Transcript Ann Thorac Surg

Karyn P. Luna, M.D.
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Updates on the indications, timing and outcome of
univentricular repair.
Present surgical options.
Discuss the controversies regarding the modifications of the
Fontan operation.
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A heart that lacks two well-developed ventricles:
Hypoplastic left heart syndrome
Hypoplastic right heart
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Tricuspid atresia+/- pulmonary atresia with intact ventricular
septum, double inlet left ventricle, double outlet right
ventricle, unbalanced atrioventricular septal defects and
hypoplastic left heart syndrome.
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Generally, the risk to siblings and offspring of affected
individuals is 2% to 5% (19% -33% of siblings HLHS) .
Clinical manifestations and initial management hinge on the
presence or absence of pulmonary and systemic outflow
obstruction.
Natural history
Left ventricle dominant= 70% died before age 16
Right ventricle dominant =50% died before 4 years
Fontan operation: 20-year survival rate and freedom from
heart transplantation of 80%-82%.
Paul Khairy, MD, PhD et al.Circulation. 2008
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Staged repair allows progressive adaptation of the heart and
lungs and reduces the overall perioperative morbidity and
mortality.
SandeepNayak,PD Booker MBBS MD FRCA
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Create unobstructed systemic outflow, unobstructed
systemic and pulmonary venous return
Provide balanced pulmonary and systemic circulations.
Totally separate the systemic and pulmonary venous return
and provides pulmonary blood flow without a ventricular
pumping chamber.
Effective strategies to preserve pulmonary, ventricular and
valvular function.
Paul Khairy, MD, PhD et al.Circulation.2007
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Balanced circulation: No early intervention
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The aim of the initial palliation is to provide complete relief
of any systemic obstruction, if it exists, and provide
pulmonary blood flow just sufficient to allow adequate
oxygen delivery to tissues and pulmonary arterial growth.
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Procedures:
Pulmonary Artery banding
Modified Blalock-Taussig shunt
Modified Norwood 1/Sano repair
Hybrid procedure
Setting:Pulmonary overcirculation
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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.
Setting: Hypoxemia
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Modified Blalock-Taussig
shunt
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PDA stenting
Advantages of mBTS:
 Improve oxygen saturation
 Facilitate growth of pulmonary
arteries
Disadvantages of mBTS:
 Ventricular volume overload
 Increase in pulmonary arterial
pressure
 Distortion of pulmonary arteries
The long-term effects of marked
single-ventricle preload and
inefficient oxygenation via an
arterial shunt rarely allowed
survival beyond the second or
third decade of life.
Welton M. Gersony, MD Circulation 2008
Setting: HLHS
 Modified Norwood 1 or
Sano repair
 Hybrid procedure
 First 2 weeks of life
 Permanent systemic outflow
utilising the right ventricle
 Temporary pulmonary blood
supply to allow the pulmonary
vasculature to develop and
mature.
 Up to 20-25% mortality rate
U Theilen, L Shekerdemian. Arch Dis Child Fetal Neonatal Ed 2005
Modified Norwood 1
The main pulmonary
artery(MPA) is divided
 Proximal MPA is anastomosed to
the ascending aorta
 Aortic arch is repaired
 Augmented pulmonary blood
flow is maintained via a
mBTS
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Sano repair:
Similar to NW 1 but with RV-PA
conduit instead of mBTS.
 Theoretical advantage of
avoidance of aorto-pulmonary
runoff, resulting in higher
coronary and systemic perfusion
pressures and reducing the
incidence of ventricular.
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Risk factors for midterm mortality were cardiorespiratory
failure requiring ventilation and ACS ≥20, but not shunt type.
Increased number of shunt-related interventions before the
Glenn procedure were noted with Sano.
CONCLUSIONS: Preoperative risk factors, regardless of
shunt type, influence midterm survival after the Norwood
procedure with an excellent outcome in low-risk patients,
while high-risk cases still incur a significant mortality.
Given the possible negative effects of ventriculotomy on
right ventricle function, the widespread use of Sano shunt
should be reconsidered.
Does the shunt type determine mid-term outcome after Norwood operation?
Joachim Photiadisa, et al November 15, 2011.
