Download Management of the postoperative Fontan patient

Progress in Pediatric Cardiology 17 (2003) 73–79
Management of the postoperative Fontan patient
Deborah R. Gersony*, Welton M. Gersony
College of Physicians and Surgeons, Columbia University, 630 W. 168th Street PH-3-343, New York, NY 10032, USA
Abstract
The purpose of the Fontan operation is to effectively normalize the circulation pathway in patients with a single ventricle.
Pulmonary blood flow is established by a direct systemic venous connection to the pulmonary artery without an interposed
ventricular chamber; the single ventricle provides systemic blood flow. The modern Fontan procedure consists of a direct superior
vena cava to pulmonary artery anastomosis with the inferior vena cava blood reaching the pulmonary artery by means of an
internal tunnel through the right atrium or an extra cardiac conduit from the inferior vena cava to the pulmonary artery. The
Fontan operation is performed in patients from late infancy to adulthood, and is now one of the most common surgical procedures
for congenital heart disease over the age of two years. Excellent palliation, and increased life expectancy is expected for most
patients. However, over time, complications may occur and they require careful diagnostic evaluation and specific management.
Some manifestations may appear in patients with any form of single ventricle, whereas others are specifically related to the
morphology of the original single ventricle anatomy. Potential late complications include: obstruction in the systemic venous
pathway, competitive pulmonary collateral blood flow, significant A–V valvar insufficiency, bulboventricular foramen obstruction,
ventricular dysfunction, arrhythmia, protein losing enteropathy, and thromboembolism. This monograph addresses the clinical and
laboratory assessment of patients who have had a Fontan operation, and discusses the various complications and management
options.
䊚 2003 Elsevier Science Ireland Ltd. All rights reserved.
Keywords: Single ventricle; Fontan procedure; Glenn operation
1. Management of the postoperative Fontan patient
Prior to the development of the Fontan procedure,
pulmonary blood flow in patients with single ventricle
and pulmonary stenosis was surgically augmented by
means of systemic to pulmonary artery shunts. These
shunts improved life expectancy remarkably in the short
term, but survival past the second decade remained
unusual w1x. The complications of continued cyanosis,
ventricular volume overload, and ventricular failure
remained significant risks with these procedures.
The concept that the right ventricle could be bypassed
allowing systemic blood to enter the pulmonary artery
directly was first established on experimental animals in
1970 w2x. A right atrial to pulmonary artery surgical
procedure in humans with tricuspid atresia was introduced almost simultaneously by Fontan w3x and Kreutzer
in 1971 w4x. Over the years, the indications for the
Fontan operation gradually expanded to all patients with
*Corresponding author. Tel.:q1-212-305-0629; fax: q1-212-3054429.
E-mail address: [email protected] (D.R. Gersony).
single ventricle physiology, and has become the most
common operation performed for congenital heart disease after the age of two years. Originally, the procedure
included anastomosis of the right atrium to the pulmonary artery directly (Fig. 1), with valves placed in the
superior vena cava andyor inferior vena cava. It soon
became clear that the valve placements, rather than
being advantageous, resulted in obstruction of the lowpressure vena cava to pulmonary artery circulation.
Furthermore, long-term follow-up of patients with the
direct right atrial appendage to pulmonary artery anastomosis indicated that they were prone to late complications. These were most often related to turbulent flow
within a markedly dilated right atrium that resulted in
an energy loss state, and stagnant right sided blood flow.
The patients developed exercise intolerance and other
symptoms of low cardiac output, and had a predilection
to cardiac arrhythmias associated with the dilated atrium.
On the basis of this experience, the original Fontan
operation has undergone numerous changes. In the
modern era, the modified Fontan operation often
includes an earlier bi-directional Glenn (SVC-PA anas-
1058-9813/03/$ - see front matter 䊚 2003 Elsevier Science Ireland Ltd. All rights reserved.
