Transcript Univentricular heart and Fontan principle

FONTAN OPERATION:
HOW I DO IT?
Piya Samankatiwat
Cardiothoracic Unit
Ramathibodi hospital
What is the ‘single ventricle’?
 Prof. Richard van Praagh
 ‘One ventricular chamber receives blood from
both tricuspid and mitral valve or a common
atrioventricular valve’
 Therefore, this excludes tricuspid atresia and
mitral atresia.
So !!! you are in doubt what the
univentricular physiology is.
 Prof. Robert H Anderson emphasised that
 ‘the entire atrioventricular junction is connected
to only one chamber in the ventricular mass’
 DILV
 Absent of one atrioventricular connection
 Common AV valve with one completely well-developed
ventricule
 Only one fully developed ventricle
 Heterotaxy syndrome
 Rare miscellaneous forms
Univentricular heart
Congenital heart surgery nomenclature and database
Common inlet atrioventricular connection
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Absence of one atrioventricular connection
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DILV
DIRV
Tricuspid atresia
Mitral atresia
Common atrioventricular valve with one welldeveloped ventricle (unbalanced AVSD)
Heterotaxia syndrome with only one fully developed
ventricle
Other rare forms of univentricular heart
R Kaulitz, M Hofbeck. Arch Dis Child 2005;90:757–762.
Evolution of surgery for single
functioning ventricle
 1940s Blalock-Taussig shunt
 1952 PA banding by Muller WH and
Dammann JF
 1954 Shumacker and 1st cavopulmonary
connection
 1956 separation of single ventricle at Mayo
Clinics
 1971 Fontan’s and Kreutzer’s
Fontan procedure
Background
 1971 Fontan and Baudet first introduced a
new procedure for repair of tricuspid atresia
in clinical practice
 1970s to 1980s became widely accepted as
the palliative procedure of choice in
univentricular heart physiology
 Physiological, not anatomical correction
First report in Thorax 1971
by F Fontan and E Baudet
Ten commandments
(Fontan and Baudet)
 Age above 4 years
 No distortion of pulmonary arteries from prior shunt
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surgery
Normal systemic venous drainage
Normal ventricular function
Adequate pulmonary artery size
No atrio-ventricular valve regugitation
Low pulmonary artery pressure (below 15 mmHg)
Low PVR
Normal sinus rhythm
Adequate size of right atrium
Candidate for Fontan
 Body size
 Systemic ventricular function
End diastolic pressure < 10 mmHg
 Mean PAP < 15 mmHg
 PVR < 2.5 Woods unit (2)
 Unrepairable pulmonary
artery distortion ???
Abnormal IVC course: proceed to
Fontan operation???
Principle of surgery for UVH
 Protect the lung: prevent pulmonary HT with
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PA banding
Correct cyanosis: BT shunt
Atrial septostomy/septectomy
Relieve ventricular outflow tract obstruction
Correct obstruction of pulmonary venous
connection
Correct valvular regurgitation
Operations: pre-Fontan palliation
 Pulmoanry artery banding
 Modified BT shunt
 Atrioventricular valve repair
 Correction of TAPVC (if present)
 DKS or Norwood principle
 Glenn’s shunt: superior cavopulmonary
connection
Palliative procedures
Pulmonary artery banding (PAB)
Source: www.ucch.org
Blalock-Taussig shunt
DKS and Stage-I Norwood procedure
Glenn procedure
 SVC to pulmonary artery
 Patient selection: good candidate
 Pulmonary arterial pressure (PAP) mean < 20
mmHg
 Size of branch pulmonary arteries
 Preparation
 Bypass circuit: aortic/ SVC –RA venous
cannulation
Special considerations for Glenn
 Additional sources of pulmonary blood flow
 Bilateral BDG
 Kawashima operation
 Hemi-Fontan procedure
Cavopulmonary shunt (Glenn)
Source: www.ucch.org
Glenn shunt
Surgical technique: How I do it?
 With/without CPB
 CPB: aortic cannulation and SVC-RA venous
cannulation
 Essential steps:
 Extensive mobilisation of the SVC
 Cannulate the SVC at the SVC-innominate junction
 Keep azygos vein intact until starting the SVC-RPA
anatomosis
 Limited PA dissection – no need for extensive
dissection
Palliative definitive surgery:
Fontan procedure
 Total cavopulmonary connection
 Fontan: IVC to PA
 Extracardiac conduit (ECC)
 Lateral tunnel (intracardiac baffle, LT)
Types of TCPC
R Kaulitz, M Hofbeck. Arch Dis Child 2005;90:757–762.