Setting: HLHS
 The Hybrid Procedure
 Interventional ductal
stenting & surgical bilateral
pulmonary artery banding
 An alternative to the NW in HLHS
for high-risk patients
 This approach has been extended
to borderline left heart structures.
David Anderson, Conal Austin, Shakeel Qureshi et al.
European Journal of Echocardiography (2010)
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Hybrid vs NW:
Hybrid has lower 1st stage
mortality but higher interstage
mortality. Possible causes are
closing PFO, short stent,
myocardial ischemia.
= the 2nd stage is a
more difficult operation.
Pizarro et al. Eur J
CTS. 2008
Overall, no difference in outcome
at 6 months.
Pizarro et al Thorac CVS 2010
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The advantage of the BDG/HemiFontan over
the Blalock-Taussig shunt is to allow an increase of effective
pulmonary blood flow without an increase in total pulmonary
blood flow and cardiac work.
Davide F. Calvaruso, et al Ann Thorac Surg 2008
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An interstage mortality of 10–15%
Most interstage deaths are sudden and apparently
unpredictable.
U Theilen, L Shekerdemian. Arch Dis Child Fetal Neonatal Ed 2005
Setting: Cyanosis or pulmonary
overcirculation or balanced
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CavoPulmonary Connection
Bidirectional Glenn Shunt
or
Hemi-Fontan operation :
Duration of 15 months because
after surgery, the patient is at risk
of developing intrapulmonary
arteriovenous shunts
SandeepNayak, PD Booker MBBS MD FRCA
Benefits of Cavopulmonary
connection:
 Decreased cyanosis
 Decreased intracardiac
volume overload
O MonteinNgodngamthaweesuk et al Asian
CardiovascThorac Ann 2007
HEMIFONTAN OPERATION
HEMIFONTAN OPERATION
Procedures
Cardiopulmonary Bypass
Bidirectional Glenn
Hemi-Fontan
+/-
+
SVC-PA connection
+
+
Transection of SVC
+
-
+/-
+
Repair of TV & PA
Procedures
Bidirectional Glenn
Hemi-Fontan
Early Mortality rate
5%-10%
Same
Ideal type of Fontan
Extracardiac
Lateral Tunnel
+
+
21%
2%-5%
Noncompetitive Fontan
flow pattern
Post-Fontan Mortality rate
Operative mortality rate of BDG/HemiFontan is 5%-7%
Freedom from failure at 10 years was 99.2% for the
Fontan group and 67.4% for the BDG patients.
Jenkins KJ.Eur J Cardiothorac Surg 2010
Modified Fontan operation:
Total cavopulmonary
connection (TCPC)
 18 months – 4 yrs. old (or 6-18
months post-Glenn or hemifontan)
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ORIGINAL FONTAN
MODIFIED FONTAN
LATERAL TUNNEL FONTAN
EXTRACARDIAC FONTAN
LATERAL TUNNEL
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Intra- atrial baffle from IVC
to SVC
EXTRACARDIAC
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A tube graft from the IVC
to RPA outside the heart.
LATERAL TUNNEL
EXTRACARDIAC
Age/Weight
Lateral Tunnel
Extracardiac
>/= 2 years old
>/= 5 years old (13 kg)
CPB
+
+/-
Growth potential
+
-
Exercise tolerance
Similar
Similar
Arrhythmia
Similar
Similar
Thrombosis
Low risk
High risk
Effusions
Less prolonged
Prolonged
Early mortality
2%-5%
Similar
20-yr. survival
82%
Similar
STS Congenital Heart Surgery Database: 2,747 Fontan
operations (2000 to 2009) in 68 centers
A right-dominant ventricle 45%.
Extracardiac conduit Fontan vs lateral tunnel)was performed
in 63%; in all, 65% were fenestrated.
In multivariable analysis the extracardiac conduit Fontan was
associated with significantly higher Fontan
takedown/revision and Fontan failure and longer
postoperative hospital stay.
 Conclusions: The lateral atrial tunnel Fontan may be
associated with superior early outcomes.
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Constantine Mavroudis, Marshall L. Jacobs, et al January 3, 2012.
