Current Management of Infants and Children

With Single Ventricle Anatomy

Patricia OBrien, RN, MSN, PNP
Jeanne T. Boisvert, RN, BSN

Children with single ventricle anatomy are among the most complicated and challenging patients encountered in pediatric
cardiology. Current management involves staged surgical procedures, beginning with neonatal palliation and followed by a
bidirectional cavopulmonary anastomosis in infancy and culminating in the Fontan procedure. The Fontan procedure, despite
separating the circulation, remains a palliative procedure with many long-term concerns. This report discusses the staged
surgical management of patients with single ventricle anatomy and the nursing issues relevant to each stage.
Copyright 2001 by W.B. Saunders Company

ANY CHILDREN WITH complex congenital heart disease have only one functional
ventricle. Single ventricle anatomy (SVA) is the
term used to describe a functional single ventricle
regardless of anatomic subtype (Tweddell, Litwin,
Thomas, & Mussatto, 1999) (Table 1). The functional ventricle provides a common mixing chamber and must pump both the pulmonary and systemic circulations; it is thus volume overloaded.
The majority of defects are diagnosed within the
first days or weeks of life because of cyanosis or
congestive heart failure. An increasing number are
diagnosed prenatally by fetal echocardiography.
Without intervention, all patients with SVA will
develop sequelae associated with chronic cyanosis
(i.e., clubbing, polycythemia, increased risk of cerebral emboli, etc.) and eventually will develop
decreased ventricular function. The goal of therapy
for these children is to improve hemodynamics by
surgically separating the systemic and pulmonary
circulations and thereby relieving cyanosis and
volume overload. This goal is ultimately accomplished with the Fontan procedure.
Children with SVA and their families are freFrom the Cardiovascular Program, Childrens Hospital,
Boston, MA 02115.
Address reprint requests to Patricia OBrien, Cardiovascular Program, Childrens Hospital, 300 Longwood Ave., Boston,
MA.
E-mail: [email protected]
Copyright 2001 by W.B. Saunders Company
0882-5963/01/1605-0007$35.00/0
doi:10.1053/jpdn.2001.26573
338

quent visitors to the health care setting throughout

their lives. Because of their unique cardiac anatomy, they challenge their caregivers to fully understand their hemodynamics and to plan every
intervention with an eye toward the future. Because they must undergo multiple tests, catheterizations, and surgeries, they demand compassionate, individualized care. This report discusses the
staged surgical management of patients with SVA
and the nursing issues relevant to each stage.
There has been a shift in emphasis from selection for Fontan procedure to preparation for Fontan
procedure (Jacobs, 2000). In the current era, patients with SVA are carefully managed from birth
with a combination of staged surgical procedures
to minimize future complications; these are outlined in Figure 1.
Initial neonatal palliation seeks to provide complete relief of systemic obstruction and manipulation of pulmonary blood flow to achieve a balanced
circulation. An acceptable balance between the
pulmonary and systemic output provides enough
pulmonary blood flow for adequate oxygen delivery to prevent acidosis without an excessive volume load on the single ventricle. Some infants with
SVA have a well-balanced circulation and need no
neonatal palliation.
A second palliative procedure often consists of a
cavopulmonary anastomosis in the form of a bidirectional Glenn shunt or Hemi Fontan procedure.
This intervention provides passive pulmonary
blood flow and decreases the volume load on the
single ventricle.
The final stage is the Fontan procedure. Critical
Journal of Pediatric Nursing, Vol 16, No 5 (October), 2001

MANAGEMENT OF PATIENTS WITH SVA

339

Figure 1. Current staged surgical management of infants with single ventricle anatomy. (Abbreviations: SVA, single ventricle anatomy; MBTS,
modified blalock taussig shunt; PA Band, pulmonary artery band.)

to the success of the Fontan procedure is an unobstructed pulmonary circulation with low pulmonary artery pressures and pulmonary vascular resistance (PVR) and normal ventricular function.
Risk factors for poor outcome after the Fontan
procedure include ventricular hypertrophy and abnormal ventricular systolic or diastolic function,
elevated right atrial pressure or PVR, pulmonary
artery distortion, and atrioventricular valve regurgitation (Tweddell, 1999) (Wernovsky & Bove,
1998).

flow and obstruction to pulmonary flow, (b) those

without obstruction to either systemic or pulmonary blood flow, and (c) those with obstruction to
systemic flow and unobstructed pulmonary flow. In
most patients, there is obstruction either to the
pulmonary or systemic outflow tracts. Having no
obstruction or obstruction to both outflows is rare.
All exhibit some degree of cyanosis because of
mixing in the single ventricular chamber.

NEONATAL PALLIATION

Infants with unobstructed systemic flow and obstruction to pulmonary flow are cyanotic at birth.
The degree of cyanosis is determined by the severity of pulmonary obstruction. Children with severe
pulmonary stenosis or pulmonary atresia may be
completely dependent on the patent ductus arteriosus (PDA) to provide pulmonary blood flow and
may become critically ill when the ductus begins to
close. Administration of prostaglandins keeps the
ductus open temporarily. Children with moderate
obstruction to pulmonary flow may do well without intervention for years. These children are vis-

SVA patients may be divided into three broad

categories: (a) Those with unobstructed systemic
Table 1. Types of Single Ventricle Anatomy
Tricuspid atresia
Hypoplastic left heart syndrome
Heterotaxy with common AV valve
Unbalanced AV canal
Double outlet right ventricle with mitral atresia
Double inlet left ventricle
Abbreviations: AV, atrioventricular.

