patient was identified with end-stage renal disease, investigated by coronary angiography, and matched ..... H, Nord#{233}nG, Friman. 5, Nyberg. G: Autosomal.
Coronary Polycystic
Aneurysms in Patients Kidney Disease HENRIK
HADIMERI,*
*Depart,,ie,,t Transplant
Abstract.
Patients
disease
(ADPKD)
rysms.
Reports
not been tigate
an
The
the prevalence
aneurysms
ADPKD
patient,
coronary 15 after
a control
dominant
present
with
ment
of
often
a variety
vascular
diagnosed
lenee
is
has
a large
also
in
A
(6,7).
been
aneurysms
failure
ADPKD in
has
an
also
ducted
without
onary
aneurysms
not known.
ADPKD
present
was
not
separate with
however,
verified
of
was
in been
reports
coronary
end-stage
that
renal
study
was
eon-
Whether coris therefore
initiated
and
Methods
We
searched
angiography. are presented
for
ADPKD
patients
who
Thirty were identified. in Table 1 . Twenty-four
seventeen
of whom
transplant
patients
were (1985
had
undergone
Demographic were kidney
members
of a cohort
to
whose
1993)
coronary
background data transplant patients,
of 1 15 ADPKD
records
have
been
siveby reviewed with respect to renal and extrarenab manifestations the disease (9). Three more ADPKD patients received their transplants in the period 1982 to 1983 period 1993 to 1996. The angiographies of 1.2 yr before
transplantation
in nine
and four other patients had been performed patients
(range,
renal extenof first
from the a median
0.2 to 3.5 yr)
and
a median to 14.4 been
one
belonged
was
I 046-6673/0905-0837$03.00/0 Journal of the American Society of Nephrobogy Copyright © 1998 by the American Society of Nephrology
subjects,
had aortic
The
young.
with
very
had
siblings
the
of extracranial Nephrol
not
yet
large
In the
same
angiography nephropathy.
was
history
(range,
five
patients and
dialysis
shown
in the
in Table
in
all
cases.
Two
1 , there
were
based
others
incompletely
findings kidneys
on
had
a
parents
investigated In each
in two,
of the six cases,
made before
the diagnosis they became
reported
to have
of patients
groups
unequivocal: uremic. four
liver
and
with
cysts.
Two
between
ADPKD transplantation
patients
investigated
with
and and
before
and one control
subject
from
was
closest
in age
was
less
Table
the
study no
significant
subjects
in the
population
with
re-
time
interval
separately One
for
ADPKD
origin
other
5 yr how
difference
transplantation. The
in age than
I shows
as calculated
East.
patients
who
was of Finnish/Estonian
the Middle
of coronary
group.
was
control after
each
pairs.
angiography, and
disease
undergone
the time
Within
There
patients
was
to sex and
difference
compares
demographics.
had
diagnoses other than ADPKD or and the ADPKD patients, were
3 yr in twenty-five
population
renal
who
the control
The
less than
end-stage
identified
according
matched
in all pairs
between
with
were
to transplantation.
of investigation.
and one
56 patients
were
by origin.
radiology
reevaluated
were units
by one
requested
where
they
experienced
and received had
been
investigator
in original performed.
(Dr.
Lamm).
was considered significant if the vessel diameter was 50%. The number of vessels affected was determined ment
cvi-
in AD-
transplantation,
of the disease.
Three
at the time
group
aortic
patients
peritoneal
obvious
and had renal These patients.
four
to basic
of to the
1998)
undergone. with
of
patient
died adds
patients,
in 24 patients.
individuals
were
control
was
heredity
in relation
ADPKD
ADPKD
in fifteen
As also
populations
coronary diabetic
into
and
none
the disease.
fifty-seven
angiography
erite-
for all angiographies.
kidneys.
with
this patients
but
9: 837-841,
treated
to or
aneurysms
transplantation
of clinical polycystic
(ESRD),
eight
study
but
a family
The angiograms Univer-
another
This
to the 24 transplant
for.
the constellation All had enlarged
patient
and
surgery.
of inheritance
and two lacked
speet
aneurysm,
diagnosis
who
the
One
Soc
indication
mode
ADPKD
ectasias.
risk
respect by 50%
not fulfilling
in four
minor
to a population
ADPKD
with
patients,
In addition
dominant
with
ectasias
Universitetssjukhuset.
died
and
diameter
ADPKD
of 4.8 yr after
evaluated
reevaluated in artery
more
bypass (J Am
a clinical
Swedish 12, 1997. Accepted November 13, 1997. to Dr. Gudrun Nyberg, Transplant Unit. Sahlgrenska 5-413 45 Goteborg, Sweden.
