Key result
Children with mitochondrial disease and Duchenne muscular dystrophy exhibited significant left ventricular myocardial atrophy, with LV mass-for-height z scores of -1.02 (P<0.001) and -0.82 (P=0.002) respectively, compared to normal references.
Cross-Sectional (n=132)
No
p-value: p=<0.001
Left ventricular myocardial atrophy is present in children with mitochondrial disease and Duchenne muscular dystrophy, is associated with low BMI, and correlates with deterioration of systolic function.
LV atrophy may signal early cardiac involvement needing monitoring; leaves open its prognostic role and therapeutic implications in these myopathies.
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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Lee et al. (2014) conducted a cross-sectional in Mitochondrial disease and Duchenne muscular dystrophy (n=132). Mitochondrial disease and Duchenne muscular dystrophy vs. Normal reference population was evaluated on Left ventricular mass-for-height z score (p=<0.001). Children with mitochondrial disease and Duchenne muscular dystrophy exhibited significant left ventricular myocardial atrophy, with LV mass-for-height z scores of -1.02 (P<0.001) and -0.82 (P=0.002) respectively, compared to normal references.
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