Obstruction To Pulmonary Blood Flow

340

ibly cyanotic but are able to grow and thrive. For

children with SVA, the ideal pulmonary blood flow
is between one and two times the systemic flow
and produces an arterial saturation that averages
from 76% to 85% (Fyler, 1992).
Infants who require early intervention because
of inadequate pulmonary blood flow will receive
some type of aorta-to-pulmonary shunt, usually a
modified Blalock-Taussig shunt (MBTS) (Figure
2). An MBTS allows blood to flow from the aorta
to the lungs through the placement of a tube graft
(usually 3.5 mm in neonates) between the subclavian artery and the pulmonary artery. The size of
the shunt limits pulmonary blood flow and the
shunt is purposely kept small to prevent an increase in PVR. A shunt is a time-limited intervention, as it has no growth potential. As the infant
grows, the shunt will become restrictive, and further palliation will be needed.
No Pulmonary or Systemic Obstruction
With no obstruction to pulmonary or systemic
flow, infants with SVA may develop congestive
heart failure (CHF) because of excessive pulmonary blood flow. Over the first few weeks of life,
PVR falls with a subsequent increase in pulmonary
blood flow. With no obstruction to pulmonary or
systemic blood flow, blood will follow the path of
least resistance and preferentially flow to the lower

Figure 2. Right modified Blalock-Taussig shunt. Goretex tube

graft (W.I. Gore & Associates, Flagstaff, AZ) from the subclavian
artery to the right pulmonary artery to provide pulmonary blood
flow.

OBRIEN AND BOISVERT

resistance pulmonary circulation. With the large

pulmonary flow, cyanosis may be slight, and signs
of CHF including tachypnea, tachycardia, diaphoresis, poor weight gain, and respiratory infections are prominent. If medical management of
CHF is unsuccessful, pulmonary blood flow must
be limited to control CHF and prevent further
increases in PVR. A pulmonary artery band (PAB)
may be placed around the main pulmonary artery
and tightened to reduce pulmonary artery pressure
to one-third to one-half the systemic pressure with
a minimal reduction of systemic arterial saturation
to between 75% and 90%. Potential disadvantages
of banding include distortion of the main pulmonary artery, development of subaortic stenosis, and
damage to the pulmonary valve (Wernovsky &
Bove, 1998).
Obstruction to Systemic Flow
Hypoplastic left heart syndrome (HLHS) is a
prime example of the third category of neonates
with SVA: those infants with obstruction to systemic flow and unobstructed pulmonary flow. This
syndrome comprises underdeveloped or atretic mitral and aortic valves, a very small left ventricle
that is unable to sustain systemic circulation, and a
hypoplastic ascending aorta. In these infants, systemic circulation is supplied entirely through the
PDA. A left to right shunt at the level of the atrium,
most often a patent foramen ovale, is necessary to
allow oxygenated blood from the left atrium to
enter the systemic circulation via the PDA.
The neonate with HLHS gets into difficulty
early and usually presents for treatment as the
ductus arteriosus begins to close. Partial closure of
the ductus results in decreased cardiac output, as
blood cannot get to the aorta; subsequently, pulmonary blood flow increases. Infants with decreased cardiac output develop metabolic acidosis
and show signs of lethargy and poor peripheral
perfusion. In addition, the increased pulmonary
blood flow causes tachypnea and respiratory distress. These infants, if untreated, succumb to profound cardiogenic shock within the first few weeks
of life.
The immediate treatment is administration of
prostaglandins to maintain ductal patency and correct metabolic acidosis. Intubation, mechanical
ventilation, and inotropic support are often needed.
If pulmonary overcirculation is severe, ventilation
with a hypoxic gas mixture (17% to 20% oxygen),
which is achieved by adding nitrogen to the gas
mixture, may be used to increase PVR and decrease pulmonary blood flow. Surgical intervention

MANAGEMENT OF PATIENTS WITH SVA

is aimed at establishing systemic circulation and

controlling pulmonary blood flow. Heart transplantation is another treatment option.
The stage 1 palliation developed by Norwood,
Lang, and Hansen (1983) provides systemic circulation by transecting the main pulmonary artery
and attaching it to the hypoplastic ascending aorta.
This allows the right ventricle to pump blood to the
systemic circulation. As the main pulmonary artery
is now used in the systemic circulation, pulmonary
circulation is established by placing a modified
Blalock Taussig shunt. The existing PDA is ligated
and the atrial septum is opened to allow flow of
oxygenated blood from the left atrium to reach the
right atrium and single right ventricle and then to
the systemic circulation (Fig 3). This surgical technique is also performed in infants with other forms
of SVA that include severe aortic obstruction or
aortic arch hypoplasia.
Post-operative care. Postoperatively, hemodynamic stability is dependent on maintaining a balance between pulmonary and systemic flow. Complications present as symptoms of low cardiac
output, CHF, and excessive cyanosis (Table 2).
Providing adequate systemic blood flow may require inotropic support in the early postoperative
period. As PVR continues to fall, there may be
excessive flow through the shunt, resulting in CHF.
Aggressive treatment with diuretics and afterload
reduction may be required to manage CHF. Exces-

341
Table 2. Complications After Stage 1 Palliation for HLHS
Symptom

Potential Cause

Low cardiac output

Decreased ventricular function

Excessive pulmonary blood flow
Atrioventricular valve regurgitation
Pleural effusions
Pulmonary edema
Anemia
Elevated PVR
Restrictive atrial septal defect
Pulmonary artery distortion
Small or thrombosed aorta
Pulmonary shunt
Excessive flow through aorto-pulmonary
shunt
Aortic arch obstruction

