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patch and the superior vena cava was sutured directly to the
right pulmonary artery.1 Performing the anastomosis between
the superior vena cava and the right pulmonary artery
(bidirectional Glenn) at an earlier age as an intermediate step
decreased total mortality and morbidity to achieve a final
Fontan circulation.4 The most recent modification of the
technique consisted in the replacement of the intra-atrial
routing of the venous blood by the insertion of an extracardiac conduit (EC) between the inferior vena cava and the
right pulmonary artery (Figure 1).2
An increasing number of Fontan patients are now entering adulthood, and these patients are facing an uncertain
future. It is clear from the 20 years follow-up of the
operations performed in the initial era that this operation
remains palliative. Patients are prone to developing arrhyth-
From the Departments of Cardiac Surgery (Y.dU., A.J.I., A.D.C., C.P.B.) and Cardiology (D.J.P.), Royal Childrens Hospital and the Department of
Pediatrics, the University of Melbourne; Department of Cardiology (L.G.), Royal Melbourne Hospital; Department of Cardiology (J.M.R.), Princess
Margaret Hospital for Children, Perth; Department of Cardiology (G.R.W.), Adelaide Womens and Childrens Hospital, Adelaide; Australia and New
Zealand Childrens Heart Research Center, Australia.
Presented at the American Heart Association Scientific Sessions, Chicago, Ill, November 1215, 2006.
Correspondence to Yves dUdekem, Department of Cardiac Surgery, Royal Childrens Hopital, Flemington Road, Parkville, Melbourne 3052, Victoria
Australia. E-mail yves.dudekem@rch.org.au
2007 American Heart Association, Inc.
Circulation is available at http://circ.ahajournals.org
DOI: 10.1161/CIRCULATIONAHA.106.676445
I-157
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Circulation
Figure 1. Fontan surgical techniques: Classical atriopulmonary connection (A), Lateral tunnel (B), and extracardiac conduit (C).
Surgical Procedures
The characteristics of the patients undergoing each of the 3 techniques applied are given in Table 1. A total of 307 prior palliative
procedures aiming at adjusting pulmonary blood flow were performed in 249 patients. Eighty additional procedures were performed
in 60 patients before Fontan completion: 4 arterial switches, 27
pulmonary artery reconstructions, 19 Damus-Kaye-Stansel anastomoses, 20 coarctation repairs, and 10 aortic arch reconstructions.
Since 1990, the majority of patients (91/149 versus 1/156; P0.001)
underwent a bidirectional Glenn as a staged procedure before Fontan
completion. The bidirectional Glenn was performed at a median age
of 1.3 years (0.8 to 2.4 years), and the median interval time between
this procedure and Fontan completion was 2.9 years (1.9 to 4 years).
All patients underwent a cardiac catheterization before Fontan
completion.
Hospital Mortality
Hospital mortality was defined as mortality within the hospital or in
the first 30 postoperative days. All the preoperative and procedural
variables were tested for their impact on hospital mortality by
univariate analysis and the significant ones were entered in a
multivariate analysis (Table 2).
Follow-Up Study
Follow-up information was gathered for all Australian hospital
survivors, whereas foreign patients were excluded from the
follow-up study. The postoperative variables given in Table 2 were
obtained from the hospital database or their referring cardiologists.
Kaplan-Meier curves were calculated for the following adverse
events: death, tachyarrhythmias, thromboembolic events, and Fontan
failure defined as death, takedown of the Fontan procedure, orthotopic heart transplantation, or NYHA functional class III or IV. All
the perioperative variables were tested by univariate and multivariate
analysis for their ability to predict these adverse events using Cox
proportional hazard methods.
Statistical Analysis
Data were reported as median and interquartile ranges and in means
and standard deviations. All tests were 2-tailed, and a probability
value 0.05 was considered significant.
The authors had full access to the data and take responsibility for
its integrity. All authors have read and agree to the manuscript as
written.
dUdekem et al
TABLE 1.
