Single Ventricle & Fontan

Pathway
UCSF Congenital Cardiac Anesthesia Tutorials

William C. K. Ng
Division of Congenital Cardiac Anesthesia

2017
 Normal Cardiopulmonary Anatomy
Paediatric to Adult circulation

PFO
PDA
RV (Pulmonary Vascular System) in series with LV (Systemic
Vascular System)
RV is the auxiliary pump, synchrony, interdependence
Preload to LV and reduced afterload to LV (RAP < LAP)
Contribution to CO
Pulmonary vascular volume reserve: recruitment
Further contribution to CO in exercise

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 Neonatal Circulation
Transition from Foetal to Neonatal circulation: 2 shunts

Bruce Blaus - Own work, CC

BY-SA 4.0,
https://commons.wikimedia.or
g/w/index.php?curid=449681
62

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 The classic TA and the early Fontan
Fontan and Baudet, French. (First reported in Thorax. 1971, 26:240-248)
26/11/2016, 17)59

Superior
vena cava

Aorta
Pulmonary
artery

Pulmonary
Left vein
atrium
Right
atrium

Atresic
tricuspid
valve

Rudimentary
right ventricle Diagram_of_the_human_hea
Ventricle rt_(cropped).svg: Yaddah
cropped image by Wapcaplet
derivative work: Rupert
Millard (talk) -
Inferior vena cava
Diagram_of_the_human_hea
rt_(cropped).svg, CC BY-SA
3.0,
https://commons.wikimedia.or
g/w/index.php?curid=610370
0

4 Single Ventricle and Fontan Pathway - William C. K. Ng 2017

 Congenital Cardiac Lesions
Various lesions may benefit from Single Ventricular Surgical
Palliation Pathway:

DORV, DILV
HRHS
PA/IVS
TA
HLHS
MA
Schones, Williams
Unbalanced CAVC, Truncus Arteriosus

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 Staged Palliation for HLHS management

Using HLHS as the context for understanding Single Ventricles:

Stage I Norwood + Sano/AP shunt/BTS
Stage II Glenn (bidirectional Glenn shunt, superior
cavopulmonary connection)
Stage III Fontan (superior & inferior cavopulmonary connections)

N.B. Damus-Kaye-Stansel Shunt for conversion to Univentricular

conversion, such as DORV.

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 Stage I: The Norwood Norwood, Lang, Hansen. Physiologic
Repair of Aortic AtresiaHypoplastic
Left Heart Syndrome. NEJM.
1981;308:23-26

Sano et al. Right ventricle-pulmonary artery shunt in

first-stage palliation of hypoplastic left heart
syndrome. Semin Thorac Cardiovasc Surg Pediatr Card
Surg Annu. 2004;7:22-31

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 Goals of the Norwood
Goals and effects
Relieve LVOTO, increased organ perfusion and growth
Increased oxygenation by decreased mixing
Maintained but limited pulmonary blood flow, growth, maturity
Non-distorted pulmonary arteries
Unrestricted pulmonary venous return
Preparation for cavopulmonary connections
Performed in neonatal period, within first-week of life

Adjunct include PA band

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 The classic -> bidirectional Glenn shunt
W. Glenn. Circulatory Bypass of the Right Side of the Heart. NEJM 1958; 259:117-120.

Khairy P, et al. Univentricular heart. Circ 2007;115:800-812

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 Goals of the Glenn in Staged Palliation
Ventricular volume unloading in the transitional period is key.

Decreased end-diastolic ventricular volume

Decreased ventricular wall-thickness
Favourable geometry (prevent eccentric hypertrophy, dilation, dysfunction)
Diuretics
Age-dependent
Ventricular load change
Stage I 300% normal/BSA, stage II 90%, stage III 70%.

Volume load paradox: adequate PV load vs. SV off-load

3-6 Months
Sats 75-85% (IVC/hepatic blood is deoxygenated)

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 F. Fontan and M. Baudet. Thorax.
1971, 26:240-248

Surgical repair of
tricuspid atresia
has been carried
out in three
patients; two of
these operations
have been
successful.

11 Presentation Title and/or Sub Brand Name Here 4/12/17

 Gross Anatomy of Fontan
Cavopulmonary anastomosis: lateral tunnel or extra-cardiac

Jolley M, Colan S, Rhodes J, Dinardo J. Fontan Physiology

Revisited. Anes Analg 2015;121:172-82.