Age above 4 years
 Normal ventricular function
 Adequate pulmonary artery size
 No distortion of pulmonary arteries from prior shunt surgery
 Low pulmonary artery pressure (below 15 mmHg)
 Low pulmonary vascular resistance
 Normal systemic venous drainage
 No atrioventricular valve leak
 Normal heart rhythm
 No right atrial enlargement
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Negative prognostic factors for both early and late outcome:
Ventricular function
Pulmonary artery pressure
William J. Brawn.Circulation. 2001
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Three groups of PA sizes using the PA index (PAI)
Hypoplastic PA (<180 mm2/m2)=impedance increase
especially at 100mm2/m2
Relatively small PA (180-250 mm2/m2)
Good PA (250mm2/m2)
Jae Suk Baek et al. Ann Thorac Surg 2011
ACC/AHA 2008 GUIDELINES GUCH
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Recent 15-year survival reports post-Fontan:
85% was recently reported by the Boston Children’s
Hospital , USA
82 ± 3% recently reported by Birmingham Children’s
Hospital, UK
Preoperatively impaired ventricular function and elevated
pulmonary artery pressures have an adverse influence on
both early and late outcome.
Long-term viability after the creation of a Fontan circulatory
arrangement is most dependent on maintenance of sinus
rhythm, the behavior of the pulmonary vasculature, and
the performance characteristics of the ventricle
Alvin J. Chin et al. World Journal for Pediatric and Congenital Heart Surgery 2010
Riad B.M. Hoseina, William J. Brawn J ThoracCardiovascSurg 2012
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Norwood 1 operation (n=9):100% early mortality rate
Glenn shunts (n=128): 5.4% mortality rate
HemiFontan (n=11): 100% early midterm survival rate
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BTS to Fontan operation (n=6) : 17%
Glenn to Fontan operation (n=9): 25%
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Modified Fontan operation (n=36): 5.5% mortality rate
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A 15 year follow-up on somatic growth after Fontan
operation:
 Body weight and BMI have significantly improved by 1 year
after the operation.
 Height has significantly improved by 2 years postoperatively.
 Patients with BDG shunts showed significantly better weight
and BMI at the time of the Fontan operation compared with
those without prior BDG shunts .
 Fenestration is beneficial suggesting that mild arterial
desaturation is well tolerated and permits catch-up growth.
 The pace of catch-up growth after the Fontan operation is
much slower than that after biventricular repair.
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Masamichi Ono et al. J Thorac Cardiovasc Surg 2007
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Oxygen delivery index, at least through their preadolescent
years, was normal or near normal to reach a relatively
normal height.
Alvin J. Chin et al. World Journal for Pediatric and Congenital Heart Surgery 2010
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Physical and Psychosocial Summary scores of the Child
Health Questionnaire Parent Form were within the normal
range for over 80% of patients.
Carl Lewis Backer.J. Am. Coll. Cardiol. 2008
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Greater differences between parent and child-completed
scores for the domains of physical functioning and impact of
physical limitations.
Children may “adapt” better to their situation and rate the
impact of the illness to be better.
Recommendation: Clinicians should consider both parent
and patient viewpoints when counseling patients.
Parent- Versus Child-Reported Functional Health Status After the Fontan Procedure
Linda M. Lambert et al. Pediatrics2009
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Finding of apparently impaired systolic function in the RV
subgroup relative to the LV and mixed subgroups is
consistent with the general opinion that the structure of the
RV is suboptimal for a systemic ventricle.
The tricuspid valve is more likely to fail as a systemic AV
valve.
Page A. W. Anderson, MD, et al. J Am CollCardiol 2008
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Arrhythmia occur in 10-40% of patients regardless of the
type of Fontan.
Cardiac rhythm is important in this circulation:
Loss of atrio-ventricular synchronisation will cause an
increase of the pulmonary venous atrial pressure and/or
a diminished ventricular preload,
Both of which are known to have negative effects on a
Fontan circuit.
Marc Gewilliga et al. European Association for Cardio-Thoracic Surgery 2010.
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Ventricular tachycardia was detected 15 years post surgery,
especially in older patients with older age at Fontan
operation.
Yuki Nakamura, MD .J Thorac Cardiovasc
Surg 2010
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Lymphatic circulation may be affected by high venous
pressure and impaired thoracic duct drainage.
Increased pulmonary lymphatic pressure may result in
interstitial pulmonary edema, lymphedema &/or protein
losing enteropathy.
