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

Velde, Nienke M van de; Gegenava, T; Koeks, Z.; Butcher, Steele C; Roest, Arno A.W.; Bax, Jeroen J.; Atsma, Douwe E.; Spitali, Pietro; Marsan, Nina Ajmone; Niks, Erik H.

doi:10.1016/j.amjcard.2021.09.016

Cited by 2 publications

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“…Several authors mention that left ventricle ejection fraction and other conventional echocardiographic tools are not sensitive in detecting cardiac dysfunction at an early age. [15][16][17][18][19][20][21] Our work provides a quantitative model for the age effects on left ventricle ejection fraction, showing a non-linear progression for the average left ventricle ejection fraction and the corresponding uncertainty. In clinical practice, left ventricle ejection fraction is usually dichotomised with a cut-off value around 52-55%.…”

Section: Discussionmentioning

confidence: 99%

“…9 Several studies have reported decreased values of global longitudinal strain on patients with Duchenne muscular dystrophy, even though left ventricle ejection fraction was within the normal range. [15][16][17][18][19][20][21] Being a progressive disease, the cardiac function of Duchenne patients deteriorates with age. These age effects are unknown and probably not linear.…”

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confidence: 99%

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Early assessment of cardiomyopathy in Duchenne patients by means of longitudinal strain echocardiography

et al. 2023

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Introduction: The diagnosis of Duchenne-linked cardiomyopathy may be challenging. Conventional echocardiographic measurements typically show deterioration beyond the second decade. Global longitudinal strain has been proposed as an earlier marker than left ventricular ejection fraction. Material and methods: A prospective, observational, cross-sectional, case-control study was carried out. Both Duchenne patients and control subjects underwent transthoracic echocardiogram in order to assess left ventricle function. Bayesian linear regression was the main tool for inference. Age effects were parameterised by means of a spline function because of its simplicity to characterise continuous variables and smooth contributions. The posterior distribution of the marginal age effects was used to assess the earliest age of deterioration of each marker. Results: Sixteen Duchenne patients and twenty-two healthy male subjects were enrolled. On overage, cardiac function measures were found for ejection fraction and longitudinal strain among different groups. Age effects on global longitudinal strain are more reliably found at ages of 6 and above, while ejection fraction starts to deteriorate at an older age. Progressive left ventricular dysfunction in Duchenne patients is one of the key issues and starts at an early age with subtle symptoms. Conclusion: This cross-sectional study provides supporting evidence that global longitudinal strain is an earlier marker of disease progression than ejection fraction in Duchenne patients.

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