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 gery or in the operating room concomitant with the Fontan
of evaluation before surgical repair of congenital heart disease. procedure) are required.
Past studies have demonstrated the accuracy of echocardiog- The overall management strategy for patients with func-
raphy alone for preoperative diagnosis in many lesions includ- tional single-ventricle heart disease has evolved since the
ing atrial septal defects, complete atrioventricular (AV) canal, enunciation of the so-called Fontan laws (10). At our institu-
and tetralogy of Fallot (1–5). tion, most patients proceed to Fontan surgery after antecedent
Echocardiography is also commonly used as the sole diag- bidirectional cavopulmonary anastomosis. It has become un-
nostic modality before the first palliative procedure in patients common to exclude patients from Fontan based on hemody-
with single-ventricle lesions (6,7). Diagnostic evaluation before namic parameters obtained at catheterization (11). We hy-
Fontan procedure has typically included noninvasive assess- pothesized that, after a previous bidirectional cavopulmonary
ment with echocardiography as well as invasive hemodynamic anastomosis, a subset of patients could be identified in whom
and angiographic evaluation by cardiac catheterization (8,9). catheterization was of little added benefit before total cavopul-
The goals of this diagnostic assessment are: 1) to identify monary anastomosis. Based on clinical experience we delin-
patients in whom the Fontan operation should not be per- eated a series of criteria to discriminate between patients who
formed because of excessive risk; and 2) to identify patients in would benefit from catheterization before Fontan (cath) and
those who would not (no-cath). We then tested the value of
whom additional interventions (either by catheter before sur-
these criteria in predicting either poor outcome or the need for
additional intervention.
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 METHODS
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 The surgical database at our institution was queried to identify
Echocardiography, June 11 to 14, 2000, Chicago, Illinois.
Manuscript received March 18, 2003; revised manuscript received January 7, 2004, all patients who underwent bidirectional superior cavopulmo-
accepted February 24, 2004. nary anastomosis between January 1992 and October 1997.
JACC Vol. 44, No. 1, 2004 Ro et al. 185
July 7, 2004:184–7 Evaluation for Fontan Without Catheterization

Table 2. Characteristics Predicted to Discriminate Need for

Abbreviations and Acronyms Catheterization
AV ⫽ atrioventricular Number of Patients
NPV ⫽ negative predictive value Meeting Criteria
PPV ⫽ positive predictive value Characteristic for Cath (n ⴝ 53)*
Clinical data
Pulse oximetry ⬍76% 8
For inclusion in the study we required that patients have a Hemoglobin ⬎18 g/dl 9
complete echocardiogram performed within three months of Echocardiographic data
cardiac catheterization in anticipation of completion of the Left pulmonary artery not seen or stenotic 32
(19 not visualized)
Fontan procedure. According to our laboratory standards, (13 stenotic)
complete echocardiograms in this setting include assessment of Significant atrioventricular valve 10
systemic and pulmonary venous return, flow at the atrial regurgitation (⬎1⫹)
septum, AV and semilunar valve function, ventricular perfor- Significantly diminished ventricular 6
mance, pulmonary artery architecture, and aortic arch anatomy contractility
Aortic coarctation 1
and flow velocity. Medical records, operative notes, and cardiac Restrictive atrial septal defect 1
catheterization data were reviewed. Independent observers Evidence of a decompressing vessel 1
blinded to outcomes reviewed echocardiograms and angio- *Nine patients met two criteria and three patients met three criteria.
grams.
