Modified Fontan Without Use of Cardiopulmonary
Bypass
Vincent K. H. Tam, MD, Bruce E. Miller, MD, and Kathy Murphy, MSN
Section of Cardiothoracic Surgery and Department of Anesthesiology, Emory University School of Medicine; and Sibley Heart
Center, Egleston Children’s Hospital, Atlanta, Georgia
Background. Direct cavopulmonary connection using
an extracardiac conduit has a number of theoretical
advantages in the staged management of children with
single ventricular congenital heart defects. With appro-
priate planning, completion Fontan using an extracardiac
connection may be accomplished without the use of
cardiopulmonary bypass.
Methods. From January 1995 to October 1997, 32 con-
secutive patients underwent completion Fontan using an
extracardiac cavopulmonary connection. Twenty-one of
these patients had completion Fontan without the use of
cardiopulmonary bypass (No CPB group). Their postop-
erative outcome was retrospectively compared with a
second group of 11 patients who underwent completion
Fontan with an extracardiac conduit with the use of
cardiopulmonary bypass.
Results. There was no operative or hospital mortality
S ince the original description of right heart bypass by
Fontan and Baudet in 1971 [1] as treatment for
children with tricuspid atresia, many modifications have
been suggested. De Leval and colleagues [2] proposed
the concept of direct cavopulmonary connection as a way
to minimize kinetic energy loss in the Fontan circuit.
Interest on the use of an extracardiac inferior vena cava
to pulmonary artery conduit has recently been rekindled
by Marcelleti and colleagues [3, 4].
The extracardiac modified Fontan has a number of
theoretical advantages. Because the conduit is con-
structed outside the heart, the operation may be per-
formed with the patient supported on cardiopulmonary
bypass, without arresting the heart. In selected patients,
the extracardiac Fontan may be accomplished without
the use of cardiopulmonary bypass. In January 1995, an
18-month-old 11-kg child with hypoplastic left heart
syndrome successfully underwent placement of an extra-
cardiac inferior vena cava to pulmonary artery conduit
without the use of cardiopulmonary bypass. This patient
had bilateral bidirectional Glenn shunts placed at the age
of 6 months, and the presence of dual sources of blood
flow to the lungs allowed the construction of an inferior
vena cava to pulmonary artery conduit without the use of
Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic
Surgeons, New Orleans, LA, Jan 26 –28, 1998.
Address reprint requests to Dr Tam, Emory Clinic, 1365 Clifton Rd, Suite
A2236, Atlanta, GA 30322; e-mail: vtam01@emory.edu.
© 1999 by The Society of Thoracic Surgeons
Published by Elsevier Science Inc
in either group. Early postoperative hemodynamics ap-
pear to be significantly improved in the No CPB group.
Transfusion of cryoprecipitate and platelets was signifi-
cantly less in the group without the use of cardiopulmo-
nary bypass (p ⴝ 0.026, p < 0.001, respectively). Review of
the most recent 12 patients also demonstrated a substan-
tially shorter extubation time and intensive care unit
stay. The length of hospital stay was significantly shorter
(p ⴝ 0.036).
Conclusions. Completion Fontan without the use of
cardiopulmonary bypass results in improved immediate
postoperative hemodynamics, and decreased use of
blood and blood products. The most recent group ap-
pears to demonstrate a more rapid recovery time and
shorter hospital stay (p ⴝ 0.036).
(Ann Thorac Surg 1999;68:1698 –704)
© 1999 by The Society of Thoracic Surgeons
cardiopulmonary bypass. The early gratifying result
stimulated us to plan for and develop techniques to
accomplish the extracardiac Fontan without the use of
cardiopulmonary bypass. This report describes the first
21 consecutive patients and compares their early out-
come with 11 other consecutive patients who underwent
an extracardiac Fontan with the use of cardiopulmonary
bypass during the same time period.
Material and Methods
Patient Population
From January 1995 to October 1997, 32 consecutive pa-
tients between the ages of 18 months and 5 years,
weighing between 8.6 to 19 kg, underwent the modified
Fontan operation with an extracardiac inferior vena cava
to pulmonary artery connection (Table 1). Twenty-one
patients underwent surgery without the use of cardiopul-
monary bypass (No CPB group), while 11 patients had
surgery with the use of cardiopulmonary bypass (CPB
group). All patients except 1 had previously undergone
placement of a superior vena cava to pulmonary artery
anastomosis. One patient in each group had bilateral
bidirectional Glenn shunts. Cardiac catherization was
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Ann Thorac Surg TAM ET AL 1699
1999;68:1698 –704 FONTAN WITHOUT CARDIOPULMONARY BYPASS

