Jorge L. Pinzon, MD #{149} Patricia E. Burrows, MD, FRCPC #{149} Lee N. Benson, MD, FACC, FRCPC C. A. Frederic Mo#{235}s,MD #{149} Nancy E. Lightfoot, PhD #{149} William G. Williams, MD, FRCSC Robert M. Freedom, MD, FACC, FRCPC

Repair ofCoarctation in Children: Postoperative To determine the morphologic sequelae after surgical repair of coarctation of the aorta, the authors retrospectively reviewed angiograms and hemodynamic and clinical data on 215 patients who underwent cardiac catheterization after surgical repair of coarctation of the aorta during a 13year period. Ninety-seven patients (45%) underwent coarctation reseclion with end-to-end anastomosis; 92

ofthe

Aorta Morphology’

variety of surgical repairs of coarctation of the aorta are in current use (1-3). Coarctation resection with end-to-end anastomosis, in spite of recent improvements in technique, is associated

with

a significant

mci-

of repair site to diaphragmatic aorta greater than 1.5. Transverse-arch or isthmic hypoplasia or recoarctation was detected in 86 patients (40%) and was most commonly

(4-6). The suba popular has not (7-10), and aneurysms at the repair site after this procedure have recently been reported (11). Technical modifications have been introduced to avoid redoarctation (12), preserve the arterial blood flow to the left arm (13,14), and increase the diameter of a hypoplastic transverse arch (14-17). Synthetic patch aortoplasty carries the added risks of thrombosis and infection and

associated

may

(43%),

subclavian-flap

angioplasties;

and 26 (12%), synthetic-patch Sixty-four patients (30%) had “aneurysm,”

ment

defined

repairs. an

as a measure-

ratio

with

septal

defects

or ob-

dence of recoarctation clavian-flap arterioplasty, approach in newborns, avoided recoarctation

be associated

with

late

aneurysm

struction of the left ventricular outflow tract. Pullback systolic pressure gradients at catheterization were significantly higher (P .0001) in the

formation (18,19). Recent reports have also described a significant incidence of aneurysms after balloon dilation angioplasty of native coarctation

patients

with

transverse-arch

(20-23).

poplasia

and

recoarctation

=

those

with

diaphragmatic Significant structions

surement diameter

ratios

hy-

than

of transverse

aorta

greater

postoperative

in arch

than arch

to

0.9.

ob-

can be predicted with mearatios on the basis of the of the abdominal aorta.

Index

terms: Aneurysm, aortic, 562.73 #{149} Aorta, stenosis or obstruction, 562.1511 #{149} Aorta, surgery, 562.4545 #{149}Aorta, transluminal angioplasty, 942.454 #{149} Children, cardiovascular system

Radiology

1991; 180:199-203

This study was undertaken to determine the relative incidence of aneurysm formation as well as residual obstruction following three surgical repairs and whether a relationship exists between the development of so-called “aneurysms” and the type of or time since repair. At the same time, this study was to test the usefulness of measurement ratios in predicting postoperative residual arch obstructions. MATERIALS

AND

The records of patients angiography after repair the aorta December

duningJanuany 1988 were

METHODS who underwent of coarctation of

1976 obtained

through from the

From the Department of Diagnostic Imaging (P.E.B., C.A.F.M.), Divisions of Cardiology (J.L.P., L.N.B., N.E.L., R.M.F.) and Cardiovascular Surgery (W.G.W.), and Variety Club Cardiac Cathetenization Laboratories, The Hospital for Sick Children and the University of Toronto, Toronto. From the 1989 RSNA scientific assembly. Received February 9, 1990; revision requested March 27; revision received January 7, 1991; accepted March 21. Address reprint requests to P.E.B., Department of Radiology, The Hospital for Sick Children, 555 University Aye, Toronto, Ont, Canada M5G 1X8. ( RSNA, 1991

computerized

the

angiographic

Hospital

Cardiac

for Sick

data

Children,

catheterization

grams

of 231

cations for phy included lesions sessment

data

patients

recurrent

angio-

reviewed.

mdi-

required further signs of residual

obstruction.

Enlarging

eurysms were diac catheterization

not

before of these

tients.

