Extending the limits for modified Fontan procedure : J.E. Mayer, H. Hlgaso, R.A. Jonas, P Lang, F.J. Vargas, and A.R. Castaneda

J
THoRAc CARDIOVASC SURG
92:1021-1028, 1986
Extending the limits for modified Fontan
procedures
During the early development of atriopulmonary anastomotic operations (Fontan-Kreutzer), a number of
physiologic and anatomical limits were proposed by the Fontan group as selection criteria. Among 167
consecutive patients undergoing modified Fontan procedures from 1973 through 1985, 109 (65%)
patients exceeded one or more of the original selection criteria in areas of age, anomalies of systemic or
pulmonary venous connection, pulmonary artery distortion, and pulmonary artery pressure. Twenty-six
patients had a mean pulmonary artery pressure greater than 15 mm Hg, with 16 operative survivors
(62%). Nineteen patients had anomalies of systemic and/or pulmonary venous connection, and 16
survived (84 %). There were 44 patients under the age of 4 years, and 26 survived (59 %). Twenty-five
patients were older than 15 years, and 23 (92 %) survived the Fontan procedure. Pulmonary artery
distortion, relating to prior palliative operations, was found in 34 patients. Seventeen of these 34 surviveda
modified Fontan procedure (SO %). Twenty-six patients had a pulmonary arteriolar resistance more than
2 Wood units times square meter, and 14 survived(54%), whereas 81 of 93 with a pulmonary arteriolar
resistance of less than 2 U . m' survived(87 % ). Multivariate analysis showed that pulmonary arteriolar
resistance and pulmonary artery distortion had a significant, negative impact on survival, but age and
anomalies of systemic and/or pulmonary venous connection did not. Pulmonary artery pressure was not
an independent predictor of outcome. The results show that the original criteria may be exceeded in the
areas of age and anomalies of pulmonary or systemic venousconnection. Pulmonary artery pressure alone
should not contraindicate a Fontan procedure if pulmonary arteriolar resistance is low. Pulmonary artery
distortion from a prior palliative operation and elevated pulmonary arteriolar resistance increase the risk
of a Fontan procedure.
John E. Mayer, Jr., M.D. (by invitation), Hrodmar Helgason, M.D. (by invitation),
Richard A. Jonas, M.D. (by invitation), Peter Lang, M.D. (by invitation),
Florentino J. Vargas, M.D. (by invitation), Nancy Cook, D.Sc. (by invitation),
and AIdo R. Castaneda, M.D., Boston, Mass.
During the early development of atriopulmonary
anastomotic operations, a number of criteria were
suggested by Choussat, Fontan, and co-workers' to aid
in the selection of patients for these procedures. However, the extent to which these criteria can be extended has
not been evaluated. In a review of all 167 consecutive
patients undergoing modified Fontan procedures at
From the Departments of Cardiology and Cardiovascular Surgery,
The Children's Hospital, and the Departments of Pediatrics and
Surgery, Harvard Medical School, Boston, Mass.
Read at the Sixty-sixth Annual Meeting of The American Association
for Thoracic Surgery, New York, N. Y., April 28-30, 1986.
Address for reprints: John E. Mayer, Jr., M.D., Cardiovascular
Surgery, The Children's Hospital, 300 Longwood Ave., Boston,
Mass. 02115.
Children's Hospital, Boston, from 1973 through 1985, a
total of 109 (65%) patients were identified who
exceeded one or more of the original ten criteria in the
areas of age, anomalies of systemic and/or pulmonary
venous connection, pulmonary artery deformity, and
pulmonary vascular hemodynamics. This report
describes the surgical results in this group of patients
and explores the relative importance of these four
criteria in predicting surgical outcome.
Methods
The hospital records and catheterization data of all
167 patients undergoing modified Fontan procedures at
Children's Hospital between 1973 and 1985 were
reviewed. For this report, the following variables were
recorded:
1021
1 0 2 2 Mayer et al.
