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Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy.

Authors
 Tae Ho Lee  ;  Lucy Youngmin Eun  ;  Jae Young Choi  ;  Hye Eun Kwon  ;  Young Mock Lee  ;  Heung Dong Kim  ;  Seong Woong Kang 
Citation
 Korean Journal of Pediatrics, Vol.57(5) : 232-239, 2014 
Journal Title
 Korean Journal of Pediatrics 
ISSN
 1738-1061 
Issue Date
2014
Keywords
Duchenne muscular dystrophy ; Echocardiography ; Mitochondrial diseases ; Myocardium
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.
Files in This Item:
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DOI
10.3345/kjp.2014.57.5.232
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Rehabilitation Medicine (재활의학교실) > 1. Journal Papers
Yonsei Authors
Kang, Seong Woong(강성웅) ORCID logo https://orcid.org/0000-0002-7279-3893
Kwon, Hye Eun(권혜은)
Kim, Heung Dong(김흥동) ORCID logo https://orcid.org/0000-0002-8031-7336
Eun, Lucy Youngmin(은영민) ORCID logo https://orcid.org/0000-0002-4577-3168
Lee, Young Mock(이영목) ORCID logo https://orcid.org/0000-0002-5838-249X
Choi, Jae Young(최재영) ORCID logo https://orcid.org/0000-0002-1247-6669
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/138518
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