Sixteen

patients

from

the series

diagnosed in any were

owing

tion 215

of the aortic arch remaining children

the

total

population

coarctation

repair

the

period

13-year The

215

125)

=

were

the the ing

outlines nearest aortograms

made

aortic

mon

with

=

if the aortic arch Measurements

electronic

arch

carotid

systolic (Fig

just

artery;

that portion subclavian

90) or lateral reviewed, and

(ti

were

used only visualized.

toyo, Japan) from following locations

verse

calipers

gin of the ductus aorta at the level the narrowest aorta and their

distal (b) the

to the left cornaortic

errors

arteriosus; of the diaphragm.

caused

ratios

used for the aorta relatively

statistical analysis. at the level of the constant in relation

size

is unaffected

by

diovascular anomalies normalize the following (a) the transverse aortic

the

data

From was

(fewer

of the

aorta,

of Clarkson

data,

determined

than

for were

As the size diaphragm to patient

associated

and from were

and

a ratio to be

two

of the were

of is

car-

(24), it was used to measurements: arch, (b) the wid-

measurements projections

these

Also,

by correction

measurement

est segment isthmus. Normal giographic

isthmus,

to the left to the onand (c) the

and widest segments location and position

magnification,

and

(Mitu-

frames in the 1): (a) the trans-

of the aorta distal artery and proximal

determined. To avoid

accord-

repair. Cineangreat vessels in

of the aortas were traced to 0.5 mm. In most cases, ascendwere used, while ventnicu-

lograms were was adequately

were

over

grouped

oblique

projections

undergoing

review.

ing to mode of coarctation giograms of the aorta and (ii

The of

27%

institution

of this

patients

visualiza-

796)

=

ancarpa-

at angiography. represent (ii

asor

excluded

to poor

at this

the left anterior

at

and angiogracardiovascular

1) that clinical

arch

and

were

catheterization associated

(Table and

base

Toronto.

standard

(c) the

aortic

similar obtained

Brandt

of less

anfrom

(24).

than

pathologically

deviations

0.9 small

from 199

IA

Table

1

Associated

Cardiac

Lesions

TAA

No. of Patients Type Ventricular

Bicuspid

of Lesion

(n

septal defect aortic valve without

Left ventricular outflow Atrial septal defect Aortic stenosis Mitralstenosis Dextrocardia

aortic stenosis obstruction

tract

Other

Table Figure

1.

Schematic representation aorta. AA = ascending aorta, DA ing aorta, TAA = transverse aortic isthmus, IA = innominate artery,

common tery, C

carotid,

LSA

a = transverse diaphragmatic

=

=

LCC left subclavian

=

arch, aorta.

b

aortic

=

of Aortic

Dilatation

in Three

Type

of Repair

isthmus,

isthmal

hypoplasia

or

aorta

greater

Measurements

from

recordings

were

second

catheterization

the

were

earlier

Type

± 30

those

the

aortic

compared dent

arch

by means

unpaired

5%

level

was

test

was

used

proportions; significant.

t test. considered 5%

for

each

±

mean

2.6 (range,

age

were

level

Stuat the The

differences was

x2

in

29(13) 9(4)

were was

0.002-14.8

5.6 years ± 5.5 (range, 0.02-19.7 years). The elapsed time between

#{149} Radiology

in parentheses

64(30)

are percentages.

between

Type

of Repair

and

of the Aorta at Repair

Morphology with Focal

Site

Bulge

Diffuse (n = 20)

of Repair

Posterior (n = 20)

Anterior (n = 24)

Total (n

Group

215)

=

12

11

3

97

Subclavian-fiap

7

7

15

92

Synthetic-patch

1

2

6

26

Of the 215 patients, 97 (45%) underwent coarctation resection with an end-to-end anastomosis, 92 (43%) underwent

as a first

1.3

years),

was ne-

pair and study averaged 4.2 years ± 4.1 (range, 0.002-15.6 years). Isolated coarctation of the aorta was observed in 43 patients (20%). Associated cardiac lesions were observed in most of the patients (n 172) and are listed in order of frequency (Table 1).

200

215(100)

subclavian-flap

angioplasty,

and 26 (12%) underwent syntheticpatch repair. In 24 patients, syntheticpatch repair was performed to correct recoarctation; in two, it was used to enlarge a hypoplastic transverse arch

considered

at catheterization

=

Site:

± 1

group

A cutoff value significant.