Patients were divided into those in whom catheterization
2.9 months to 14 years). The median age at echocardiogram
was predicted to be unnecessary (no-cath) and those who were
was 19.1 months (4 months to 14 years), and the median age
predicted to require catheterization (cath) before proceeding to
at cardiac catheterization was 19.1 months (4 months to 14
the Fontan operation. Patients were included in the cath group
years), with the median time between echocardiogram and
if they met any one of the criteria, including two clinical
cardiac catheterization being one day (range 0 to 82 days). The
features (pulse oximetry ⬍76%, hemoglobin concentration
median age at Fontan procedure was 21 months (range 11
⬎18 g/dl) and six echocardiographic features (Table 1). The
months to 14 years). A total of 46 patients met criteria for the
clinical course of all patients was reviewed to determine which
no-cath group, and 53 were stratified to cath. Among the latter
patients were subsequently excluded from proceeding to Fon-
group, more than one criterion was present in 12 patients
tan, required additional interventions (at catheterization or
(Table 2).
surgery), or died within 30 days of Fontan. Predictive value and
Because a large number of patients were excluded from this
sensitivity of the discriminating criteria were determined.
study, excluded patients were compared with those in the study
Summary data are expressed as mean (range).
population. The median age at hemi-Fontan or bidirectional
Glenn among the 206 patients excluded from this study was
RESULTS 6.9 months (p ⫽ NS compared with study group). Mortality
Patient population. A total of 305 patients underwent among the excluded patients was 15% (n ⫽ 31), higher than
bidirectional cavopulmonary anastomosis between January the study population. However, this apparent difference is
1992 and October 1997. Of the total, 101 patients had an explained by the fact that most of these deaths occurred early
echocardiogram and cardiac catheterizations within three after hemi-Fontan, before the age where patients would have
months of each other after bidirectional cavopulmonary anas- been eligible for entrance into this study. Of the 31 deaths, 23
tomosis with data available for review. In two cases, echocar- occurred before 19 months of age (the average age at cathe-
diography studies were inadequate as defined (see the Methods terization in this study). This leaves eight patients who died
section). Thus, the study population consisted of 99 patients. more than 19 months after hemi-Fontan, a mortality rate of
Of these, 94 patients had undergone hemi-Fontan and 5 had 4%, not significantly different from that in the study group.
undergone bidirectional Glenn procedures. The median age at Outcomes. In 17 of the 99 patients, the subsequent course
hemi-Fontan or bidirectional Glenn was 6.7 months (range was complicated. Five died (two before Fontan and three
after Fontan), and one did not proceed to Fontan. Other
Table 1. Characteristics of Low-Risk Patients interventions in addition to Fontan surgery were performed
Clinical data in 11 patients; these are outlined in Table 3.
Room air pulse oximetry ⱖ76% Forty-five of the 46 patients meeting no-cath criteria
Hemoglobin ⱕ18 g/dl proceeded to Fontan; three had additional interventions.
Echocardiographic data
Left pulmonary artery visualized without stenosis
One patient did not proceed, secondary to a previous
No significant atrioventricular valve insufficiency (ⱕ1⫹) cerebrovascular accident and the parents’ wish for no further
No significant ventricular dysfunction (qualitative) surgery. Two patients underwent coil embolization of de-
No aortic coarctation compressing veins: one had a 2 mm left superior vena cava;
An unrestrictive atrial communication the other had a 3 mm vein to the left atrium (both patients
No evidence of a decompressing vessel
had arterial oxygen saturations over 80%). One patient had
186 Ro et al. JACC Vol. 44, No. 1, 2004
Evaluation for Fontan Without Catheterization July 7, 2004:184–7

Table 3. Additional Surgical or Transcatheter Interventions in received additional interventions: small decompressing veins
Patients Undergoing Bidirectional Superior Cavopulmonary were embolized in two patients, and one patient underwent
Anastomosis
valvuloplasty at the time of Fontan. It is not possible to
“No Cath” “Cath” definitively assess the importance of these interventions.
Intervention Group Group However, it is unlikely that failure to embolize the decom-
Coil embolization of decompressing vessel 2 2* pressing veins would have resulted in excess cyanosis after
Atrioventricular valvuloplasty 1 3 Fontan; in both instances the vessels originated from the
Balloon dilation of left pulmonary artery 0 3*
superior cavopulmonary circuit, and neither patient had an
Balloon dilation of coarctation 0 1*
Atrial septectomy 0 1 unusually low arterial saturation before Fontan.