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Characteristic
LT
(105 pts)
ECC
(48 pts)
Difference,
P value
Total
(305 pts)
0.001*
4.4 (3.07.0)
Demographics
Male:Female
80:72
64:41
25:23
5.0 (3.08.6)
3.6 (2.85.1)
5.4 (4.37.5)
Tricuspid atresia
31 (20.4)
21 (20)
15 (31.3)
44 (28.9)
21 (20)
8 (16.7)
169:136
Morphology, n (%)
67 (22)
73 (23.9)
21 (13.8)
19 (18.1)
7 (14.6)
47 (15.4)
17 (11.2)
19 (18.1)
6 (12.5)
42 (13.8)
12 (7.9)
7 (6.7)
2 (4.2)
21 (6.9)
Straddling AV valve
10 (6.6)
3 (2.9)
2 (4.2)
2 (1.3)
2 (1.9)
5 (10.4)
15 (9.9)
13 (12.4)
3 (6.3)
15 (4.9)
0.02
9 (3)
31 (10.2)
111 (73)
59 (56.2)
31 (64.6)
0.005*
201 (65.9)
32 (21.1)
33 (31.4)
9 (18.8)
0.002
74 (24.3)
9 (5.9)
13 (12.4)
8 (16.7)
30 (9.8)
Atrial isomerism
20 (13.2)
15 (14.3)
7 (14.6)
42 (13.8)
Bilateral SVC
39 (25.7)
17 (16.2)
8 (16.7)
64 (21)
Common AV valve
18 (11.8)
19 (18.1)
6 (12.5)
43 (14.1)
46 (43.8)
46 (95.8)
Right
Biventricular
Other morphological characteristics, n (%)
0 (0)
0.001*
92 (30.2)
29 (19.1)
35 (33.3)
12 (25)
Right BT shunt
54 (35.5)
47 (44.8)
15 (31.3)
Left BT shunt
16 (10.5)
6 (5.7)
5 (10.4)
Waterston shunt
12 (7.9)
1 (1)
0 (0)
Potts shunt
1 (0.7)
0 (0)
1 (2.1)
2 (0.7)
Central shunt
4 (2.6)
0 (0)
1 (2.1)
5 (1.6)
Norwood stage I
0.009*
76 (24.9)
116 (38)
27 (8.9)
0.05 *
0.01
13 (4.3)
2 (1.3)
2 (1.9)
6 (12.5)
34 (22.4)
14 (13.3)
8 (16.7)
51 (33.6)
12 (11.4)
3 (6.3)
0.001*
66 (21.6)
Oxygen saturation80%
54 (35.5)
70 (66.7)
25 (52.1)
0.04 *
149 (48.9)
2 (1.3)
3 (2.9)
5 (10.4)
0.05
10 (3.3)
1 (0.7)
39 (37.1)
0.001*
52 (17)
None
10 (3.3)
56 (18.4)
12 (25)
AP indicates atriopulmonary; LT, lateral tunnel; ECC, extra-cardiac conduit; AV, atrio-ventricular.
* indicates a difference between AP and LT; , AP and ECC; , LT and ECC.
Results
Hospital Survival
There were 10 hospital deaths within 98 days of the operation
for a hospital mortality of 3%. Nine occurred after an
atriopulmonary connection and 1 after a lateral tunnel.
Hospital mortality was 6.3% between July 1980 and June
1990. After that date there was no more hospital death. Seven
patients had their Fontan taken down to a bidirectional Glenn
between 3 hours and 14 days of the operation. The indication
for Fontan takedown was low cardiac output in 5 patients,
complete thrombosis of left pulmonary artery in 1, and
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TABLE 2.
