Surgery for Congenital Heart Disease

The Fontan Procedure

Contemporary Techniques Have Improved Long-Term Outcomes
Yves d’Udekem, MD, PhD; Ajay J. Iyengar, BmedSci; Andrew D. Cochrane, MD, FRACS;
Leeanne E. Grigg, MBBS, FRACP; James M. Ramsay, MD, FRACP;
Gavin R. Wheaton, MD, FRACP; Dan J. Penny, MD, PhD, FRACP; Christian P. Brizard, MD

Background—To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the
Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection.
Methods and Results—Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution.
There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were
preoperative elevated pulmonary artery pressures (P⫽0.002) and common atrioventricular valve (P⫽0.04). Fontan was
taken down during hospital stay in 7 patients. A mean of 12⫾6 years of follow-up was obtained in the 257 nonforeign
Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%).
Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73%
to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P⫽0.004). Nine pts required heart transplantation (8
atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased
occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary
connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P⫽0.02). Freedom from Fontan failure (death,
take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac
conduits, no death, SVT, or failure was observed.
Conclusions—The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection
minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less
arrhythmia and are likely to have failure of their Fontan circulation postponed. (Circulation. 2007;116[suppl I]:I-157–
I-164.)
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Key Words: Fontan procedure 䡲 follow-up studies 䡲 pediatrics

T he Fontan procedure is today the last staged operation for

all children born with congenital heart disease who
cannot be offered a 2-ventricle repair. Originally designed by
Fontan for treatment of tricuspid atresia, the procedure has
undergone 2 major successive technical modifications.1–3 In
its first version, the atriopulmonary connection (AP), the right
atrial chamber was isolated by the closure of the atrial septal
defect and the hypoplastic tricuspid valve. The right atrial
appendage was then anastomosed to the right pulmonary
artery.3 It was later understood that better streaming of the
blood flow in the systemic venous pathway to the lungs
improved the patients hemodynamics and might avoid com-
plications related to progressive atrial dilatation. The opera-
tion was therefore modified to the lateral tunnel technique
(LT), whereby the right atrium was baffled with an intraatrial
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 extra-
cardiac conduit (EC) between the inferior vena cava and the
right pulmonary artery (Figure 1).2
An increasing number of “Fontan patients” are now enter-
ing 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.d’U., A.J.I., A.D.C., C.P.B.) and Cardiology (D.J.P.), Royal Children’s 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 Women’s and Children’s Hospital, Adelaide; Australia and New
Zealand Children’s Heart Research Center, Australia.
Presented at the American Heart Association Scientific Sessions, Chicago, Ill, November 12–15, 2006.
Correspondence to Yves d’Udekem, Department of Cardiac Surgery, Royal Children’s 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
 I-158 Circulation September 11, 2007
Figure 1. Fontan surgical techniques: Classical atriopulmonary connection (A), Lateral tunnel (B), and extracardiac conduit (C).
mias, heart failure, and progressive rise of their pulmonary
vascular resistances.5–9 Conversion of the atriopulmonary
connection or the lateral tunnel technique to the extracardiac
technique has been successful in treating failing Fontan
patients, but the indication of this new procedure are still
under investigation.10,11 Because it is still unclear whether the
technical modifications brought to the initial Fontan opera-
tion translated into long-term clinical benefits, we decided to
review our clinical experience with these three techniques.
Patients and Methods
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Study Group
The design of the study was approved by the local hospital ethics
committee. We reviewed the medical records of all patients under-
going a Fontan procedure between July 1980 and December 2000 in
the Royal Children’s Hospital, Melbourne, Australia. Three hundred
twenty-seven patients were identified. Fifteen of them had under-
gone a Bjork procedure consisting in baffling the right atrium to a
hypoplastic right ventricle. This operation was not considered as
being a Fontan procedure, and these patients were excluded from the
study. Ten patients had atypical Fontan procedures. The superior
vena cava was transected and both ends were anastomosed to the
right pulmonary artery, but no intraatrial baffling was performed.
Because the blood from the inferior vena cava was still transiting
through large nonseptated atria, these 10 patients were considered to
not have benefited from optimal blood streaming and were excluded
from the study. Two patients had their Fontan taken down immedi-
ately intraoperatively to a bidirectional Glenn because of elevated
pulmonary artery pressures after Fontan completion. The remaining
305 patients constitute the core of the study.
Surgical Procedures
The characteristics of the patients undergoing each of the 3 tech-
niques applied are given in Table 1. A total of 307 prior palliative
procedures aiming at adjusting pulmonary blood flow were per-
formed 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 anasto-
moses, 20 coarctation repairs, and 10 aortic arch reconstructions.
Since 1990, the majority of patients (91/149 versus 1/156; P⬍0.001)
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.
From 1980 to 1995, 152 patients (50%) underwent a classical
atriopulmonary connection. In 31 patients, isolation of the right
atrium was achieved by the direct closure of the ASD and the
tricuspid valve, and in 121, patches of Gore-Tex (WL Gore &
Associates, Inc) were used. From 1988 to 1999, 105 patients (34%)
had a lateral tunnel modification. In 48 patients (16%) from 1998 to
2000, the Fontan procedure consisted in the implantation of an
extracardiac conduit. The conduit consisted of a Gore-Tex prosthetic
tube in 42 patients and an aortic homograft in 6. Fenestration
between the systemic venous blood circuit and the pulmonary venous
atrium was performed according to the surgeon preference, only in
patients undergoing lateral tunnel technique and extracardiac
conduit.
Fifty patients underwent an additional concomitant procedure.
Thirty-three had pulmonary artery patch arterioplasty. Eight patients
underwent a repair, and 2 a replacement of an atrioventricular valve
and 1 patient a repair of an aortic valve. A Damus-Kaye-Stansel
anastomosis was performed in 8 patients.
During the study period, all patients were initially prescribed
lifetime warfarin anticoagulation.
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, ortho-
topic 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.
 d’Udekem et al Improved Outcomes After Fontan Procedures I-159

