1. Total Cavo-pulmonary Connection
SNU Children’s Hospital
Total Cavo-pulmonary Connection
Yong Jin Kim, M.D.
Department of Thoracic & Cardiovascular Surgery
Seoul National University Hospital
Seoul , Korea
TCPC

2. SNU Children’s Hospital
Basic Concepts
Single ventricle physiology
1. Separation the systemic and pulmonary venous return
2. Establishment of the pathway of a passive, direct, and
unobstructed connection between the systemic venous
return and the pulmonary artery
TCPC

3. Historical Context of Fontan Operation
SNU Children’s Hospital
Historical Context of Fontan Operation
1971 Fontan Fontan operation
1973 Kreuzer Modified Fontan op.(APC)
1979 Bjork RA-RV connection
1984 Kawashima TCPS
1988 de Leval TCPC with lateral tunnel
1990 Bridges Fenestrated Fontan op.
1990 Giannico Extra-cardiac Fontan op.
TCPC

4. As a Result Fontan operation Technical modifications and advances
SNU Children’s Hospital
As a Result
Fontan operation
in 1971, Fontan et al, for tricuspid atresia
Technical modifications and advances
Better understanding of this physiology
Improvement of pre and postoperative management
For a wide spectrum of complex
congenital heart disease
TCPC

5. Atriopulmonary Connection
SNU Children’s Hospital
Atriopulmonary Connection
Fontan & Baudet. 1971
Physiologic correction
Glenn shunt
RA-RPA with homograft
ASD closure
IVC-RA with homograft
MPA ligation
TCPC

6. Intra-cardiac Lateral Tunnel
SNU Children’s Hospital
Intra-cardiac Lateral Tunnel
de Leval et al. 1988
Atrial partitioning with PTFE
or Dacron tube graft
Creating a straight pathway
: laminar flow without turbulence
& energy loss
TCPC

7. SNU Children’s Hospital
Extra-cardiac Lateral tunnel
Giannico et al. 1990
Using Gore-Tex tube graft
Concomitant PA enlargement with conduit patch
Shunt formation
Between conduit and RA
With Gore-Tex tube graft
TCPC

8. Surgical Options for Arrhythmia
SNU Children’s Hospital
Surgical Options for Arrhythmia 1 3 2
Kao et al. 1994
Conversion to TCPC in AFL
Gandhi et al. 1996
Fontan suture lines alone induce AFL
Mavroudis et al / Deal et al. 1999
1) Area between coronary sinus & IVC
2) Lateral tunnel suture line
3) Superior rim of the prior ASD patch
TCPC

9. Optimization of TCPC Geometry
SNU Children’s Hospital
Optimization of TCPC Geometry
Flow separation of caval inlets (in vitro)
Offset : de Leval et al. 1996
Flaring ,offset : Ensley et al. 1999
Curvature : Sharma et al. 1996, Gerdes
Balance of mixing
1) Energy conservation
2) Optimal distribution of SVC & IVC flows
Needs a further investigation & clinical application
TCPC

10. Lateral Tunnel Technique (SNUCH)
SNU Children’s Hospital
Lateral Tunnel Technique (SNUCH)
TCPC

11. Lateral Tunnel Technique (SNUCH)
SNU Children’s Hospital
Lateral Tunnel Technique (SNUCH)
Right atriotomy
extending to coronary sinus
Cryoablation
between atriotomy & TV anulus TV CS
Cryoablation
TCPC

12. Lateral Tunnel Technique (SNUCH)
SNU Children’s Hospital
Lateral Tunnel Technique (SNUCH)
Closure of atriotomy
incorporating the Gore-Tex patch
(Sandwich technique)
To reduce atrial suture line
Third, to reduce the atrial suture line , the remainder of the
baffle is sandwiched between the free edges of the atrium
and included with the atriotomy closure . CS
TCPC

13. Video Demonstration (SNUCH)
SNU Children’s Hospital
Video Demonstration (SNUCH)
This patient was 26 months old. His diagnosis was complex heart disease with criss-cross heart, double-outlet right ventricle, and coarctation of aorta. When he was one month old, he underwent coarctoplasty and pulmonary arterial banding At the age of 5 months, bidirectional cavo- pulmonary connection was performed with cardiopulmonary bypass.
The final Fontan operation was performed under routine cardiopulmonary bypass and intermittent cold blood cardioplegic arrest during moderate systemic hypothermia.
click the play button
Right atrial incision is made obliquely about 2 cm parallal to the sulcus terminalis and extended to coronary sinus.
After running suture of extended atriotomy to coronary sinus, cryoablation was done between the tricuspid valve and lower right atrial incision .
Shoe-shaped baffle is cut from a sheet of PTFE cardiovascular patch
and 4 mm fenestration is created with an aortic punch.
The baffle is sewn into position with running suture beginning at the middle,
and continued around the entrance of the IVC into the atrium , leaving the
coronary sinus on the pulmonary venous side of the baffle.
The suture line is then continued to the entrance site of the SVC.
The remainder of baffle is sandwiched between the free edges of the atrium
and included with the atriotomy closure.
TCPC

