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Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience.
[congenital diaphragmatic hernia]
To
evaluate
the
impact
of
congenital
heart
anomaly
(
CHA
)
and
its
potential
contribution
to
morbidity
and
mortality
in
infants
with
congenital
diaphragmatic
hernia
(
CDH
)
.
In
the
present
retrospective
cohort
study
,
prenatal
and
postnatal
data
of
all
newborns
diagnosed
with
CDH
between
January
2004
and
November
2012
in
a
single
center
were
reviewed
.
Cases
were
classified
into
two
groups
:
those
with
"
isolated
"
CDH
and
those
with
both
CDH
and
CHA
.
Patients
with
CHA
were
further
sub-classified
into
those
with
a
major
or
minor
CHA
based
on
the
Risk
Adjustment
for
Congenital
Heart
Surgery
1
(
RACHS-
1
)
,
and
the
Society
of
Thoracic
Surgeons-
European
Association
for
Cardiothoracic
Surgery
score
(
STS
-EACTS
)
scoring
systems
.
Patients
with
associated
non-
cardiac
anomalies
(
including
'
syndromic
cases
'
)
were
excluded
from
the
analysis
.
Primary
and
secondary
outcomes
were
survival
up
to
1
year
of
life
and
need
for
Extracorporeal
Membrane
Oxygenation
(
ECMO
)
,
respectively
.
Out
of
180
infants
with
CDH
,
41
patients
were
excluded
because
of
the
presence
of
non-
cardiac
associated
anomalies
.
118
infants
had
isolated
CDH
and
21
had
CDH
+
CHA
(
16
with
minor
CHA
and
5
with
major
CHA
)
.
Receiver
operating
characteristic
(
ROC
)
analyses
demonstrated
the
best
cut-off
for
survival
was
when
the
scoring
value
was
≤
than
2
for
both
RACHS-
1
(
area
under
the
curve
:
0
.
74
;
p
=
0
.
04
;
sensitivity
=
80
.
0
%
;
specificity
=
87
.
5
%
)
and
STS
-EACTS
(
area
under
the
curve
:
0
.
83
;
p
=
0
.
03
;
sensitivity
=
100
%
;
specificity
=
87
.
5
%
)
.
The
survival
rate
at
1
year
of
life
was
significantly
lower
in
the
group
of
major
CHA
+
CHD
(
40
.
0
%
;
p
=
0
.
04
)
in
contrast
with
'
isolated
CDH
'
(
77
.
1
%
)
and
'
CDH
+
minor
CHA
'
(
81
.
3
%
)
.
We
found
no
significant
differences
among
the
groups
concerning
the
need
for
ECMO
.
In
general
,
mild
forms
of
CHA
do
not
appear
to
impact
negatively
on
survival
for
infants
with
CDH
.
However
,
mortality
appears
significantly
higher
in
infants
with
major
forms
of
CHA
in
infants
with
CDH
.
The
scoring
systems
appear
to
be
useful
predictors
to
classify
the
effects
of
CHA
in
this
population
of
patients
.
Diseases
Validation
Diseases presenting
"mild forms"
symptom
classical phenylketonuria
congenital adrenal hyperplasia
congenital diaphragmatic hernia
dentinogenesis imperfecta
lamellar ichthyosis
neonatal adrenoleukodystrophy
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