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 |
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Pages (from-to) | 930-936 |
Number of pages | 7 |
Journal | Journal of Cardiac Failure |
Volume | 17 |
Issue number | 11 |
DOIs | |
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