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Relation of burden of myocardial fibrosis to malignant ventricular arrhythmias and outcomes in Fabry disease.
[fabry disease]
The
aim
of
this
study
was
to
investigate
the
impact
of
myocardial
fibrosis
in
Fabry
disease
.
Seventy
-
three
patients
with
genetically
confirmed
Fabry
disease
were
followed
for
4
.
8
±
2
.
4
years
.
In
accordance
with
current
guidelines
,
57
patients
received
enzyme
replacement
therapy
(
ERT
)
after
study
inclusion
,
whereas
16
did
not
.
At
baseline
and
latest
possible
follow-up
,
myocardial
fibrosis
was
assessed
noninvasively
by
cardiac
magnetic
resonance
,
and
biomarkers
of
collagen
metabolism
were
determined
.
Holter
electrocardiography
and
clinical
follow-up
at
yearly
intervals
were
used
to
monitor
malignant
ventricular
arrhythmias
(
MVAs
;
nonsustained
and
sustained
ventricular
tachycardia
and
sudden
cardiac
death
)
.
In
total
,
48
patients
(
66
%
)
showed
fibrosis
assessed
by
late
enhancement
(
LE
)
at
baseline
,
and
4
patients
developed
new
LE
during
follow-up
,
2
of
them
despite
ERT
.
The
2
patients
receiving
ERT
(
1
.
4
±
1
.
9
%
vs
2
.
5
±
2
.
6
%
,
p
<
0
.
001
)
and
the
patients
not
receiving
ERT
(
0
.
5
±
0
.
8
%
vs
0
.
7
±
1
.
0
%
,
p
=
0
.
035
)
showed
a
progression
of
LE
during
follow-up
.
None
of
the
patients
displayed
reductions
of
LE
during
follow-up
.
Collagen
biomarkers
were
elevated
in
patients
with
and
without
LE
but
did
not
correlate
with
LE
amount
.
Thirteen
LE-
positive
patients
at
the
baseline
examination
had
documented
MVAs
(
including
5
sudden
cardiac
deaths
)
,
whereas
none
of
the
patients
without
LE
had
MVAs
.
The
yearly
increase
in
fibrosis
was
0
.
9
±
0
.
6
%
in
patients
with
MVAs
and
0
.
2
±
0
.
3
%
in
patients
without
MVAs
(
p
<
0
.
001
)
.
Logistic
multivariate
regression
analysis
revealed
that
the
annual
increase
in
fibrosis
during
follow-up
was
the
only
independent
predictor
of
MVAs
.
In
conclusion
,
myocardial
fibrosis
in
Fabry
disease
is
progressive
,
apparently
not
modified
by
ERT
,
and
a
crucial
outcome
determinant
.
Diseases
Validation
Diseases presenting
"late enhancement"
symptom
fabry disease
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