Collected Variables
Morphology
Surgical characteristics
Preoperative haemodynamics
Preoperative AV valve regurgitation
Type of Fontan procedure
Procedure-related
Postoperative
Surgical intervention
Arrhythmia
Thrombo-embolic events
Echocardiographic findings
Catheterisation data
Other
Sex
Age at operation (grouped: 4, 48, 8)
Year of operation
Diagnosis
Presence of bilateral SVC
Presence of interrupted IVC
Presence of complete AVSD-type AV valve
Morphology of dominant ventricle (Left, right, biventricular)
Initial palliative surgical procedure
Initial pulmonary artery band
Systemic-to-pulmonary shunt vs central shunts
Previous BCPS staging
Interval between BCPS and Fontan completion
Other procedures performed before or after Fontan procedure
Mean pulmonary artery procedure
Oxygen saturation
None, trivial/mild, moderate, severe
Atrio-pulmonary
Lateral tunnel
Extra-cardiac conduit
Total cavopulmonary connection without intra-atrial baffle
Presence of intra-atrial baffle
Baffle fenestration
Concomitant procedures
Hospital mortality
Early Fontan take-down
Length of hospital stay
Major re-operation
Survival status
New York Heart Association (NYHA) functional class (I-IV)
Pregnancy
Medications
Anti-coagulation (None, aspirin, warfarin)
Takedown
Orthotopic heart transplantation
Late revision or conversion
Arrhythmia surgery
Other reoperation
New-onset supraventricular tachyarrhythmia
Interval between Fontan and onset of SVT
Pacemaker requirement
Interval between Fontan and pacemaker implantation
Anti-arrhythmic therapy
Reversible ischaemic neurological defecit (RIND)
Stroke
Pulmonary embolism
Deep vein thrombosis
Severe bleeding
Interval between Fontan procedure and echocardiogram
AV valve regurgitation (None, trivial/mild, moderate, severe)
Presence of inter-atrial shunting/fenestration
Interval between Fontan procedure and catheterisation
Intervention
Mean PA, systemic venous and systemic atrial pressure
Systemic saturation
Protein-losing enteropathy
Phrenic nerve palsy
dUdekem et al
TABLE 3.
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Variable
Multivariate Analysis
P Value
P Value
Odds Ratio
95% CI
0.001
0.002
1.35
1.111.63
0.023
0.04
7.5
1.1349.9
0.027
Surgical era
0.03
Fontan type
0.045
Long-Term Survival
Thirty-three patients (14 with an atriopulmonary connection,
15 with a lateral tunnel, and 4 with an extracardiac conduit)
who had been referred from foreign countries were excluded
from the study of the long-term impact of the Fontan
procedure. The total cohort of patients valid for the long-term
follow-up studies consisted of 257 Australian hospital survivors with a Fontan circulation. Six of the 257 were lost to
follow-up. The completeness of concurrent follow-up (2003
2006) was 96% for a mean follow-up of 126 years.
Twenty-eight late deaths occurred a median of 7 years (1 to
11 years) after the Fontan procedure, 25 with an atriopulmonary connection, and 3 with a lateral tunnel. Two patients
died of intractable protein-losing enteropathy 6 and 9 years
after Fontan completion. Six patients died shortly after
cardiac reoperation. One patient died after 1 year when his
Fontan was taken down to a bidirectional Glenn. Two died
shortly after the conversion of an atriopulmonary connection
to an extracardiac conduit, 1 patient after the revision of the
systemic venous pathway, 1 of early graft rejection 1 day
after orthotopic heart transplantation, and 1 after a mitral
valve replacement. The cause of death of the remaining 18
patients were sudden death (5), end-stage heart failure (6),
pulmonary embolism (3), subdural hemorrhage (1), cerebrovascular accident (1), humoral graft rejection after orthotopic
heart transplantation (1), asthma (1), pneumonia (1), and
motor-vehicle accident (1). Ten- and 20-year Kaplan-Meier
survival of hospital survivors were, respectively, 91% (95%
CI: 86.7%93.9%) and 84% (95%CI: 78.5% 89.3%). The
15-year survival after classical Fontan was 81% (95% CI:
73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral
TABLE 4.
