Coronary Aneurysms in Patients with Autosomal Dominant Polycystic ...

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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

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autosomal

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aortie kidney

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of

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unclear.

in a minority seen

the

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Sehnaack

12.

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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

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has in

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