FONTAN

PROCEDURE
History

 1971 for tricuspid atresia, published by Francis

Fontan
 Fontan procedure final state of palliation.
 Principle  bypasses the right heart
 Goal  to re protect the functional single ventricle
(FSV) from pressure overload, volume overload,
and improve oxygen levels
 Once the patients get beyond the BCPS stage they
are then considered for the final Fontan stage.
 Common-Inlet
AV connection

Absence of one
AV connection

Functional Common AV valve &

only well-developed
Single Ventricle ventricle

Only one fully

developed ventricle &
heterotaxia syndrome

Other rare forms

Surgery strategy
I. Initial surgical palliation
1. Systemic to Pulmonary Shunt
2. BAS / Septectomy
3. Pulmonary Artery Banding
4. Damus Kaye Stansel Procedure
5. Norwood Operation

II. Stage 2 palliation

■ BCPS

III. Stage 3 palliation

■ Fontan procedure
Stages toward a Univentricular Pathway:
Early Infancy
Setting: Ductus dependent circulation
■ Prostaglandin E (alprostadil/PGE1) : Patency of DA must be keep by giving PGE1 with
titrated dose 0,01 mcg/kg/min
■ PDA stenting
Setting: Pulmonary undercirculation

■ Modified Blalock-Taussig-Thomas shunt

■ Mee shunt
■ Potts shunt
■ Waterston shunt
Stages toward a Univentricular Pathway:
Early Infancy
Setting: Pulmonary overcirculation

•Pulmonary artery banding : Pulmonary blood flow

must be minimized to ensure that PVR is kept low and
the ventricle does not have an excessive volume load.
Stages toward a Univentricular Pathway:
Late Infancy
The purpose of superior cavopulmonary connection (SCPC) is to allow an increase of
effective pulmonary blood flow without an increase in total pulmonary blood flow and
cardiac work.
SCPC :
• Bidirectional cavopulmonary shunt (BCPS/modified Glenn shunt)
• Hemi Fontan procedure

SCPC is usually undertaken as soon as the pulmonary arteries have grown sufficiently to
allow a low PVR, usually between 4–8 months or earlier but not less than 3 months

Should lasts no more than 15 months to proceed to final stage  the patient is at risk of
developing intrapulmonary arteriovenous shunts, related either to endothelial dysfunction
secondary to chronic non-pulsatile pulmonary blood flow or because the lungs are not
perfused by some unidentified factor produced by the liver.
• SandeepNayak, PD Booker MBBS MD FRCA
BCPS/modified Glenn Shunt
BCPS/modified Glenn shunt
10 Commandments (Choussats, 1977)

1. Age >4 years

2. Sinus rhythm
3. Normal systemic venous return
4. Normal right atrial volume
5. Mean pulmonary artery pressure <15 mm Hg
6. Adequate pulmonary artery size
7. Left-ventricular ejection fraction >0.60
8. No atrioventricular valve leak
9. Absence of pulmonary artery distortion
10. Pulmonary arteriolar resistance <4 Wood units/m2
Fontan Criteria (PPK Harapan Kita)

■ PA pressure <15 mmHg, PARi <4 Woods.U

■ PA konfluens, adequate PA Size (kirklin table)
■ Systemic ventricle EDP < 15 mmHg
■ No severe regurgitation AV valve except can be repaired/replaced
■ Good systemic ventricle function
■ Embolized MAPCAs if possible
■ Age >3 th
Technique of Surgery
Lateral Tunnel Intraatrial Baffle
Lateral Tunnel Intraatrial Baffle
Extracardiac Conduit
Fenestrated Fontan procedure
Hillel Laks and Bridges (1990)
■ When the Fontan operation has to be done
in children who have an elevated lung
blood vessel resistance, the outcome is
unlikely to be good. This is because lung
blood flow after operation will be low.
Also, the pressure of blood in the veins
will increase to maintain lung blood flow.
And this elevated vein pressure has its
own ill effects.

■ To avoid such problems, it was suggested

that a "hole" be left in the fabric patch
used to create the tunnel. Through this
hole, the tunnel could "decompress" into
the left atrium whenever the pressure in
the tunnel becomes dangerously high. Disadvantage :
■ Significant decrease in pleural effusions lower systemic oxygen saturation
dan hospital stay compared to non
fenestrated patients.

Doorn CV, de Leval MR, Management of Single Ventricle and Cavopulmonary Connections, in Sabiston & Spencer – Surgery of
The Chest, 7 edition, Volume 2, 2005, p. 2211-2212
Postoperative management

■ Haemodynamic monitoring
■ Cardiac output optimalization (preload, inotropik and vasopressor)
■ Increase fontan flow (low PEEP, early spontaneous breathing, early extubation)
■ Echo if LCOS happened, evaluate fontan system and fenestration flow
Post Operative Management (PPK
Harapan Kita)
■ Afterload reduction  keep systemic EDP low
■ Lung Vasodilator  optimal dose 3 month after surgery or longer if needed  keep
pulmonary pressure low
■ Warfarin  INR 2-2.5 or antiplatelet 1 x 5 mg/kgBW/day
Complication
• Bleeding
• Infection
• Thromboembolic
• Pleural Effusion
• Arrhytmia
• Protein Losing Enteropathy
• Neurologic
• Phrenic nerve injury
Thank You