Table 1. Patient Characteristics

Heart
Defects
Age (mo) Weight (kg) HLHS TA Others

No CPB 24.2 ⫾ 5.1 10.8 ⫾ 1.1 14 4 3

CPB 26.8 ⫾ 14.6 12.1 ⫾ 2.8 4 1 6
p 0.57 0.17

CPB ⫽ cardiopulmonary bypass; HLHS ⫽ hypoplastic left heart

syndrome; TA ⫽ tricuspid atresia.

performed before surgery in all patients. The calculated

pulmonary vascular resistance for the No CPB group was
2.0 ⫾ 0.5, while the calculated pulmonary vascular resis-
tance for the CPB group was 2.2 ⫾ 0.9. One patient in the
No CPB group has a calculated pulmonary vascular
resistance greater than 3.0 Woods unit, while 2 of the CPB
group had similarly elevated calculated pulmonary vas-
cular resistance. These differences were not statistically
significant. Additional features that may confer increased
risk for the completion Fontan operation are listed for
both groups in Table 2.
It has been our practice to address all potential ana-
tomical issues at the bidirectional Glenn stage, such that
at completion Fontan, connection between inferior vena
cava and pulmonary artery only needs to be established.
Of these 32 consecutive patients, 26 patients needed
inferior vena cava (IVC) to pulmonary artery connections
only, and were candidates for completion Fontan without
the use of cardiopulmonary bypass. Six patients required
pulmonary artery reconstruction and were not felt to be
candidates. Of the 26 patients for whom no cardiopulmo-
nary bypass was planned, in 5 patients, cardiopulmonary
bypass was used because of difficulty with placement of
the IVC cannula.
Surgical Technique
In all patients, abnormal systemic arterial to pulmonary
arterial collateral vessels are divided as much as possible.
In the cardiopulmonary bypass group, standard bicaval
venous and ascending aortic cannulation is used. In both
groups, right and left branch pulmonary arteries are
dissected completely to the hilar branches. All patients
Table 2. Risk Factors for Completion Fontan
No CPB AVVR
Decreased systemic RV function
Diffusely small branch PA, AVVR
LPA stent, SVT, renal failure, pleural effusion
after Glenn
SVT, pacemaker, AVVR, decreased ventricular
function
CPB AVVR
No previous Glenn
AVVR ⫽ moderate atrioventricular valve regurgitation; CPB ⫽ car-
diopulmonary bypass; LPA ⫽ left pulmonary artery; PA ⫽ pulmo-
nary artery; RV ⫽ right ventricle; SVT ⫽ supraventricular tachy-
cardia requiring transcatheter ablation.
Fig 1. Right branch pulmonary artery has been isolated and a longi-
tudinal arteriotomy made. A vascular clamp is used to direct supe-
rior vena cava blood flow to the left lung only.
are heparinized initially with a 400-U/kg dose of heparin.
A vascular clamp is used to isolate the right branch
pulmonary artery, diverting superior vena cava blood
flow to one lung only (Fig 1). The extracardiac conduit to
pulmonary artery anastomosis is then performed using a
continuous polypropylene suture technique. Once this
anastomosis is completed, superior vena cava blood flow
is reestablished to both lungs (Fig 2). Next, an inferior
vena cava to atrial shunt is constructed using two right-
angle metal-tip venous cannulas (DLP, Grand Rapids,
MI). This inferior vena cava to atrial shunt is first placed
in the inferior vena cava, and then the atrium, to provide
for an alternative pathway for inferior vena cava blood
flow to the atrium (Fig 3). A vascular clamp is then placed
across the inferior portion of the right atrium, cephalad to
the inferior vena cava junction. The inferior vena cava to
extracardiac conduit anastomosis is performed (Fig 4).
The conduit is then deaired and blood flow from the
inferior vena cava is established to the branch pulmonary
arteries. The inferior vena cava to atrial shunt is then
removed (Fig 5). All patients had a 4-mm fenestration
created between the IVC conduit and the pulmonary
venous atrium (Fig 6). Because of the concern with the
lack of growth of the extracardiac conduit, typically a 20-
to 22-mm conduit is used.
Analysis
The early postoperative course for these two groups of
patients were compared. Variables analyzed include in-
traoperative and early postoperative hemodynamics,
transfusion of blood and blood products in the first 24
1700 TAM ET AL Ann Thorac Surg
FONTAN WITHOUT CARDIOPULMONARY BYPASS 1999;68:1698 –704

Fig 3. The inferior vena cava is divided from the right atrium and
the cardiac end oversewn. The inferior vena cava to the right atrial
shunt allows venous blood to continue to return to the right atrium.

different. However, when the last 12 patients in the No

CPB group were compared with the CPB group, the
Fig 2. The extracardiac conduit to pulmonary artery anastomosis length of ventilator hours, intensive care unit stay, and
has been completed. Superior vena cava blood flow is now reestab-
lished to both right and left lungs. An inferior vena cava to right
atrial shunt has been placed.

hours of mechanical ventilation, length of intensive care

unit stay, and length of hospital stay. Categorical vari-
ables were compared using Fisher’s exact test, while
continuous variables were compared using Student’s t
test. All results for the continuous variables are reported
as mean ⫾ standard deviation. Results were considered
significant if the p value was less than 0.05.

