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- W2124799148 abstract "<h3>OBJECTIVE</h3> To investigate changes in the alignment of the sarcomeric filaments in hypertrophic cardiomyopathy and the effects of cardiac β myosin heavy chain (β-MHC) mutation on the sarcomeric ultrastructure. <h3>DESIGN</h3> A retrospective analysis. <h3>PATIENTS</h3> Endomyocardial biopsy samples were examined by transmission electron microscopy in seven patients with hypertrophic cardiomyopathy and β-MHC mutation, six with hypertrophic cardiomyopathy but without the mutation, and five controls (with chest pain syndromes). <h3>MAIN OUTCOME MEASURE</h3> Alignment of the sarcomeric filaments and the distance between neighbouring thick myosin filaments. <h3>RESULTS</h3> In controls, cross sections of the sarcomere at the A band showed a highly organised orthohexagonal array with 6 thin actin filaments surrounding one thick myosin filament, whereas in hypertrophic cardiomyopathy the alignment of the sarcomeric filaments was sparse and disrupted. In hypertrophic cardiomyopathy with a mutation, the distance between neighbouring thick myosin filaments was greater than in controls (mean (SD) 45.3 (4.7)<i>v</i> 38.5 (3.5) nm, p < 0.05), and the variance of the distance was greater than in controls (8.0 (0.7)<i>v</i> 4.8 (1.0) nm, p < 0.001) or in patients with hypertrophic cardiomyopathy without a mutation (6.7 (0.6) nm, p < 0.05). In the latter, the variance of the distance was also greater than in the controls (p < 0.01). A significant correlation was found between the grade of the myocyte hypertrophy and the variance of the distance (<i>r</i> = 0.654; p < 0.01). <h3>CONCLUSIONS</h3> The alignment of the sarcomeric filaments is disrupted in hypertrophic cardiomyopathy, particularly when there is β-MHC mutation." @default.
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- W2124799148 date "1999-11-01" @default.
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- W2124799148 title "Malalignment of the sarcomeric filaments in hypertrophic cardiomyopathy with cardiac myosin heavy chain gene mutation" @default.
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- W2124799148 doi "https://doi.org/10.1136/hrt.82.5.625" @default.
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