Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy

Chun An Chen; Yin Hsiu Chien; Wuh Liang Hwu; Ni Chung Lee; Jou Kou Wang; Lei Ru Chen; Chun Wei Lu; Ming Tai Lin; Shuenn Nan Chiu; Hsin Hui Chiu; Mei Hwan Wu

doi:10.1016/j.cardfail.2011.07.011

Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy

Chun An Chen, Yin Hsiu Chien, Wuh Liang Hwu, Ni Chung Lee, Jou Kou Wang, Lei Ru Chen, Chun Wei Lu, Ming Tai Lin, Shuenn Nan Chiu, Hsin Hui Chiu, Mei Hwan Wu

Research output: Contribution to journal › Article › peer-review

23 Citations (Scopus)

Abstract

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

Original language	English
Pages (from-to)	930-936
Number of pages	7
Journal	Journal of Cardiac Failure
Volume	17
Issue number	11
DOIs	https://doi.org/10.1016/j.cardfail.2011.07.011
Publication status	Published - Nov 2011
Externally published	Yes

Keywords

dyssynchrony
enzyme replacement therapy
hypertrophic cardiomyopathy
Pompe disease

ASJC Scopus subject areas

Cardiology and Cardiovascular Medicine

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10.1016/j.cardfail.2011.07.011

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Chen, C. A., Chien, Y. H., Hwu, W. L., Lee, N. C., Wang, J. K., Chen, L. R., Lu, C. W., Lin, M. T., Chiu, S. N., Chiu, H. H., & Wu, M. H. (2011). Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy. Journal of Cardiac Failure, 17(11), 930-936. https://doi.org/10.1016/j.cardfail.2011.07.011

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title = "Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy",

abstract = "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 ",

keywords = "dyssynchrony, enzyme replacement therapy, hypertrophic cardiomyopathy, Pompe disease",

author = "Chen, {Chun An} and Chien, {Yin Hsiu} and Hwu, {Wuh Liang} and Lee, {Ni Chung} and Wang, {Jou Kou} and Chen, {Lei Ru} and Lu, {Chun Wei} and Lin, {Ming Tai} and Chiu, {Shuenn Nan} and Chiu, {Hsin Hui} and Wu, {Mei Hwan}",

year = "2011",

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doi = "10.1016/j.cardfail.2011.07.011",

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T1 - Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy

AU - Chen, Chun An

AU - Chien, Yin Hsiu

AU - Hwu, Wuh Liang

AU - Lee, Ni Chung

AU - Wang, Jou Kou

AU - Chen, Lei Ru

AU - Lu, Chun Wei

AU - Lin, Ming Tai

AU - Chiu, Shuenn Nan

AU - Chiu, Hsin Hui

AU - Wu, Mei Hwan

PY - 2011/11

Y1 - 2011/11

N2 - 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

AB - 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

KW - dyssynchrony

KW - enzyme replacement therapy

KW - hypertrophic cardiomyopathy

KW - Pompe disease

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Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy

Abstract

Keywords

ASJC Scopus subject areas

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