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Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy

PURPOSE: Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution...

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Autores principales: Lee, Tae Ho, Eun, Lucy Youngmin, Choi, Jae Young, Kwon, Hye Eun, Lee, Young-Mock, Kim, Heung Dong, Kang, Seong-Woong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Pediatric Society 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4102686/
https://www.ncbi.nlm.nih.gov/pubmed/25045366
http://dx.doi.org/10.3345/kjp.2014.57.5.232
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author Lee, Tae Ho
Eun, Lucy Youngmin
Choi, Jae Young
Kwon, Hye Eun
Lee, Young-Mock
Kim, Heung Dong
Kang, Seong-Woong
author_facet Lee, Tae Ho
Eun, Lucy Youngmin
Choi, Jae Young
Kwon, Hye Eun
Lee, Young-Mock
Kim, Heung Dong
Kang, Seong-Woong
author_sort Lee, Tae Ho
collection PubMed
description PURPOSE: Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution and functional changes of the myocardium in patients with MD or DMD. METHODS: We retrospectively evaluated the echocardiographic data of 90 children with MD and 42 with DMD. Using two-dimensional echocardiography, including time-motion (M) mode and Doppler measurements, we estimated the LV mass, ratio of early to late mitral filling velocities (E/A), ratio of early mitral filling velocity to early diastolic mitral annular velocity (E/Ea), stroke volume, and cardiac output. A "z score" was generated using the lambda-mu-sigma method to standardize the LV mass with respect to body size. RESULTS: The LV mass-for-height z scores were significantly below normal in children with MD (-1.02±1.52, P<0.001) or DMD (-0.82±1.61, P=0.002), as were the LV mass-for-lean body-mass z scores. The body mass index (BMI)-for-age z scores were far below normal and were directly proportional to the LV mass-for-height z scores in both patients with MD (R=0.377, P<0.001) and those with DMD (R=0.330, P=0.033). The LV mass-for-height z score correlated positively with the stroke volume index (R=0.462, P<0.001) and cardiac index (R=0.358, P<0.001). CONCLUSION: LV myocardial atrophy is present in patients with MD and those with DMD and may be closely associated with low BMI. The insufficient LV mass for body size might indicate deterioration of systolic function in these patients.
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spelling pubmed-41026862014-07-18 Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy Lee, Tae Ho Eun, Lucy Youngmin Choi, Jae Young Kwon, Hye Eun Lee, Young-Mock Kim, Heung Dong Kang, Seong-Woong Korean J Pediatr Original Article PURPOSE: Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution and functional changes of the myocardium in patients with MD or DMD. METHODS: We retrospectively evaluated the echocardiographic data of 90 children with MD and 42 with DMD. Using two-dimensional echocardiography, including time-motion (M) mode and Doppler measurements, we estimated the LV mass, ratio of early to late mitral filling velocities (E/A), ratio of early mitral filling velocity to early diastolic mitral annular velocity (E/Ea), stroke volume, and cardiac output. A "z score" was generated using the lambda-mu-sigma method to standardize the LV mass with respect to body size. RESULTS: The LV mass-for-height z scores were significantly below normal in children with MD (-1.02±1.52, P<0.001) or DMD (-0.82±1.61, P=0.002), as were the LV mass-for-lean body-mass z scores. The body mass index (BMI)-for-age z scores were far below normal and were directly proportional to the LV mass-for-height z scores in both patients with MD (R=0.377, P<0.001) and those with DMD (R=0.330, P=0.033). The LV mass-for-height z score correlated positively with the stroke volume index (R=0.462, P<0.001) and cardiac index (R=0.358, P<0.001). CONCLUSION: LV myocardial atrophy is present in patients with MD and those with DMD and may be closely associated with low BMI. The insufficient LV mass for body size might indicate deterioration of systolic function in these patients. The Korean Pediatric Society 2014-05 2014-05-31 /pmc/articles/PMC4102686/ /pubmed/25045366 http://dx.doi.org/10.3345/kjp.2014.57.5.232 Text en Copyright © 2014 by The Korean Pediatric Society http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Lee, Tae Ho
Eun, Lucy Youngmin
Choi, Jae Young
Kwon, Hye Eun
Lee, Young-Mock
Kim, Heung Dong
Kang, Seong-Woong
Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
title Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
title_full Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
title_fullStr Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
title_full_unstemmed Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
title_short Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
title_sort myocardial atrophy in children with mitochondrial disease and duchenne muscular dystrophy
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4102686/
https://www.ncbi.nlm.nih.gov/pubmed/25045366
http://dx.doi.org/10.3345/kjp.2014.57.5.232
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