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[Perioperative management of lung transplantations in patients with pulmonary arterial hypertension compared with that in patients with lymphangiomyomatosis].
[lymphangioleiomyomatosis]
The
number
of
lung
transplantation
has
tended
to
increase
as
a
treatment
for
patients
with
pulmonary
arterial
hypertension
(
PAH
)
and
lymphangiomyomatosis
(
LAM
)
in
Japan
.
However
,
we
have
little
evidence
about
the
comparison
of
perioperative
management
in
patients
with
PAH
and
that
in
patients
with
LAM
.
In
this
retrospective
study
,
ten
patients
with
PAH
and
seventeen
patients
with
LAM
who
underwent
the
lung
transplantations
between
2006
and
2011
were
enrolled
.
PAH
patients
received
double
lung
transplantation
with
intraoperative
cardiopulmonary
bypass
(
CPB
)
support
.
Before
anesthesia
induction
,
percutaneous
cardiopulmonary
support
(
PCPS
)
was
begun
.
Most
of
LAM
patients
received
single
lung
transplantation
without
using
CPB
support
and
PCPS
support
before
anesthesia
induction
.
But
sometimes
during
an
operation
PCPS
support
was
necessary
.
Postoperative
PCPS
support
showed
no
significant
differences
between
PAH
and
LAM
.
The
four
year
survival
rate
was
80
%
(
PAH
)
and
87
.
8
%
(
LAM
)
.
Pao
2
/
FIo
2
in
patients
with
PAH
and
in
those
with
LAM
,
%
FEV
10
in
those
with
LAM
after
surgery
improved
when
compared
to
those
before
surgery
.
The
large
difference
in
perioperative
management
between
patients
with
PAH
and
those
with
LAM
is
an
important
knowledge
for
anesthesist
.