Results
There was no operative or hospital mortality in either
group. Patients in the No CPB group have significantly
lower pulmonary venous atrial pressures at the end of
surgery, 6 hours, and 12 hours postoperatively (Table 3).
By 24 hours, these differences disappeared (Fig 7). Al-
though proportionately more patients were treated with
milrinone in the CPB group, this difference was not
statistically significant. Cryoprecipitate and platelet
transfusions were significantly more common in the CPB
group (Table 4). Although there was a substantial differ-
ence in the use of more packed red blood cells for the
CPB group, this difference was not statistically signifi-
cant. Time to extubation, length of intensive care unit Fig 4. The inferior vena cava to extracardiac conduit anastomosis is
stay, and length of hospital stay were not statistically performed.
Ann Thorac Surg TAM ET AL 1701
1999;68:1698 –704 FONTAN WITHOUT CARDIOPULMONARY BYPASS

Table 3. Perioperative Hemodynamics

CVP Atrial Pressure
(mm Hg) (mm Hg)

End of surgery
No CPB 12.7 ⫾ 2.6 4.9 ⫾ 1.4
CPB 14.6 ⫾ 2.4 6.6 ⫾ 2.1
p 0.053 0.040
6 Hours postop
No CPB 11.2 ⫾ 3.5 4.2 ⫾ 2.7
CPB 12.6 ⫾ 2.3 6.5 ⫾ 2.1
p 0.22 0.016
12 Hours postop
No CPB 10.9 ⫾ 3.4 4.6 ⫾ 2.8
CPB 13.2 ⫾ 3.1 7.3 ⫾ 3.0
p 0.086 0.031
24 Hours postop
No CPB 13.5 ⫾ 5.0 6.7 ⫾ 4.4
CPB 13.5 ⫾ 2.2 6.7 ⫾ 2.4
p 1.00 1.00

CPB ⫽ cardiopulmonary bypass; CVP ⫽ central venous pressure.

tions. One patient developed refractory ventricular

Fig 5. Completion Fontan using an extracardiac conduit has been
accomplished without the use of cardiopulmonary bypass. A fenes-
tration may be placed between the extracardiac conduit and the
atrium.
hospital stay were all substantially different, with the
length of hospital stay achieving statistical significance.
Four patients in the No CPB group suffered complica-
tachycardia and fibrillation secondary to digoxin toxicity
approximately 18 hours after admission to the intensive
care unit. A loading dose of digoxin had been given
intravenously over a short period of time, 16 to 18 hours
postoperatively because of the development of supraven-
tricular tachycardia (even though this patient was on
maintenance digoxin preoperatively). Because this was a
witnessed event in the intensive care unit, cardiopulmo-
nary resuscitation was begun immediately and the pa-
tient brought emergently to the operating room. The
child was quickly placed on cardiopulmonary bypass.
Despite being on CPB, multiple attempts at defibrillation
were unsuccessful. This rhythm was resistant to multiple