TABLE 1. Patient Characteristics by Fontan Type

AP LT ECC Difference, Total
Characteristic (152 pts) (105 pts) (48 pts) P value (305 pts)
Demographics
Male:Female 80:72 64:41 25:23 169:136
Age at Fontan operation in years, median (IQ range) 5.0 (3.0–8.6) 3.6 (2.8–5.1) 5.4 (4.3–7.5) 0.001*‡ 4.4 (3.0–7.0)
Morphology, n (%)
Tricuspid atresia 31 (20.4) 21 (20) 15 (31.3) 67 (22)
Double-inlet left ventricle 44 (28.9) 21 (20) 8 (16.7) 73 (23.9)
Double-outlet right ventricle 21 (13.8) 19 (18.1) 7 (14.6) 47 (15.4)
Complete atrioventricular canal 17 (11.2) 19 (18.1) 6 (12.5) 42 (13.8)
Pulmonary atresia with intact ventricular septum 12 (7.9) 7 (6.7) 2 (4.2) 21 (6.9)
Straddling AV valve 10 (6.6) 3 (2.9) 2 (4.2) 15 (4.9)
Hypoplastic left heart syndrome 2 (1.3) 2 (1.9) 5 (10.4) ⬍0.02 †‡ 9 (3)
Other 15 (9.9) 13 (12.4) 3 (6.3) 31 (10.2)
Predominant ventricular morphology, n (%)
Left 111 (73) 59 (56.2) 31 (64.6) 0.005* 201 (65.9)
Right 32 (21.1) 33 (31.4) 9 (18.8) 0.002† 74 (24.3)
Biventricular 9 (5.9) 13 (12.4) 8 (16.7) 30 (9.8)
Other morphological characteristics, n (%)
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)
Prior staging with bidirectional Glenn, n (%) 0 (0) 46 (43.8) 46 (95.8) ⬍0.001*†‡ 92 (30.2)
Initial palliation to adjust pulmonary blood flow, n (%)
PA band 29 (19.1) 35 (33.3) 12 (25) 0.009* 76 (24.9)
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Right BT shunt 54 (35.5) 47 (44.8) 15 (31.3) 116 (38)

Left BT shunt 16 (10.5) 6 (5.7) 5 (10.4) 27 (8.9)
Waterston shunt 12 (7.9) 1 (1) 0 (0) ⱕ0.05 *† 13 (4.3)
Pott’s 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 2 (1.3) 2 (1.9) 6 (12.5) ⬍0.01 †‡ 10 (3.3)
None 34 (22.4) 14 (13.3) 8 (16.7) 56 (18.4)
Preoperative variables, n (%)
Pulmonary artery pressureⱖ15 mm Hg 51 (33.6) 12 (11.4) 3 (6.3) ⬍0.001*† 66 (21.6)
Oxygen saturationⱖ80% 54 (35.5) 70 (66.7) 25 (52.1) ⱕ0.04 *† 149 (48.9)
Moderate-severe AV valve regurgitation 2 (1.3) 3 (2.9) 5 (10.4) ⱕ0.05 †‡ 10 (3.3)
Operative variables, n (%)
Fenestration created 1 (0.7) 39 (37.1) 12 (25) ⬍0.001*† 52 (17)
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 capillary leak syndrome in 1. Death was subsequent to Fontan