PII: S 1 0 5 8 - 9 8 1 3 Ž 0 3 . 0 0 0 1 1 - 0
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D.R. Gersony, W.M. Gersony / Progress in Pediatric Cardiology 17 (2003) 73–79
in the right sided circulation can cause significant
obstruction, leading to symptoms such as peripheral
edema and fatigue.
Whether morphology of the single ventricle affects
ventricular function over the long term remains unclear
w6x. It may seem axiomatic that a single systemic left
ventricle would be more likely to have preserved function in the long-term, as compared to a single right
ventricle, but this has yet to be convincingly demonstrated. Late ventricular dysfunction over time appears to be
significant in both groups w7x. However, tricuspid insufficiency in a patient with a systemic right ventricle may
result in earlier heart failure then mitral incompetence
in a single left ventricle.
Adults with the Fontan circulation offer unique challenges in that they are prone to the long term complications of ventricular dysfunction and atrial arrhythmias
w5,8x. Thus, careful monitoring of their functional status
is mandatory.
2. Anatomy of single ventricle
Patients who have had the Fontan procedure may
have one of several forms of single ventricle. The Fontan
operation was first designed for tricuspid atresia. In this
defect, the tricuspid valve is absent, and the presence of
Fig. 1. The classic Fontan operation is shown diagramatically in the
context of a double inlet single left ventricle with a bulboventricular
foramen and rudimentary RV outflow chamber. The right atrial
appendage is sewn directly into the right pulmonary artery. The systemic venous circulation is separated from the pulmonary circulation
by oversewing the right A–V valve and atrial communication. Ao:
aorta; RA: right atrium; RV: right ventricle; SLV; single left ventricle.
tomosis) or hemi-Fontan procedure. Large systemic to
pulmonary artery shunts are avoided. The Fontan operation is completed later by an internal tunnel from the
inferior vena cava through the right atrium to the
pulmonary artery (Fig. 2). Many centers will utilize an
extracardiac conduit from the inferior vena cava to the
pulmonary artery to avoid manipulation and suturing in
the right atrium (Fig. 3). Fenestration of the intracardiac
or exracardiac conduit is often carried out to allow
systemic venous blood to vent to the left atrium. This
procedure potentially increases cardiac output postoperatively, although at the cost of lower arterial O2 saturation. Pleural effusions have been reported to be less
prominent after fenestration.
The Fontan operation is still considered to be a
palliative procedure, although one which potentially will
remain effective for decades. It has been established
that the Fontan operation prolongs life expectancy in
patients with single ventricle w5x. However, late complications are frequent. With direct communication from
the vena cava system to the pulmonary artery without a
right sided pumping chamber, even minimal gradients
Fig. 2. The lateral tunnel Fontan is demonstrated in this illustration
of a double inlet single left ventricle. A prior bidirectional Glenn
anastomosis of the SVC to the right pulmonary artery is shown. For
completion of the Fontan, the IVC is tunneled within the right atrium
to the right pulmonary artery.
D.R. Gersony, W.M. Gersony / Progress in Pediatric Cardiology 17 (2003) 73–79
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3. Preoperative assessment
Fig. 3. The extracardiac Fontan operation is shown in a patient with
a double inlet single left ventricle. An external conduit from the IVC
is connected to the right pulmonary artery after a prior bi-directional
Glenn shunt.
a large atrial communication is required whereby both
systemic venous and pulmonary venous blood enters the
left atrium, left ventricle, and subsequently the aorta.