Excessive cyanosis

Elevated oxygen
saturations

Abbreviation: PVR, pulmonary vascular resistance.

sive cyanosis may result from structural limits to

pulmonary blood flow or pulmonary causes of poor
oxygenation such as edema or effusions.
Oxygen therapy for decreased oxygen saturations must be used with extreme caution in infants
with HLHS both before and after repair. Oxygen is
a potent pulmonary vasodilator, and blood will
preferentially flow to the lungs at the expense of
the systemic circulation. Effects of oxygen therapy
must be carefully evaluated and, even during resuscitation attempts, 100% oxygen is rarely used in
this population. Oxygen saturations of 75% to 85%
are ideal and imply a good balance between systemic and pulmonary blood flow. Saturations
above 90% indicate excessive pulmonary blood
flow, and symptoms of CHF may be evident.
Although mortality for patients with HLHS who
undergo stage 1 palliation is relatively high compared with other neonatal repairs, it has decreased
significantly in the last decade. Hospital survival
after repair at a large volume center was recently
reported at 76% (Bove & Lloyd, 1996), and survival to second stage repair was 77% at another
center (Tweddell, Hoffman, & Fedderly, 2000).
Patients with cardiac defects other than HLHS who
undergo Stage 1 repair have better outcomes (Daebritz, et al., 2000).
Nursing Care

Figure 3. Stage 1 palliative reconstruction (Norwood operation).

Main pulmonary artery is ligated and anastomosed to the hypoplastic aorta connecting the right ventricle to the systematic circulation.
Pulmonary circulation is established with a modified Blalock-Taussig
shunt. The ductus arteriosus is ligated and an atrial septectomy is
done.

Infants with SVA are fragile and require knowledgeable nursing care to attain the best outcomes.
Conservation of energy, maintenance of normothermia, and aggressive treatment of fevers are
important. Increased body temperature increases
oxygen consumption, which causes decreased arterial saturations. The tachycardia associated with
fever places added stress on an already overworked

342

ventricle. Dehydration, which can increase the risk

of thrombosis, should be avoided.
Infants with SVA are notoriously poor feeders.
They tire easily, may have poor or inconsistent
sucks, and may be unable to meet their caloric
needs orally. Nurses can encourage smaller, more
frequent feedings and help parents to position infants in a more upright position to increase the ease
of feeding. Healthy infants require about 110
kcals/kg per day for normal weight gain. Infants
with SVA have higher caloric needsat least 130
kcals/kg per day. Breast milk, which contains essential immunologic properties, balanced nutrients,
and digestive substances, is the recommended infant food and should be used whenever possible.
Although some infants with SVA can nurse easily,
those who cannot nurse can benefit from receiving
breast milk by bottle or gavage feedings. High
calorie formulas can meet increased caloric needs
with less fluid volume. Calories can be added to
pumped breast milk when needed. Nutritionists can
help parents with recipes for formulas and nutritional assessments of their infants. Many infants
will require assistance with nasogastric or nasojejunal tube feedings until adequate oral feeding can
be established. Some infants will require placement of gastrostomy tubes for longer-term nutritional support.

OBRIEN AND BOISVERT

CAVOPULMONARY ANASTOMOSIS
The second palliative procedure for SVA usually occurs between 4 and 9 months of age. Preoperatively, a cardiac catheterization is done to
assess pulmonary pressures, PVR, and ventricular
function, and interventional procedures may be
performed. The operative procedure is a bidirectional cavopulmonary anastomosis (BCPA), either
bidirectional Glenn shunt or hemi-Fontan. The bidirectional Glenn shunt (BDG) involves a direct
end-to-side anastomosis of the superior vena cava
(SVC) to the right pulmonary artery (Fig 4). With
the hemi-Fontan, the lower SVC is opened vertically to the right atrial appendage and anastomosed
to the right pulmonary artery. A patch is placed in
the superior vena cava-right atrial junction to block
blood flow to the atrium and divert blood into the
pulmonary arteries (Fig 5). In cases in which pulmonary artery stenosis is present, pulmonary artery
dilatations and stenting may be done preoperatively in the cardiac catheterization laboratory, or
pulmonary artery plasty may be done at the time of
the bidirectional cavopulmonary anastomosis. Any

Parent Support and Education

Parents are understandably stressed and overwhelmed when their newborn is diagnosed with
severe congenital heart disease. Nurses can make a
significant impact on how effectively parents cope
with the stresses brought on by the diagnosis and
hospitalization of the infant. Transition to home is
also a stressful time as families become independent in their childs care. Nurses are in a unique
position to provide parental education about the
care of these fragile infants and role-model interactions with these infants that will promote parentinfant bonding. Family education includes well
baby care and nutritional concerns, as well as medication administration and assessment of signs and
symptoms of cardiac problems and respiratory distress. Maintaining open communication with the
health care team is important. Referrals to community agencies and early intervention programs
(EIP) can help ease the transition from hospital to
home and promote the infants growth and development. EIP can be especially helpful in promoting
gross motor skills, which tend to be delayed in this
population.

Figure 4. Bidirectional cavopulmonary anastomosis: bidirectional Glenn shunt. The superior vena cava is ligated and anastomosed end-to-side to the right pulmonary artery. The lower part of
the superior vena cava is oversewn. The main pulmonary artery is
usually ligated, though sometimes left open as an additional source
of pulmonary blood flow.