Two-thirds of the patients male. The mean age at repair and

Repair

Diaphragmatic Aorta Ratio > 1.5

26 (12)

End-to-end

RESULTS

years

with

No. of Patients

of

of the two-tailed

to determine the

of Repair

catheterization.

All values are expressed as mean standard deviation. Systolic gradients across

62

patient,

40 months

postoperative

Types

No. of Patients Dilated-Aortic-Segment

were calculated. underwent a

with

1

153

Synthetic-patch

pressure

for each

compared

7

5

26(13)

Table 3 Relationship

after the initial study; the angiographic measurement ratios and systolic catheter gradients

10

17

97 (45)

1.5 (11,19).

pullback

and the systolic gradients A subgroup of 33 patients

27

86)

=

recoarcta-

than

obtained

39 12 24 18

92 (43)

Note-Values

tion in our population. Aneurysmal dilatation was defined as in the literature: a ratio of the dilated aortic segment to diaphragmatic

100 51 40 39

End-to-end Subclavian-flap

Total

and

(n

ar-

the mean) for both transverse arch:diaphragmatic aorta and isthmus:diaphragmatic aorta ratios and was used to define the presence of transverse-arch hypoplasia

Transverse.Arch

Hypoplasia

Patients

left

=

215)

2

Prevalence

of the descendarch, I =

=

No. of Patients with

procedure.

The

mean

ratio

ences in the percentage of patients with an aneurysm after the three types of repair (P = .659). However, the morphology of the aorta at the repair site appeared to be related to the type of repair. Most aneurysms in patients with subclavian-flap angioplasties

and

synthetic-patch

repairs

were

of

repair site to diaphragmatic aorta measurement for the group of 215 patients was 1.45 ± 0.3. Sixty-four patients (30%) had an aneurysm (ratio > 1.5 at the repair site) (Table 2). The morphology of the aorta at the repair site consisted of diffuse enlargement without focal bulging of the wall in 20 patients, a focal posterior bulge in 20, and an anterior focal bulge in 24. There were no significant differ-

directed anteriorly (Fig 2), while 88% of the patients with an aneurysm after coarctation resection with endto-end anastomosis showed a predominance of posterior bulges or diffuse enlargement (Figs 3, 4); anterior bulges developed in just three patients (Table 3). There was no relationship between the presence of an aneurysm and time elapsed after repair, repair type, or age at the time of repair. Thirty-three patients in this series underwent a second postoperative

July 1991

Table 4 Measurement

Ratios Ratios Ratios and Ranges for Total Group (n = 215)

Ratios Transverse aortic arch to diaphragmatic aorta Repair site to diaphragmatic Isthmus

0.96

0.23

aorta

± 0.25 (0.45-1.78) 1.44 ± 0.27 (0.80-2.44)

Note-Ratios

are presented

as means

1 standard

±

deviation.

Values in parentheses

of Repair

Synthetic-Patch (n

0.90 ± 0.18 (0.53-1.40) 1.44 ± 0.30 (0.73 ± 2.52) 1.23 ± 0.28

1.18 ± 0.26 (0.51-1.91)

(0.48-1.95)

and Ranges by Type Subclavian-Flap (n = 92)

0.99

(0.45-1.96) 1.46 ± 0.29 (0.74-2.52) 1.20 ± 028

aorta

to diaphragmatic

±

End-to-End (n = 97)

26)

=

1.08 ± 0.26 (0.67-1%) 1.57 ± 0.34 (1.05-2.28)

1.25

(0.48-1.90)

0.35

±

(0.53-1.95)

are ranges.

Figure 4. Aortogram obtained in axial left anterior oblique projection shows focal

Figure

2. Aortogram shows anterior 3.6 years after

jection patient plasty.

obtained in lateral probulge in a 3.7-year-old subclavian-flap arterio-

bulge, predominantly synthetic-patch-repair year-old patient Figure 3. Aortogram obtained in left antenor oblique projection shows transverse-arch hypoplasia plus postcoarctotomy posterior bulge with end-to-end anastomosis in a 7.7year-old patient 7.6 years after repair.

struction;

catheter study the initial one.