Protein losing enteropathy is characterized by excessive loss
of proteins from serum into the intestinal lumen with
mesenteric vascular inflammation.
Manifestations include oedema, immunodeficiency, ascites,
malabsorption of fat, hypercoagulopathy, hypocalcaemia,
and hypomagnesaemia.
SandeepNayak , PD Booker MBBS MD FRCA
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During a 10-yr follow-up, PLE incidence is about 13%.
Poor prognosis (60% 5-yr and 20% 10-yr survival after
diagnosis).
Treatment: Diet low in salt and high in calories, protein
content, and medium chain triglycerides.
Diuretics, corticosteroids, heparin, and
octreotide (a somatostatin analogue)
Fontan fenestration; Fontan takedown or
revision; or cardiac transplantation.
SandeepNayak , PD Booker MBBS MD FRCA
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Plastic bronchitis : less than 1%–2% of patients.
Noninflammatory mucinous casts that form in the
tracheobronchial tree and obstruct the airway.
Related to the increased enteric loss of alpha-1-antitrypsin
Alvin J. Chin et al. World Journal for Pediatric and Congenital Heart Surgery 2010
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Clinical manifestations are dyspnea, cough, wheezing, and
expectoration of casts, which may cause asphyxia, cardiac
arrest, or death.
Medical management is difficult; often require repeat
bronchoscopy to remove the thick casts.
Treatment: Fontan fenestration; Fontan takedown or
revision or transplantation.
Tyler B. Fredenburg, Mervyn D. Cohen, MBChB .Ann Thorac Surg 2005
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Circulating ‘‘hepatic factors,’’
which are responsible for either
stimulating a repressor of
pulmonary arteriovenous
malformations (PAVMs) or
repressing an activator of PAVMs.
Alvin J. Chin et al. World Journal for Pediatric and
Congenital Heart Surgery 2010
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The ventricle evolves from being volume overloaded and
overstretched, to overgrown and (severely)underloaded.
The deprived ventricle in a Fontan circuit shows systolic and
diastolic dysfunction.
The ventricle may now enter a vicious cycle whereby the low
preload results in remodelling, reduced compliance, poor
ventricular filling, and eventually continuously declining
cardiac output.
This phenomenon of progressive ‘‘disuse hypofunction’’
occurs at a chronic preload of less than 70% of the ‘‘due’’
preload.
Marc Gewillig. Heart 2005
Freedom from failure at 10 years was 99.2% for the
Fontan group and 67.4% for the BDG patients.
Jenkins KJ.Eur J Cardiothorac Surg 2010
 The mean age at the time of failure symptoms 17.2 ±
6.3 years.
 The indications for transplantation was protein-losing
enteropathy, arrhythmia with ventricular dysfunction ,
and heart failure
Paolo Ferrazzi. Ann Thorac Surg 2001
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Staged vs. Primary Fontan operation (or total
cavopulmonary connection)
Antenatal diagnosis
Age
Fenestrated vs. non-fenestrated Fontan
Arrhythmia
Anticoagulation
Cognitive, psychosocial and somatic development
Pregnancy
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SVC and IVC to PA connections originally were
performed at the same time, which in many patients
resulted in a marked increase in blood flow to the lungs,
pulmonary lymphatic congestion, and prolonged
problems due to pleural effusions.
Currently, surgeons create total cavopulmonary Fontan
circulation in at least two stages to allow the patient's
body to adapt to the different hemodynamic states and
reduce overall surgical morbidity and mortality.
Tyler B. Fredenburg,Mervyn D. Cohen, MBChB. Ann Thorac Surg 2005
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Better outcome for staged TCPC because of the gradual
ventricular unloading and remodelling.
An excessive acute volume load reduction gives rise to a fatal
afterload mismatch.
Cardiovascular deaths and heart transplantation occurred
less frequently when the Fontan procedure was performed in
patients with a previous bidirectional Glenn/ hemi-Fontan
procedure.
Doty . Int J Cardiol,2006
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Advantages of staging towards Fontan operation are almost
universally accepted.
Yorikazu Harada et al. Interactive CardioVascular and Thoracic Surgery 2009
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One-stage modified Fontan in low-risk patients (n=15): at
least 4 years for the lateral tunnel, and older than 6 years
for the extracardiac conduit; McGoon ratio > 1.8 or PA index
> 200, low PA pressure (< 15 mm Hg), and no AVVR.
