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

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20 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 languageEnglish
Pages (from-to)930-936
Number of pages7
JournalJournal of Cardiac Failure
Volume17
Issue number11
DOIs
Publication statusPublished - Nov 2011
Externally publishedYes

Keywords

  • dyssynchrony
  • enzyme replacement therapy
  • hypertrophic cardiomyopathy
  • Pompe disease

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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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