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ORIGINAL ARTICLE
Korean J Pediatr 2014 May;57(5) :232-239.
Published online 2013 October 22.       
Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
Tae Ho Lee1, Lucy Youngmin Eun1, Jae Young Choi1, Hye Eun Kwon2, Young-Mock Lee2, Heung Dong Kim2, Seong-Woong Kang3
1Divisions of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
2Division of Pediatric Neurology, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
3Department of Rehabilitation Medicine and Rehabilitation Institute of Muscular Disease, Yonsei University College of Medicine, Seoul, Korea
Corresponding Author: Lucy Youngmin Eun ,Tel: +82-2-2019-3350, Fax: +82-2-3461-9473, Email: lucyeun@yuhs.ac
Copyright © 2014 by The Korean Pediatric Society
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 (–1.021.52; P<0.001) or DMD (–0.821.61; 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.
Keywords: Myocardium | Mitochondrial diseases | Duchenne muscular dystrophy | Echocardiography
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