Journal of the American College of Cardiology Vol. 44, No.

1, 2004
© 2004 by the American College of Cardiology Foundation ISSN 0735-1097/04/$30.00
Published by Elsevier Inc. doi:10.1016/j.jacc.2004.02.058

Diagnostic Assessment Before

Fontan Operation in Patients With
Bidirectional Cavopulmonary Anastomosis
Are Noninvasive Methods Sufficient?
Pamela S. Ro, MD, Jack Rychik, MD, FACC, Meryl S. Cohen, MD, FACC,
William T. Mahle, MD, FACC, Jonathan J. Rome, MD, FACC
Philadelphia, Pennsylvania
OBJECTIVES This study was designed to determine if a subset of patients who have undergone bidirectional
cavopulmonary anastomosis could be identified in which catheterization was of little benefit
before completion of the Fontan procedure.
BACKGROUND Diagnostic evaluation before Fontan procedure has typically included cardiac catheterization.
However, the overall management strategy for patients with functional single ventricle has
evolved to include staging bidirectional cavopulmonary anastomosis in most, and it has
become uncommon to exclude patients from Fontan based on catheterization data.
METHODS Patients who underwent bidirectional cavopulmonary anastomosis and had complete echo-
cardiograms and catheterizations within three months of each other between January 1992
and October 1997 were evaluated with a series of clinical and echocardiographic character-
istics to identify a subset in whom catheterization was predicted to be of little added value
(“no-cath” group). The predictive value and sensitivity of these criteria in excluding patients
who required additional intervention, were excluded from Fontan, or died within 30 days of
Fontan was determined.
RESULTS A total of 99 patients who underwent bidirectional cavopulmonary anastomosis at 6.7 months
(range 2.9 months to 14 years) were studied; 46 met criteria for the “no-cath” group.
Noninvasive criteria stratified all patients who died (n ⫽ 5) or did not proceed to Fontan (n
⫽ 1) and 9 of 11 who required additional interventions to the “cath” group. Thus, the
negative predictive value of these criteria was 93%.
CONCLUSIONS Our data suggest that catheterization before Fontan could be avoided in a large percentage of
patients without adversely affecting outcome; prospective evaluation of this strategy is
warranted. (J Am Coll Cardiol 2004;44:184 –7) © 2004 by the American College of
Cardiology Foundation

Noninvasive techniques are commonly used as the sole means
of evaluation before surgical repair of congenital heart disease.
Past studies have demonstrated the accuracy of echocardiog-
raphy alone for preoperative diagnosis in many lesions includ-
ing atrial septal defects, complete atrioventricular (AV) canal,
and tetralogy of Fallot (1–5).
Echocardiography is also commonly used as the sole diag-
nostic modality before the first palliative procedure in patients
with single-ventricle lesions (6,7). Diagnostic evaluation before
Fontan procedure has typically included noninvasive assess-
ment with echocardiography as well as invasive hemodynamic
and angiographic evaluation by cardiac catheterization (8,9).
The goals of this diagnostic assessment are: 1) to identify
patients in whom the Fontan operation should not be per-
formed because of excessive risk; and 2) to identify patients in
whom additional interventions (either by catheter before sur-
From the Division of Cardiology, The Children’s Hospital of Philadelphia,
Philadelphia, Pennsylvania. Dr. Ro is currently affiliated with the Division of
Cardiology, Columbus Children’s Hospital, Columbus, Ohio. Dr. Mahle is currently
affiliated with Sibley Heart Center, Children’s Health Care of Atlanta, Atlanta,
Georgia. Presented at the 11th Annual Scientific Sessions of the American Society of
Echocardiography, June 11 to 14, 2000, Chicago, Illinois.
Manuscript received March 18, 2003; revised manuscript received January 7, 2004,
accepted February 24, 2004.
gery or in the operating room concomitant with the Fontan
procedure) are required.
The overall management strategy for patients with func-
tional single-ventricle heart disease has evolved since the
enunciation of the so-called Fontan laws (10). At our institu-
tion, most patients proceed to Fontan surgery after antecedent
bidirectional cavopulmonary anastomosis. It has become un-
common to exclude patients from Fontan based on hemody-
namic parameters obtained at catheterization (11). We hy-
pothesized that, after a previous bidirectional cavopulmonary
anastomosis, a subset of patients could be identified in whom
catheterization was of little added benefit before total cavopul-
monary anastomosis. Based on clinical experience we delin-
eated a series of criteria to discriminate between patients who
would benefit from catheterization before Fontan (cath) and
those who would not (no-cath). We then tested the value of
these criteria in predicting either poor outcome or the need for
additional intervention.
METHODS
The surgical database at our institution was queried to identify
all patients who underwent bidirectional superior cavopulmo-
nary anastomosis between January 1992 and October 1997.
