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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 68 y/o male with Fabry variant demonstrates delayed myocardial enhancement and wall thickening (>16mm) in multiple LV segment, including segment 7,8, 10, 11 and 12 (red arrows). (Other segments were also involved, but not shown in this image.)
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Fig1: A 68 y/o male with Fabry variant demonstrates delayed myocardial enhancement and wall thickening (>16mm) in multiple LV segment, including segment 7,8, 10, 11 and 12 (red arrows). (Other segments were also involved, but not shown in this image.)


MRI assessment of cardiomyopathy in Taiwan Chinese late-onset Fabry mutation (IVS4+919G > A)
A 68 y/o male with Fabry variant demonstrates delayed myocardial enhancement and wall thickening (>16mm) in multiple LV segment, including segment 7,8, 10, 11 and 12 (red arrows). (Other segments were also involved, but not shown in this image.)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig1: A 68 y/o male with Fabry variant demonstrates delayed myocardial enhancement and wall thickening (>16mm) in multiple LV segment, including segment 7,8, 10, 11 and 12 (red arrows). (Other segments were also involved, but not shown in this image.)

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.