2-stage procedure for high-risk patients (n=13).
Result: 4 hospital deaths in the 1-stage group (operative
mortality, 26.6%)
No death occurred in the 2-stage group ( p < 0.05).
Three of the 4 deaths were due to elevated CVP, despite
preoperative cardiac catheterization demonstrating mean
PA pressure < 20 mm Hg.
O MonteinNgodngamthaweesuk et al. Asian CardiovascThorac Ann 2007
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The 1-stage modified Fontan procedure may put patients at
higher surgical risk due to poor cardiovascular adaptation.
O MonteinNgodngamthaweesuk et al. Asian CardiovascThorac Ann 2007
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An intermediate Glenn shunt and lower postoperative
pulmonary artery pressure as significant predictors of better
outcome.
Nelson Alphonso, David Anderson
Presented at the joint 17th Annual Meeting of the European Associationfor Cardio-thoracic Surgery and the 11th Annual
Meeting of the EuropeanSociety of Thoracic Surgeons, Vienna, Austria, October 12-15, 2003.
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The reported impact of prenatal diagnosis on overall
survival is variable
Infants diagnosed prenatally are certainly less likely to
experience the sequelae of pulmonary overcirculation or
acidosis.
Prenatal diagnosis is associated with
improved neurological outcome and with a lower
incidence of preoperative acidosis and ventricular
dysfunction, and the need for less inotropic support.
U Theilen, L Shekerdemian. Arch Dis Child Fetal Neonatal Ed 2005
< 7 yrs old
> 7 yrs old
Operative mortality
Similar
5.4%
Ventricular dysfunction
Late
Early
Arrythmia
Similar
Similar
Arteriovenous
malformation
Rare
More frequent
Carlo Pace Napoleone Eur JCardiothorac Surg 2010
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All patients see a progressive decline in their exercise
capacity, but the decrease in exercise capacity was
accelerated if the Fontan surgery was performed in
adolescents and adults rather than in children.
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In a serial angiographic study comparing Fontan
surgerybefore and after the age of 3 years, it was
demonstrated that the cardiac index was preserved if
Fontan surgery took place before 3 years of age, and
progressively deteriorated if performed at a later age.
Yves d’Udekem, Eur J Cardiothorac Surg 2011
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Fifteen patients underwent TCPC completion: extracardiac
conduit (n = 13), lateral tunnel (n =1), and direct anastomosis
of the inferior vena cava and pulmonary trunk (n = 1)
The mean age at operation was 27 +/- 9 years (range, 16-52).
The mean follow-up period was 57 +/- 45 months (0 to 154).
All patients had at least 2 risk factors (2 to 8).
Mean pulmonary artery pressure of 20 mm Hg or higher is
their only contraindication to TCPC .
Mean PVR of 4.0 wood units, pulmonary arterial index of 150,
and ejection fraction of 40% are usually acceptable.
Midterm to Long-Term Outcome of TCPC in High-Risk Adult Candidates
Shunji Sano, MD, et al . Ann Thorac Surg 2009
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Overall mortality rate was 13.3%.
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Conclusion: To improve quality of life and long-term
prognosis, TCPC completion should be performed as soon as
possible, and TCPC conversion should be performed at an
early stage of the morbidities.
: TCPC could be offered to high-risk adult
Fontan candidates, with an acceptable mortality rate.
Midterm to Long-Term Outcome of TCPC in High-Risk Adult Candidates
Shunji Sano, MD, et al . Ann Thorac Surg 2009
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Long-standing cyanosis and volume overload to the
ventricle are believed to cause progressive ventricular
fibrosis by inducing histopathologic changes, which must
result in both systolic and diastolic dysfunction.
The ventricular EF and cardiac indexes were significantly
higher at 5 and 10 years after the Fontan procedure had been
completed in younger patients.
Earlier elimination of hypoxia allows better growth of
respiratory and skeletal muscles, which would subsequently
increase the cardiac index and exercise capacity, especially
at 5 and 10 years after the Fontan procedure.
Shuichi Shiraishi et al
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Only 13% to 16% of patients who underwent Fontan at 3
years of age had moderate to severe AV valve
regurgitation, versus 23% to 26% who underwent Fontan
at 3 years of age (p 0.01) due to prolonged volume
overloading in the patients who were older at Fontan.