JACC Vol. 44, No. 1, 2004
July 7, 2004:184–7
Abbreviations and Acronyms
AV ⫽ atrioventricular
NPV ⫽ negative predictive value
PPV ⫽ positive predictive value
For inclusion in the study we required that patients have a
complete echocardiogram performed within three months of
cardiac catheterization in anticipation of completion of the
Fontan procedure. According to our laboratory standards,
complete echocardiograms in this setting include assessment of
systemic and pulmonary venous return, flow at the atrial
septum, AV and semilunar valve function, ventricular perfor-
mance, pulmonary artery architecture, and aortic arch anatomy
and flow velocity. Medical records, operative notes, and cardiac
catheterization data were reviewed. Independent observers
blinded to outcomes reviewed echocardiograms and angio-
grams.
Patients were divided into those in whom catheterization
was predicted to be unnecessary (no-cath) and those who were
predicted to require catheterization (cath) before proceeding to
the Fontan operation. Patients were included in the cath group
if they met any one of the criteria, including two clinical
features (pulse oximetry ⬍76%, hemoglobin concentration
⬎18 g/dl) and six echocardiographic features (Table 1). The
clinical course of all patients was reviewed to determine which
patients were subsequently excluded from proceeding to Fon-
tan, required additional interventions (at catheterization or
surgery), or died within 30 days of Fontan. Predictive value and
sensitivity of the discriminating criteria were determined.
Summary data are expressed as mean (range).
RESULTS
Patient population. A total of 305 patients underwent
bidirectional cavopulmonary anastomosis between January
1992 and October 1997. Of the total, 101 patients had an
echocardiogram and cardiac catheterizations within three
months of each other after bidirectional cavopulmonary anas-
tomosis with data available for review. In two cases, echocar-
diography studies were inadequate as defined (see the Methods
section). Thus, the study population consisted of 99 patients.
Of these, 94 patients had undergone hemi-Fontan and 5 had
undergone bidirectional Glenn procedures. The median age at
hemi-Fontan or bidirectional Glenn was 6.7 months (range
Table 1. Characteristics of Low-Risk Patients
Clinical data
Room air pulse oximetry ⱖ76%
Hemoglobin ⱕ18 g/dl
Echocardiographic data
Left pulmonary artery visualized without stenosis
No significant atrioventricular valve insufficiency (ⱕ1⫹)
No significant ventricular dysfunction (qualitative)
No aortic coarctation
An unrestrictive atrial communication
No evidence of a decompressing vessel
Ro et al. 185
Evaluation for Fontan Without Catheterization
Table 2. Characteristics Predicted to Discriminate Need for
Catheterization
Number of Patients
Meeting Criteria
Characteristic for Cath (n ⴝ 53)*
Clinical data
Pulse oximetry ⬍76% 8
Hemoglobin ⬎18 g/dl 9
Echocardiographic data
Left pulmonary artery not seen or stenotic 32
(19 not visualized)
(13 stenotic)
Significant atrioventricular valve 10
regurgitation (⬎1⫹)
Significantly diminished ventricular 6
contractility
Aortic coarctation 1
Restrictive atrial septal defect 1
Evidence of a decompressing vessel 1
*Nine patients met two criteria and three patients met three criteria.
2.9 months to 14 years). The median age at echocardiogram
was 19.1 months (4 months to 14 years), and the median age
at cardiac catheterization was 19.1 months (4 months to 14
years), with the median time between echocardiogram and
cardiac catheterization being one day (range 0 to 82 days). The
median age at Fontan procedure was 21 months (range 11
months to 14 years). A total of 46 patients met criteria for the
no-cath group, and 53 were stratified to cath. Among the latter
group, more than one criterion was present in 12 patients
(Table 2).
Because a large number of patients were excluded from this
study, excluded patients were compared with those in the study
population. The median age at hemi-Fontan or bidirectional
Glenn among the 206 patients excluded from this study was
6.9 months (p ⫽ NS compared with study group). Mortality
among the excluded patients was 15% (n ⫽ 31), higher than
the study population. However, this apparent difference is
explained by the fact that most of these deaths occurred early
after hemi-Fontan, before the age where patients would have
been eligible for entrance into this study. Of the 31 deaths, 23
occurred before 19 months of age (the average age at cathe-
terization in this study). This leaves eight patients who died
more than 19 months after hemi-Fontan, a mortality rate of
4%, not significantly different from that in the study group.
Outcomes. In 17 of the 99 patients, the subsequent course
was complicated. Five died (two before Fontan and three
after Fontan), and one did not proceed to Fontan. Other
interventions in addition to Fontan surgery were performed
in 11 patients; these are outlined in Table 3.
Forty-five of the 46 patients meeting no-cath criteria
proceeded to Fontan; three had additional interventions.