Five
yr).
had
of Radiology
and
detected
subjects.
patients.
in each
Received May Correspondence sitetssjukhuset,
were
of an increased
separated
Patients
retrieved
as to pathologic
during
0.4
*Depart,nent
as an increase
a dissecting
dissection
to provide
information.
Materials
the control
Sahlgrenska
aortie
ease
subjects (8). of ADPKD
investigation
preva-
have
prevalence
population
published;
comparable control are a manifestation
The
but
two
the
most of
aneurysms
in
on
may
the
were
control
had
and
defined
as well
dence
Involve-
where
NYBERG
Aneurysms
PKD
aneurysms, incidence
(3,4),
patients
been
with
(ADPKD)
region increased
Coronary
report
two
manifestations.
reported (5).
ADPKD
to transplantation.
arterial
more, non.
angiography,
disease
subarachnoid
study
preliminary
relation
cause An
have to inves-
identified
by coronary
extrarenal
(1,2).
screening
described
such
10%
Thirty
was
kidney
may
in the
5 to
aneurysms
of
walls
locations
Dominant
Sweden.
aneurysms,
angiography on clinical renal transplantation. For
patient
polycystic
GUDRUN Eksj#{246},Sweden:
All angiograms
aneu-
initiated
aneurysms.
end-stage renal disease, investigated and matched for age, sex, and time
Autosomal
was
Autosomal
Goteborg,
kidney
of intracranial
in other
study
of coronary
who had undergone were identified,
polycystie
risk
present
and
Hoglandssjukhuset, Universitetssjukhuset,
dominant
increased
on arterial
LAMM,t
Medicine,
Sahlgrenska
autosomal
have
conclusive.
patients indication each
Unit,
with
CARL
of internal
with
of the
right
coronary
artery
(RCA),
the circumflex
from They
the were
A stenosis reduced
by
by involveleft
artery,
838
Journal
Table
I.
of the American
Background
data
coronary
Society
for
30
angiography
of Nephrology
patients
and
with
30
autosomal
matched
dominant
control
polycystie
subjects
with
kidney
end-stage
disease
renal
(ADPKD)
disease
who
of other
underwent
origin
Indication Group
Age Median. Range (yr)
Gender (M/F)
Number Investioated . 0 after Transplantation
Angina/Silent Ischemia
Heart Failure
Valvular Disease
ADPKD
(,i
=
30)
56,
45
to 71
20/10
15
25
3
2
Controls
(ii
=
30)
56,
45
to 73
20/10
15
26
4
0
and
the left anterior
an increase
descending
in the artery
in several
surveys
coronary
of
the
angiograms
artery.
diameter
prevalence
(10-13).
vessel
Statistical
was
was
or more,
of
Ectasias
when judged to be pathologic but aneurysm. either because the dilation reference
An aneurysm
by 50c4
aneurysms
were
defined
as
a definition
used
or ectasias
also
noted
in
as “minor”
not fulfilling the criterion of an was less pronounced or a normal
not available.
Analyses
The
Mann-Whitney
test was
used
U test
to compare
was
used
frequencies
to compare
values,
in ADPKD
and
patients
the
and control
subjects.
Results Table and
2 shows
control
angiograms
The
the angiography
subjects.
Coronary
of four
difference
is not
saccular
aneurysm,
anterior
descending
This
patient
The
angiogram
ADPKD
also
is depicted
in
aneurysms,
these
successive
aneurysms
the
other
IgA
had
nephritis
and
spectively.
The
aneurysms
(of
subelavian
artery).
