Fontan Circulation

• Fontan Procedure
• Fontan Physiology
• Patient Selection
• Post-Fontan Complications
• Fontan Outcomes
• Ventilation Implications
Fontan Procedure
• First described in 1971 by Francis Fontan
• It is used to treat complex congenital heart diseases
when bi-ventricular repair is not possible:
– Tricuspid atresia
– Pulmonary atresia
– Severe pulmonic stenosis
– Single ventricle
– Hypoplastic left or hypoplastic right heart
• The above conditions rely on one functional ventricle to
maintain systemic and pulmonary circulations that are
not connected in series but in parallel
Fontan Procedure
• A single ventricle parallel circuit creates 2 major
disadvantages:
– Systemic arterial desaturation at rest
– Chronic volume overload to the ventricle
• Without surgical intervention, there is about a
90% mortality before age 1
• A completion Fontan is the definitive palliative
step in a series of surgeries used to improve
oxygenation and cardiac function
How to achieve a Fontan Circuit

• At birth, it is impossible to create a Fontan

circulation:
– PVR is elevated for several weeks
– SVC and IVC veins and pulmonary arteries
may be too small
• A staged approach allows the body to
adapt progressively to the different
hemodynamic conditions
How to achieve a Fontan Circuit
• Neonatal period
– Achieve unrestricted flow from the heart to the aorta
– Improve limited flow to the lungs
– Achieve unrestricted blood return to the ventricle
– The infant is allowed to grow for several months
• Pulmonary vasculature will develop more
• PVR will stabilize
• The heart will be subjected to chronic volume overload
• Ventricular function may deteriorate
• Expect mild progressive desaturation of the infant
How to achieve a Fontan Circuit
Normal Heart Single Ventricle – BT Shunt

http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/sv.htm
How to achieve a Fontan Circuit

• At age 4-12 months

– The superior vena cava will be connected to
the pulmonary artery (Glenn)
• This will decrease the volume load to the
heart
– The patient will remain cyanotic as the
desaturated blood from the IVC is still allowed
to flow to the aorta
How to achieve a Fontan Circuit

Single Ventricle – BT Shunt Single Ventricle – Bidirectional

Glenn

http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/sv.htm
How to achieve a Fontan Circuit
• At 1-5 years of age
– The Fontan circuit is completed by connecting the IVC
to the pulmonary artery

Single Ventricle – Single Ventricle –

Bidirectional Glenn Completed Fontan

http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/sv.htm
 Fontan Modifications
Fontan surgical techniques: Classical atriopulmonary connection (A), Lateral tunnel (B),
and extracardiac conduit (C)

d'Udekem, Y. et al. Circulation 2007;116:I-157-I-164

Copyright ©2007 American Heart Association

Fontan Physiology
• The sub-pulmonary ventricle is bypassed
• Systemic venous return is diverted directly into
the pulmonary arteries
– Goal is to provide adequate pulmonary blood flow
and cardiac output with minimal elevation in venous
pressure
• Systemic and pulmonary venous returns are
separated:
– Cyanosis is relieved
– Volume loading on the ventricle is significantly
reduced
Patient Selection
• After a Fontan operation, the left atrial pressure and the
transpulmonary gradient must be low in order for the
repair to be successful
• Pre-op cardiac requirements:
– Unobstructed ventricular inflow
– Unobstructed ventricular outflow
– Reasonable ventricular function
• Pre-op pulmonary requirements:
– Good sized pulmonary arteries
– Near normal pulmonary vascular resistance
– Unobstructed pulmonary venous return
Fontan Circulation Complications

• Complications after Fontan repair are

common and are related to:
– Increased venous pressure
– Increased venous congestion
– Chronic low cardiac output
Fontan Circulation Complications
• Early and late mortality
• Mild to moderate exercise intolerance
• Residual cardiomegaly
• Ventricular dysfunction
• Rhythm and conduction disturbances
• Hepatomegaly
• Lymphatic dysfunction with protein losing enteropathy
• Systemic venous thrombi
• Ascites
• Peripheral edema
Fontan Circulation Complications

• Ventricular Function
– All post-Fontan hearts have a ventricle that is
dilated, hypertophic and hypocontractile
– Dysfunction may be caused by the congenital
malformation itself, previous surgery or the
abnormal hemodynamic changes encountered
through the various stages of palliation
Fontan Circulation Complications