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 Goals of Fontan
And some major circulatory side-effects,
Near normal saturation: implications for survival, growth
Performed 18-24 months: improved outcomes after staged BDG
But increased afterload, increased hydraulic power (Wt/CI), reduced preload,
reduced contractile reserve.
Smaller venous capacitance (smaller baseline unstressed volume)
Inefficient pulmonary vascular bed
SVC/IVC collision, shear stress,
APC (80%) -> turbulence, increased Fontan pathway pressures
Lack of pulmonary vascular growth
Non-pulsatile flow (physical and molecular mechanisms)
Lack of hepatic factor -> AV malformations
Increased hepatic, splanchnic pressures
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 Schematic diagram of Series Circulation
SVR, PVR and the CPVR

Jolley M, Colan S, Rhodes J, Dinardo J. Fontan Physiology

Revisited. Anes Analg 2015;121:172-82.

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 Scheme of SV pressures
CVP, RAP, PAP/LAP, AoP
Jolley M, Colan S, Rhodes J, Dinardo J. Fontan Physiology
Revisited. Anes Analg 2015;121:172-82.

Gewillig M, Brown SC. The Fontan circulation after 45 years.

Heart 2016; 102:1081-6.

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 Fontan: Cavopulmonary Bottleneck
Absence of RV, normal PV vasculature, pulsatility PBF is bad
Gewillig: the critical bottleneck to flow is the neoportal
impedance, causing upstream congestion and downstream
limitation.

Gewillig M, Brown SC. The Fontan circulation

after 45 years. Heart 2016; 102:1081-6.

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 Fontan: associated conditions
PLE (3.7 24%)
Hepatic and Portal venous congestion: Fontan liver-associated disease
Plastic bronchitis
SVT and other Arrhythmias (7-50%; major cause of death outside
perioperation; 23% pacemaker)
Atrio-pulmonary connection > lateral tunnel > extra-cardiac Fontan
Heterotaxy
Spleen, GI, SVC/IVC/azygous
Cavopulmonary failure but rarely ventricular dysfunction
Failed Fontan: low CO, increased systemic venous pressure,
Diastolic (overgrown, stiff); Systolic
Pulmonary vascular system already discussed

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 Anesthesia Strategem
Despite the spectrum of badness, therere common elements:
Pre- & Post-Stage I
PGE1 infusion.
Balancing PVR : SVR, CO; systemic venous saturation; coronary (diastolic) steal in
BTS
Post-Stage II
Cerebral venous return, SVC, positioning
CO incompletely dependent on PBF. Qp:Qs 0.5-0.7 & inhalation induction
Ventilation, PAW, pCO2
Post-Stage III
CO is dependent on PBF: PVR or PV impedance is the single most important factor
AW pressures: 30% respiratory dependent c.c. 15% in biventricular system),
MAW inversely related to CO.
Preload deprivation
Fenestration (pop-off), saturation
Ventricular diastolic and systolic function

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 CHD for non-cardiac surgery scenario

5 y.o. for laparoscopic appendicectomy

HLHS, post completion of FONTAN
What are your anaesthetic
Preoperative concerns & optimisations?
Intraoperative concerns & optimisations?
Post-operative concerns & optimisations?

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 Anaesthetic Consideration continued
Pre-op
Function and ET. PE: SPO2, associated conditions.
Meds: ASA, PDE-I/ACE-I/ETA/PGI2
Labs: HCT, plt. Echo. CXR.
Access: previous procedures, BTS, difficult IV,
Intra-op: TIME-OUT (intraop. pitfalls, disposition)
Premed: benzo +/- ketamine. Induction: opioid; tolerance.
Preload sensitive. Systemic-Pulmonary venous return and ventilation (CO2, PEEP,
venous tone). Low-threshold for OPEN procedure.
Inotropy: Dopamine PIV/EJV temporarily. CPR strategy: PADS, drugs, help.
Monitoring: NIRS, standard +/- 5-lead ECG
Post-op:
PACU or PICU. Spontaneous ventilation.
Other: Dexmedetomidine.
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 Outcomes of Fontan
Good, Bad, and Failing
HLHS pre 1980s
<30% survival into adulthood
HLHS post- 1990s
Up to 70% survival into adulthood
90% of hospital survivors remain in NYHA I & II for two or more
decades

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 References

Selected reviews
Gewillig M, Brown C. The Fontan circulation after 45 years: update in physiology. Heart
2016;102:10811086
Jolley M, Colan S, Rhodes J, Dinardo J. Fontan Physiology Revisited. Anesth Analg
2015;121:17282
Feinstein et al. Hypoplastic Left Heart Syndrome - Current Considerations and
Expectations. J Am Coll Card. Vol. 59, No. 1, Suppl S, 2012
Freedom R, Nykanen D, Benson LN. The physiology of the bidirectional cavopulmonary
connection. Ann Thorac Surg. 1998;66:664-7.

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