40 months The mean

operative

site:diaphragmatic 0.25 at the first

ratios study and

were 1.45 1.4 ± 0.26

the

second

surement

ratio

The

increased

±

at

mea-

significantly

in only one child, from 1.34 to 1.51, oven 21 months. The configuration of the aneurysm did not change. Measurement ratios for each repair are

in Table

poplasia

4. Transverse-arch

was

present

hy-

in 86 (40%)

of

and four (2%), a synrepair. There was no rela-

tionship

and (P

had sia;

between

patient .129).

=

age One

recoarctation 101

Volume

of these

180

arch

at the

hypoplasia

time

hundred

of repair fifty

or isthmic also

had

#{149} Number

1

patients

hypopla-

a hypoplas-

tic transverse

arch.

These

was

used

to

obstruction.

DISCUSSION

outcomes

were not related to type of surgical repair. The hemodynamic impact of the angiographic observations was assessed

with

systolic

gradients

time of catheterization mean systolic arch similar

215 patients: 36 (17%) had undergone coarctotomy with end-to-end anastomosis; 46 (21%), a subclavian-flap

angioplasty; thetic-patch

of 0.9

residual

at the site of in a 10.2repair.

30 after

±

catheterization.

10.1

a ratio

define

posterior, angioplasty years after

at the

among the three (P = .5362) (Fig 5).

types

of re-

ratio

(arch

or isth-

mus:diaphragmatic

aorta)

to 0.9 correlated

less

with

than

a clini-

observed in patients with recoarctation, transverse-arch

hypoplasia, (P = .0001)

and

than

recoarctation

in those

without

ob-

of co-

are influenced

of the lesions,

aortic arch, and mode

of repair attempted

(11,18,19,25,26). Our to define whether

study there

was

relationship

repair

any

late

aneurysm

of coanctation

iso-

correction

aorta

among

type, aneurysm formation time elapsed after repair. Previous studies have on

cally significant obstruction measured by means of catheter gradient. Significantly higher gradients (P = .0002)

were lated

of surgical

of the

by the morphology associated cardiac

(Table 5). The gradients were

pair A measurement equal

Results

arctation

thetic

materials

(1), and identified

formation

of the

after

aorta

(18,19,25,27)

repair

with and

synafter

subclavian-flap arterioplasty (11), defining the presence of an aneurysm with a measurement ratio greater than 1.5 for the repair site:diaphragmatic aorta (11,19). Many of these aneurysms

are

detected

as enlarging

Radiology

#{149} 201

masses

on

routine

chest

radiographs,

but some have ruptured, with fatal outcome. A true aneurysm is one in which the wall is formed by the stretched tissue of the artery itself, whereas a false aneurysm develops when blood leaks out of the artery

and

excavates

rounding

Our in

a cavity

tissues

review 30%

of

all

trihution

or

in the

identified

true

aneurysms

patients,

with

equal

to repair

type,

suhclavian-flap

66%

with

of synthetic-patch

70

60

50%

an

a few

repairs

were

present

weeks

after

aneurysms

probably

liberate achieve as

attempt as large

possible

end-to

at

angiogra-

surgery.

These

represent

or a combination

of preex-

dilatation

by Olsson et al (27). These implicated the effect of dif-

tensile

strengths

a noncompliant Other studies, have suggested lack

the

with

the

lamina

the

patch

of a collagen

scar

may

factors (29). More has been expressed intimal

in the and

the

aortic

resection

carried

to resect Infection

have other

also been considered among possible stimuli of aneurysm (18). early

after

The

the and

presence

surgery,

relationship with time after and the lack of significant serial examinations of some children indicate that most aneurysms do not progress during childhood, but grow child. The factors that cause

202

#{149} Radiology

wall

exout

posterior thrombosis

of aneulack of repair, change at of these of these or regress with the some of

the

have In 40% and

Pullback

flap

synthetic

patch

systolic

aneurysms

gradients

to grow

later

by type

OF

REPAIR

of repair.

in life

not been revealed by this study. our study group of 215 patients, had transverse-arch hypoplasia 29% had recoarctation. The study was biased as residual

toward obstruction

interpreting

other

aminations,

such

these

diagwas one

raphy

and

aortic-imaging as computed

magnetic

extomog-

resonance

imag-

ing. rate

and

residual

after

three

Last,

this

report

of so-called

outlines

a

aneurysms

aortic-anch different

obstructions types

measurement

the diameter

concern the

repair (25,26).

rysms

subclavian

of repair.