Table
2.
elongated
stage
in the
each
measuring
both
aorta,
is the
disease
only
the
thoracie
patient
other
Vessels Significant Group
I
two
control
subjects,
fulfilling
the
re-
2
(arrow)
in the
with
a
in 30
of main
patients
with
Aneurysms
grams
segments
=
30)
4
2
2
18h
Controls
(ii
=
30)
8
12
5
5
.1
PTCA,
h
Numbers
percutaneous transluminal higher than in control
coronary group. P
anterior
polyeystic
of an
was
higher
coronary
As shown
ADPKD
and
in each
groupu
30
with
Minor
ectasias.
in patients
arteries
and were
in Table
matched
angioplasty. 0.0 1.
example
found
were
control
subjects
in
of patho(P = 0.01).
significant
in most
2, they
not
is given
ADPKD
ectasias,
of the
dilation
the prevalence with
none
steno-
patients more
and
frequent
with
end-
Revascularization Ectasias
5h
2
An
observed
but
pathologic
aneurysm.
the minor
were
patients,
with
PTCA
Bypass First
(ii
left
dominant
all others
ADPKD
to the aneurysms
subjects.
procedures Angio
3
ses
more
had
dilations
control
a coronary
revascularization
ADPKD
proximal
autosomal
in the clinic;
Five
criteria
In addition
and
with Sten osis
review.
3. Including
logic
diagnosed
performed
the
Figure
had
aorta,
was diagnosed
during
patients
in whom
origin:
aneurysm
with 8 mm,
previously
angiograms of
of
had
approximately
three
aneurysm
in
One
patient two
1. Saccular
descending artery of a patient kidney disease (ADPKD).
a similar
subjects
one
Figure
1).
aneurysm.
ectasias.
The
left
aneurysms
control
RCA;
the
gbomerulonephritis.
had
in coronary renal
two
2 X 3 cm.
abdominal
Findings
In
seen
patient
This
artery
or fusiform
2.
in
(Figure
showed
other
biopsy-verified
latter the
the
distinctive
found
a renal
in
subjects.
patient
patient
whereas
not
was
to have
measuring
control
A
ADPKD
ADPKD
Figure
two
patients detected
significant.
one
known
were
one
and
8 mm, of
another
but smaller aneurysm, ADPKD patients were these
were
statistically
measuring
of
in ADPKD
patients
artery
was
findings aneurysms
0
Choice
Surgery After
PTCA
6
13
1
9
9
3
Coronary
posterolateral
branch
life-threatening
of the
As
of
June
subjects
malignancy
(two).
circumflex
aneurysm.
heart (two), One
died
control
patients
aneurysms
has
of the
ischemic
with
patients
disease dilated
undergone
and
four the
was
the
are alive;
surgery
with
two
with
a
aneurysms Two
of
control
uremia.
Both
the one with
to correct
of
aneurysm (one), and
are alive.
disease,
control causes
patient
patients
arteries
heart
no
dissec-
(two), dissecting cardiomyopathy latter
aneurysms
group,
patients,
of nine
of coronary
of
control peripheral
ADPKD
Seven
dilatations
patients
the
839
but one of the patients
ADPKD
Among
death were ischemic of the thoracic aorta
In the
a moderate
seven
died.
in ADPKD
occurred,
developed
1997,
have
or minor
RCA.
complications
(without aneurysm) tion during PTCA.
Aneurysms
multiple
it.
Discussion Coronary
aneurysms
ADPKD Figure
2. Fusiform
coronary
artery
aneurysm
(arrows)
of a patient
in the proximal
part of the right
ADPKD.
with
in two
connection
(6,7).
genesis
form
32
(34%).
lower
The
in our
still
higher
known.
