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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...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Pediatric Society
2014
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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. |
format | Online Article Text |
id | pubmed-4102686 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | The Korean Pediatric Society |
record_format | MEDLINE/PubMed |
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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