The presence of a focal enlargement of the aorta at the repair site appears to be a common angiographic finding.

presence

during shelf

formation

of

be contributing

recently, regarding

these

5.

In summary,

aor-

use

Figure

similar

foreign material. based on histology, that the additional

of elastic

opposite

tensive

across

in conjunction

a

obstruction and an angiographic ratio of 0.9 or less for the transverse arch on isthmus. These data may be useful in

after synthetic patch repair is compliance mismatch, or maldistribution of mechanical forces in the aortic wall,

tic wall

S

of the indications for cardiac catheterization. No relationship was observed among arch hypoplasia, type of repair, aneurysm formation, or age. Our data have shown a good correlation between significant hemodynamic

type of repair raises questions concerning etiology. A widely accepted theory about aneurysm formation

ferent

end

TYPE

group noses,

a de-

by the surgeon to a diameter of repair

poststenotic

postulated authors

!---

8

show-

and redundant patch. The lack of relationship between the presence of an aneurysm and the isting

-

bulge

anterior

aneurysms

phy

-

of

0

and

at the repair site in the left anterior oblique or lateral projection at angiography. The antenor bulge may be due to a combination of the ductal ampulla and the oblique aortoplasty suture line. There was no clear relationship between the presence of an aneurysm and time elapsed after repair. In some patients, ing

I

dissurgical of the to be

arterioplasties

0

U

(28).

of the three repair groups. The morphology aneurysm, however, appeared

0

80

sur-

adventitia

in each

related

90

of the

ratios

involving

aorta

at the

level

of the diaphragm as the normalizing factor are useful in predicting hemodynamically significant arch obstructions. U Acknowledgments: sistance Scorizzi

We acknowledge

of Cameron Finlay, in the preparation

3.

4.

5.

the as-

BSc, and Susan of this manuscript.

6.

References 1.

2.

Trinquet F, Vouhe PR, Vernant F, et al. Coarctation of the aorta in infants: which operation? Ann Thorac Surg 1988; 45:186191. Ziemer C, Jonas RA, Perry SB, Freed MD, Castaneda AR. Surgery for coarctation of the aorta in the neonate. Circulation 1986; 74:1-25.

7.

8.

Hesslein PS, McNamara DG, Morniss MJH, Hallman GL, Cooley DA. Comparison of resection versus patch aortoplasty for repair of coarctation in infants and children. Circulation 1981; 64:164-168. Metzdorff MT, Cobanoglu A, Grunkemeier CL, Sunderland CO, Starr A. Influence of age at operation on late results with subclavian flap artenioplasty. J Thorac Cardiovasc Surg 1985; 89:235-241. Williams WG, Shindo G, Trusler GA, Dische MR. Olley PM. Results of repair of coarctation of the aorta during infancy. Thorac Cardiovasc Sung 1980; 79:603-608. Pellegrino A, Deverall PB, Anderson RH, et al. Aortic coarctation in the first three months of life: an anatomopathological study with respect to treatment. J Thorac Cardiovasc Sung 1985; 89:121-127. Moulton AL, Brenner JI, Roberts G, et a!. Subclavian flap repair of coarctation of the aorta in neonates: realization of growth potential. J Thorac Cardiovasc Sung 1984; 87:220-235. Sanchez CR, Rohinton BK, Dunn JM, Mehta AV, O’Riordan AC. Recurrent obstruction after subclavian flap repair of co-

July 1991

9.

10.

arctation of the aorta in infants: can it be predicted or prevented? J Thorac Cardiovasc Sung 1986; 91:738-746. Brown JW, Fiore AC, King H. Isthmus flap aortoplasty: an alternative to subclavian flap aortoplasty for long-segment coarctation of the aorta in infants. Ann Thorac Sung 1985; 40:274-279. Penkoske PA, Williams WG, Olley PM, et al. Subclavian arterioplasty, repair of coarctation of the aorta in the first year of life.

J Thorac 11.

12.

13.

15.

Sung

1984;

18.

