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Postoperative cardiac homograft involvement in Erdheim-Chester disease.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
multisystem
disorder
which
is
known
to
affect
the
skin
,
lungs
,
bone
,
pituitary
gland
,
retroperitoneum
and
cardiovascular
system
.
The
case
is
described
of
a
patient
with
ECD
who
had
previously
undergone
a
Ross
procedure
for
presumed
endocarditis
involving
the
aortic
valve
and
aortic
root
.
The
patient
subsequently
developed
arthralgias
,
abdominal
pain
(
requiring
an
exploratory
laparotomy
)
and
polydipsia
.
Furthermore
,
he
developed
progressive
,
symptomatic
stenosis
of
the
pulmonic
homograft
.
A
reoperative
replacement
of
the
homograft
was
required
.
The
clinically
suspected
diagnosis
of
ECD
was
confirmed
by
a
pathologic
analysis
of
the
explanted
pulmonary
homograft
,
and
also
(
retrospectively
)
of
previously
resected
mesenteric
tissue
.
It
is
postulated
that
the
patient
may
have
developed
ECD
as
a
result
of
an
immunologic
reaction
to
the
homograft
tissue
used
for
the
Ross
procedure
.
Diseases
Validation
Diseases presenting
"subsequently developed arthralgias"
symptom
erdheim-chester disease
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