There is usually a restrictive ventricular septal defect,
and an underdeveloped rudimentary right ventricle
resulting in limited pulmonary blood flow. The double
inlet left ventricle patient has both atrioventricular valves
empty into a morphological left ventricle and only a
rudimentary right ventricular outlet chamber exists. A
bulbo-foramen (BVF) ‘VSD’ connects the left ventricle
to the small right ventricular outlet, which in turn is
connected to the transposed aorta. The BVF may be
small, and this would result in associated subaortic
stenosis. Hypoplastic left heart syndrome (HLHS) is
also a form of single ventricle. This anomaly is initially
palliated by the Norwood procedure in which the pulmonary artery and narrow ascending aorta are anastomosed; the pulmonary artery serves as the aorta and the
right ventricle remains the systemic ventricle. An arterial-PA or right ventricular-PA shunt supplies pulmonary
blood flow. HLHS patients eventually undergo a Fontan
procedure, after a second stage bi-directional SVC-PA
shunt. Less common forms of univentricular hearts
include unbalanced atrioventricular canal defects as well
as the Holmes heart in which the ventricular septum is
absent, but the intracardiac anatomy is normal.
The success of the Fontan procedure is related to the
selection criteria. The ideal low risk candidate will have
normal pulmonary arteries with low pulmonary pressure
and resistance. There would be no significant atrioventricular regurgitation and the single ventricular function
would be normal with a low ventricular end-diastolic
pressure w7x.
Originally the requirements for potential Fontan candidates were strict; most patients who underwent the
procedure had tricuspid atresia and excellent hemodynamics. Over the past decade it has become clear that
the early selection criteria were too inflexible and
excluded a significant number of patients who could
potentially benefit from the procedure. Non-fulfillment
of some of the original criteria are now regarded as risk
factors, rather than absolute contraindications w9x. It is
important to identify the potential anatomical and functional risks for they may need to be addressed at
preoperative catheterization or at the time of surgery.
Excess collateral pulmonary artery blood flow from
systemic arterial collaterals or a previous systemic arterial shunt may result in competitive pulmonary blood
flow and increased PA pressure after a Fontan operation.
Coiling of intermediate and large collaterals andyor
persistent surgical systemic arterial shunts can usually
be achieved during preoperative catheterization w11x.
Distortion of the pulmonary arteries as a result of earlier
shunts or PA banding, often requires pulmonary arterioplasty as a component of Fontan surgery. Anomalies
of the systemic or pulmonary venous system have been
incorporated into the Fontan circulation with minimal
additional risk w10x.
Significant atrioventricular valve regurgitation is poorly tolerated by patients with a Fontan circulation. Elevated pulmonary venous pressure, inevitably results in
a mandatory increase in pressure in the Fontan systemic
venous circulation in order to perfuse the lungs. In these
cases, the clinical findings of low cardiac output syndrome, peripheral edema, fatigue and abdominal discomfort due to a congested liver may become manifest.
Patients with atrioventricular valve regurgitation can
have the insufficient valve surgically repaired or
replaced at the time of the Fontan operation. Preoperative A–V insufficiency is considered an important risk
factor, although satisfactory surgical results have been
reported w12,13x. If there is ventricular outflow obstruction, enlargement of the bulboventricular foramen or a
Damus–Kaye–Stansel (DKS) procedure to merge the
great arteries may be required. It is important to note
that if there is mild morphologic obstruction to systemic
outflow, the gradients may progress over time. Even a
moderate gradient may be poorly tolerated in the Fontan
circulation, if ventricular function is affected. An appropriate strategy to relieve obstruction to systemic blood
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flow should be considered concomitant with the Fontan
procedure, but reoperation may be necessary if obstruction progresses over time. Both enlargement of BVF
and the DKS procedure have been reported to be
successful in this regard w14,15x.
Significant pulmonary hypertension is an absolute
contraindication to Fontan palliation. However, mild
pulmonary hypertension in the presence of increased
flow from a systemic shunt or in a patient after a less
than optimal pulmonary artery banding is not a contraindication for a Fontan procedure.