Conclusion: Support current trends toward primary Fontan
at an early age.
Carl Lewis Backer, MD, FACC . J. Am. Coll. Cardiol. 2008;52;114-116
ESC GUIDELINES ON GUCH 201O
 Indications: Small pulmonary arteries
Borderline CVP 15 mmHg
Poor ventricular function (EF <50%)
Moderate-to-severe AVVR
Adult patients
HLHS
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Advantages: Improve cardiac output
Decrease venous pressure (lessen effusions and
Protein-losing enteropathy)
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Disadvantages: Desaturation
Paradoxical embolization
Strategy of closure: Transcatheter device
John W. Brown et al Ann ThoracSurg2010
Indications for fenestration closure
 Persistent O2 sat’n of <90%
 Passed the occlusion test:
Does not experience a fall in blood pressure or mixed
venous saturation ,
No rise in right atrial pressure >20 mmHg
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Jenkins KJ.Eur J Cardiothorac Surg 2010
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After closure of Fontan fenestration: No change in peak
exercise capacity as demonstrated by percent of predicted
VO2, percent predicted ventilatory anaerobic threshold,
heart rate, or O2 pulse.
This is despite a 12% increase in O2 saturation at peak
exercise.
Finding demonstrates that exercise tolerance in the Fontan
patient is limited by peak blood flow in the pulmonary
vascular bed, which is passively perfused by systemic venous
pressure.
Carl Lewis Backer, MD, FACC. J. Am. Coll. Cardiol. 2008;52;114-116
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All patients with clinical thromboembolic events were
taking warfarin at the time of the event.
Warfarin (INR 1.5 - 2.0.)
Aspirin: daily antiplatelet therapy for most Fontan
patients
Welton M. Gersony, MD Circulation 2008
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Warfarin + Aspirin in higher risk: RA-PA connection,
external conduit, sluggish venous circulation, or low
cardiac output, history of thromboembolism.
Yves d’Udekem .Eur J Cardiothorac Surg 2007
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Most individual patients palliated with the Fontan procedure
in the 1970s and 1980s have cognitive outcome and
academic function within the normal range, but the
performance of the cohort is lower than that of the general
population.
Jonas RA, et al.Ann Thorac Surg 2011
By 20–28 weeks gestation, myocardial oxygen
consumption and heart rate normally increase by 20%
and stroke volume by 40%
The physiological increase in blood volume result in atrial
distension.
 Left uterine displacement help avoid sudden
hypotension
 Although women can successfully complete pregnancy
with a Fontan circulation, the associated physiological
haemodynamic changes have a significant influence on
their abnormal heart function.
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SandeepNayak , PD Booker MBBS MD FRCA
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Women can successfully complete pregnancy after
adequate Fontan palliation without important longterm sequelae, although it is often complicated by
clinically significant (non-)cardiac events. I
Subfertility or infertility and menstrual disorders were
common.
W Drenthen et al. Heart 2006
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In the absence of Eisenmenger physiology, pregnancy in the
context of cyanotic heart disease has been associated with
30% incidence of maternal cardiovascular
complications and prematurity.
Paul Khairy, MD, PhD. Circulation 2007
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Pregnancy is contraindicated in patients with severely
reduced pulmonary blood flow or with severe pulmonary
vascular disease (Eisenmenger syndrome) or if ventricular
function is poor.
Cyanosis poses a significant risk to the foetus, with a live
birth unlikely (12%) if oxygen saturation is <85%.
ESC Guidelines on GUCH 201o. European Heart Journal
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Early staged operation:
mBTs for diminutive right-sided structures
Norwood operation for diminutive left-sided structures
Pulmonary artery band for pulmonary overcirculation
No procedure in early infancy for balanced circulations.
BDG/HemiFontan at 6 months of age
Total cavopulmonary connection at >2 yrs old
At least on aspirin
ACE inhibitor for signs of ventricular dysfunction
Aggressive repair of AV valve regurgitation
Fenestration of Fontan circuit for high-risk candidates
Counselling and close follow-up.
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The average 80% twenty-year survival rate of
Fontan operation is based on a mix of
patients who had their procedures done
before the advent of and after the TCPC,
hence, the outcome of current surgical trends
could be better.
