The Fontan Procedure - Contemporary Techniques Have Improved Long-Term Outcomes

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The Fontan ProcedureContemporary Techniques Have Improved Long-Term Outcomes

Yves dUdekem, 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

BackgroundTo 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 ResultsBetween 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 (P0.002) and common atrioventricular valve (P0.04). Fontan was

taken down during hospital stay in 7 patients. A mean of 126 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 (P0.004). 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 (P0.02). 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.

ConclusionsThe 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.)

Key Words: Fontan procedure follow-up studies pediatrics

The Fontan procedure is today the last staged operation forall children born with congenital heart disease whocannot be offered a 2-ventricle repair. Originally designed by

Fontan for treatment of tricuspid atresia, the procedure has

undergone 2 major successive technical modifications.13 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 uncertainfuture. 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.), PrincessMargaret Hospital for Children, Perth; Department of Cardiology (G.R.W.), Adelaide Womens and Childrens Hospital, Adelaide; Australia and NewZealand 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 [email protected]

2007 American Heart Association, Inc.

Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.106.676445

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Surgery for Congenital Heart Disease

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mias, heart failure, and progressive rise of their pulmonary

vascular resistances.59 Conversion of the atriopulmonaryconnection 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

Study GroupThe design of the study was approved by the local hospital ethicscommittee. We reviewed the medical records of all patients under-

going a Fontan procedure between July 1980 and December 2000 inthe Royal Childrens Hospital, Melbourne, Australia. Three hundredtwenty-seven patients were identified. Fifteen of them had under-gone a Bjork procedure consisting in baffling the right atrium to ahypoplastic right ventricle. This operation was not considered asbeing a Fontan procedure, and these patients were excluded from thestudy. Ten patients had atypical Fontan procedures. The superiorvena cava was transected and both ends were anastomosed to theright pulmonary artery, but no intraatrial baffling was performed.Because the blood from the inferior vena cava was still transitingthrough large nonseptated atria, these 10 patients were considered tonot have benefited from optimal blood streaming and were excludedfrom the study. Two patients had their Fontan taken down immedi-ately intraoperatively to a bidirectional Glenn because of elevatedpulmonary artery pressures after Fontan completion. The remaining

305 patients constitute the core of the study.

Surgical ProceduresThe characteristics of the patients undergoing each of the 3 tech-niques applied are given in Table 1. A total of 307 prior palliativeprocedures aiming at adjusting pulmonary blood flow were per-formed in 249 patients. Eighty additional procedures were performedin 60 patients before Fontan completion: 4 arterial switches, 27pulmonary 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; P0.001)underwent a bidirectional Glenn as a staged procedure before Fontancompletion. The bidirectional Glenn was performed at a median ageof 1.3 years (0.8 to 2.4 years), and the median interval time betweenthis procedure and Fontan completion was 2.9 years (1.9 to 4 years).

All patients underwent a cardiac catheterization before Fontancompletion.

From 1980 to 1995, 152 patients (50%) underwent a classical

atriopulmonary connection. In 31 patients, isolation of the rightatrium was achieved by the direct closure of the ASD and thetricuspid 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 to2000, the Fontan procedure consisted in the implantation of an

extracardiac conduit. The conduit consisted of a Gore-Tex prosthetictube in 42 patients and an aortic homograft in 6. Fenestrationbetween the systemic venous blood circuit and the pulmonary venousatrium was performed according to the surgeon preference, only in

patients undergoing lateral tunnel technique and extracardiacconduit.

Fifty patients underwent an additional concomitant procedure.Thirty-three had pulmonary artery patch arterioplasty. Eight patientsunderwent a repair, and 2 a replacement of an atrioventricular valve

and 1 patient a repair of an aortic valve. A Damus-Kaye-Stanselanastomosis was performed in 8 patients.

During the study period, all patients were initially prescribedlifetime warfarin anticoagulation.

Hospital MortalityHospital mortality was defined as mortality within the hospital or inthe first 30 postoperative days. All the preoperative and procedural

variables were tested for their impact on hospital mortality byunivariate analysis and the significant ones were entered in amultivariate analysis (Table 2).

Follow-Up StudyFollow-up information was gathered for all Australian hospitalsurvivors, whereas foreign patients were excluded from the

follow-up study. The postoperative variables given in Table 2 wereobtained from the hospital database or their referring cardiologists.