MANAGEMENT OF PATIENTS WITH SVA

Figure 5. Bidirectional cavopulmonary anastomosis: Hemi-Fontan. Incisions are made in the central pulmonary artery and in the
lower superior vena cava to the right atrial appendage. The incision
in the superior vena cava and the pulmonary artery are joined
creating a wide anastomosis between them. A patch is placed in the
superior vena cava at the entrance to the right atrium to prevent flow
into the atrium.

pre-existing aorto-pulmonary shunts or pulmonary

artery bands are taken down at this time. The main
pulmonary artery may be ligated and oversewn or
left as an additional source of pulmonary blood
flow, in some situations. Both procedures relieve
the volume load on the single ventricle, which
helps to prevent the development of atrioventricular valve regurgitation and preserve ventricular
function. Effective pulmonary blood flow is increased. Numerous studies have shown that outcomes after the Fontan procedure are improved
when the patient had a prior cavopulmonary anastomosis (Forbess, et al., 1997; Reddy, McElhinney,
Moore, Haas, & Hanley, 1997; Van Arsdell, et al.,
2000). An improvement in growth and development is usually noted in children after Stage 2
palliation.
The postoperative course following BCPA is
usually uncomplicated, and the mortality rate is
very low. Immediate postoperative fluid retention
may result in edema and decreased oxygen saturations. Diuretic therapy usually resolves the edema
over the first 48 hours or so, and oxygen saturations rise to between 75% and 85%. Increased SVC
pressure may occur postoperatively either transiently secondary to volume overload or because of
obstruction of the anastomosis or distal pulmonary
artery distortion. The increased pressure can result
in facial and upper extremity edema. Hypertension,
headaches, and irritability are common. Diuretics
and elevation of the head may help relieve increased pressures. Continuous analgesia may be

343

needed for headache. Pleural effusions secondary

to an increase in central venous pressure occur in
some patients.
Long-term issues after BCPA procedures include the development of venovenous collaterals and pulmonary arteriovenous malformations,
which cause increasing cyanosis. Venovenous collaterals carrying blood from the higher pressure
SVC to the lower pressure inferior vena cava
(IVC) lead to increased cyanosis over time. The
absence of hepatic venous return to the pulmonary
circulation after BCPA results in the development
of pulmonary arteriovenous malformations (AVMs).
(Srivastava, et al., 1995).
Prior to the Fontan procedure (stage 3), patients
have a cardiac catheterization to measure pulmonary artery pressure, PVR, end diastolic pressures
and to assess atrioventricular valve insufficiency.
Interventional procedures, such as balloon dilation
of pulmonary artery stenosis or coiling of collateral
vessels, may be done in preparation for the Fontan
procedure.
FONTAN PROCEDURE
The underlying principle of the Fontan circulation is that the pulmonary circulation can be perfused without a ventricular pump. The Fontan procedure separates the systemic and pulmonary
circulations to achieve normal (or near normal)
oxygen saturations and decrease the volume load
on the single ventricle. The single ventricle becomes the systemic pump, pumping blood through
the aorta to the body. The pulmonary circulation
receives passive nonpulsatile blood flow directly
from the vena cava (SVC and IVC), without a
ventricular pump. If pulmonary artery pressure is
normal, the systemic venous pressure in the vena
cava is able to exceed pulmonary artery resistance
to allow forward flow into the pulmonary arteries.
First described by Fontan and Baudet in 1971
for repair of tricuspid atresia, the original operative
technique has had many modifications and has
been applied to all types of single ventricle anatomy. Currently, the Fontan procedure is often performed at 18 months to 4 years of age. There are
several operative techniques used to create the
Fontan circulation, which are outlined in Table 3.
A significant modification of the Fontan procedure was the addition of a fenestration into the
atrial baffle (Bridges, Lock, & Castaneda, 1990). A
similar idea was an adjustable intra-atrial communication with surgical ligatures (Laks, et al., 1991),
A small hole is placed in the baffle to act as a
pop-off to decrease high pressure in the lateral

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OBRIEN AND BOISVERT

Table 3. Modifications of the Fontan Procedure
Operative Techniques

Comments

Atriopulmonary Anastomosis (RA-PA anastomosis)

(see Figure 6)

Entire RA subjected to increased pressure causing atrial

distension

Direct anastomosis between RA and PA

Atrial distension and multiple suture lines contributed to

atrial arrhythmias
Systemic venous congestion and pleural/pericardial
effusions common
Swirling sluggish flow patterns impeded flow to PA
increases risk of thromboembolism

Systemic venous return enters RA and exits through new anastomosis directly
to the PAs
ASD closed or LA blood flow baffled to the right AV valve (as in HLHS)
Commonly performed in 1980s, rarely done now
Total Cavopulmonary Connection
(see Figure 7)
An intra-atrial baffle creating a lateral tunnel within the RA is combined with
a BCPA (may have been done at a previous operation)
Baffles the IVC blood up through the baffle to the SVC, then to the PAs
Intra-atrial baffle frequently fenestrated

Improved flow patterns within the baffle

Increases velocity of PA flow
Decreases thrombus risk
Fewer atrial suture lines and less atrial distension may
reduce incidence of atrial arrhythmias

Extracardiac Conduit
(see Figure 8)
Prosthetic or homograft tube graft to carry IVC flow behind the heart to the PA
Combined with a BCPA (may have been done at a previous operation)