Age
Year of operation
Diagnosis
Anatomy of systemic atrioventricular (AV) valve (left
or right sided; mitral, tricuspid, or AV canal)
Spatial relationship of atrial baffle and systemic AV
valve
Prior shunt (yes/no)
Prior pulmonary artery band (yes/no)
Anomaly of systemic venous return (yes/no)
Anomaly of pulmonary venous return (yes/no)
Hemoglobin concentration
Arterial saturation
Mean pulmonary artery pressure (PAP)
Ventricular end-diastolic pressure
Pulmonary arteriolar resistance (indexed) (PARI)
Pulmonary-systemic flow ratio
Pulmonary artery distortion (yes/no)
Type of atriopulmonary connection
Presence of valve in atriopulmonary connection or in
right side of circulation (yes/no)
History of heart failure
History of pulmonary hypertension
A diagnosis was assigned to each patient on the basis
of a synthesis of preoperative echocardiography and
cardiac catheterization, operative findings, and autopsy
results (when available). Tricuspid atresia was the
diagnosis assigned in patients with situs solitus, an
atretic right AV valve, and dextro-loop of the ventricle,
with or without transposition of the great arteries. The
diagnosis of single ventricle was assigned to patients
with both atria emptying into a single ventricular
chamber (double-inlet left or right ventricle). One or
both AV valves could be patent, and there could be an
"infundibular" chamber connected to the dominant
ventricle via a bulboventricular foramen. Either dextroloop or levo-loop of the ventricle could be present.
Heterotaxy syndrome was identified when typical systemic and/or pulmonary venous connection anomalies,
atrial septal defect, and AV valve anomalies (generally
complete AV canal) were present. Pulmonary atresia
with intact ventricular septum was diagnosed when
there were two ventricles with dextro-loop, each fed by a
patent AV valve, and there was atresia of the outlet
valve of the right ventricle. Hypoplastic left heart
syndrome was defined as a prohibitively small left
ventricle (end-diastolic volume less than 20 ml/m') in
association with mitral and/or aortic atresia or stenosis
and an intact ventricular septum. A diagnostic category
of hypoplastic right ventricle and ventricular septal
defect was used for patients with a right AV valve that
straddled the ventricular septum and an underdeveloped
The Journal of
Thoracic and Cardiovascular
Surgery
right ventricle. Included in this group were patients with
dextro-transposition and straddling tricuspid valve and
patients with superoinferior ventricles. A final cetegory
of "other" single ventricle was used for patients with a
functionally single ventricular mass who did not fit into
one of the other categories.
Hemodynamic variables were derived from cardiac
catheterization data acquired by standard methods.
Pulmonary and systemic blood flows were determined
by the Fick method. Pulmonary arteriolar resistance
(indexed) (PARI) was determined from mean PAP,
pulmonary venous atrial pressure, and calculated pulmonary blood flow (indexed) and expressed as Wood
units times square meter. No calculation of pulmonary
blood flow or resistance was made when the pulmonary
arteries were not entered unless the only source of
pulmonary flow was from a single source (single ventricle without systemic-pulmonary shunt or single systemic-pulmonary shunt with pulmonary atresia). Pulmonary venous wedge pressures were used to approximate
mean PAP if the pulmonary arteries were not entered.
The presence of pulmonary artery distortion was
recorded when a review of the radiologic and catheterization reports combined with the recorded operative
findings were found to show distorted or markedly
hypoplastic peripheral or central pulmonary arteries. In
doubtful cases the original angiograms were reviewed
and a qualitative assessment was made by two of the
authors.
Anomalous systemic venous drainage was identified
when there existed systemic venous connections to the
heart other than a right-sided superior and inferior vena
cava (or a left-sided superior and inferior vena cava in
situs inversus). Included among these were connections
of a left superior vena cava to the left atrium or coronary
sinus. Anomalous pulmonary venous connection was
defined when the pulmonary veins made no direct
connection to the left atrium but drained to a systemic
vein or to the same side of the atrium as the venae
cavae.