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
take down in 2 patients. The cause of death in the remaining
8 patients was low cardiac output in 4, septic shock in 2,
pneumonia in 1, and pulmonary embolism in 1. Early Fontan
revision was undertaken for obstruction of the systemic
venous pathway in 2 patients (2 and 6 days postoperatively)
and for fenestration in 2 patients (2 hours and 34 days
postoperatively). Phrenic nerve palsy occurred in 2 patients,
necessitating diaphragm plication in 1.
Significant risk factors for hospital mortality are displayed
in Table 3. By logistic regression analysis, only elevated
preoperative pulmonary artery pressure and presence of a
 I-160 Circulation September 11, 2007
TABLE 2. Collected Variables
Perioperative and procedural variables
Patient demographics
Morphology
Surgical characteristics
Preoperative haemodynamics
Preoperative AV valve regurgitation
Type of Fontan procedure
Procedure-related
Postoperative
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Variables collected at follow-up
Clinical
Surgical intervention
Arrhythmia
Thrombo-embolic events
Echocardiographic findings
Catheterisation data
Other
Sex
Age at operation (grouped: ⬍4, 4–8, ⬎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
 d’Udekem et al Improved Outcomes After Fontan Procedures
TABLE 3. Risk Factors for Hospital Mortality
Univariate Analysis
Variable P Value
Preop pulmonary artery pressure* 0.001
Bilateral superior venae cava 0.023
Common atrioventricular valve 0.027
Surgical era 0.03
Fontan type 0.045
*Odds ratio for every 1 mm Hg increment of pulmonary artery pressure.
common atrioventricular valve were independent predictors
of hospital mortality. Surgical era, previous staging with
bidirectional Glenn, and fenestration of the Fontan could not
be tested because there was no mortality after 1990, at the
time staging and fenestration were implemented.
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 survi-
vors 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 12⫾6 years.
Twenty-eight late deaths occurred a median of 7 years (1 to
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11 years) after the Fontan procedure, 25 with an atriopulmo-
nary 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), cerebro-
vascular 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. Risk Factors for Late Mortality
Univariate Analysis
Variable P Value
Fontan type 0.004
Prior staging with bidirect Glenn 0.026
Pulmonary artery reconstruction 0.036
Bidirect indicates bidirectional.
I-161
Multivariate Analysis
P Value Odds Ratio 95% CI
0.002 1.35 1.11–1.63
0.04 7.5 1.13–49.9
tunnel (P⫽0.004). During the 10 years of follow-up available
for the patients undergoing an extra-cardiac conduit, no death
occurred. Identified predictors of late mortality are listed in
Table 4. Prior staging with bidirectional Glenn improved
survival (P⫽0.026). Kaplan-Meier curves of late survival
according to the Fontan technique used are displayed in
Figure 2.
Reinterventions
Forty-two reoperations were performed in 34 hospital survi-
vors (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 trans-
plantation 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.
Late Functional Status
Protein-losing enteropathy was diagnosed in 4 patients be-
tween 5.5 and 12.3 years after the Fontan procedure, resulting
in death in 2 patients.
Two hundred twenty-three patients were alive at last
follow-up. Seven were in NYHA class I after heart transplan-
tation, and 1 was in class II 12 years after Fontan take-down
to a bidirectional Glenn. Among the remaining 215 patients,
177 (82%) were in NYHA class I, 32 (15%) in class II, and
6 (3%) in class III.
Multivariate Analysis
P Value Hazard Ratio 95% CI
No independent predictors
 I-162 Circulation September 11, 2007
Figure 2. Kaplan-Meier survival curves of hospital survivors by
Fontan techniques.
Four female patients had a total of 7 successful pregnan-
cies. 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
11.9⫾4.9 years. Some degree of atrioventricular valve regur-
gitation 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.
Thromboembolic and Bleeding Events
At the time of follow-up of the 215 patients with a Fontan
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circulation, 176 (82%) were on warfarin, 18 on aspirin only
(8%), and 21 were not taking any anticoagulation (10%).
Thirteen patients had a documented history of clinical throm-
boembolic events. All patients were taking warfarin at the
time of the event. Eleven of these patients had a classical
atriopulmonary connection, 1 a lateral tunnel, and 1 an
extracardiac conduit. Nine patients had clinical evidence of
pulmonary embolism, 7 of them being in supraventricular
tachycardia at the time (8 atriopulmonary connections and
one extra-cardiac conduit). One stroke and 1 transient ische-
mic attack occurred during cardiac catheterization at 6
months and 15 years postoperatively. One patient had a
