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 | |
| Publication status | Published - Nov 2011 |
| Externally published | Yes |
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Keywords
- dyssynchrony
- enzyme replacement therapy
- hypertrophic cardiomyopathy
- Pompe disease
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
Cite this
Left ventricular geometry, global function, and dyssynchrony in infants and children with pompe cardiomyopathy undergoing enzyme replacement therapy. / Chen, Chun An; Chien, Yin Hsiu; Hwu, Wuh Liang; Lee, Ni Chung; Wang, Jou Kou; Chen, Lei Ru; Lu, Chun Wei; Lin, Ming Tai; Chiu, Shuenn Nan; Chiu, Hsin Hui; Wu, Mei Hwan.
In: Journal of Cardiac Failure, Vol. 17, No. 11, 11.2011, p. 930-936.Research output: Contribution to journal › Article
}
TY - JOUR
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
UR - http://www.scopus.com/inward/record.url?scp=80255137080&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=80255137080&partnerID=8YFLogxK
U2 - 10.1016/j.cardfail.2011.07.011
DO - 10.1016/j.cardfail.2011.07.011
M3 - Article
C2 - 22041330
AN - SCOPUS:80255137080
VL - 17
SP - 930
EP - 936
JO - Journal of Cardiac Failure
JF - Journal of Cardiac Failure
SN - 1071-9164
IS - 11
ER -