Kaplan-Meier curves were calculated for the following adverseevents: death, tachyarrhythmias, thromboembolic events, and Fontanfailure 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 multivariateanalysis for their ability to predict these adverse events using Coxproportional hazard methods.

Statistical AnalysisData were reported as median and interquartile ranges and in meansand standard deviations. All tests were 2-tailed, and a probabilityvalue 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 aswritten.

Figure 1. Fontan surgical techniques: Classical atriopulmonary connection (A), Lateral tunnel (B), and extracardiac conduit (C).

I-158 Circulation September 11, 2007

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Results

Hospital SurvivalThere 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

capillary leak syndrome in 1. Death was subsequent to Fontan

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 elevatedpreoperative pulmonary artery pressure and presence of a

TABLE 1. Patient Characteristics by Fontan Type

Characteristic

AP

(152 pts)

LT

(105 pts)

ECC

(48 pts)

Difference,

Pvalue

Total

(305 pts)

Demographics

Male:Female 80:72 64:41 25:23 169:136

Age at Fontan operation in years, median (IQ range) 5.0 (3.08.6) 3.6 (2.85.1) 5.4 (4.37.5) 0.001* 4.4 (3.07.0)

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)

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)

0.05 * 13 (4.3)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 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 pressure15 mm Hg 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)

Moderate-severe AV valve regurgitation 2 (1.3) 3 (2.9) 5 (10.4) 0.05 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.

dUdekem et al Improved Outcomes After Fontan Procedures I-159



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TABLE 2. Collected Variables

Perioperative and procedural variables

Patient demographics Sex

Age at operation (grouped: 4, 48, 8)

Year of operation

Morphology Diagnosis

Presence of bilateral SVC

Presence of interrupted IVC

Presence of complete AVSD-type AV valve

Morphology of dominant ventricle (Left, right, biventricular)

Surgical characteristics 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

Preoperative haemodynamics Mean pulmonary artery procedure

Oxygen saturation

Preoperative AV valve regurgitation None, trivial/mild, moderate, severe

Type of Fontan procedure Atrio-pulmonary

Lateral tunnelExtra-cardiac conduit

Total cavopulmonary connection without intra-atrial baffle

Procedure-related Presence of intra-atrial baffle

Baffle fenestration

Concomitant procedures

Postoperative Hospital mortality

Early Fontan take-down

Length of hospital stay

Major re-operation

Variables collected at follow-up

Clinical Survival status

New York Heart Association (NYHA) functional class (I-IV)

Pregnancy

Medications

Anti-coagulation (None, aspirin, warfarin)

Surgical intervention Takedown

Orthotopic heart transplantation

Late revision or conversion

Arrhythmia surgery

Other reoperation

Arrhythmia New-onset supraventricular tachyarrhythmia

Interval between Fontan and onset of SVT

Pacemaker requirement

Interval between Fontan and pacemaker implantation

Anti-arrhythmic therapy

Thrombo-embolic events Reversible ischaemic neurological defecit (RIND)

Stroke

Pulmonary embolism

Deep vein thrombosis

Severe bleeding

Echocardiographic findings Interval between Fontan procedure and echocardiogram

AV valve regurgitation (None, trivial/mild, moderate, severe)

Presence of inter-atrial shunting/fenestration

Catheterisation data Interval between Fontan procedure and catheterisation

Intervention

Mean PA, systemic venous and systemic atrial pressure

Systemic saturation

Other Protein-losing enteropathy

Phrenic nerve palsy




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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 126 years.

Twenty-eight late deaths occurred a median of 7 years (1 to

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 aftercardiac 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

tunnel (P0.004). 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 (P0.026). Kaplan-Meier curves of late survival

according to the Fontan technique used are displayed in

Figure 2.

ReinterventionsForty-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 6Damus-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 StatusProtein-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.

TABLE 3. Risk Factors for Hospital Mortality

Variable

Univariate Analysis Multivariate Analysis

PValue PValue Odds Ratio 95% CI

Preop pulmonary artery pressure* 0.001 0.002 1.35 1.111.63

Bilateral superior venae cava 0.023

Common atrioventricular valve 0.027 0.04 7.5 1.1349.9Surgical era 0.03

Fontan type 0.045

*Odds ratio for every 1 mm Hg increment of pulmonary artery pressure.

