Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy

  • Lee, Tae Ho (Divisions of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Eun, Lucy Youngmin (Divisions of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Choi, Jae Young (Divisions of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Kwon, Hye Eun (Divisions of Pediatric Neurology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Lee, Young-Mock (Divisions of Pediatric Neurology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Kim, Heung Dong (Divisions of Pediatric Neurology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Kang, Seong-Woong (Department of Rehabilitation Medicine and Rehabilitation Institute of Muscular Disease, Yonsei University College of Medicine)
  • 투고 : 2013.09.20
  • 심사 : 2013.10.21
  • 발행 : 2014.05.15
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초록

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 ($-1.02{\pm}1.52$, P<0.001) or DMD ($-0.82{\pm}1.61$, 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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참고문헌

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피인용 문헌

  1. Dystrophic Cardiomyopathy: Complex Pathobiological Processes to Generate Clinical Phenotype vol.4, pp.3, 2014, https://doi.org/10.3390/jcdd4030014
  2. Gene Therapy Rescues Cardiac Dysfunction in Duchenne Muscular Dystrophy Mice by Elevating Cardiomyocyte Deoxy-Adenosine Triphosphate vol.4, pp.7, 2019, https://doi.org/10.1016/j.jacbts.2019.06.006
  3. Persistent myocardial atrophy despite LV reverse remodeling in Duchenne cardiomyopathy treated by LVAD vol.25, pp.2, 2021, https://doi.org/10.1111/petr.13890