Reinterventions
Forty-two reoperations were performed in 34 hospital survivors (24 after an atriopulmonary connection, 8 after lateral
tunnel, and 2 after extra-cardiac conduit) after a median time
of 5.7 years (2 to 14 years). Two patients had their Fontan
taken down to a bidirectional Glenn. Six patients had a
revision of their Fontan circuit (3 atriopulmonary connections
and 3 extracardiac conduits) resulting in 1 death, and 8
underwent a conversion of an atriopulmonary connection to
an extra-cardiac conduit (5 with antiarrhythmic surgery)
resulting in 3 deaths and 2 patients undergoing heart transplantation within 2 years. The remaining procedures were 6
Damus-Kaye-Stansel anastomoses (1 with concomitant aortic
and mitral valve repair), 4 resections of left ventricular
outflow tract obstruction, 4 aortic valve replacements, 1
aortic valve repair, 1 roofing of the coronary sinus, 1
pulmonary artery thrombectomy, and 9 heart transplantations.
Variable
P Value
Fontan type
Multivariate Analysis
P Value
Hazard Ratio
0.004
0.026
0.036
No independent predictors
95% CI
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Four female patients had a total of 7 successful pregnancies. At the time of the follow-up, no patient was listed for
heart transplantation.
Echocardiographic reports were available in 202 hospital
survivors with an intact Fontan circulation after a mean of
11.94.9 years. Some degree of atrioventricular valve regurgitation was noted in 137 (67.8%) of them, being quoted as
trivial to mild in 119, moderate in 16, and severe in 2.
Shunting between the systemic venous pathway and the
pulmonary venous chamber could be seen in 13 patients who
had a fenestration at the time of the Fontan procedure, and in
an additional 3 patients who had no fenestration. In 27
patients who had a fenestrated Fontan, no more shunting
could be seen.
Fontan Failure
Late Fontan failure occurred in 42 patients after a median of
8.5 years (2.3 to 15.6 years). There were 25 deaths (22
atriopulmonary connections, 1 lateral tunnel), 2 Fontan takedowns (1 atriopulmonary connection, 1 lateral tunnel), 9 heart
transplantations (8 atriopulmonary connections, 1 lateral
tunnel), and 6 patients were in NYHA class III or IV (all
atriopulmonary connections). Thirty-seven patients undergoing failure had an atriopulmonary connection and 5 had a
lateral tunnel technique. Freedom from late Fontan failure
was 84.6% (95% CI: 78.4 to 89.1%) at 15 years and 69.6%
(95% CI: 58 to 78.6%) at 20 years. Predictive risks factors for
failure are displayed in Table 7.
Discussion
Despite its widespread application, there have been increasing concerns that the Fontan procedure is merely a palliative
operation. Many in the medical community believe that most
of these patients are doomed to death or heart transplantation
in the decades that will follow this procedure. Since its
Variable
Age group (0 to 3, 4 to 8, 8 years)
Fontan conversion
Current AV valve regurgitation*
Multivariate Analysis
P Value
P Value
0.02
0.19
0.001
0.015
0.002
Hazard Ratio
95% CI
14.17
2.2, 90.7
0.44
AV indicates atrioventricular.
*AV Valve regurgitation present at the time of follow-up.
dUdekem et al
TABLE 6.
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Variable
P Value
0.005
Common AV valve
0.02
0.036
0.026
Hazard Ratio
95% CI
0.047
0.28
0.080.98
0.011
4.0
1.411.5
0.036
0.001
Fontan conversion
0.001
P Value
0.004
0.001
Fontan type
Multivariate Analysis
0.009
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Circulation
TABLE 7.
Variable
P Value
Fontan conversion
0.001
Multivariate Analysis
P Value
Hazard Ratio
95% CI
0.003
3.7
1.588.66
0.013
0.017
0.018
Thrombo-embolic events
0.04
0.044
Disclosures
None.
References
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