transient ischemic attack and the last patient had a renal
infarct. Freedom from thromboembolic events was 96.9%
(95% CI: 93.7 to 98.5%) at 10 years and 94.3% (95% CI: 89.2
to 97.1%) at 15 years. Risks factors predictive of thrombo-
embolic events have been displayed in Table 5.
Two bleeding events were reported. One patient had a
thigh compartment syndrome and one a subdural hemorrhage.
TABLE 5. Risk Factors for Thromboembolic Events
Univariate Analysis
Variable
Age group (0 to 3, 4 to 8, ⬎8 years)
Fontan conversion
Current AV valve regurgitation*
AV indicates atrioventricular.
*AV Valve regurgitation present at the time of follow-up.
Late Occurrence of Arrhythmia
Supraventricular tachyarrhythmia was reported in 62 hospital
survivors (52 with atriopulmonary connections and 10 with a
lateral tunnel) after a mean of 9.1⫾5.2 years with a Fontan
circulation. Freedom from supraventricular tachycardia was
84.5% (95% CI: 78.9 to 88.8%) at 10 years and 70.8% (95%
CI: 62.9 to 77.4%) at 15 years. Risk factors predictive of
these late arrhythmias are displayed in Table 6. Undergoing a
Fontan modification independently predicted decreased oc-
currence of arrhythmia. The 15-year freedom of supraven-
tricular tachyarrhythmia was 61.4% (95% CI: 51.4 to 69.9%)
for atriopulmonary connections compared with 87.3% (95%
CI: 76.2 to 93.4%) for the lateral tunnel technique (P⫽0.02,
Figure 3). At the time of follow-up, no SVT were observed
after extracardiac conduit. Twenty patients underwent at least
1 cardioversion during follow-up. Four patients had 3 or more
cardioversions. At the last follow-up, 31 patients were treated
with 1 medication, 3 with 2, and 2 with 3. Percutaneous
radiofrequency ablation therapy was attempted in 8 patients
with results lasting more than 3 months in 3 patients.
At last follow-up, a permanent pacemaker had been im-
planted in 28 patients (23 atriopulmonary connections, 4
lateral tunnels, and 1 extra-cardiac Fontan). Four were im-
planted at the time or before Fontan surgery for congenital
heart block. The indications for implantation in the remaining
patients were atrioventricular block (7), sinus node dysfunc-
tion (13), tachycardia-bradycardia syndrome (4).
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 take-
downs (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 undergo-
ing 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 increas-
ing 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
Multivariate Analysis
P Value P Value Hazard Ratio 95% CI
0.02 0.19
⬍0.001 0.015 14.17 2.2, 90.7
0.002 0.44
 d’Udekem et al
TABLE 6. Risk Factors for Supraventricular Tachyarrhythmia
Univariate Analysis
Variable
Age group (0 to 3, 4 to 8, ⬎8 years)
Fontan type
Atrial isomerism
Common AV valve
Interrupted inferior vena cava
Prior staging with bidirect Glenn
Pre-op pulmonary artery pressure*
History of thrombo-embolic event
Fontan conversion
Currrent AV valve regurgitation
AV indicates atrioventricular; bidirect, bidirectional.
*Odds ratio for every 1 mm Hg increment of pulmonary artery pressure.
original description, the Fontan operation has undergone
several modifications aimed at improving streaming of the
systemic venous blood to the lungs, namely the lateral tunnel
and the extracardiac conduit. So far, the long-term benefits of
these modifications have not been clearly ascertained. The
present study now shows that the experience gained over time
with the Fontan procedure benefited patients not only in
terms of short-term survival, but also long-term outcome.
Patient selection has clearly varied with time. In the 1980s,
Fontan procedures were offered to patients with elevated
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pulmonary pressures. It is likely that the low hospital mor-
tality observed after 1990 was related to improved patient
selection, better adjustment of pulmonary blood flow, and to
staging with bidirectional Glenn. Staging might have been
beneficial because of the well-documented relief of ventric-
ular volume loading, but also might have added a further
opportunity for careful patient selection.4 Because hospital
mortality was eliminated in the second part of the study, at the
time when staging with bidirectional Glenn and surgical
fenestration were implemented, the impact of these measures
could not be evaluated due to a lack of end points.
Two patients had their Fontan taken down at the operation,
7 during the hospital stay, and 2 in the first years following its
completion. It is possible that this aggressive approach to
patients showing signs of failure spared us some mortality.
The morphological features determining patients’ poorer
outcomes are similar to those identified previously. Patients
with a common atrioventricular valve were more likely to die
after the procedure. In Gentles et al’s study, this feature was
Figure 3. Freedom from SVT by Fontan techniques.
Improved Outcomes After Fontan Procedures I-163
Multivariate Analysis
P Value P Value Hazard Ratio 95% CI
0.004
⬍0.001 0.047 0.28 0.08–0.98