The technique of operation has been described previously.' All operations were performed with cardiopulmonary bypass and moderate hypothermia (25 0 to 28 0
C), and in 31 patients, weighing less than 10 kg, a period
of less than I hour of profound hypothermia and
circulatory arrest was used. Atriopulmonary connections
were fashioned by a variety of techniques including right
atrial-right "ventricular" outflow chamber connection
(direct anastomosis or conduit) and right atrial-pulmonary artery connections (direct anastomosis or conduit).
A "modified Glenn procedure" was defined as a direct
Volume 92
Modified Fontan procedures
Number 6
December 1986
Table I. Causes of death after Fontan operation
(N = 167)
No.
Cause of death
Low cardiac output
Elevated pulmonary vascular resistance
Failure of single ventricle
Systemic venous obstruction
Pulmonary venous obstruction
Uncertain origin of low cardiac output
Tamponade
Noncardiac
Total
12
9
2
4
7
I
3
38 (23%)
connection between a superior vena cava and a pulmonary artery, either end of cava to side of pulmonary
artery or end of pulmonary artery to side of vena cava
(without ligation of superior vena cava-atrial junction in
the latter). Techniques for dealing with anomalous
systemic or pulmonary venous connection have been
previously described. 3
1023
Table II. Fontan procedures
Diagnosis
Success
Total
55
43
9
65
54
12
19
8
6
3
Tricuspid atresia
Single ventricle
Heterotaxy
Hypoplastic RV + VSD
PAjlVS
HLHS
Other
13
4
2
I
~
85
80
75
68
50
33
33
Legend: RV, Right ventricle. VSD, Ventricular septal defect. PA, Pulmonary
atresia. IVS, Intact ventricular septum. HLHS, Hypoplastic left heart syndrome.
Table m. PARI: Effect on survival after Fontan
operation
PARI (V. m 2)
o
-0.99
I -1.49
1.5-1.99
~2.0
Success
Total
%
40
18
22
14
42
22
29
26
95
82
76
54
Statistical methods
The outcome variable of interest in this analysis was
the failure or success of the Fontan procedure. An
operation was considered a failure if the patient died of
any cause within 30 days of the operation with or
without the Fontan connection in place or if the Fontan
connection was taken down after the initial operation.
The relationship of this outcome with each of the
potential predictor variables was examined with a
general chi square test of association. When appropriate,
categories were consolidated because of small numbers
or similarity in meaning. Continuous predictor variables
were categorized roughly into quartiles. When categories were ordered, a chi square test for trend in
proportions was used.
All predictor variables were then tested in a stepwise
logistic regression model with the Statistical Analysis
System on an IBM mainframe computer.' Indicator
variables were created for levels of categorical variables,
and trends across quartiles were tested for continuous
variables. For variables with several missing values, a
missing value indicator was created and included in the
test for that variable. A significance level of 0.05 was
required for entry into the logistic regression model.
Results
In the total group of 167 patients, 129 survived the
operative period (77%). In two survivors the atriopulmonary connection was taken down in the operating
room with reestablishment of a systemic-pulmonary
shunt. Thus 127 patients had a successful modified
Fontan operation (76%). The causes of death are shown
in Table I.
The results by diagnostic group are shown in Table II.
The best survival rate occurred in patients with tricuspid
atresia (85% operative success). The results in the single
ventricle and heterotaxy groups were nearly equal to
those of the tricuspid atresia group.