Fig 6. Angiogram demonstrating direct inferior vena cava to main

pulmonary artery connection, accomplished without the use of car-
diopulmonary bypass. Because of the slight rotation of the patient,
the branch pulmonary artery confluence is superimposed on the
Glenn anastomosis, which had been placed in the very distal right Fig 7. Pulmonary venous atrial pressures at the end of surgery, 6,
branch pulmonary artery. 12, and 24 hours postoperatively. * p ⬍ 0.05.
1702 TAM ET AL
FONTAN WITHOUT CARDIOPULMONARY BYPASS
Table 4. Number of Patients Who Received Perioperative
Transfusion (24 Hours)
PRBC FFP Cryo
No CPB 17/20 (369 ⫾ 227 mL) 7/20 2/20
CPB 10/10 (563 ⫾ 271 mL) 3/10 5/10
p 0.532 1.00 0.026
CPB ⫽ cardiopulmonary bypass; Cryo ⫽ cryoprecipitate;
fresh-frozen plasma; PRBC ⫽ packed red blood cells.
phamacologic medications. Dilantin briefly interrupted
the ventricular tachycardia. Digoxin binding antibody,
however, terminated the ventricular tachycardia upon
administration. The digoxin level before binding anti-
body, while the patient was on cardiopulmonary bypass,
was well above 5.0 ng/mL. He subsequently developed
multiple complications, including renal failure, empy-
ema, and remained in the intensive care unit for a period
of 50 days. Fortunately, he has recovered since with no
discernable neurologic deficit. One patient suffered a
perioperative stroke with the development of left upper
extremity paresis. The etiology of this insult remains
unclear. Two patients developed superficial wound in-
fections, requiring treatment with antibiotics only. In the
CPB group, 1 patient developed a severe tracheitis and
required continuous sedation, paralysis and prolonged
endotracheal intubation for approximately 13 days. Per-
sistent pleural effusions occurring after 2 weeks, requir-
ing catheter drainage, occurred in 4 patients in the No
CPB group and in 5 patients in the CPB group. This
difference was not statistically significant.
Comments
Surgical management of children with single ventricular
physiology has continued to evolve. Many institutions
have adopted various approaches with improved early
Fig 8. Optimal growth and development of the right and left branch
pulmonary arteries in an infant 5 months after the Norwood opera-
tion for hypoplastic left heart syndrome.
Ann Thorac Surg
1999;68:1698 –704
Platelets
4/20
9/10
⬍ 0.001
FFP ⫽
Fig 9. Construction of the Glenn anastomosis toward the left branch
pulmonary artery in the same patient with hypoplastic left heart
syndrome.
clinical outcomes. Staging the Fontan operation with the
bidirectional Glenn anastomosis, and the deliberate cre-
ation of a residual right-to-left atrial shunt, have un-
doubtedly contributed significantly to the improved early
outcome of these patients. The investigations of De Leval
and associates [2] and Sharma and associates [5] have
added further guidance in optimizing the geometry of
the cavopulmonary connections. The use of an extracar-
diac inferior vena cava to pulmonary artery connection
has a number of theoretical advantages. It avoids exten-
sive atrial suture lines, exposure of the atrium to higher
venous pressures, and theoretically better preservation
of kinetic energy in the Fontan circuit. In addition,
because the connection is created outside the heart,
aortic cross-clamping could be entirely avoided. These
theoretical advantages have prompted the application of
the extracardiac cavopulmonary connection at our
institution.
The outcome after completion Fontan operations may
be further optimized with appropriate planning. Metic-
ulous attention is given to achieving appropriate growth
and development of branch pulmonary arteries with
avoidance of any significant pulmonary artery stenosis
(Fig 8). In the newborn period, excessive pulmonary
blood flow is avoided. A bidirectional Glenn anastomosis
is constructed in early to mid infancy. We have not
utilized the hemi-Fontan approach. The superior vena
Table 5. Hematology, Coagulation (Postoperative Day 1,
3:00 a.m.)
Hgb Hct PT PTT
No CPB 13.3 ⫾ 1.9 39.1 ⫾ 5.6 15.7 ⫾ 3.4 47.6 ⫾ 19.2
CPB 14.5 ⫾ 1.9 42.9 ⫾ 6.1 15.4 ⫾ 2.1 42.9 ⫾ 10.7
p 0.10 0.11 0.79 0.40
CPB ⫽ cardiopulmonary bypass; Hgb ⫽ hemoglobin (gm/100 ml);
Hct ⫽ hematocrit; PT ⫽ prothrombin time (in seconds); PTT ⫽
partial thromboplastin time (in seconds).
Ann Thorac Surg
1999;68:1698 –704
cava (SVC) anastomosis is either diverted toward the
right, or more typically toward the left branch pulmonary
artery (Fig 9). This tends to encourage appropriate
growth of the left branch pulmonary artery, simplifying
the later completion Fontan procedure. Having the SVC
anastomosis in the very proximal right branch pulmo-
nary artery, occasionally into the proximal left branch
pulmonary artery, easily allows construction of the extra-
cardiac Fontan without the use of cardiopulmonary by-
pass. Need for simultaneous extensive pulmonary artery
reconstruction, atrioventricular valve repair, and other
procedures would exclude the use of this technique.