19.

BG, Rutherford

20.

21.

180

#{149} Number

1

JD, Kerr AR, Neutze

23. 24.

22.

25.

26.

JM.

Prosthetic repair of coarctation of the aorta with particular reference to dacron onlay patch grafts and late aneurysm formation. Am J Cardiol 1985; 56:342-346. Cooper RS, Ritter SB, Rothe WB, Chen CK, Griepp R, Golinko RJ. Angioplasty for coarctation of the aorta: long term results. Circulation 1987; 75:600-604. Brandt B, Marvin WJ, Rose EF, Mahoney IT. Surgical treatment of coarctation of

Isner

Payne

JM, Donaldson

DD, Cleveland

RF, Fulton

RJ.

Cystic

D, Bhan

medial

necrosis in coarctation of the aorta: a potential factor contributing to adverse consequences observed after percutaneous balloon angioplasty of coarctation sites. Circulation 1987; 75:689-695.

Ritter

SB.

flated

or realistic?

Coarctation

balloons: in13:696-699. Brandt PWT. Aortic diameand young children: norma-

Clarkson PM, tens in infants

tive angiographic

the aorta after balloon angioplasty. J Thorac Cardiovasc Sung 1987; 94:715-719.

92:1005-1012.

Mendonca JT, Carvalho MR. Costa RK, Filho EF. Coarctation of the aorta: a new surgical technique. J Thonac Cardiovasc Sung 1985; 90:445-447. HantJC, Waldhausen JA. Reversed subclavian flap angioplasty for arch coarcta-

Volume

17.

87:894-900.

Martin MM, Beekman RH, Roochini AP, Crowley DC, Rosenthal A. Aortic aneurysm after subclavian angioplasty repair of coanctation of the aorta. Am J Cardiol 1988; 61:951-953. Dietl CA, Tomes AR. Coarctation of the aorta: Anastomotic enlargement with subclavian artery: two new surgical options. Ann Thorac Sung 1987; 43:224-225. Meier MA, Lucchese FA,Jazbik W, Nesnalla IA, Mendonca JT. A new technique for repair of aortic coarctation: subclavian flap aortoplasty with preservation of blood flow to the left arm. J Thorac Cardiovasc Surg 1986;

14.

Cardiovasc

16.

tion of the aorta. Ann Thorac Sung 1983; 36:715-717. Elliott MJ. Coarctation of the aorta with arch hypoplasia: improvements on a new technique. Ann Thorac Surg 1987; 44:321323. VincentJG, Daniels 0, Oort VA, Lacquet LK. Hypoplastic aortic arch with aortic coarctation: surgical correction. J Thorac Cardiovasc Surg 1985; 89:465-468. Kirsk MM, Perry B, Spooner E. Management of pseudoaneurysm following patch grafting for coarctation of the aorta. J Thorac Candiovasc Sung 1977; 74:636-639. Clankson PM, Brandt PWT, Barratt-Boyes

27.

29.

Pediatr

Cardiol

of coanctation

of the aorta:

a fol-

low-up study. Scand J Thorac Cardiovasc Sung 1976; 10:139-143. TitusJL, Kim H. Blood and lymphatic vessels. In: Kissane JM, ed. Anderson pathology. 9th ed. Vol 1. St Louis: Mosby, 1990; 752-803. Del Nido PJ, Williams WG, Wilson GJ, et

al.Synthetic I,

data.

1985; 6:3-6. DeSanto A, Bills RG, King H, Waller B, Brown JW. Pathogenesis of aneurysm formation opposite prosthetic patches used for coarctation repair: an experimental study. J Thorac Cardiovasc Sung 1987; 94: 720-723. Hehrlein FW, Mulch J, Rautenburg HW, Schlepper M, Scheld HH. Incidence and pathogenesis of late aneurysm after patch graft aortoplasty for coarctation. J Thorac Candiovasc Sung 1986; 92:226-230. Olsson P, Soderlung 5, Dubiel WT, Ovenfors CO. Patch grafts or tubular grafts in

the repair

28.

and

JACC 1989;

coarctation aneurysm 74(suppl

patch

angioplasty

for repair

of the aorta: experience formation. Circulation 1):1-32.

Radiology

of

with 1986;

#{149} 203