PARI had an important impact on outcome after the
modified Fontan procedure. Among the 119 patients for
whom PARI was available, the success rate declined
from 95% for PARI of less than 1 U . m 2 to 54% for
PARI of 2 U . m' or more (Table III). In Table IV, the
relationship of mean PAP to PARI and to successful
outcome with a Fontan procedure is shown. When PAP
was 15 mm Hg or less, the success rate was 80%, and
this declined to 62% in the group with a PAP of more
than 15 mm Hg, The greater importance of PARI is
shown by the success rate of 80% in patients with a PAP
of more than 15 mm Hg but with a PARI of less than 2
U . m', compared to a success rate of only 65% in the
group with a PAP of less than 15 mm Hg but a PARI of
2 U . m' or more. These data are also displayed in Fig.
1, which shows the distribution of PAP and PARI.
The results of modified Fontan procedures in patients
with anomalies of systemic and/or pulmonary venous
connection are shown in Table V. Eight patients had left
1024
The Journal of
Thoracic and Cardiovascular
Surgery
Mayer et al.
Table IV. PAP: Operative success after Fontan operation
PARI
PAP (mm Hg)
SIT
~15
>15
69/78
12/15
Totals
80/93
I
%
SIT
87
80
11/17
3/9
86
14/26
I
Total
No PARI data
;;;2 U. m2
<2 U. m'
%
SIT
65
33
27/35
1/2
54
28/37
I
%
SIT
80
50
106/130
16/26
76
122/156
I
%
82
62
78
Legend: S, Success. T, Total.
Table V. Anomalies of venous connection-Fontan
procedures
Survivors
LSVC to CS
Left Glenn
Atrial baffle
Ligation
LSVC to LA
Left Glenn
Atrial baffle/conduit
Occlusion (cath, lab.)
TAPVC
Total
Total
3
3
3
4
I
I
3*
3*
3
5
I
I
2
2
16 (84%)
Table VI. Pulmonary artery distortion-i-Foman
procedures
19
Legend: LSVC, Left superior vena cava. CS, Coronary sinus. LA, Left atrium.
Cath. lab., Catheterization laboratory. TAPVC, Total anomalous pulmonary
venous connection.
'One patient each with associated TAPVC.
superior vena cava draining to the coronary sinus. This
was managed by placing the atrial baffle which separated systemic from pulmonary venous return to the left
of the coronary sinus in four. In one patient in whom the
right AV valve was an important part of the pathway
between the pulmonary veins and the ventricle, death
occurred when the baffle partially obstructed the right
AV valve orifice. In three patients an end-to-side
connection between the left superior vena cava and left
pulmonary artery (modified Glenn) was created to
prevent obstruction of the right AV valve, and all three
patients survived. In one patient with an innominate
vein, the left superior vena cava was ligated. Nine
patients had a left superior vena cava connecting directly
to the left atrium. In five patients, a complex atrial
baffle was sewn to segregate all of the systemic venous
return on one side of the baffle and connect the
pulmonary venous drainage to the AV valve(s). Three
survived (60%). In three others a modified left Glenn
anastomosis was created, and all three survived. In one
patient the diagnosis of left superior vena cava was not
made preoperatively or intraoperatively. The cava was
Success
Total
%
No distortion
Distortion
110
17
133
34
83
50
Total
127
167
76
successfully occluded in the catheterization laboratory
postoperatively. Two patients with left superior vena
cava-left atrial connections had associated total anomalous pulmonary venous connection (TAPVC) , which
was successfully managed by a modified left Glenn and
intra-atrial baffle in one (TAPVC to left superior vena
cava) and by an atrial baffle in the other (TAPVC to
right atrium). Two others had isolated TAPVC managed by pulmonary vein-atrial anastomosis in one
(supracardiac TAPVC) and by atrial baffle placement
in the other (TAPVC to right atrium). In the total group
of 19 patients with venous anomalies, there were 16
survivors (84%).
