https://scholars.lib.ntu.edu.tw/handle/123456789/525156
標題: | Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy | 作者: | CHUN-AN CHEN YIN-HSIU CHIEN WUH-LIANG HWU NI-CHUNG LEE JOU-KOU WANG Chen L.-R. CHUN-WEI LU MING-TAI LIN SHUENN-NAN CHIU Chiu H.-H. MEI-HWAN WU |
公開日期: | 2011 | 卷: | 17 | 期: | 11 | 起(迄)頁: | 930-936 | 來源出版物: | Journal of Cardiac Failure | 摘要: | Background: Enzyme replacement therapy (ERT) for infantile-onset Pompe disease effectively reduces the left ventricular (LV) mass. This study sought to explore detailed process of LV reverse remodeling after ERT with the use of tissue Doppler and stain rate imaging. Methods and Results: Nine infants and children with Pompe cardiomyopathy undergoing ERT for ?1 year, as well as 36 healthy control subjects, were studied. Global systolic and diastolic function was evaluated by peak systolic and early-diastolic velocity at mitral annulus. Temporal systolic and diastolic dyssynchrony was evaluated by the coefficient of variation of the time from the QRS complex to peak systolic and early-diastolic strain rate among 12 LV segments. All pre-ERT patients had impaired global systolic and diastolic function as well as increased regional dyssynchrony (P <.001 for each of all). During the regression of LV hypertrophy, all of these functional indices improved (P for trend <.001), with temporal diastolic dyssynchrony being a significant factor linking to LV mass index in multivariate analysis (P <.001). Conclusions: ERT improved global LV function and dyssynchrony in Pompe patients. The relationship between LV mass and temporal diastolic dyssynchrony during reverse remodeling suggested a pathophysiologic role of dyssynchrony in Pompe cardiomyopathy. ? 2011 Elsevier Inc. All rights reserved. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-80255137080&doi=10.1016%2fj.cardfail.2011.07.011&partnerID=40&md5=33f8cc6d30b6e3ed624a4a3c8da3173e https://scholars.lib.ntu.edu.tw/handle/123456789/525156 |
ISSN: | 1071-9164 | DOI: | 10.1016/j.cardfail.2011.07.011 | SDG/關鍵字: | recombinant glucan 1,4 alpha glucosidase; article; cardiomyopathy; clinical article; controlled study; enzyme replacement; female; geometry; heart left ventricle; heart left ventricle function; heart left ventricle hypertrophy; heart left ventricle mass; human; infant; infantile onset pompe cardiomyopathy; male; mitral valve; multivariate analysis; pompe cardiomyopathy; priority journal; QRS complex; tissue Doppler imaging; Adrenergic beta-Antagonists; Age Factors; Arrhythmias, Cardiac; Cardiomyopathy, Hypertrophic; Case-Control Studies; Child, Preschool; Diastole; Disease Progression; Enzyme Replacement Therapy; Female; Glycogen Storage Disease Type II; Humans; Hypertrophy, Left Ventricular; Infant; Infant, Newborn; Male; Multivariate Analysis; Prospective Studies; Statistics, Nonparametric; Systole; Time Factors; Ultrasonography, Doppler |
顯示於: | 醫學系 |
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