Fontan operation
Extracardiac conduit
Completion of TCPC
Surgical technique: How I do it?
 On CPB or Off-pump
 Dissection of IVC and PA
 Tailoring the PTFE conduit (at least size 16
mm should be selected for adequate IVC flow)
 Anastomosis: the IVC anastomosis is done
first.
 Side-to-side fenestration
Fontan procedure I
Fontan procedure II
Fenestration and completion
Anomalous systemic venous return
Challenges
Anatomical and technical
 Branch pulmonary artery stenosis and
disconnected pulmonary artery
 Dextrocardia and Mesocardia
 Abnormal anatomy or position of the IVC
 Fenestration or non-fenestration
Physiological
 Pulmonary hypertension
Advantage of fenestration
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Debate on routine fenestration
Beneficial in high-risk Fontan
Standard-risk group  controversial
Randomised-controlled trial
49 cases underwent Fontan (< 2 risk factors)
25 fenestrated, 24 non-fenestrated
6 LT, 43 ECC
Fenestrated group: less hospital stay, shorter
duration of pleural drainage and less additional
procedures
Lemler MS, Scott WA, Leonard SR, et al.
Circulation 2002; 105; 207-212
Factors associated with
poor outcome
 Systemic ventricular dysfunction (EDP > 12)
 Increased pulmonary arterial pressure (mean
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> 15)
Increased pulmonary vascular resistance (>2)
Atrioventricular valve regurgitation
Distorted pulmonary artery
Anomalous pulmonary venous connection
Postoperative care
Cardiac output depends primarily on
pulmonary vascular resistance (PVR) and
systemic ventricular function
 Positioning
 Extubation as early as possible
 Inotropes
 Vasodilators
 Reduction of pulmonary arterial pressure
 Anticoagulation: warfarin (how long???)
 Only aspirin: enough??? Ann Thorac Surg. 2002
Jan;73(1):64-8. (St Christopher Hospital, Philadelphia)
Sequelae of Fontan procedure
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Low cardiac output
Pleural effusion
Arrhythmia
Protein losing enteropathy (PLE)  immunity loss
Hepatic dysfunction
Pulmonary arteriovenous malformation (AVM) in
Kawashima
 Progressive cyanosis
 Chronic exercise intolerance
 Plastic bronchitis
Ramathibodi’s experience on
surgical treatment of UVH
Between 2007-2010: 64 operations in 52 patients
Defects
N
Double inlet ventricle:
• DILV
• DIRV
6
1
Absence of one AV connection
• TA
• MA
14
3
Unbalanced AVSD
5
Heterotaxia syndrome
• Asplenia
11
Miscellaneous
• PA/IVS
• Complex DORV
• Other rare conditions (e.g. hypoplastic LV & AA ,
hypoplastic RV & TGA, dextrocardia/PS/common ventricle)
4
3
5
Ramathibodi’s experience on
surgical treatment of UVH
Operations
N
Results
Glenn procedure
32
All alive
Inferior cavo-pulmonary connection
3
All alive
19 (S= 2/19)
Dead 2
Kawashima operation
2
Alive
Other palliative procedures
•Systemic to pulmonary shunt
•CoA repair +/- PAB
6
2
Late dead 1
All alive
PA plasty or reconnection
8
Dead 2
Completion of TCPC
Case discussion: case I
 A male premature baby, birth weight 2.1 kg
 Respiratory distress with heart murmur
 No dysmorphic feature
 Normal S1, S2 with SEM gr 2/6 at LPSB
Investigation
 Echocardiography
Single functioning morphologic RV, DIRV,
DORV, rudimentary LV, large bulboventricular
foramen
Mild MS,No AS, No PS
hypoplastic aortic arch 3 mm, severe CoA 2.5
mm, PDA 3.3 mm
 CTA heart
Operation and findings
 Severe coarctation of the aorta and
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hypoplastic aortic arch
Hypoplastic LV
Deep hypothermia + circulatory arrest
Resection and end-to-end anastomosis
Pulmonary artery banding
Case II
 1-y-2-mo-old boy
 Cyanotic since birth
 Systolic ejection murmur gr 4/6 at LPSB
Investigation
Echocardiography
truncus arteriosus type I with moderate
truncal valve regurgitation
Cardiac catheterisation
truncus arteriosus type I with PHT
PVR 6 Woods unit
Findings and procedure
 Truncus type I, right sided aortic arch
 RPA & LPA 10 mm
 Tricuspid truncal valve with regurgitation due
to redundancy of posterior truncal cusp
Rastelli operation (using 13 mm pulmonary
homograft)