1. Fontan operation: How I do it?
Piya Samankatiwat
Cardiothoracic Unit
Ramathibodi hospital

2. 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.

3. 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

4. Univentricular heart
Congenital heart surgery nomenclature and database
Common inlet atrioventricular connection
DILV
DIRV
Absence of one atrioventricular connection
Tricuspid atresia
Mitral atresia
Common atrioventricular valve with one well- developed ventricle (unbalanced AVSD)
Heterotaxia syndrome with only one fully developed ventricle
Other rare forms of univentricular heart

5. R Kaulitz, M Hofbeck. Arch Dis Child 2005;90:757–762.

6. 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

7. 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

8. First report in Thorax 1971 by F Fontan and E Baudet

11. Ten commandments (Fontan and Baudet)
Age above 4 years
No distortion of pulmonary arteries from prior shunt 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

12. 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???

13. Principle of surgery for UVH
Protect the lung: prevent pulmonary HT with PA banding
Correct cyanosis: BT shunt
Atrial septostomy/septectomy
Relieve ventricular outflow tract obstruction
Correct obstruction of pulmonary venous connection
Correct valvular regurgitation

14. 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

15. Palliative procedures
Pulmonary artery banding (PAB)
Source:

16. Blalock-Taussig shunt

17. DKS and Stage-I Norwood procedure

18. 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

19. Special considerations for Glenn
Additional sources of pulmonary blood flow
Bilateral BDG
Kawashima operation
Hemi-Fontan procedure

20. Cavopulmonary shunt (Glenn)
Source:

22. 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

23. Palliative definitive surgery: Fontan procedure
Total cavopulmonary connection
Fontan: IVC to PA
Extracardiac conduit (ECC)
Lateral tunnel (intracardiac baffle, LT)

24. Types of TCPC
R Kaulitz, M Hofbeck. Arch Dis Child 2005;90:757–762.

26. Fontan operation

27. Extracardiac conduit

28. 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

29. Fontan procedure I

30. Fontan procedure II

31. Fenestration and completion

32. Anomalous systemic venous return

33. 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

34. Advantage of fenestration
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;

35. Factors associated with poor outcome
Systemic ventricular dysfunction (EDP > 12)
Increased pulmonary arterial pressure (mean > 15)
Increased pulmonary vascular resistance (>2)
Atrioventricular valve regurgitation
Distorted pulmonary artery
Anomalous pulmonary venous connection

36. 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)

37. Sequelae of Fontan procedure
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

38. Ramathibodi’s experience on surgical treatment of UVH
Between : 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

39. Ramathibodi’s experience on surgical treatment of UVH
Operations N
Results
Glenn procedure 32
All alive
Inferior cavo-pulmonary connection 3
Completion of TCPC
19 (S= 2/19)
Dead 2
Kawashima operation 2
Alive
Other palliative procedures
Systemic to pulmonary shunt
CoA repair +/- PAB 6
Late dead 1
PA plasty or reconnection 8

41. 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

43. 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

47. Operation and findings
Severe coarctation of the aorta and hypoplastic aortic arch
Hypoplastic LV
Deep hypothermia + circulatory arrest
Resection and end-to-end anastomosis
Pulmonary artery banding

49. Case II 1-y-2-mo-old boy Cyanotic since birth
Systolic ejection murmur gr 4/6 at LPSB

51. Investigation
Echocardiography truncus arteriosus type I with moderate truncal valve regurgitation Cardiac catheterisation truncus arteriosus type I with PHT PVR 6 Woods unit

58. 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)