Pulmonary artery distortion, related to previous palliative operations, was identified in 34 patients. Pulmonary artery reconstruction, in conjunction with the
modified Fontan procedure, was attempted in 27. The
results are shown in Table VI. Only 50% of patients with
pulmonary artery distortion had a successful outcome,
whereas 83% of those without pulmonary artery distortion were treated successfully. There were no survivors
among the seven patients in whom a pulmonary artery
reconstruction was not possible. The success rate was
63% (17/27) among those undergoing satisfactory pulmonary artery reconstruction in conjunction with the
Fontan procedure. In six patients the stenosis was
enlarged at the site of the atriopulmonary connection,
and all survived. All but one of the remainder had
patches placed across the stenotic area. One patient with
a proximal right pulmonary artery stenosis had a
right-sided Glenn anastomosis (without caval ligation)
Volume 92
Number 6
December 1986
Modified Fontan procedures 1025
Table VII. Age-operative success after Fontan
operation
36
Success
Total
%
0-3.99
4-15.99
26*
78
23
44
98
25
59
80
92
Total
127
167
76
"Two additional patients survived but with the Fontan shunt taken down.
-:-..
28
~ 24
~20
and a right atrium-main pulmonary artery conduit
placed successfully.
The impact of age on operative success is shown in
Table VII. A total of 69 patients were outside the 4 to 15
year age range and 74% survived, although two patients
in the under 4 year group had early takedown of their
atriopulmonary connection and their operations were
considered failures. Age under 4 years did not exert an
independent influence on survival in the multivariate
analysis. A partial explanation for this is apparent from
an examination of Table VIII. Fourteen of the 44
patients under 4 years of age had a PARI of 2 Wood
units or more, and the survival rate in this group was
43% (6/14). Of those under 4 years of age with a low
PARI (1 Wood unit or less), all five survived.
The relationship between the number of ChoussatFontan criteria exceeded (maximum of four in this
study) and the outcome is shown in Table IX. Clearly,
one criterion can be exceeded without adversely affecting results.
The relative importance of the four factors (age,
pulmonary artery distortion, pulmonary hemodynamics,
and venous anomalies) is addressed by the multiple
logistic regression analysis (Table X). In this analysis,
both pulmonary artery distortion and elevated PARI
were associated with increased operative risk, which
confirms the impression from the univariate assessment.
The presence of a left-sided AV valve with mitral valve
morphology was associated with a reduced risk. Included in this group of patients were those with classical
tricuspid atresia (63) as well as eight patients with
pulmonary atresia and intact ventricular septum, 21
patients with single ventricle, and 14 patients with
hypoplastic right ventricle and ventricular septal defect.
This factor, which was associated with reduced risk, was
of greater significance than any of the standard diagnostic groups, explaining why the diagnosis of tricuspid
atresia did not appear as an independent risk factor (see
Table II). Neither age nor the presence of anomalies of
systemic or pulmonary venous connection was associated with increased risk. The values for these variables
•
~
~
~ 16fo12
8
0
Ieo Success
I
Failure
32
Age (yr)
~16
0
0
•
•
••
•••
0
•
0
•
•
•
•t •• ••• 0 ~···U
e.l..
• I
.0
0
~
• ': •
•
•• •
••
o•
•• •;: •• t ••••••
• •
b
..
~
00
•
• 0
~
2
PARI(IJ·rrIJ
>15112/15 (80%)
II
0
•
0
0
0
••
3
3/9 (33%)
4
I
<15169/78(87%) 1111/17(65%) 1
L - <2------..J l - - . - >2----...l
Fig. 1. Relationship between mean pulmonary artery pressure (PjiA), pulmonary arteriolar resistance indexed (PARI),
and outcome after Fontan procedure.
shown in the table were obtained only by forcing these
variables into the model. The decreased risk associated
with the later years of operation is attributed to the
acquisition of experience in the operative and postoperative management of these patients.
Discussion
The dismal natural history of tricuspid atresia' and
univentricular heart"!' combined with the decreasing
mortality rates for modified Fontan procedures and the
symptomatic improvement which most patients experience after this procedure have led to attempts to extend
this procedure to patients who do not meet one or more
of the ten criteria originally proposed by Choussat,
Fontan, and their associates. I The results of the current
review help to define the areas in which these criteria
can and cannot be safely extended in applying the
Fontan principle.
