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MRI assessment of cardiomyopathy in Taiwan Chinese late-onset Fabry mutation (IVS4+919G > A)

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We categorized these patients into three groups (Group I, II, III) according to their myocardial thickness(all segments<12mm, at least one segment 12~16mm, at least one segment>16mm, respectively )... Among the all patients, we used Χ square test to evaluate if there was significant difference in presence of delayed enhancement according to different myocardial wall thickness... In Group III, there were 15 segments>16mm involving the septum and 13 segments in range of 13~16mm... Besides, there were 37 segments with delayed enhancement in Group III, with 14 in posteriolateral wall, 13 in anterior wall and 10 involving the septum... In group I and II, two patients with 4 segments demonstrated delayed enhancement and the 4 segments (segment 4, 10, 6, 11) are all in the normal range of thickness(<12mm)... In the Χ square test evaluation, there was significant difference in presence of delayed enhancement dependent on various myocardial wall thickness (P<0.05)... In our study, patients with Fabry variant demonstrated some different patterns of delayed myocardial enhancement and wall thickness in cardiac MR as compared with classic Fabry disease.

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A 55 y/o male with Fabry variant shows delayed myocardial enhancement in LV segment 10, 11 and 12 (red arrows). These segments were only 12~13mm in thickness.
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Fig2: A 55 y/o male with Fabry variant shows delayed myocardial enhancement in LV segment 10, 11 and 12 (red arrows). These segments were only 12~13mm in thickness.


MRI assessment of cardiomyopathy in Taiwan Chinese late-onset Fabry mutation (IVS4+919G > A)
A 55 y/o male with Fabry variant shows delayed myocardial enhancement in LV segment 10, 11 and 12 (red arrows). These segments were only 12~13mm in thickness.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4328876&req=5

Fig2: A 55 y/o male with Fabry variant shows delayed myocardial enhancement in LV segment 10, 11 and 12 (red arrows). These segments were only 12~13mm in thickness.

View Article: PubMed Central

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

We categorized these patients into three groups (Group I, II, III) according to their myocardial thickness(all segments<12mm, at least one segment 12~16mm, at least one segment>16mm, respectively )... Among the all patients, we used Χ square test to evaluate if there was significant difference in presence of delayed enhancement according to different myocardial wall thickness... In Group III, there were 15 segments>16mm involving the septum and 13 segments in range of 13~16mm... Besides, there were 37 segments with delayed enhancement in Group III, with 14 in posteriolateral wall, 13 in anterior wall and 10 involving the septum... In group I and II, two patients with 4 segments demonstrated delayed enhancement and the 4 segments (segment 4, 10, 6, 11) are all in the normal range of thickness(<12mm)... In the Χ square test evaluation, there was significant difference in presence of delayed enhancement dependent on various myocardial wall thickness (P<0.05)... In our study, patients with Fabry variant demonstrated some different patterns of delayed myocardial enhancement and wall thickness in cardiac MR as compared with classic Fabry disease.

No MeSH data available.