Abstract:
:Pompe's disease is a glycogen storage disease (type II) characterized by inherited autosomal recessive transmission. The right ventricular (RV) function is a determinant parameter of clinical outcome in patients with heart failure. We sought to characterize the RV function using Doppler-echocardiography completed by Doppler tissular imaging and tricuspid annular plane systolic excursion (TAPSE) measurement. We analyzed retrospectively clinical and Doppler-echocardiographic data of patients with adult late onset Pompe disease and compared to a control group. Ten patients with late onset Pompe disease were included in our study and were compared to a control group (seven patients). Mean age was 56.7 ± 10.2 years in late onset Pompe disease versus 55 ± 21 years in control group (p = 0.65). Left ventricular ejection fraction (LVEF) was similar in the two groups (LVEF 63.7 ± 9 vs 63.7 ± 6.6 % in control group p = 0.99). LV end diastolic diameter was 40.8 ± 6 mm in Pompe disease versus 45.8 ± 6 mm in control group (p = 0.11). Mean TAPSE was similar in the two groups (25.6 ± 6.2 vs 21.5 ± 2.7 mm p = 0.23). Mean peak systolic RV velocity Sm was not significantly different in the two groups (17.11 ± 3.4 cm/s in Pompe disease vs 16.14 ± 3.8 cm/s in control group p = 0.61). Mean peak early diastolic Ea velocity in the RV were not significantly different in the two groups (15.6 ± 5.6 vs 18.2 ± 4.9 cm/s p = 0.34). According to our data, RV systolic function seems preserved in late-onset Pompe disease.
journal_name
J Clin Monit Computjournal_title
Journal of clinical monitoring and computingauthors
Fayssoil A,Nardi O,Annane D,Orlikowski Ddoi
10.1007/s10877-014-9551-0subject
Has Abstractpub_date
2014-08-01 00:00:00pages
419-21issue
4eissn
1387-1307issn
1573-2614journal_volume
28pub_type
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journal_title:Journal of clinical monitoring and computing
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journal_title:Journal of clinical monitoring and computing
pub_type: 杂志文章,meta分析,评审
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journal_title:Journal of clinical monitoring and computing
pub_type: 杂志文章
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journal_title:Journal of clinical monitoring and computing
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journal_title:Journal of clinical monitoring and computing
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更新日期:2006-12-01 00:00:00
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journal_title:Journal of clinical monitoring and computing
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journal_title:Journal of clinical monitoring and computing
pub_type: 杂志文章,评审
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更新日期:2006-04-01 00:00:00
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更新日期:2020-06-01 00:00:00
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journal_title:Journal of clinical monitoring and computing
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