The results in patients outside the 4 to 15 year age
group suggest that modified Fontan procedures can be
offered to patients outside this age range. The very
favorable results in the over 15 year age group suggest
that this limit alone should not contraindicate a Fontan
procedure. Others have reported good results in this age
group.' The group under 4 years of age requires closer
examination. In this younger age group, choices frequently must be made between a palliative shunt
procedure (which may be the second or third palliative
procedure) and a modified Fontan procedure. In the
1026
The Journal of
Thoracic and Cardiovascular
Surgery
Mayer et al.
Table VIII. Age and PARI-Operative success after Fontan operation
PARI (Wood units indexed)
ND
0-0.99
Age (yr)
(SfT)
(S/T)
o - 3.99
6/10*
22/32
5/6
5/5
25/27
10/10
3/5
9/11
6/6
34/48
40/42
18/22
4.0-15.99
~16
Total
1.0-1.49
I
I
(S/T)
1.5-1.99
(S/T)
6/10*
13/16
3/3
~2.0
I
22/29
(SfT)
6/14
8/11
0/1
14/26
Legend: ND, No PARI data. S, Success. T, Total.
'One patient survived takedown in the operating room (counted as failed Fontan).
Table IX
Survival rate
No. of
criteria exceeded
o
I
2
3
Table X. Risk factors for poor outcome of Fontan
procedure (N = 167)
(%)
49
58
22
o
58
72
33
4
84
81
67
o
Variable
PA distortion
PARI (vs. 0.99)
o -0.99
I -1.49
1.5-1.99
~2.0
current group under 4 years, 12 of 14 (86%) patients
with low PARI and nondistorted pulmonary arteries
underwent a successful Fontan procedure. Age alone
was not an independent risk factor when pulmonary
artery distortion and PARI were taken into account.
These results are in contrast to those of Fontan,"
Cleveland," Mair,' and their colleagues, all of whom
found young age alone to be an independent risk factor.
However, for an equivalent level of PARI, results are
less favorable in the younger group although the number
of patients is relatively small. Because of these data, we
believe that patients under 4 years of age can be offered
a Fontan procedure when low PARI (less than 2
U . m') and nondistorted pulmonary arteries are
present.
The encouraging results in patients with anomalies of
pulmonary and systemic venous connection suggest that
their presence should not necessarily contraindicate a
Fontan procedure. These results are in contrast with
those of King," DjCarlo.!' and their co-workers. Several
technical features are important in this group of patients
and have been detailed in a prior publication.' An
end-to-side left superior vena cava-left pulmonary
artery anastomosis (modified left Glenn) has been very
useful. Complex inta-atrial baffles should be used
cautiously because of the risk of distortion by the right
atrial-left atrial pressure difference that will occur
postoperatively. This distortion can obstruct the pulmo-
Left-sided mitral valve (vs. any other
systemic AV valve)
Year of operation (vs. 84-85)
73-79
80-81
82-83
84-85
Age (vs. 4-15)
o -3.99
4 -15.99
~16
ASVC
APVC
p Value
Odds ratio
0.0164
3.50
0.0724
0.0221
0.0047
6.14
9.40
15.82
0.22
0.0537
0.001
0.6552
3.69
8.55
1.36
0.2326
1.87
0.4472
0.7773
0.9046
0.57
0.87
0.82
om
Legend: PA, Pulmonary artery. PARI, Pulmonary arteriolar resistance (indexed). AV, Atrioventricular. ASVC, Anomalous systemic venous connection.
APYC, Anomalous pulmonary venous connection.
nary venous pathway, particularly when a right-sided or
common AV valve will serve as the systemic AV valve.
The anatomy of the venous connections should be
clearly delineated by preoperative echocardiography and
catheterization, but with careful preoperative study and
an individualized operative approach, favorable results
can be achieved.
