Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Quantification of circumferential, longitudinal, and radial global fractional shortening using steady-state free precession cines: A comparison with tissue-tracking strain and application in fabry disease.
[fabry disease]
Conventional
calculation
of
myocardial
strain
requires
tissue-tracking
.
A
surrogate
for
strain
called
global
fractional
shortening
(
GFS
)
is
proposed
based
on
changes
in
dimensions
of
endocardial
and
epicardial
surfaces
without
tissue-tracking
.
Three
-dimensional
endocardial
and
epicardial
left
ventricular
surfaces
traced
at
end-diastole
and
end-systole
using
conventional
steady-
state
free
precession
cine
images
were
used
to
calculate
GFScc
(
circumferential
)
,
GFSll
(
longitudinal
)
,
and
GFSrr
(
radial
)
using
fractional
length
changes
in
each
direction
over
the
heart
surface
.
GFS
values
were
validated
using
finite
element
models
(
FEM
)
and
in
vivo
using
tagging-derived
strains
(
εcc
,
εll
,
εrr
)
in
patients
with
a
wide
range
of
ejection
fraction
(
EF
)
and
diagnosis
(
n
 
=
 
32
)
.
GFS
was
also
measured
in
31
patients
with
Fabry
disease
and
matched
healthy
controls
.
GFS
values
were
within
3
%
of
average
FEM-derived
Lagrangian
strains
and
had
good
agreement
in
vivo
(
GFScc
 
=
 
-
14
 
±
 
4
%
,
εcc
 
=
 
-
14
 
±
 
4
%
,
R
(
2
)
 
=
 
0
.
85
;
GFSll
 
=
 
-
12
 
±
 
4
%
,
εll
 
=
 
-
12
 
±
 
4
%
,
R
(
2
)
 
=
 
0
.
72
;
GFSrr
 
=
 
46
 
±
 
21
%
)
.
εrr
could
not
be
measured
reliably
from
tagging
.
Compared
with
healthy
controls
with
matched
EF
,
patients
with
Fabry
disease
had
significantly
increased
GFScc
(
Endo
)
(
-
28
 
±
 
3
%
versus
-
25
 
±
 
2
%
)
,
decreased
GFScc
(
Epi
)
(
-
10
 
±
 
2
%
versus
-
11
 
±
 
2
%
)
and
decreased
GFSll
for
all
components
.
GFS
yields
similar
values
to
conventionally
measured
strains
without
requiring
tissue-tracking
.
Compared
with
controls
,
patients
with
Fabry
disease
have
significant
differences
in
several
GFS
components
.
Magn
Reson
Med
,
2014
.
©
2014
Wiley
Periodicals
,
Inc
.
Diseases
Validation
Diseases presenting
"wide range"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
adrenomyeloneuropathy
alexander disease
allergic bronchopulmonary aspergillosis
alpha-thalassemia
aromatase deficiency
benign recurrent intrahepatic cholestasis
cadasil
carcinoma of the gallbladder
congenital toxoplasmosis
cowden syndrome
cystinuria
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
fabry disease
gm1 gangliosidosis
harlequin ichthyosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
legionellosis
neonatal adrenoleukodystrophy
oral submucous fibrosis
pendred syndrome
phenylketonuria
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
proteus syndrome
pyruvate dehydrogenase deficiency
scrub typhus
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
trochlear dysplasia
well-differentiated liposarcoma
werner syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom