Yonsei Med J.  2018 Jan;59(1):128-134. 10.3349/ymj.2018.59.1.128.

Myocardial Layer-Specific Strain Analysis in Children with Mitochondrial Disease

• Eun LY
• Lee YM

• KMID: 2418859
• DOI: http://doi.org/10.3349/ymj.2018.59.1.128

Abstract

PURPOSE
Children with mitochondrial disease (MD) have clinical phenotypes that are more severe than those found in adults. In this study, we assessed cardiac function in children with MD using conventional and advanced echocardiographic measurements, explored any unique patterns present, and investigated the development of early cardiomyopathy (CMP).
MATERIALS AND METHODS
We retrospectively reviewed the medical records of 33 children with MD. All patients underwent transthoracic echocardiography with conventional and advanced myocardial analysis. We compared all data between patients and an age-matched healthy control group.
RESULTS
Conventional echocardiographic diastolic measurements of mitral E, E/A, and tissue Doppler E"² were significantly lower and E/E"² was significantly higher in children with MD, compared with the measurements from the control group. There was no significant difference in longitudinal and radial strain between the groups. Circumferential strain in the endocardium (p=0.161), middle myocardium (p=0.008), and epicardium (p=0.042) were lower in patients, compared to the values in controls. Circumferential strain was correlated with E"² (p < 0.01, r>0.60).
CONCLUSION
In children with MD, myocardial circumferential strain may develop early in all three layers, even with normally preserved longitudinal and radial strain. This may be an early diagnostic indicator with which to predict CMP in this patient population.

Keyword

Mitochondrial disease; echocardiography; cardiomyopathy; children; myocardial strain

MeSH Terms

Biomechanical Phenomena
Child
Echocardiography, Doppler
Female
Humans
Male
Mitochondrial Diseases/diagnostic imaging/*pathology/*physiopathology
Myocardium/*pathology

Figure

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  Fig. 1 Correlation of circumferential strain of the endocardial layer with conventional and tissue Doppler parameters.

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  Fig. 2 Correlation of circumferential strain of the middle myocardial layer with conventional and tissue Doppler parameters.

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  Fig. 3 Correlation of circumferential strain of the epicardial layer with conventional and tissue Doppler parameters.

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