The dominant effects of the pulmonary vascular
hemodynamics on outcome after Fontan procedures are
confirmed by this group of patients. PAP as an isolated
measurement is less useful than calculated PARI,
because an 80% survival rate was achieved in the group
with a PAP of more than 15 mm Hg but a PARI of less
than 2 U . m-, In addition, PAP did not appear as an
Volume 92
Number 6
December 1986
independent risk factor in the multivariate analysis.
Similar results have been obtained by the Mayo Clinic
group.' The current group of patients contains only two
patients with a PARI of 4 U . m' or more, and both
died. The level of PARI of 2 U . m 2 or more leading to
increased operative risk in our patients is lower than the
4 U . m 2 level suggested by Choussat, Fontan, and their
colleagues', In the group with a PARI of 2 U . m' or
more, the anatomic diagnosis seems to be related to
outcome, as eight of 10 with tricuspid atresia survived
operation but only six of 16 non-tricuspid atresia defects
survived. This difference was possibly related to the type
of atriopulmonary connection, as five of seven with right
atrial-right "ventricular" connections survived and nine
of 19 with right atrial-pulmonary arterial connections
survived. However, in the total group of 167 patients, the
type of atriopulmonary connection (to "ventricle" or
pulmonary artery) or the presence or absence of a valve
anywhere in the right side of the circulation did not have
an independent effect on outcome. Intermittent abdominal compression to support the circulation in the early
postoperative period (when pulmonary arteriolar resistance may be temporarily elevated) may be useful in
managing patients with a high PARI, but we have not
found this technique to be consistently useful. The
calculation of PARI is not always possible, particularly
when there are multiple sources of pulmonary blood
flow, and the fact that PARI is a derived variable must
also be kept in mind.
Finally, the importance of pulmonary artery distortion was emphasized by this review. The disastrous
impact of residual pulmonary artery distortion at the
completion of the Fontan procedure is shown by the
100% mortality in the group in whom pulmonary artery
reconstruction was not possible. However, when a good
pulmonary artery reconstruction can be performed,
reasonable results can be achieved (63% survival). Some
authors have emphasized the importance of the size of
the pulmonary arteries in the outcome of Fontan
procedures,14 but others have not reproduced these
results." In our group of patients, all of the distortions in
the pulmonary artery were related to prior palliative
procedures. This finding has provided additional impetus toward minimizing or eliminating palliative procedures before a modified Fontan procedure. When
deformities more than a simple, centrally located, and
readily accessible stenosis are identified, we now prefer
to reconstruct the pulmonary arteries in preliminary
operations. The technique reported by Uretzky, Puga,
and Danielson," in which a Glenn shunt is combined
with a connection between the right atrium and right
Modified Fontan procedures
1 027
ventricle or main pulmonary artery, is an alternative to
patching a stenotic area and was used successfully once
in this series.
In conclusion, the results of this review suggest that
one or more of at least four of the original ten criteria of
Choussat and Fontan may be exceeded, except in the
area of PARI. PAP should not be considered alone as a
contraindication to a Fontan procedure as long as PARI
is low. Anomalies of venous connection may be successfully dealt with, and easily accessible localized distortions of the pulmonary may be repaired, but should be
viewed with caution. Age alone does not contraindicate
a Fontan procedure although criteria for younger
patients should be more strict than those for the older
age groups.
2
3
4
5
6
7
8
9
10
11
REFERENCES
Choussat A, Fontan F, Besse P, Vallot F, Chauve A,
Bricaud H: Selection criteria for Fontan's procedure,
Paediatric Cardiology, RH Anderson, EA Shinebourne,
eds., Edinburgh, 1978, Churchill Livingstone, pp 559566
Mayer J, Jonas R, Castaneda A: Modified Fontan procedure. Modern techniques in surgery. Cardiac/Thorac
Surg. 78:1-11, 1985
Vargas F, Mayer J, Jonas R, Castaneda A: Anomalous
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(For Discussion see page 1044.)