14. Surgical Results of Total Cavopulmonary Connection - SNUCH -
SNU Children’s Hospital
Surgical Results of Total Cavopulmonary Connection - SNUCH -
TCPC

15. SNU Children’s Hospital
Patients Profile
1990 ~ July (except AP Fontan)
195 patients
M:F = 128:67
Age : 39.2 ± 34.7months (Median: 26.6mo)
Bwt : 13.8 ± 8.2Kg (Median: 11.9Kg)
TCPC

16. SNU Children’s Hospital
Patients Profile
Diagnosis
Tricuspid atresia
Double inlet ventricle
DIRV (79.4%)
DILV (16.9%)
DIUV ( 3.7%)
Others
TCPC

17. Pre-Fontan Palliations
SNU Children’s Hospital
Pre-Fontan Palliations
101 procedures in 95 patients (48.7%)
BT shunt ; 63, LMBT (n= 35) RMBT (n=28)
PAB (n= 30)
PAB only (n= 24)
with ASO (n=2)
with septectomy (n=2)
with coarctoplasty (n=1)
with TAPVR (n=1)
BAS (n= 3)
Norwood (n=2)
ASO (n=1)
Atrial septectomy (n= 1)
TCPC

18. SNU Children’s Hospital
Staged Approach
in 75 patients (38.5%)
BCPS (61.3%)
Pulsatile BCPS (26.7%)
Hemi-Fontan ( 8.0%)
TCPS (Kawashima) ( 2.7%)
Classic Glenn shunt ( 1.3%)
TCPC

19. Palliation to Complete Fontan
SNU Children’s Hospital
Palliation to Complete Fontan
n=60(63.2%)
with palliation
n=95
n=35
Staged Fontan
n=75
n=75
Complete Fontan
n=195
n=40
without palliation
n=100
n=60(60%)
TCPC

20. Type of Fontan Operation
SNU Children’s Hospital
Type of Fontan Operation
Intracardiac (78.5%)
: including
Conversion to lateral tunnel from AP Fontan 4
Hepatic inclusion ( in pts with previous TCPS) 2
Extracardiac (21.5%)
Conduit
EELT(extracardiac epicardial)
Direct anastomosis (IVC to MPA)
TCPC

21. Concomitant Procedures
SNU Children’s Hospital
Concomitant Procedures
Fenestration
PA angioplasty
Atrial septectomy
Prophylactic Cryoablation after 1997 Feb
AVVP
AVVR
PPM insertion
TAPVR
Diaphragm plication
Widening of pulmonary venous opening
Aortic annuloplasty
TCPC

22. SNU Children’s Hospital
Complication
Prolonged c-tube drain (>2wk) (21.5%)
Chylothorax (11.3%)
Diaphragm palsy ( 6.2%)
HIE ( 5.1%)
Bleeding ( 3.6%)
Protein-losing enteropathy ( 2.1%)
TCPC

23. SNU Children’s Hospital
Results
Mortality
Early ( <30days) % (12/195)
Late ( >30days) % ( 7/183)
Follow-up duration
Mean : 46.5±34.5 months
Range : 0.4~ months
TCPC

24. SNU Children’s Hospital
Annual Trends
TCPC

25. Additional Procedures during F/U
SNU Children’s Hospital
Additional Procedures during F/U
PPM insertion
Closure of RL shunt
Stenosis of pulmonary venous opening
Widening
Pneumonectomy
AVVR
Widening of Fontan pathway
Angioplasty for LPA stenosis
Collateral obliteration (transpleural)
TCPC

26. SNU Children’s Hospital
Arrhythmia during F/U
13 of 176 patients (7.4%)
Supraventricular tachyarrhythmia
Bradyarrhythmia
4 patients needed PPM insertion
* Before or at the time of Fontan operation
: PPM insertion in 7 patients
TCPC

27. Cumulative Survival Rate
SNU Children’s Hospital
Cumulative Survival Rate
Time (months)
Survival (%) 80
100
90.1 ± % 20
120 60 40
90.8 ± %
87.8 ± %
TCPC

28. SNU Children’s Hospital
Conclusion
TCPC in present series
Applicable to single ventricle physiology
Favorable early results
Low (but non-zero) arrhythmia incidence
Low late failure rate
Unresolved issues
Longer term arrhythmia
Longer term survival
Comparison between intra vs extra
Further modification of surgical technique
TCPC