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22q11.2 Deletion syndrome is associated with increased perioperative events and more complicated postoperative course in infants undergoing infant operative correction of truncus arteriosus communis or interrupted aortic arch.
[22q11.2 deletion syndrome]
The
effect
of
genotype
on
the
outcomes
of
infant
cardiac
operations
has
not
been
well
established
.
The
purpose
of
the
present
study
was
to
investigate
the
effect
of
22
q
11
.
2
deletion
(
22
q
11
del
)
on
infants
with
truncus
arteriosus
communis
(
TA
)
and
interrupted
aortic
arch
(
IAA
)
undergoing
operative
correction
during
infancy
.
We
conducted
a
retrospective
cohort
study
of
all
infants
who
had
undergone
operative
correction
of
TA
or
IAA
at
the
Children
's
Hospital
of
Philadelphia
from
1995
to
2007
,
comparing
the
perioperative
outcomes
(
hospital
length
of
stay
,
intensive
care
,
mechanical
ventilation
,
risk
of
cardiac
and
noncardiac
events
,
number
of
consultations
,
and
number
of
discharge
medications
)
by
22
q
11
del
status
.
A
total
of
104
patients
were
studied
(
55
with
TA
and
49
with
IAA
)
,
of
whom
40
(
38
%
)
were
22
q
11
del
positive
.
The
22
q
11
del
status
was
unknown
in
9
(
7
with
TA
and
2
with
IAA
)
.
In
patients
with
known
deletion
status
,
those
with
22
q
11
del
had
a
longer
hospital
and
intensive
care
length
of
stay
.
Subjects
with
22
q
11
del
also
required
more
frequent
operative
reintervention
and
more
consultations
and
were
prescribed
more
medications
at
discharge
.
No
significant
difference
was
found
in
method
of
feeding
between
those
with
and
without
22
q
11
del
at
discharge
.
In
this
study
,
22
q
11
del
is
associated
with
perioperative
outcomes
in
infants
undergoing
operative
correction
of
TA
and
IAA
,
with
longer
hospital
stays
and
greater
resource
utilization
in
the
perioperative
period
.
These
findings
should
inform
counseling
and
risk
stratification
and
warrant
additional
study
to
identify
genotype-
specific
management
strategies
to
improve
outcomes
.
Diseases
Validation
Diseases presenting
"known deletion status, those with 22q11"
symptom
22q11.2 deletion syndrome
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