The Univentricular repair, however, remains a
challenge to the entire perioperative team.
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In 1971, Fontan and Baudet described an atriopulmonary anastomosis as definitive
palliation for tricuspid atresia.
Mortality rates with the classic atrial pulmonary connection approximated 15% to 25%.
It was originally thought that the right atrium, a pulsatile chamber, would improve
pulmonary blood flow. However, in a series of experiments by de Leval et al , it was
discovered that the right atrium dilated and then lost contractile function, which resulted in
turbulence and energy loss and actually decreased pulmonary blood flow
Right Atrium with Classic Fontan Circulation Patients with atriopulmonary Fontan
circulation are predisposed to development of complications. The right atrium is exposed
to elevated systemic and right atrial pressure, which leads to right atrial dilatation and
hypertrophy (Fig 11). Dilatation may be severe, and it may lead to complications such as
arrhythmia and swirling of blood in the enlarged atrium, which causes stasis and results in
poor blood flow to the lungs. Dilatation also may be a predisposing factor for clot
formation.Secondary dilatation of the coronary sinus also is seen.
Total cavopulmonary connections have the advantage of saving energy compared with the
original RA-PA connections,eliminating less effective blood flow via a large, turbulent
atrium that sends blood directly to the pulmonary artery.
Welton M. Gersony, MD Circulation 2008
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In 1988, de Leval and colleagues proposed total cavopulmonary connection by a lateral
tunnel as an alternative to the Fontan-type operation with hemodynamic advantages and
reduced atrial complications.
1990 when Marcelletti and colleagues described total cavopulmonary connection using an
extracardiac conduit from the inferior vena cava to the PA. This has beneficial
hemodynamic effects on systemic venous flow, while the entire atrium is left at low
pressure with minimal atrial sutures.
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CXR: assess lung disease
ECG: rhythm
Echocardiography: systemic valvular status,
ventricular function, central pulmonary
arterial anatomy ,PAP, SVC and IVC anatomy
MRI: pulmonary arterial anatomy and flows,
collateral circulation, other data to confirm
echocardiographic questions
Cardiac cath: pulmonary arterial anatomy,
PVR
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Although echocardiography is widely used in patients with
single-ventricle physiology, its ability to comprehensively
image all the relevant anatomy, particularly the thoracic
vasculature, may be limited in some patients.
Four patients in the catheterization group (10%) had findings
that were not appreciated at the initial procedure, including
a large systemic venovenous collateral vessel, a diffusely
small left pulmonary artery requiring intraoperative
arterioplasty, a RV aneurysm at a shunt insertion site, and an
abscess around a Blalock-Taussig shunt.
No new intraoperative or postop. findings in the CMR group.
In the absence of evidence of pulmonary hypertension,
routine measurement of PVR is not necessary before BDG.
Pedro J. del Nido, MD et alCirculation. 2007
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Age
The Fontan operation in adults has
acceptable early and late mortality.
Functional class, systolic ventricular function,
atrioventricular regurgitation, and
arrhythmia deteriorate late after surgery but
to a lesser degree than in non-Fontan
patients with a single ventricle.
Veldtman et al. Heart 2001
 All patients with a Fontan circulation have an
abnormal cardiorespiratory response to exercise.
 They have a blunted heart rate response, and
 Limited ability to increase stroke volume with
exercise, due to impaired ventricular function and
difficulty in increasing ventricular preload.
SandeepNayak, PD Booker MBBS MD FRCA
Functional health status: Over 80% of subjects scored in
the normal range on the CHQ.
 Parents perceived their children as having lower physical and
psychosocial functional status.
 The lower Physical Summary scores are similar to those for
children who have undergone thoracic organ transplantation
or cardioverter defibrillator implantation
 Exercise performance. Maximal exercise performance was
lower than normal and worse in older subjects due to:
 absence of a subpulmonary pumping chamber, abnormal
endothelial cell function, increased PVR and SVR, decreased
muscle mass, & deconditioning
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Page A. W. Anderson, MD, et al. J Am CollCardiol 2008
Augment Preload:
Increase systemic venous return: Low intrathoracic
pressure (low respiratory rates, short inspiratory times,
low PEEP, and tidal volumes of 5–6 ml kg, normocarbia,
and a low PVR).