4. The postoperative adult patient
Most post-operative Fontan patients are asymptomatic, leading active quality lives. However, late symptoms
in the adult are not uncommon. These most often consist
of diminished exercise tolerance, fatigue, and palpitations. Obstruction in the Fontan circulation, ventricular
dysfunction, increased A–V valve insufficiency or an
arrhythmia most often present as fatigue or dyspnea on
exertion. Complaints of lower extremity or abdominal
swelling are important to investigate as they may represent the relatively uncommon but serious complication
of protein losing enteropathy (PLE).
A patient with an uncomplicated course will have an
unremarkable physical examination. The cardiovascular
examination will demonstrate no significant murmurs
and a single S2. The jugular venous pressure is often
elevated, reflecting high venous pressure in the Fontan
circulation. In patients with significant hemodynamic
abnormalities (e.g. A–V valve insufficiency, subaortic
stenosis, significant pulmonary collateral blood flow,
etc.), classic physical findings will emerge. For example,
a loud systolic ejection murmur at the base could suggest
BVF obstruction; a left lower sternal border or apical
pansystolic murmur is usually associated with A–V
valve insufficiency. A specific diagnosis can be made
on the basis of laboratory studies (e.g. Chest X-ray,
ECG, 2D echo, catheterization, Holter studies, O2 sat.
etc.)
The postoperative Fontan patient should have normal
or near normal oxygen saturation. If significant cyanosis
persists, it could have several different etiologies. If
there was an atrial fenestration, a persistent right-to-left
shunt may be present. Fenestrations may be closed by a
device in the cardiac catheterization laboratory to both
correct cyanosis and prevent paradoxical embolization.
Before such a device is placed, temporarily balloon
occlusion of the fenestration may be useful in order to
determine the effect of closure on hemodynamics. If
aortic pressure remains stable and right atrial pressure
does not increase significantly, it is considered appropriate to close the fenestration. A 10 year follow-up
study by Goff and colleagues of 154 patients, suggests
that device closure of the Fontan fenestration is safe,
and results in improved oxygenation and a reduced need
for anticongestive medication w16x. The incidence of
death or significant clinical decompensation in this study
was rare (1.3 and 3.2%, respectively). This paper did
not address the issue as to whether all Fontan patients
should have an atrial fenestration as part of the original
operation. With the advent of external conduits, some
centers are carrying out fewer fenestrations w17x.
Another etiology of persistent cyanosis in a Fontan
patient is a leak of the IVC to right pulmonary artery
baffle (e.g. an unintentional fenestration). These baffle
leaks are often small and do not cause significant
cyanosis, but if necessary, they can be closed by transcatheter device with the same preclosure techniques to
observe and evaluate the hemodynamic effects as with
fenestrations. Occasionally, a persistent left SVC may
cause significant right-to-left shunting by draining
venous blood into the coronary sinus or directly into the
left atrium. Finally, intrapulmonary shunting through AV
fistulae can be encountered in patients who have had
either a bi-directional or, more commonly, a classic
Glenn anastomosis w18x.
5. Complications
5.1. Arrhythmia
Late atrial tachyarrhythmias are frequently seen in
post-Fontan population, especially in patients who have
had a classic atrial-pulmonary artery anastomosis. Ghai
and colleagues w19x reviewed the outcomes of 94 consecutive patients who underwent the Fontan procedure
between 1977 and 1994. Forty-one percent of patients
developed sustained atrial tachycardia. The authors
found that many were likely to exhibit atrial enlargement
and have a right atrial thrombus. A higher frequency of
significant systemic valve regurgitation was also demonstrated and ventricular dysfunction was not uncommon. It remains to be seen whether the extracardiac
Fontan, which requires less direct manipulation or suturing of atrial tissue, may result in a decrease in the
incidence of atrial arrhythmias. Preliminary early and
mid-term reports have been encouraging, but not definitive w20x.
Given the long-term risks of ventricular dysfunction
and thrombus formation, it is important to reinstitute
sinus rhythm in patients with sustained atrial arrhythmia.
