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Distribution of late gadolinium enhancement in various types of cardiomyopathies: Significance in differential diagnosis, clinical features and prognosis.
[fabry disease]
The
recent
development
of
cardiac
magnetic
resonance
(
CMR
)
techniques
has
allowed
detailed
analyses
of
cardiac
function
and
tissue
characterization
with
high
spatial
resolution
.
We
review
characteristic
CMR
features
in
ischemic
and
non-ischemic
cardiomyopathies
(
ICM
and
NICM
)
,
especially
in
terms
of
the
location
and
distribution
of
late
gadolinium
enhancement
(
LGE
)
.
CMR
in
ICM
shows
segmental
wall
motion
abnormalities
or
wall
thinning
in
a
particular
coronary
arterial
territory
,
and
the
subendocardial
or
transmural
LGE
.
LGE
in
NICM
generally
does
not
correspond
to
any
particular
coronary
artery
distribution
and
is
located
mostly
in
the
mid-wall
to
subepicardial
layer
.
The
analysis
of
LGE
distribution
is
valuable
to
differentiate
NICM
with
diffusely
impaired
systolic
function
,
including
dilated
cardiomyopathy
,
end-
stage
hypertrophic
cardiomyopathy
(
HCM
)
,
cardiac
sarcoidosis
,
and
myocarditis
,
and
those
with
diffuse
left
ventricular
(
LV
)
hypertrophy
including
HCM
,
cardiac
amyloidosis
and
Anderson-
Fabry
disease
.
A
transient
low
signal
intensity
LGE
in
regions
of
severe
LV
dysfunction
is
a
particular
feature
of
stress
cardiomyopathy
.
In
arrhythmogenic
right
ventricular
cardiomyopathy
/
dysplasia
,
an
enhancement
of
right
ventricular
(
RV
)
wall
with
functional
and
morphological
changes
of
RV
becomes
apparent
.
Finally
,
the
analyses
of
LGE
distribution
have
potentials
to
predict
cardiac
outcomes
and
response
to
treatments
.
Diseases
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"arrhythmogenic right ventricular cardiomyopathy/dysplasia"
symptom
fabry disease
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