Normovolemia
 Decrease afterload:
Vasodilators
 Hyperventilation tends to impair pulmonary blood flow,
despite the induced respiratory alkalosis, because of the
increased mean intrathoracic pressure.

SandeepNayak,PD Booker MBBS MD FRCA

Arrhythmia incidence is similar between EC & LT due to:
Right atrial dilatation
Sinus node dysfunction

High risk to develop arrhythmia:
ventricular dysfunction, bilateral superior venae cavae, and
heterotaxy syndrome

Management: Surgical Maze operation
Catheter ablation

Due to the absence of pulsatile blood flow and underfilling of
the pulmonary vascular bed, patients with Fontan circulation
are at increased risk for formation of pulmonary
arteriovenous malformations.
Tyler B. Fredenburg, MD, Mervyn D. Cohen, MBChB . Ann Thorac Surg 2005
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Effect of residual shunts: volume overload on the ventricle
and may induce an irreversible increase in PVR secondary to
high regional pulmonary blood flow.
Sources of shunts: Fenestration, drainage of coronary sinus
aorta-pulmonary collaterals & incomplete occlusion of
previous artificial shunts.
SandeepNayak , PD Booker MBBS MD FRCA
Typically, cardiac output in a Fontan circulation at rest is
decreased to 70% (range 50–80%) of normal for body surface
area.
 Preload to the ventricle is determined by transpulmonary
flow (PVR) and a fenestration if present.
 Since the 1990s when excessive volume overloading and
acute unloading have been avoided, ventricular dysfunction
has become a less important risk factor.
 A decrease of afterload without preload reserve will not
result in increase of output, but may cause hypotension.
 Excessive afterload on the other hand may be
detrimental,especially in the systemic RV.

Marc Gewilliga, European Association for Cardio-Thoracic Surgery. 2010.

Decreasing age, as well as intervals, in staged Fontan
palliation have beneficial influence on major complications
and outcome.
K Francois, M Tamim, T Bove, PediatrCardiol, July 1, 2005.
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Subjects who were older at time of Fontan had worse AV
valve function and decreased likelihood of
being in sinus rhythm.
Poorer valve function and a decrease in sinus rhythm might
be related to a longer period of volume overloading .
Recommendation: To complete the Fontan at an earlier age.
Page A. W. Anderson, MD, et al. J Am CollCardiol 2008
Indications to leave a pulmonary
antegrade flow:
Low pre-BDG PA pressure
Borderline sizes of the native Pas
 Definitive palliation for high
risk patients for Fontan.
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Davide F. Calvaruso, et al Ann Thorac Surg 2008
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Those without APBF did not enjoy the
same degree of PA growth as those
with APBF. These differences did not
correlate with appreciable differences
in clinical outcome after BDG or
Fontan.
Robert G. Gray, et al. Ann ThoracSurg 2007
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Advantages :
Hepatic flow & pulsatile hemodynamics are maintained that
may decrease the tendency for collaterals/AVMs
Preserves the ability to catheterize the pulmonary
arteries from the femoral veins.
Disadvantages :
Excessive pulmonary flow
Elevated pressures resulting in persistent effusions
Brian E. Kogon et al.
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The incidence of clinical superior vena caval syndrome
did not differ between groups with or without antegrade
flow.
Robert G. Gray, et al. Ann ThoracSurg 2007
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
Kawashima procedure
results in a ‘near complete’
Fontan circulation
The Spo2 early after postKawashima Fontan was
not as high as after control
Fontan in most cases, but
often improved
throughout the
postoperative follow-up.
Shelby Kutty MD, James S. Tweddell, MD
Ann ThoracSurg 2010
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Early redirection of hepatic flow after the Kawashima
procedure may actually decrease or result in resolution of
PAVMs.
The resolution of hypoxemia after incorporation of the
hepatic veins into the cavopulmonary circuit is likely due to a
combination of PAVM resolution and elimination of hepatic
venoatrial right-to-left shunting.
Extracardiac or lateral tunnel connection are the most
common methods for completion Fontan
Comparative survival rates after Fontan procedure in
heterotaxy and nonheterotaxy patients have been similar.
Shelby Kutty MD, James S. Tweddell, MD Ann ThoracSurg 2010