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[Pulmonary hypertension in chronic respiratory diseases].
[lymphangioleiomyomatosis]
Pulmonary
hypertension
is
frequent
in
advanced
chronic
respiratory
diseases
,
with
an
estimated
prevalence
at
the
time
of
pulmonary
transplantation
of
30
-
50
%
in
idiopathic
pulmonary
fibrosis
,
30
-
50
%
in
chronic
obstructive
pulmonary
disease
,
50
%
in
combined
pulmonary
fibrosis
and
emphysema
,
75
%
in
sarcoidosis
,
and
more
than
75
%
of
cases
in
pulmonary
Langerhans
cell
histiocytosis
.
Histologic
features
include
varying
degrees
of
pulmonary
arterial
remodeling
(
prominent
)
,
vascular
rarefaction
(
emphysema
)
,
fibrosis
or
specific
involvement
of
the
pulmonary
arteries
(
idiopathic
pulmonary
fibrosis
,
sarcoidosis
,
lymphangioleiomyomatosis
,
pulmonary
Langerhans
cell
histiocytosis
)
,
in
situ
thrombosis
,
and
frequently
associated
involvement
of
the
pulmonary
veins
(
idiopathic
pulmonary
fibrosis
,
sarcoidosis
)
.
Pulmonary
hypertension
is
usually
detected
using
echocardiography
with
Doppler
,
however
right
heart
catheterisation
is
required
to
confirm
precapillary
pulmonary
hypertension
defined
by
pulmonary
artery
pressure
≥
25
mm
Hg
,
with
pulmonary
artery
wedge
pressure
≤
15
mm
Hg
.
When
present
,
it
is
associated
with
decreased
exercise
capacity
and
worse
mortality
.
Pulmonary
hypertension
in
chronic
respiratory
disease
is
almost
invariably
multifactorial
;
hypoxia
is
one
of
its
main
determinants
,
however
supplemental
oxygen
therapy
rarely
reverses
pulmonary
hypertension
.
Management
of
pulmonary
hypertension
in
chronic
respiratory
disease
is
mostly
based
on
the
optimal
treatment
of
the
underlying
disease
.
Available
data
do
not
support
the
use
of
drug
therapies
specific
for
pulmonary
hypertension
in
the
setting
of
chronic
respiratory
diseases
,
however
very
few
clinical
studies
have
been
conducted
so
far
specifically
in
this
context
.
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hirschsprung disease
lymphangioleiomyomatosis
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