One patient did not proceed, secondary to a previous
cerebrovascular accident and the parents’ wish for no further
surgery. Two patients underwent coil embolization of de-
compressing veins: one had a 2 mm left superior vena cava;
the other had a 3 mm vein to the left atrium (both patients
had arterial oxygen saturations over 80%). One patient had
186 Ro et al.
Evaluation for Fontan Without Catheterization
Table 3. Additional Surgical or Transcatheter Interventions in
Patients Undergoing Bidirectional Superior Cavopulmonary
Anastomosis
“No Cath” “Cath”
Intervention Group Group
Coil embolization of decompressing vessel 2 2*
Atrioventricular valvuloplasty 1 3
Balloon dilation of left pulmonary artery 0 3*
Balloon dilation of coarctation 0 1*
Atrial septectomy 0 1
*One patient had three interventions.
surgical valvuloplasty of the AV valve at the time of Fontan.
This patient had mild AV valve regurgitation by echocar-
diogram and moderate by angiography. Of the 53 patients
meeting criteria for cath, 5 died and 8 underwent additional
interventions. The ability of these criteria to appropriately
stratify patients to cath (negative predictive value [NPV])
was 93% (95% confidence interval 86% to 100%) with 81%
sensitivity. Specificity, however, was only 52%, and the
positive predictive value (PPV) was only 25% with many
patients stratified to catheterization in whom no pertinent
findings emerged.
DISCUSSION
Many patients were excluded from the Fontan procedure in
the early decades of its application based on selection criteria
that could be accurately obtained only by cardiac catheter-
ization (9,10). Since then, patient population, surgical
approach, and diagnostic tools have dramatically changed.
In our practice the vast majority of patients considered for
Fontan have undergone prior bidirectional superior cavo-
pulmonary anastomosis (12). The goal of diagnostic evalu-
ation before completion of the Fontan procedure is to
identify those few patients in whom the Fontan operation
should not be performed, as well as those who require
additional intervention before or at the time of Fontan.
Despite these changes, current practice continues to include
cardiac catheterization before Fontan. Although catheter-
ization can be performed safely, it is not without risk.
Recent studies report complications in 5% to 10% and death
in 0.1% of diagnostic catheterizations (13,14). Catheteriza-
tion is costly and is an additional procedure for children who
are already subjected to many such procedures. Thus, we
sought to determine whether noninvasive criteria could be
used to define a subset of patients who could forgo preop-
erative catheterization without an adverse effect on out-
come. Eight criteria were defined, retrospectively applied,
and evaluated for predictive value. Forty-six percent of
patients met criteria to avoid catheterization. Overall, 17%
of patients died, required additional intervention, or did not
proceed to Fontan. The selection criteria appropriately
stratified all 5 patients who died before or after Fontan
procedure and 8 of the 11 patients who underwent addi-
tional intervention.
Three patients who were stratified to the no-cath group
JACC Vol. 44, No. 1, 2004
July 7, 2004:184–7
received additional interventions: small decompressing veins
were embolized in two patients, and one patient underwent
valvuloplasty at the time of Fontan. It is not possible to
definitively assess the importance of these interventions.
However, it is unlikely that failure to embolize the decom-
pressing veins would have resulted in excess cyanosis after
Fontan; in both instances the vessels originated from the
superior cavopulmonary circuit, and neither patient had an
unusually low arterial saturation before Fontan.
Overall, the ability of our criteria to discriminate patients
who could forgo catheterization (NPV) was acceptable
(93%). However, the positive predictive value of this strat-
egy was rather low; the majority of patients stratified to
undergo catheterization required no additional intervention
and had unremarkable courses at Fontan. The limited
ability of echocardiography to adequately assess branch
pulmonary artery architecture contributed to this poor
predictive value (19% of cases). Application of magnetic
resonance imaging to these cases would substantially im-
prove PPV of these criteria.
Other important limitations of this work should be
noted. This study is retrospective and subject to all short-
comings inherent in retrospective analyses. We chose as
outcomes the need for any additional intervention and
deaths. We did not attempt to determine to what extent
catheterization predicted these outcomes. This approach
was taken because we reasoned that attempts at such
determinations would be too subject to bias in a retrospec-
tive analysis. Clearly a prospective evaluation of this man-
agement strategy must follow before its generalized appli-
cation. Finally, it should be noted that at our institution coil
embolization of small systemic to pulmonary arteries (com-
monly found in this patient group) is not routinely per-
formed before Fontan. Some have suggested that the
presence of collaterals correlates with worse outcome after
Fontan and, therefore, they routinely embolize these vessels
(15,16). These findings have not been reproduced in other
studies (17). We reserve this intervention for instances with
abundant collateral flow and hemodynamic evidence of their
significance: high pulmonary artery pressure or elevated
ventricular filling pressure. It seems unlikely that noninva-
sive criteria would correctly identify patients with more
extensive collaterals. In spite of these limitations, our data
suggest that catheterization before Fontan could be avoided
in a large percentage of patients. We suggest that prospec-
tive evaluation of this strategy is warranted.
Reprint requests and correspondence: Dr. Jonathan J. Rome,
Division of Cardiology, The Children’s Hospital, of Philadelphia,
34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania
19104. E-mail: rome@email.chop.edu.
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