Short or no cardiopulmonary bypass

Avoids atrial suture lines and RA distension, may
decrease incidence of arrhythmias
Preferred with complex pulmonary venous return
(pulmonary veins to RA would be obstructed with
lateral tunnel)
Preferred with complex systemic venous connections (IVC
not in alignment with SVC)
Tube graft has no growth potential (procedure
performed at 3 yrs/15 kg) so adult-sized conduit can
be useda
Potential for conduit obstruction
Potential for thromboembolism

Abbreviations: RA, right atrium; PA, pulmonary artery; SVC, superior vena cava; IVC, inferior vena cava; ASD, atrial septal defect; BCPA,
bi-directional cavopulmonary anastomosis.
aPetrossian et al., (1999).

tunnel. It also increases atrial blood flow, which

provides adequate ventricular preload. A fenestration can also be placed in an extracardiac conduit.
Oxygen saturations are decreased between 80%
and 90% in the early postoperative period because
deoxygenated blood flows right to left through the
fenestration into the atrium. Placement of a fenestration has decreased mortality after the Fontan
(Gentiles, et al., 1997) and has significantly decreased the incidence and severity of postoperative
pleural effusions (Bridges, et al., 1992). The fenestration can be closed later by a device in the
catheterization laboratory, although some will
close spontaneously.
Modified ultrafiltration is another technical improvement that is widely applied for Fontan procedures. After cardiopulmonary bypass, modified
ultrafiltration through the bypass circuit removes
excess tissue water. This decreases myocardial
edema, improves diastolic complicance and has

been shown to decrease mortality after the Fontan

procedure (Elliot, 1993, Koutlas, et al. 1997).
Postoperative Management
The general goal of treatment in the early postoperative period is to maintain cardiac output at the
lowest central venous pressure possible (Wernovsky & Bove, 1998). The central venous pressure (CVP) is equal to the pressure inside the
Fontan pathway. This pressure must be slightly
greater than the pulmonary artery pressure for
blood to flow forward into the lungs. Maintaining
low pulmonary artery pressures becomes critical to
maintaining a low CVP. Cardiac output is dependent on pulmonary venous return. If increases in
pulmonary artery pressure and pulmonary resistance reduce blood flow into the pulmonary circulation, there will be less blood returning to the
heart, causing a fall in cardiac output. A few millimeters of pressure difference in the Fontan path-

MANAGEMENT OF PATIENTS WITH SVA

345

Figure 6. Atriopulmonary anastomosis (right atrium to pulmonary artery anastomosis). Direct anastomosis between the right
atrium and pulmonary artery. All venous return enters the right
atrium and exits through the new anastomosis to the pulmonary
artery. Main pulmonary artery oversewn. Atrial septal defect closed.

way or pulmonary circulation can be the difference

between a good outcome and a failing Fontan.
Comprehensive assessment and management by
an experienced multidisciplinary team is critical in
the early postoperative period. All caregivers need
to understand the unique physiology of the postFontan procedure patient. There are many excellent reviews of the general principles of pediatric
postoperative cardiac care in the literature. The
following discussion focuses on issues unique to
this patient population.
Cardiovascular issues. Patients are often unstable in the first 24 to 48 hours after the Fontan
procedure because of the deleterious effects of
cardiopulmonary bypass (especially increases in
PVR) and fluid shifts. Pre-existing ventricular dysfunction or atrioventricular valve regurgitation
place patients at greater risk for postoperative
problems. Continuous monitoring of all hemodynamic parameters is essential in the early postoperative period. Intracardiac lines placed in the right
atrium and the left atrium are very helpful after the
Fontan procedure. The right atrial line assesses
systemic venous pressure and pressure in the Fontan pathway. It is also very helpful in fluid management. The right atrial pressure should be 12 to
15 mmHg after the Fontan procedure. The left

Figure 7. Total cavopulmonary connection. Intra-atrial lateral

tunnel baffle directs inferior vena cava flow to the superior vena
cava which is anastomosed to the right pulmonary artery. Fenestration is placed in baffle to relieve elevated pressure in lateral tunnel
in early postoperative period. Combined with bidirectional cavopulmonary anastomosis.

Figure 8. Extracardiac conduit. Conduit placed outside of right

atrium, carrying inferior vena cava flow to the pulmonary artery.
Combined with bidirectional cavopulmonary anastomosis.

346

atrial line assesses pulmonary venous return, ventricular preload, and ventricular function.
Low cardiac output is a common problem. Volume replacement to maintain adequate blood volume is initial management, along with inotropic
support. Dopamine and dobutamine are the most
common inotropes used in children. The addition
of a phosphodiesterase inhibitor, milrinone, which
offers both inotropic support and afterload reduction is becoming more common. Nitroprusside or
other vasodilators may also be needed. Echocardiography should be done to rule out anatomic obstruction, effusion, or poor ventricular function in
patients who do not respond adequately to therapy.
Arrhythmias result from pre-existing sinus node
dysfunction, mechanical injury, injury to the conduction pathway, hypoxemia, or electrolyte imbalances. Atrial and junctional rhythms are most
common. Junctional ectopic tachycardia and supraventricular tachycardia are managed with antiarrhythmic medications, overdrive pacing, or other
treatments. Slower arrhythmias are often managed
with temporary pacing. Fontan patients rely on an
adequate heart rate to maintain cardiac output.
Persistent effusions and signs of low output are
common with slow heart rates. Permanent pacing
may be necessary to alleviate bradycardia and improve hemodynamics.
Systemic venous hypertension and congestion.
Acute systemic venous hypertension is a distinctive problem after the Fontan procedure. Increased
venous pressure is the result of the sharp rise in
right atrial pressure from a mean of approximately
5 mmHg preoperative to a mean of 12 to 15 mmHg
postoperative to propel blood into the pulmonary
circulation. Elevated right atrial, SVC, and IVC
pressures result in increased pressure in the capillary bed, which causes accumulation of extracellular fluid. Common signs of systemic venous hypertension are an increased right atrial pressure,
distended neck veins, and decreasing O2 saturations. Systemic venous congestion results in pleural and pericardial effusions, hepatomegaly, ascites, and peripheral edema.
Pleural effusions are the most common manifestation of systemic venous congestion. Chest tube
drainage may last several days to several weeks
after the procedure; sometimes for longer periods.
Symptoms of pleural effusions include tachypnea,
decreased breath sounds, decreased O2 saturations,
grunting respirations, decreased exercise tolerance,
irritability, anorexia, nausea, and vomiting. Chest
radiograph confirms the clinical findings. Aggressive diuresis is often used, and mechanical drain-