In most cases, anticoagulation should be given for 4–6
weeks followed by cardioversion. Unfortunately, recurrence is common and chronic antiarrhythmic drug treatment is often needed to maintain sinus rhythm.
Antiarrhythmic therapy may be complicated by limited
drug efficacy and underlying abnormalities of the sinus
node and AV conduction system. Choice of drug options
are influenced by the concomitant presence of ventricular dysfunction. Amiodarone has been increasingly
D.R. Gersony, W.M. Gersony / Progress in Pediatric Cardiology 17 (2003) 73–79
used in patients with poor function and refractory
arrhythmias w18x. Radiofrequency ablation is an available option for recurrent tachyarryhthmias. Mapping studies have demonstrated that atrial reentry tachycardias
after the Fontan operation are significantly different
from typical atrial flutter. The arrhythmias encountered
in Fontan patients occur in areas of non-conductive
atriotomy scars or surrounding prosthetic material. Reentrant activation occurs in close proximity to these regions
and adjacent anatomic landmarks such as the SVC or
IVC, and the atrioventricular annulus w21x. Electrophysiology studies can identify these areas. These zones
may be amenable to interruption with a line of block
between surgical and anatomical barriers w22,23x. However, long-term success with radiofrequency ablation in
the catheterization laboratory has been variable in terms
of maintaining long-term sinus rhythm without recurrent
atrial tachycardia. In severe cases, a surgical MAZE
procedure with epicardiac DDD pacing can be carried
out in conjunction with revising the classic RA–PA
Fontan to a lateral tunnel or extra cardiac conduit. There
have been reports of improved functional class and
lowered incidence of recurrent arrhythmias with this
approach w24x. For patients with sinus node dysfunction
or heart block, epicardial pacing is a viable option and
results are similar to patients with biventricular physiology w25x.
5.2. Protein-losing enteropathy (PLE)
An infrequent but serious late complication of the
Fontan procedure is PLE. The findings include hypoalbuminenia, ascites, pleural effusions, diarrhea, malaise
and lymphocytopenia. The prevalence of this complication is reported to range from 1.5 to 11% w26x. Although
the etiology of PLE is not known, it has been assumed
that it is related to the elevated systemic venous pressures of the Fontan circuit. A recent retrospective analysis of 398 Fontan patients attempted to identify risk
factors for the development of PLE w27x. Potential risk
factors included postoperative systemic venous pressure,
cardiopulmonary bypass time, single right ventricle anatomy, gender, age at Fontan operation, anomalous venous
drainage, atrioventricular valve regurgitation, pulmonary
artery distortion, and pre-Fontan measurements of systemic venous pressure and pulmonary artery resistance.
In this study, longer bypass time and single right
ventricular anatomy were the only peri-operative risk
factors that were associated with the development of
PLE. The postoperative systemic venous pressure was
not significantly correlated with this complication. The
authors speculated that peri-operative cardiac injury may
serve as a potentiating factoring in the development of
PLE. At the onset of PLE, any evidence of abnormal
hemodynamics or obstruction in the Fontan circuit
should be investigated by cardiac catheterization.
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The management of PLE is challenging. Symptomatic
treatment includes, diuretics, supplemental albumin infusion, and dietary modifications to high protein and high
medium-chain triglyceride intake and some benefit has
been reported w28,29x. There are sporadic case reports
of improvement with oral steroids and heparin, which
are thought to stabilize intestinal cell membranes
w30,31x. For refractory cases, attempts to improve hemodynamics and lower the pressure in the systemic venous
system have been undertaken with fenestration of the
systemic venous baffle, and some positive results have
been forthcoming w32x. Under extreme circumstances,
in unresponsive patients, cardiac transplantation is an
effective treatment w33–35x.
5.3. Ventricular dysfunction
Several studies have demonstrated decreased systolic
ventricular performance over time after a Fontan operation. A recent prospective study evaluated the incidence
of heart failure and ventricular dysfunction in patients
with systemic right or single ventricles under various
circumstances w7x. The highest risk group were patients
who were status post the Fontan procedure, as compared
to patients with congenitally corrected transposition or
post Mustard repair of transposition of the great arteries.
