Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy

Tae Ho Lee, Lucy Youngmin Eun, Jae Young Choi, Hye Eun Kwon, Young Mock Lee, Heung Dong Kim, Seong Woong Kang

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)232-239
Number of pages8
JournalKorean Journal of Pediatrics
Volume57
Issue number5
DOIs
Publication statusPublished - 2014 May

Fingerprint

Mitochondrial Diseases
Duchenne Muscular Dystrophy
Atrophy
Body Size
Stroke Volume
Echocardiography
Body Mass Index
Cardiomyopathies
Cardiac Output
Myocardium

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Pediatrics

Cite this

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. In: Korean Journal of Pediatrics. 2014 ; Vol. 57, No. 5. pp. 232-239.
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abstract = "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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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.

In: Korean Journal of Pediatrics, Vol. 57, No. 5, 05.2014, p. 232-239.

Research output: Contribution to journalArticle

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T1 - Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy

AU - Lee, Tae Ho

AU - Eun, Lucy Youngmin

AU - Choi, Jae Young

AU - Kwon, Hye Eun

AU - Lee, Young Mock

AU - Kim, Heung Dong

AU - Kang, Seong Woong

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N2 - 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.

AB - 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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