OBRIEN AND BOISVERT

age with a chest tube or pigtail catheter may be

needed. The childs fluid balance, heart rate, blood
pressure, weight, electrolytes, and physical examination must be closely monitored. Preventing atelectasis and encouraging ambulation are important. Adequate pain management is especially
important for children with chest tubes. With a
focus on pain prevention, analgesics are given on a
regular schedule during waking hours. Providing
adequate nutrition is a challenge. Nurses, parents,
and the dietitian can plan small, frequent highcalorie and high-protein meals and snacks. Children who are losing weight or are significantly
protein depleted may need nasogastric supplemental feedings at night. If patients have high volumes
of chest tube drainage with a falling serum albumin, fluid replacement with albumin may be
needed to maintain intravascular volume. All patients with persistent effusions should have a catheterization to assess for Fontan pathway obstruction, patency of the fenestration, and the presence
of collateral vessels. Interventional catheterization
techniques may resolve these problems and improve hemodynamics.
Pericardial effusions are also seen after the Fontan procedure and can be dangerous because of
potential compression of the heart and reduction in
cardiac output. Pericardial effusions can have an
insidious onset and occur days or weeks after surgery. Symptoms include tachycardia at rest, hypotension, diaphoresis, weak pulses and poor peripheral perfusion, nausea, vomiting, and irritability.
The chest radiograph shows cardiomegaly and an
enlarging cardiac silhouette. Echocardiogram is the
definitive study to assess the size of the effusion
and the extent of hemodynamic compromise. Patients with known effusions need to be carefully
monitored for symptoms of tamponade. Smaller
effusions may be managed with diuresis, afterload
reduction, and anti-inflammatory agents such as
ASA or Motrin. Significant effusions may need to
be drained with a flexible pigtail catheter. Pericardiocentesis can be done under echo guidance in an
intensive care unit setting with adequate sedation
and monitoring or with fluoroscopy in the cardiac
catheterization laboratory. Severe recurrent pericardial effusions may require surgical creation of a
pericardial window to drain the pericardial space
into the mediastinum.
Hepatomegaly, ascites, and peripheral edema
are further evidence of systemic venous congestion
and are generally seen in patients with significant
effusions, ventricular dysfunction, or atrioventricular (AV) valve regurgitation. Management in-

MANAGEMENT OF PATIENTS WITH SVA

cludes improving cardiac output with diuresis and

afterload reduction. Comfort measures, such as
elevating the head of the bed and loose-fitting
clothing, are helpful. These patients are at increased risk for skin breakdown. Careful assessment and preventive care, such as the use of pressure reducing mattresses, position changes, and
judicious use of tape, are important.
Respiratory issues. Increases in pulmonary
vascular resistance after surgery are particularly
dangerous in patients following the Fontan procedure because they are dependent on passive, nonpulsatile blood flow into the pulmonary circulation.
Elevated PVR increases systemic venous pressures
and decreases cardiac output. In the postoperative
period, positive and expiratory pressure is avoided,
and patients are extubated within 12 hours if possible. Following extubation, careful assessment
of respiratory status includes respiratory effort,
breathe sounds, and O2 saturations. Children with
fenestrations are expected to have oxygen saturations between 80% and 85% in the early postoperative period. Patients without fenestrations
should be fully saturated. Preventing atelectasis
with deep breathing exercises, ambulation, frequent position changes, and chest physiotherapy is
important. Pulmonary complications (such as pleural effusions, pneumothorax, pneumonia or lobar
collapse) increase pulmonary vascular resistance
and may impair cardiac function. Supplemental
oxygen may be needed if there is pulmonary compromise.
Thromboembolic complications. Thromboembolic complications can occur both early and late
after the Fontan procedure. Sluggish blood flow
through the Fontan pathway and extensive atrial
suture lines and foreign material may predispose
these patients to thromboemboli. Significant morbidity and mortality can occur from occlusion of
the Fontan pathway, pulmonary embolism, and
stroke. The incidence of thromboembolic events
varies in the literature, ranging from 1% to 19%
(Monagle, et al., 1998). The management of significant thromboembolic events may include surgical embolectomy, thrombolytic therapy, or anticoagulation. The outcome of aggressive treatment
is not clear. Monagle et al. (1998), in a review of
the available research studies, identified complete
resolution of thrombosis in only half the patients,
and subsequent death in 25% of the patients. Because of the poor outcomes from thromboembolic
events, most programs use a regimen of prophylactic anticoagulation with either Coumadin (Dupont Pharmaceuticals Company, Wilmington, DE),