A series
control
aneurysms
of 30, or 13%).
four
rysm
but
bers
between
the
previous
Therefore,
with
ESRD
and
in the were
of all angiograms
for
made
The
discrepancy
comparison,
and
one
is
sus-
in
the
in num-
may
matched
this with
easy
studies
study,
by
much
of an aneu-
always
present
present
used
was
patients
is not
change.
with
was
( 1 .5%) ( 1 1). on the definition
evaluation
of atherosclerotic
of that. jects
the
found
However,
of
has
prelimi-
patients
aneurysms
population
heart disease to be agreement
in
were
ADPKD
a
patho-
to ours
and
(only
pected coronary There seems presence
similar
of definite
in a general
(10-13),
in
of
the
or ectasias
performed
with
a hint in
subjects,
prevalence
series than
only
is significant
study,
angiograms
in association
gives
ESRD
without
(8). In that
1 1 of
ESRD
is not yet
reported
which
Whether
published,
nary
been
reports,
of aneurysms
been in
have
case
be
a result
control the
sub-
evaluation
observer.
Coronary artery aneurysms were found in two of the control subjects. The difference in numbers between ADPKD patients and control subjects was only statistically significant when ectasias also Figure
parts
3. Pathologic of
the
definition
dilation
right
coronary
artery
of an aneurysm
reference
vessel
(arrows) of
of the
proximal
a patient
with
is not fulfilled
segment.
The
change
because
is reported
and
middle
ADPKD.
there
The
is no normal
as a “minor
not
fulfilling
considered.
the adjacent due
normal
not
One arterial
of the
patients (P = 0.002). However, revascubarizawere considered indicated in similar numbers
the
extracelbular
At
the
(in
patients
kidneys
ADPKD
patients
complicated
and
with
by
1 8 control
an RCA
transmural
subjects).
aneurysm,
bleeding
In one
bypass
from
one
grafts. During dissection of
resuture of a proximal anastomosis, the aorta occurred and the patient
complications
of
plasty
with
(PTCA)
a minor
occlusion, with
transluminal
occurred
ectasia
which
no observed
percutaneous
also
in the
in the
required ectasias,
angiogram
implantation occluding
surgery of
the
group.
developed of 5 stents. dissection
was venous
an extensive died. Two
coronary
ADPKD
of the
angioA patient
has
than
50%
normal
were
wider
not be classified,
the
patients This
were heredity that
such for
reported for
the that,
than
because,
reference
diameter
have
as has his
been
abdominal
ADPKD,
or
for
(14). genes
history
a general
of
four
to the
pre-
disorder
proposed
of
in ADPKD.
aortic as well
aneurysm,
his
as atrophic.
He
aneurysms,
be a representative
ADPKD cases of ADPKD
unique in addition
to be multicystic,
he might
of undetected the prevalence
had
suggests
he might
of surgery
time
no
but
of the
one
large
There is a case in populations
may
number
for studying with aneu-
rysms.
The to have
In another,
patients,
in a
control
matrix
speculate
dissecting occurred
lesions,
of an aneurysm
less
or could
glomerulonephritis,
in the ADPKD tion procedures 1 9 ADPKD
definition either
be assessed.
aneurysms.
sumed
strict were
vessel
to arteriosclerotic
could
ectasia.”
the
These
arteries
control had
patients, less
at least involved.
although
pronounced
with
respect
What
is the
otherwise
well
atherosclerosis
to number significance
matched,
than
of
the
major
of that
seem ADPKD
coronary
difference
for
840
Journal
the
of the American
outcome?
In
aneurysms
may
a population
with
have
or hidden
incidence
disrupted
of aneurysms
may
so in the ADPKD might
have
been
classified
parts
of the
artery
shown
only
consider
them
artery
dilation
ously
or
in
erosclerosis
has
demonstrated often
of
ity of the
vessel
multiple
wall
(24).
Continued
molecular
such
diversity.
general
due
aneurysms
to their such
tendency
a case.
coronary
ectasia,
when
ADPKD,
was
3. 4.
the two
and
clinical
are
not clear.
locations
the
may
The
GM,
Fick
anatomical
kidney
Chapman
for
pericardium
ventricular
of a coronary fact
been
by rupture
aneurysm
JR.
aspect
of aneurysms
has been
complicating
factor,
in a vertebral
a major
may
such
in
cause
events
In our series PKD, one died
one
aneurysm
transplant
patients
aneurysm,
patient
had
has
a coronary
aneurysm, the
link
the
organs
have No
been such
family artery
are
in adults only
reported
was
was
aneurysm
for ADPKD
remains
perhaps
a renal
aneurysms
of ADPKD
in certain
in this
material,
One
artery
share
The
patient
aneurysm
a defect
possible
patients
families but
and
the
as well.