The incidence of documented heart failure was 40% in
this group of patients w7x. The reason for the high
incidence of progessive ventricular dysfunction in the
Fontan group over time is not clear. Cheung et al. w36x
has shown evidence of progressive diastolic dysfunction
in pediatric patients, and speculate that abnormal ventricular relaxation may play a role in the development
of later systolic dysfunction. Although little data are
available regarding optimal medical treatment for ventricular dysfunction after the Fontan procedure, standard
therapy includes diuretics, digoxin and afterload reduction with ACE-inhibitors. The role of ACE-inhibitors in
patients without symptoms of heart failure is not yet
defined w37x.
For patients with a classic RA–PA Fontan, who have
severe symptomatic ventricular dysfunction despite maximal medical therapy, conversion to a lateral tunnel or
external conduit has been effective in increasing cardiac
output and alleviating symptoms. The operation is often
done in conjunction with a MAZE procedure since
chronic atrial arrhythmias are frequently associated with
Fontan failure. Heart transplantation offers a mode of
treatment when others have been exhausted, and good
results are reported for the majority of patients w38x.
5.4. Thromboembolism
Thromboembolic events are a not infrequent complication of Fontan patients. The reported rate of significant
thrombotic events has been reported to be as high as
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33% w38,39x. The etiology of embolic events has been
attributed to atrial arrhythmias, sluggish blood flow,
right-to-left shunts, and hypercoaguable states. The role
of anticoagulation for this group of patients is not clear.
The use of warfarin for prevention of thrombotic events
in patients with dense spontaneous echocontrast in the
right atrium is often recommended. Although antiplatelet
therapy is not generally recognized as being effective
w39x, Jacobs and colleagues recently found no evidence
of either thromboembolic events or asymptomatic thrombus in 72 patients treated with 81 mg of aspirin and
followed for a mean of 40 months w40x. It should be
noted that in this study, transthoracic echocardiography
was the surveillance technique used, and this is a less
sensitive test compared to a transesophageal echocardiography in the detection of intracardiac thrombus.
5.5. Pregnancy
During pregnancy cardiac output increases by 30–
40% and intravascular volume by 50%. Stroke volume
and heart rate increase, and systemic and pulmonary
resistance decreases. As a result, there are significantly
increased cardiac volume requirements. Fontan patients
have a limited ability to increase cardiac output and
have elevated venous pressures. Thus, there are concerns
regarding the hemodynamic effect of pregnancy on the
Fontan patient. Nevertheless, successful pregnancies in
patients with excellent pre-pregnancy Fontan hemodynamics have been reported w41,42x. No reports of maternal deaths have appeared, but there is an increased
incidence of spontaneous abortion and pre-term labor.
There also is an increased risk of atrial arrhythmia and
heart failure during pregnancy. Whether there are permanent changes in maternal hemodynamics is unknown.
On balance, given the risks for mother and fetus, most
cardiologists discourage pregnancy for Fontan patients.
6. Conclusion
The modern Fontan operation remains a palliative
procedure for single ventricle patients. Mortality and
morbidity are low. Outcomes are more favorable than
for those patients who have had systemic to pulmonary
artery shunts alone. Associated defects such as bulboventricluar foramen obstruction and atrioventricular
regurgitation should be treated concommitantly at the
time of the Fontan operation. However, there remain
significant long-term complications including arrhythmias, A–V valve insufficiency, ventricular dysfunction,
thromboembolism and protein-losing enteropathy. Symptomatic patients with classic Fontans can be converted
to lateral tunnel or external conduits with MAZE procedures to treat or prevent atrial arrhythmias. Cardiac
transplantation represents a final option for Fontan
patients with intractable low cardiac output.
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