Overall, the ability of our criteria to discriminate patients
*One patient had three interventions.
who could forgo catheterization (NPV) was acceptable
(93%). However, the positive predictive value of this strat-
surgical valvuloplasty of the AV valve at the time of Fontan.
egy was rather low; the majority of patients stratified to
This patient had mild AV valve regurgitation by echocar-
undergo catheterization required no additional intervention
diogram and moderate by angiography. Of the 53 patients
and had unremarkable courses at Fontan. The limited
meeting criteria for cath, 5 died and 8 underwent additional
ability of echocardiography to adequately assess branch
interventions. The ability of these criteria to appropriately
pulmonary artery architecture contributed to this poor
stratify patients to cath (negative predictive value [NPV])
predictive value (19% of cases). Application of magnetic
was 93% (95% confidence interval 86% to 100%) with 81%
resonance imaging to these cases would substantially im-
sensitivity. Specificity, however, was only 52%, and the
prove PPV of these criteria.
positive predictive value (PPV) was only 25% with many
Other important limitations of this work should be
patients stratified to catheterization in whom no pertinent
noted. This study is retrospective and subject to all short-
findings emerged.
comings inherent in retrospective analyses. We chose as
outcomes the need for any additional intervention and
DISCUSSION
deaths. We did not attempt to determine to what extent
Many patients were excluded from the Fontan procedure in catheterization predicted these outcomes. This approach
the early decades of its application based on selection criteria was taken because we reasoned that attempts at such
that could be accurately obtained only by cardiac catheter- determinations would be too subject to bias in a retrospec-
ization (9,10). Since then, patient population, surgical tive analysis. Clearly a prospective evaluation of this man-
approach, and diagnostic tools have dramatically changed. agement strategy must follow before its generalized appli-
In our practice the vast majority of patients considered for cation. Finally, it should be noted that at our institution coil
Fontan have undergone prior bidirectional superior cavo- embolization of small systemic to pulmonary arteries (com-
pulmonary anastomosis (12). The goal of diagnostic evalu- monly found in this patient group) is not routinely per-
ation before completion of the Fontan procedure is to formed before Fontan. Some have suggested that the
identify those few patients in whom the Fontan operation presence of collaterals correlates with worse outcome after
should not be performed, as well as those who require Fontan and, therefore, they routinely embolize these vessels
additional intervention before or at the time of Fontan. (15,16). These findings have not been reproduced in other
Despite these changes, current practice continues to include studies (17). We reserve this intervention for instances with
cardiac catheterization before Fontan. Although catheter- abundant collateral flow and hemodynamic evidence of their
ization can be performed safely, it is not without risk. significance: high pulmonary artery pressure or elevated
Recent studies report complications in 5% to 10% and death ventricular filling pressure. It seems unlikely that noninva-
in 0.1% of diagnostic catheterizations (13,14). Catheteriza- sive criteria would correctly identify patients with more
tion is costly and is an additional procedure for children who extensive collaterals. In spite of these limitations, our data
are already subjected to many such procedures. Thus, we suggest that catheterization before Fontan could be avoided
sought to determine whether noninvasive criteria could be in a large percentage of patients. We suggest that prospec-
used to define a subset of patients who could forgo preop- tive evaluation of this strategy is warranted.
erative catheterization without an adverse effect on out-
come. Eight criteria were defined, retrospectively applied, Reprint requests and correspondence: Dr. Jonathan J. Rome,
and evaluated for predictive value. Forty-six percent of Division of Cardiology, The Children’s Hospital, of Philadelphia,
patients met criteria to avoid catheterization. Overall, 17% 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania
of patients died, required additional intervention, or did not 19104. E-mail: rome@email.chop.edu.
proceed to Fontan. The selection criteria appropriately
stratified all 5 patients who died before or after Fontan
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