Value of Global Longitudinal Strain for Identification and Monitoring of Left Ventricular Dysfunction in Becker Muscular Dystrophy

Nienke M Van De Velde,Tea Gegenava,Zaïda Koeks,Steele C Butcher,Arno Aw Roest,Jeroen J Bax,Douwe E Atsma,Pietro Spitali,Nina Ajmone Marsan,Erik H Niks

doi:10.1016/j.amjcard.2021.09.016

Nienke M Van De Velde, Tea Gegenava + Show 8 more

Open Access

https://doi.org/10.1016/j.amjcard.2021.09.016

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Abstract

Cardiac involvement is the main cause of death in Becker muscular dystrophy (BMD). Identification of left ventricular (LV) function is crucial, but standard echocardiographic measurements such as LV ejection fraction (LVEF) might not be sensitive enough to detect early myocardial dysfunction. We explored the value of LV global longitudinal strain (GLS) as a more accurate echocardiographic parameter to detect and monitor LV dysfunction in BMD. Furthermore, we studied possible factors associated with LV dysfunction and progression. A total of 40 patients with BMD (age 39.0 ± 13.2 years) and 21 matched controls were included. Clinical variables, pulmonary tests, serum biomarkers, and echocardiograms were collected at baseline and after 2 years. LV systolic function was assessed by LVEF and LV GLS; a significant progression in LV dysfunction was defined as an absolute LV GLS deterioration ≥15%. Responsiveness to cardiac disease progression was determined using standardized response means. Patients showed impaired LVEF and LV GLS compared with controls (p <0.001). Of interest, 31 patients (77.5%) showed impaired LV GLS (defined as greater than -18%), whereas only 24 patients (60%) had reduced LVEF. LV GLS and LVEF correlated with troponin I (ρ=0.553 and -0.523) and N-terminal pro-b-type natriuretic peptide (ρ=0.506 and -0.585), but not with skeletal muscle or pulmonary function. At follow-up (2.0 ± 0.5 years, n=29), LV GLS worsened significantly (-1.3 ± 0.8%, p=0.002, standardized response mean=0.70, annually=0.60%), whereas LVEF remained stable. No risk factors for LV dysfunction progression were identified. In BMD, LV GLS is frequently impaired and shows deterioration over time compared with LVEF. LV GLS could be used as a more sensitive parameter to identify and monitor LV dysfunction.

Highlights

Global longitudinal strain (GLS), quantified by speckle tracking echocardiography (STE), has been shown to be more sensitive and reproducible compared with LV ejection fraction (LVEF) and of significant prognostic value,[3,5] and it could detect early left ventricular (LV) dysfunction in several diseases.[6−9] In this study, we explored the value of LV GLS for detecting and monitoring LV systolic dysfunction compared with LVEF and LV volumes
We explored the value of LV GLS to identify LV dysfunction and its potential progression in Becker muscular dystrophy (BMD)
Our findings suggest that LV GLS is able to detect subtle LV dysfunction in the absence of overt reduction of LVEF

Summary

Introduction

The possible low sensitivity of LVEF to detect subtle myocardial dysfunction[3] could, lead to underestimation of cardiac involvement and delay specific treatments because initiation of medication is suggested only in case of reduced LVEF.[4] Advanced echocardiographic techniques, such as speckle tracking echocardiography (STE), may improve assessment of early myocardial involvement.[3] Global longitudinal strain (GLS), quantified by STE, has been shown to be more sensitive and reproducible compared with LVEF and of significant prognostic value,[3,5] and it could detect early LV dysfunction (with preserved LVEF) in several diseases.[6−9] In this study, we explored the value of LV GLS for detecting and monitoring LV systolic dysfunction compared with LVEF and LV volumes. We studied correlations with serum biomarkers and clinical and functional parameters and tried to identify baseline characteristics associated with a deterioration in LV GLS over time

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