TABLE 4. Risk Factors for Late Mortality

Variable


PValue PValue Hazard Ratio 95% CI

Fontan type 0.004

Prior staging with bidirect Glenn 0.026 No independent predictors

Pulmonary artery reconstruction 0.036

Bidirect indicates bidirectional.




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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

11.94.9 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 EventsAt the time of follow-up of the 215 patients with a Fontan

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 thetime 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.

Late Occurrence of ArrhythmiaSupraventricular tachyarrhythmia was reported in 62 hospital

survivors (52 with atriopulmonary connections and 10 with a

lateral tunnel) after a mean of 9.15.2 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 (P0.02,

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 FailureLate 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 hearttransplantations (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.

DiscussionDespite its widespread application, there have been increas-

ing concerns that the Fontan procedure is merely a palliativeoperation. 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

TABLE 5. Risk Factors for Thromboembolic Events

Variable



Age group (0 to 3, 4 to 8, 8 years) 0.02 0.19

Fontan conversion 0.001 0.015 14.17 2.2, 90.7

Current AV valve regurgitation* 0.002 0.44

AV indicates atrioventricular.*AV Valve regurgitation present at the time of follow-up.

Figure 2. Kaplan-Meier survival curves of hospital survivors byFontan techniques.




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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

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 beenbeneficial 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. Patientswith a common atrioventricular valve were more likely to die

after the procedure. In Gentles et als study, this feature was

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 thisseries 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 protectionfrom supraventricular tachyarrhytmia. If one believes that

TABLE 6. Risk Factors for Supraventricular Tachyarrhythmia

Variable



Age group (0 to 3, 4 to 8, 8 years) 0.004

Fontan type 0.001 0.047 0.28 0.080.98

Atrial isomerism 0.005Common AV valve 0.02

Interrupted inferior vena cava 0.036

Prior staging with bidirect Glenn 0.026

Pre-op pulmonary ar tery pressure* 0.036

History of thrombo-embolic event 0.001 0.011 4.0 1.411.5

Fontan conversion 0.001

Currrent AV valve regurgitation 0.009

AV indicates atrioventricular; bidirect, bidirectional.

*Odds ratio for every 1 mm Hg increment of pulmonary artery pressure.

Figure 3. Freedom from SVT by Fontan techniques.




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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

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 atthe beginning of this experience differs from todays 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 thefailure of their Fontan circulation postponed.

DisclosuresNone.

References1. de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary

connection: a logical alternative to atriopulmonary connection for

complex Fontan operations. Experimental studies and early clinical expe-rience. J Thorac Cardiovasc Surg. 1988;96:682695.

2. Amodeo A, Galletti L, Marianeschi S, Picardo S, Giannico S, Di Renzi P,

Marcelletti C. Extracardiac Fontan operation for complex cardiac anom-

alies: seven years experience. J Thorac Cardiovasc Surg. 1997;114:

10201030; discussion 10301.

3. Fontan F, Baudet E. Surgical repair of tricuspid atresia.Thorax. 1971;

26:240248.

4. Norwood WI, Jacobs ML. Fontans procedure in two stages.Am J Surg.

1993;166:548551.

5. Gentles TL, Gauvreau K, Mayer JE, Jr., Fishberger SB, Burnett J, Colan

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10. Morales DL, Dibardino DJ, Braud BE, Fenrich AL, Heinle JS, Vaughn

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version of the failed Fontan circulation. Cardiol Young. 2006;16(suppl

1):8591.

TABLE 7. Risk Factors for Fontan Failure

Variable



Fontan conversion 0.001

Current AV valve regurgitation* 0.013

Pulmonary artery reconstruction 0.017 0.003 3.7 1.588.66Prior staging with bidirect Glenn 0.018

Thrombo-embolic events 0.04

Age group (0 to 3, 4 to 8, 8 years) 0.044

AV indicates atrioventricular; bidirect, bidirectional.

*AV Valve regurgitation present at the time of follow-up. Before or at the time of Fontan completion




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Gavin R. Wheaton, Dan J. Penny and Christian P. BrizardYves d'Udekem, Ajay J. Iyengar, Andrew D. Cochrane, Leeanne E. Grigg, James M. Ramsay,

The Fontan Procedure: Contemporary Techniques Have Improved Long-Term Outcomes

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