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

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

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

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

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

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

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

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. 1998 / Deal et al. 1999 1) Area between coronary sinus & IVC 2) Lateral tunnel suture line 3) Superior rim of the prior ASD patch TCPC

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 . 1999 Balance of mixing 1) Energy conservation 2) Optimal distribution of SVC & IVC flows Needs a further investigation & clinical application TCPC

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

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

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

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

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

SNU Children’s Hospital Patients Profile 1990 ~ 2000 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

SNU Children’s Hospital Patients Profile Diagnosis Tricuspid atresia 29 Double inlet ventricle 136 DIRV 108 (79.4%) DILV 23 (16.9%) DIUV 5 ( 3.7%) Others 30 TCPC

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

SNU Children’s Hospital Staged Approach in 75 patients (38.5%) BCPS 46 (61.3%) Pulsatile BCPS 20 (26.7%) Hemi-Fontan 6 ( 8.0%) TCPS (Kawashima) 2 ( 2.7%) Classic Glenn shunt 1 ( 1.3%) TCPC

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

Type of Fontan Operation SNU Children’s Hospital Type of Fontan Operation Intracardiac 153 (78.5%) : including Conversion to lateral tunnel from AP Fontan 4 Hepatic inclusion ( in pts with previous TCPS) 2 Extracardiac 42 (21.5%) Conduit 29 EELT(extracardiac epicardial) 10 Direct anastomosis (IVC to MPA) 3 TCPC

Concomitant Procedures SNU Children’s Hospital Concomitant Procedures Fenestration 101 PA angioplasty 68 Atrial septectomy 58 Prophylactic Cryoablation after 1997 Feb. 33 AVVP 13 AVVR 1 PPM insertion 6 TAPVR 6 Diaphragm plication 3 Widening of pulmonary venous opening 3 Aortic annuloplasty 1 TCPC

SNU Children’s Hospital Complication Prolonged c-tube drain (>2wk) 42 (21.5%) Chylothorax 22 (11.3%) Diaphragm palsy 12 ( 6.2%) HIE 10 ( 5.1%) Bleeding 7 ( 3.6%) Protein-losing enteropathy 4 ( 2.1%) TCPC

SNU Children’s Hospital Results Mortality Early ( <30days) 6.2 % (12/195) Late ( >30days) 3.8 % ( 7/183) Follow-up duration Mean : 46.5±34.5 months Range : 0.4~125.7 months TCPC

SNU Children’s Hospital Annual Trends TCPC

Additional Procedures during F/U SNU Children’s Hospital Additional Procedures during F/U PPM insertion 4 Closure of RL shunt 3 Stenosis of pulmonary venous opening 4 Widening 2 Pneumonectomy 2 AVVR 2 Widening of Fontan pathway 1 Angioplasty for LPA stenosis 1 Collateral obliteration (transpleural) 1 TCPC

SNU Children’s Hospital Arrhythmia during F/U 13 of 176 patients (7.4%) Supraventricular tachyarrhythmia 8 Bradyarrhythmia 5 4 patients needed PPM insertion * Before or at the time of Fontan operation : PPM insertion in 7 patients TCPC

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

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