347

aspirin, or other antiplatelet agents. No prospective

randomized trials have been conducted to determine the efficacy of prophylactic anticoagulation
agents or antiplatelet agents in preventing thromboembolic events or necessary length of treatment.
Nursing care. Effective nursing care to provide psychosocial support to younger children and
their parents following the Fontan procedure is
vitally important to both a successful medical and
emotional outcome. Care of the family as a unit
must be the focus of nursing interventions during a
sometimes prolonged hospital stay, as parents are
the main source of emotional support for their
children.
After surgery, parents are relieved but anxious
and continue to fear death or a life-threatening
event. They become tired, depressed, and frustrated as they attempt to balance outside family and
work demands. Many express helplessness at seeing their child undergo multiple painful procedures. Frequent explanations and realistic reassurance about their childs progress and eventual
recovery are important to maintain hope and optimism. Parents need to regain their parental role by
participating in activities of daily living with their
child, having meaningful choices in care when
possible and being a partner with the health care
team in decision-making. Serious illness and prolonged hospitalization can exacerbate dysfunctional family dynamics. Early assessment and
referrals to social work, psychiatry, or outside
agencies should be facilitated.
Toddlers and preschoolers are emotionally vulnerable when hospitalized because of normal developmental concerns about separation from parents, mutilation, and punishment. They have many
fears and fantasies and have limited verbal skills
and intellectual ability to understand events. Following surgery, they are in an unfamiliar environment and are anxious, frightened and irritable.
Pain, disruption in sleep cycles and normal rituals,
withdrawal from anesthesia and narcotics, multiple
monitoring lines, and periodic invasive procedures
compound their discomfort. During a prolonged
hospital stay, they show more signs of emotional
upset such as panic, temper tantrums, depression,
and withdrawal.
Consistent nursing care fosters a sense of familiarity, security, and trust in a strange environment.
A calm, honest approach works well, and the
childs preferences should be incorporated in his
plan of care when possible. Planning a daily schedule with the child and parent provides structure and
control over daily events. Painful procedures

348

should be avoided in a childs room, but rather

performed in a treatment room. Adequate premedication for chest tube insertions and removals
should be given. A sedative such as Versed (Roche
Laboratories, Inc., Nutley, NJ) in combination with
an analgesic works well. Whenever possible, children need days without tests or procedures; this
can be accomplished by grouping necessary tests
and treatments together. Children use play to cope
with difficult situations. Child life specialists can
provide frequent opportunities for hospital play,
drawing, fantasy play, and other diversional activities to help children master new experiences. Enhancing a childs ability to cope with the difficult
experience of hospitalization and providing for his
emotional well-being may be the most important
nursing goal.
Long-term Outcomes and Issues
Results of the Fontan operation have steadily
improved in the last three decades. Five hundred
patients with SVA operated on at Boston Childrens Hospital from 1973 to 1991 showed a
steady decline in Fontan failure during the time
period, from 27% in the first quarter to 7% in the
last quarter (Gentiles, et al., 1997). Functional outcomes were excellent, with 91% of patients free
from cardiac symptoms or mildly symptomatic
with exercise. Recent outcomes from 1990 to
present for Fontan procedures using a total cavopulmonary connection have early mortality rates of
less than 5% (VanArsdell, et al., 2000).
Even though there is excellent operative survival, the Fontan remains a palliative procedure for
patients with SVA. Despite relief of cyanosis, reduction of the volume load on the ventricle, and
division of the circulation, there remains only a
single ventricular pumping chamber. A unique
physiology is created with chronic systemic venous hypertension and chronic pulmonary artery
hypotension (deLeval, 1998). The Fontan ventricle
is chronically underloaded because of diminished
pulmonary venous return, and this may contribute
to diastolic dysfunction. Chronic systemic venous
hypertension increases vascular resistance and,
over time, is likely to contribute to ventricular
dysfunction (Tweddell, et al., 1999). The ultimate
fate of the single ventricle in the Fontan is unknown. Long-term problems are discussed in the
following sections.
Exercise. Following the Fontan operation, patients have an abnormal cardiorespiratory response
to exercise. They have a decreased aerobic exercise
capacity that decreases further with age and a low-

OBRIEN AND BOISVERT

er-than-normal anaerobic threshold. Multiple factors contribute to the exercise limits experienced
by these patients. They have a blunted heart rate
response, so their heart rate increases more slowly
with exercise and they reach lower maximal heart
rates. Their ability to increase stroke volume with
exercise is limited and may be related to impaired
ventricular function or residual Fontan obstruction.
They also experience mild systemic desaturation
with exercise, with O2 saturations of approximately
90%. Those with an open fenestration have more
pronounced desaturation with exercise. Children
should be encouraged to be physically active and
allowed to set their own exercise limits. Parents,
teachers, and other adults should be aware of the
childs exercise limitations, especially with endurance sports, and provide alternate activities and
adequate rest periods.
Arrhythmias. Atrial flutter is the most common
arrhythmia seen post-Fontan, and the incidence
increases with time after the Fontan procedure
(Fishberger, et al., 1997). The multiple atrial suture
lines near the sinus node and its blood supply,
atrial enlargement, and elevated atrial pressures
may contribute to the increased incidence of atrial
arrhythmias. Atrial flutter is poorly tolerated in the
Fontan circulation and can be fatal. Sinus node
dysfunction is also common and is associated with
a higher incidence of atrial flutter (Fishberger, et
al., 1997). Multiple treatments, including antiarrhythmic medications, radio-frequency ablation,
pacemaker placement, and surgical ablation, are
used in these patients.
A small group of patients with previous atriopulmonary anastomosistype Fontan procedures
develop worsening functional status after many
years because of atrial arrhythmias refractory to
therapy, obstruction of the Fontan pathway resulting in chronic effusions, and worsening ventricular
function. Some of these patients have undergone
conversion to a total cavopulmonary anastomosis
Fontan (either lateral tunnel or extra cardiac conduit) with a low mortality and improvement
in their clinical status ( Kreutzer, et al., 1996;
McElhinney, Reddy, Moore, & Hanley, 1996;
Marcelletti, et al., 2000). Mavroudis, Backer, Deal,
and Johnstrude (1998) have added additional arrhythmia circuit cryo-ablation and the placement
of a prophylactic antitachycardia pacemaker to
their Fontan conversion operations to achieve more
effective arrhythmia control in patients with severe, refractory arrhythmias. Heart transplantation
is another option for patients with a failing Fontan
circulation.