It may
present
17.
Transplant
12:
be
in the
Herzog
J
CA:
in patients
disease
5, Nyberg
[Abstracti.
G: Autosomal transplant
1431-1436,
1997
AJ: Aneurysmal 1983 Pridie
coronary
RB:
dom-
population.
PA, Judkins
MP, artery
Coronary
in 4993
artery
Kemp disease.
ectasia:
Br Heart
patients.
Pfeifer JF, Lopes MG, Hubtgren arteries in the adult. Radiology Stewart
and
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in
Heart
Its
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search
RAH:
disease
Coronary
Offord
KP:
artery
ectasia:
Epidemiology.
KD,
Local
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of Polvcvstic
JJ, Gardner T.
HN: Aneurysms of 1 17: 1 1-18, 1975
? (‘atheterization
Management 1985,
Fujikawa
Kidney
Kansas
City,
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Kidney
Re-
pp 49-69
Ueda
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as a first surgical
Miki
5,
Konishi
manifestation management.
T:
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aneu131:
1996 MB,
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V, Mansukhani
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Weblford dissection:
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kidney
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and significance
by Grantham
artery
A,n
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in a kidney
LD, Litwin
edited
Plumhoff
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artery
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a coronary
disease
Diagnosis
Jorgensen
aneurysms
1994
kidney
33: 116-119, 1994 Torres VE, Holley KE,
taneous
(23-25).
with not
16.
TD,
polycystie
5: 653,
PS, Fisher
618-620,
an-
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Henry
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M:
pobycystie
1993
coronary
dominant
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cells
Furthermore,
MD, of
or diffuse
Iga
or involvement
1996
Wehling
1994
MJA,
15.
polycystin,
(21,22).
with
pathology
is
other
a patient
Williams
ill
X,
dominant
7: 2483-2486,
in
Kraemer
Dial
and autosomal
arteries
autosomal
Swaye
1997
coronary
Arteriosclerosis
aortie kidney
L, Estivill
K,
incidence
Hadimeri
Thoracic
C, Perez
Sot- Nephro!
JAm
abdominal
polycystic
12: 171 1-1713, C, Bru
71 : 150-152, K, Sidaway L, Glatter with adult polycystie
5K,
and
Theisen
13.
14.
ADPKD
and
Swan
dominant
5,
Lipton MJ, the coronary
of
will
protein
unclear.
in a minority seen
the
146,
1994
Sehnaack
12.
The walls.
in
of epithelial
not complete.
had
with
formation
to be clustered
clustering
patients
with
defect
found
history
pop-
vessel
cases
1 140-1
45: 23-29,
dominant
Cliii Invest
prevalence 392-395.
of
an indication
arterial
identified
case
surgery.
A,
in autosomab
U,
Circulation 67: 134-138, 1 1 . Hartnell GG, Parnell BM,
AD-
in that
bypass
10.
consequences.
to a membrane
eurysms
lethal
Macnicol
hat 45:
Med
aortie aneurysms
disease.
disease:
inant
another
occurred
possibly of
The mechanism for aneurysmal unknown. The PKDJ gene codes which is present in the cytoplasm in various
A new
now
disclose
of autosomal
Rev
Transplant
HG, Mudd JG, Gosselin
Most
with
of
Nephrol
undiagnosed. abdominal
9.
PTCA.
( I 6,20).
C,
Kidney
autosomal
Bechtel
J Am Soc Nephro!
been
( 19) and
during
isehemia
M,
with
ar-
has
ADPKD
patients
of coronary
a predisposing abnormality Prolonged follow-up of
of dissection
Dissection
(9).
may
aneurysms
history
Anna
C, Badenas
kidney
Christ
Increased
of coronary
with
studies
AJW: Pobycystie kidneys 1: 646-647, 1980 A, Feest TG, Dudley CRK:
Dial
127: 1411-1423,
has in
phenomenon and as never been implicated
one.
our
aneurysm
as a complication
further
of
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