• Ventricular Function
– During the first months after birth, the
ventricle will always be volume overloaded
• Ventricular dilation and spherical reconfiguration
• Cardiac overgrowth
• Eccentric hypertrophy
• After the Fontan completion, some
normalization will occur
Fontan Circulation Complications
• Ventricular function evolves from being volume
loaded and overstretched to overgrown and
underloaded
• Systolic and diastolic dysfunction are common
• Reduced preload is the dominant factor
contributing to poor ventricular function
• Inotropes, afterload reducers, vasodilators and
B-blockers are generally ineffective
• Pulmonary vascular resistance will control
cardiac output
– Improving pulmonary blood flow will improve cardiac
output
Fontan Circulation Complications
• Predisposition to atrial dysrhythmias
– Up to 40% of patients 10 years post-op
– Most commonly intra-atrial re-entry
tachycardia or atypical atrial flutter
– Survival depends on ventricular contractility
and vascular resistance
– Safest treatment is immediate DC
cardioversion
– Long term treatments include medication,
ablation, pacemaker, Fontan take-down
Fontan Circulation Complications
• Predisposition to coagulopathies
– Thrombosis is more likely in patients with low
CO and venous stasis
– Increased incidence of coagulation factor
abnormalities because of hepatic congestion
• Protein C
• Protein S
• ATIII deficiency
– Anticoagulation of all patients is controversial
Fontan Outcomes

• Despite the abnormal circuit, most

patients with a Fontan circulation can lead
a nearly normal life, including mild to
moderate sport activities
• More than 90% of hospital survivors are
NYHA functional class I or II
• Patient’s remain slightly desaturated with
values in the low 90s
Fontan Outcomes

• University of Melbourne
– 305 consecutive Fontan operations between
1980-2000
– 10 hospital deaths (3%) none after 1990
– 20 year survival was 84% (257 pts in long-
term follow up)
– Freedom from Fontan Failure was 70% at age
20
Fontan Outcomes

• University of Melbourne
– Fontan failure was defined as:
• Death
• Fontan Take-down
• Transplantation
• NYHA functional class III or IV
Fontan Outcomes

• Boston Children’s Hospital

– Intermediate-term follow up study
– 220 pts aged 11 mo to 32 years
– Lateral tunnel Fontan between 1987 - 1991
– 91% survival at 10 years
– 87% Freedom from failure at 10 years
Post-Fontan Pregnancy
• Normal pregnancy
– 30-40% increase in CO by 24 weeks
– 30-40% increase in circulating blood volume
– Decreased SVR
– Myocardial oxygen consumption increases 20%
– Heart rate increases 15-20%
• Fontan patients may develop “right heart” failure
symptoms
– Atrial arrhythmias, peripheral edema, ascites
– Increased risk of venous thrombosis/PE
Post-Fontan Pregnancy

• Of 39 completed pregnancies reported in

the literature, 16 patients experienced a
decline in NYHA functional status
• Overt heart failure occurred in 4 patients
• Spontaneous abortion rate 50% (normal
population 10-15%)
• Increased risk of premature delivery
Anesthetic Implications
• Multi-disciplinary pre-operative approach is mandatory
• SBE prophylaxis
• Anticoagulation
• Maintain adequate intravascular volume
• Ventilation
– Spontaneous ventilation provides significantly increased
pulmonary blood flow when compared to positive pressure
ventilation
– Avoid hypoxemia and hypercarbia (increase PVR)
– If positive pressure ventilation is necessary, low rates, short
inspiratory times, low PEEP and moderately elevated tidal
volumes (~15/kg) are recommended
Review
• The Fontan procedure is considered palliative and
enables survival for several decades
• In a Fontan circulation, systemic venous return is
diverted directly into the pulmonary arteries
• Complications after Fontan are related to increased
venous pressure, increased venous congestion, and
chronic low cardiac output
• Post-Fontan anesthetic goals include maintaining
adequate preload and minimizing increases in pulmonary
vascular resistance
References
• D’Udekem, Y., et al. The Fontan procedure: Contemporary
techniques have improved long-term outcomes. Circulation. 2007.
116: I-157 – I-164
• Gersony, D., et al. Management of the postoperative Fontan.
Progress in Pediatric Cardiology. 2003. 17: 73-79
• Gewillig, M. The Fontan Circulation. Heart. 2005. 91: 839-846
• Redington, A. The physiology of the Fontan Circulation. Progress in
Pediatric Cardiology. 2006. 22: 179-186
• Walker, F. Pregnancy and the various forms of Fontan Circulation.
Ed. Heart. 2007. 93: 152-154