MANAGEMENT OF PATIENTS WITH SVA

Protein-losing enteropathy. Protein-losing enteropathy (PLE) is a poorly understood late complication of the Fontan procedure that entails loss
of protein through the bowel wall as evidenced by
elevated stool alpha1 antitrypsin levels, hypoalbuminemia, and hypoproteinemia. Edema, ascites,
pleural effusion, and chronic diarrhea may also be
seen. Chronic malnutrition may occur in severe
cases. The pathophysiology of PLE is unclear; the
common hypothesis is that elevated systemic venous pressure causes disturbed lymph production
and drainage in the intestinal bed, leading to bowel
wall edema and loss of protein and lymphocytes.
The severity of symptoms varies widely and may
be transient in some patients. The incidence has
been reported as between 2.5% and 13% (Feldt, et
al., 1996; Gentiles, et al., 1997; Mertens, Hagler,
Saver, Somerville, & Gewillig, 1998) and increases with longer duration of survival. (Tweddell, et al., 1999). The prognosis is poor with about
50% of patients with identified PLE eventually
dying. (Feldt, et al., 1996; Mertens, et al., 1998).
Multiple treatment approaches have been tried
with variable success. Medical management usually includes diuretics, afterload reduction, and
inotropic support in an attempt to optimize hemodynamics. Chronic albumin infusions and dietary
adjustments (high protein, low fat) may be needed
to combat chronic malnutrition. Steriods (Rychick,
Piccoli, & Barber, 1991; Mertens, et al., 1998) and
heparin therapy (Donnelly, Rosenthal, Castle, &
Holmes, 1997) have also been used with success in
some patients and with no effect in others. Interventional catheterization techniques such as balloon dilation and stenting of pulmonary artery stenosis and, particularly, creating or enlarging the
fenestration have been beneficial in some patients.
Surgical approaches such as creating a fenestration, converting to a newer Fontan pathway, and
transplantation have also been tried with some
success.
Developmental outcomes. Several recent studies have evaluated neurodevelopmental outcomes
following the Fontan procedure. Multiple factors
place this patient group at risk for neurologic and
developmental problems including chronic CHF
and cyanosis, periods of acidosis or ischemia, coexisting neurologic deficits, failure to thrive, multiple surgical procedures with cardiopulmonary bypass and periods of deep hypothermic circulatory
arrest, thromboembolic events, and other factors.
Uzark and colleagues (1998) administered neurodevelopmental tests to 32 post-Fontan patients
ages 26 months to 16 years (mean age 5.3 years).

349

They found that intellectual development is essentially within the normal range, although visual
motor deficits may be more prevalent. Wernovsky
and colleagues (2000) studied 133 children with
a median age of 11 years and median time since
Fontan of 6 years. They reached a conclusion
similar to Uzarks that the majority of individual
patients had cognitive outcomes and academic
function within the normal range but the performance as a whole was lower than the general
population.
Children with an initial diagnosis of HLHS appear to have more neurodevelopmental deficits
than the Fontan group as a whole. Uzark, et al.
(1998), Wernovsky, et al. (2000), and Goldberg, et
al. (2000) identified those with HLHS who had
deep hypothermic circulatory arrest with their initial surgical procedure as having worse outcomes.
Mahle, et al. (2000) recently reviewed outcomes in
the school-aged survivors of staged management
for HLHS and found that the mean performance of
the group was lower than the general population,
although most individual patients were in the normal range. Mental retardation (IQ less than 70) was
identified in 18%.
CONCLUSION
Children with single ventricle anatomy are
among the most complicated and challenging patients encountered in pediatric cardiology. Current
management involves staged surgical procedures
in the early years, culminating in the Fontan procedure. The outlook for these children has greatly
improved in the last 30 years since Fontan first
described this surgical approach for single ventricle anatomy. The operative survival for all surgical
repairs has improved, and the functional status of
these children is very good, with most growing and
developing normally. The Fontan procedure, despite separating the circulation, remains a palliative
procedure with many long-term concerns. The ultimate fate of the single ventricle is unknown.
Nurses have an important role in the care of these
patients and their families throughout infancy and
childhood and into adulthood.
ACKNOWLEDGMENTS
The authors gratefully acknowledge Suzanne
Reidy, RN, MN, Maeve Giangregorio, RN, and
Peter Lang, MD for their thoughtful review of the
manuscript and Emily Flynn McIntosh for the illustrations.

350

OBRIEN AND BOISVERT

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