Characterization of cardiac involvement in children with LMNA-related muscular dystrophy

Characterization of cardiac involvement in children with LMNA-related muscular dystrophy

Quantitative mechanical dyssynchrony in dilated cardiomyopathy measured by deformable registration algorithm

Left ventricular dyssynchrony in long-term childhood cancer survivors treated with anthracyclines: a retrospective cross-sectional study

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