0.005
0.02
0.036
0.026
0.036
⬍0.001 0.011 4.0 1.4–11.5
⬍0.001
0.009
the most potent predictor of late Fontan failure, most likely
because of the tendency of these valves to become regurgi-
tant.6 Patients who underwent pulmonary artery reconstruc-
tion, who presumably possessed a less favorable pulmonary
vascular bed, had a greater chance of long-term failure of
their Fontan. Some of the predictive factors identified were
contemporaneous events rather than true predictors. Throm-
bus formation and supraventricular tachycardia are both
correlates of atrial dilatation and it was not surprising that
thromboembolic events predicted occurrence of supraventric-
ular arrhythmia. Fontan conversion in this initial experience
was offered to failing patients and accordingly correlated
with occurrence of thromboembolic events.
There were relatively few thromboembolic events in this
series of patients. Apart from very few exceptions, it has been
our policy to systematically anticoagulate patients with war-
farin for life, and it is therefore difficult for us to evaluate its
benefits. This policy did not give absolute protection to our
patients, as most of those who experienced thromboembolic
events were anticoagulated at the time of the event.
The most striking point arising from the follow-up of these
patients is the improvement in their long-term outcome,
despite the fact that the Fontan procedure seems to remain a
palliative operation. The previously quoted 20-year survival
of Fontan patients with atriopulmonary connections was
65%.7 The 85% survival at 20 years achieved in our operative
cohort heralds a marked improvement, especially in view of
the inevitable improvement of results in the forthcoming
years. The 15 year-survival of the patients having a lateral
tunnel was 94%. Although there was a gradual increase in the
rate of failure of the atriopulmonary connection Fontan after
10 years, this trend was not yet observed in the time frame of
this study in the lateral tunnel Fontan.
The occurrence of supraventricular tachycardia as a con-
sequence of right atrial dilatation was the predominant reason
that motivated the modification of the original Fontan tech-
nique into the lateral tunnel technique. Eighteen years after its
description by de Leval, this technical modification has
clearly brought benefits to patients in terms of protection
from supraventricular tachyarrhytmia. If one believes that
 I-164 Circulation September 11, 2007
TABLE 7. Risk Factors for Fontan Failure
Univariate Analysis
Variable
Fontan conversion
Current AV valve regurgitation*
Pulmonary artery reconstruction†
Prior staging with bidirect Glenn
Thrombo-embolic events
Age group (0 to 3, 4 to 8, ⬎8 years)
AV indicates atrioventricular; bidirect, bidirectional.
*AV Valve regurgitation present at the time of follow-up. †Before or at the time of Fontan completion
supraventricular arrhythmia is a critical factor in the chain of
events leading to failure of a Fontan circulation, then it is
likely that failure will be observed much later in patients who
have undergone a lateral tunnel technique or an extracardiac
technique.
Consequently, this study supports the rationale of convert-
ing atriopulmonary connection to an extracardiac conduit as
been advocated initially by the team of Mavroudis and
Deal.10,11In this initial experience with Fontan conversion
only the sickest patients were offered this treatment. In
Melbourne, no patient died after this procedure, and we now
intend to convert patients in earlier stages of Fontan failure
before they experience refractory arrhythmias.
There are several obvious limitations to this study. This is
a historical series over a long time period during which
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practice and care has evolved. The improvement in patient
care may have been multifactorial, and some factors not
strictly related to the surgical procedure may not have been
analyzed. It is likely that the patient population operated at
the beginning of this experience differs from today’s practice.
In particular, we are now operating more patients with
hypoplastic left heart syndrome, and some of the conclusions
drawn may not apply to all categories of patients. By
definition, this study applies to a very heterogenous group of
patients. It is therefore possible that some parameters that
may impact outcomes were not found in sufficient numbers.
We conclude that the outcomes of patients undergoing
Fontan procedures have improved. Better patient selection
and management, as improved surgical techniques, have
contributed to minimize hospital mortality. Patients with the
lateral tunnel and extracardiac conduit variations of the
Fontan experience less arrhythmia and are likely to have the
failure of their Fontan circulation postponed.
Multivariate Analysis
P Value P Value Hazard Ratio 95% CI
⬍0.001
0.013
0.017 0.003 3.7 1.58–8.66
0.018
0.04
0.044
Disclosures
None.
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