Theoretically, we avoid the inflammatory sequelae asso-
ciated with the use of cardiopulmonary bypass. The
results in this preliminary group indeed suggest that the
immediate hemodynamics are improved compared with
the CPB group. The use of blood and blood products are
more limited. With further refinement, shorter immedi-
ate recovery time and shorter hospital stays are achiev-
able. However, because blood is exposed to the foreign
material of the IVC to right atrial shunt, we can not
assume the usual inflammatory response to cardiopul-
monary bypass is entirely avoided. A prospective ran-
domized trial with measurement of inflammatory medi-
ators may indeed provide interesting results.
The obvious single disadvantage of the extracardiac
Fontan is the lack of growth of the conduit. To minimize
the likelihood for replacement of the extracardiac con-
duit, we have utilized a conduit of 20 to 22 mm in
diameter. Whether the IVC will grow to compensate for
the fixed length of the conduit remains to be seen. In
terms of conduit material, we have generally preferred a
valveless ascending aortic homograft conduit. Typically,
the homograft is divided at or above the level of the
sinotubular ridge. Valve tissue and associated ventricular
muscle are excised. Because of the posterior location of
the IVC and the more anterior location of the right
pulmonary artery, with the potential for right upper
pulmonary vein compression, the arch of the homograft
provides the perfect geometry for this extracardiac con-
duit. Since the homograft provides a nonthrombogenic
surface, we have not routinely used anticoagulation,
except for once daily aspirin.
DISCUSSION
DR EDWARD L. BOVE (Ann Arbor, MI): It was not clear to me
in your paper just what preoperative indications you used for
patients undergoing a Fontan without the use of bypass. Can
you identify those patients before surgery and did you have
patients that had to be converted to bypass even though the
original plan was to do it without the pump.
DR TAM: What we have tried to do is to address any and all
surgical issues at the Glenn stage so that when these children
came back for the Fontan operation, generally speaking, we tend
not to have difficulty with branch pulmonary artery stenosis or
other unresolved surgical issues, eg, restrictive atrial communi-
TAM ET AL 1703
FONTAN WITHOUT CARDIOPULMONARY BYPASS
Table 6. Postoperative Course Comparing the 12 Most
Recent Patients in the No CPB Group
Ventilator ICU Stay Hospital Stay
(h) (h) (days)
No CPB 8.2 ⫾ 5.3 51.1 ⫾ 28.4 7.0 ⫾ 3.8
CPB 50.1 ⫾ 95.7 93.9 ⫾ 108.9 10.8 ⫾ 4.3
p 0.20 0.23 0.036
CPB ⫽ cardiopulmonary bypass; ICU ⫽ intensive care unit.
Conclusions
Review of our early experience with the construction of
extracardiac cavopulmonary connection without the use
of cardiopulmonary bypass has been encouraging. Early
postoperative hemodynamics appear to be improved
with less frequent use of blood and blood products.
Analysis of the most recent group has also suggested a
quicker recovery and shorter hospital stay (Tables 5, 6).
Hopefully, with further refinement, this would translate
into not only improved short-term but perhaps long-term
outcome for our patients as well.
We acknowledge the support and advise by Drs Robert A.
Guyton, Willis H. Williams, Kirk R. Kanter, James M. Bailey,
Steve Toscone, Nina Guzetta, Shiva Sharma, David Jones, and
the physicians of the Children’s Heart Center, Atlanta, Georgia.
We are also grateful for the expert assistance by Vertis Walker in
the preparation of this manuscript.
References
1. Fontan F, Baudet E. Surgical repair of tricuspid atresia.
Thorax 1971;26:240– 8.
2. De Leval M, Kilner P, Gewillig M, Bull C. Total cavopulmo-
nary connection: a logical alternative to atrial pulmonary
connection for complex Fontan operations. J Thorac Cardio-
vasc Surg 1988;96:682–95.
3. Marcelletti C, Corno A, Giannico S, Marino B. Inferior vena
cava-pulmonary artery extracardiac conduit. J Thoracic Car-
diovasc Surg 1990;100:228–32.
4. Black MD, van Son JAM, Haas GS. Extracardiac Fontan
operation with adjustable communication. Ann Thorac Surg
1995;60:716– 8.
5. Sharma S, Goudy S, Walker P, et al. In-vitro flow experiments
for the determination of the optimal geometry of the total
cavopulmonary connection for surgical repair of children
with functional single ventricle. J Am Coll Cardiol 1996;27:
1264–9.
cation. Obviously, if a child needed to have a completion Fontan
operation with, for instance, AV valve repair, then obviously that
would not be possible. In the kids who are appropriately
prepared with well-developed branch pulmonary arteries with a
Glenn anastomosis, doing the operation without bypass is quite
feasible. Early in our experience there were a few patients that
were converted to bypass because we had difficulty placing the
IVC cannula distal enough without significant blood loss for us
to do the operation.
DR J. TERRANCE DAVIS (Columbus, OH): Could you give us
a little more information about what kind of conduit you use and