PEDIATRIC CARDIOLOGY and CARDIAC SURGERY VOL. 24 NO. 5 ( ) Electrophysiological Studies and Radiofrequency Catheter Ablation before Total Cavo-p

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1 PEDIATRIC CARDIOLOGY and CARDIAC SURGERY VOL. 24 NO. 5 ( ) Electrophysiological Studies and Radiofrequency Catheter Ablation before Total Cavo-pulmonary Connection in Patients with Complex Congenital Heart Disease Keiko Toyohara, Yo Kajiyama, Jun Yoshimoto, Hitoo Fukuhara, and Yoshihide Nakamura Department of Pediatric Cardiology, Japanese Red Cross Society, Wakayama Medical Center, Wakayama, Japan Objectives: We characterized the mechanism of supraventricular tachycardia (SVT) in patients scheduled for total cavo-pulmonary connection (TCPC) and evaluated the outcome of preoperative electrophysiological studies (EPS) and radiofrequency catheter ablation (RFCA). Patients and methods: We performed EPS in 16 patients who had a history of SVT and were suspected of having an anatomic arrhythmogenic substrate (i.e., an anatomical anomaly causing tachycardia). Results: We were unable to induce supraventricular tachycardia in four patients. In 11 of 12 other patients ablation was successful; five patients had reciprocating tachycardia and twin atrioventricular nodes (twin AVNs), four had WPW syndrome, two had atrial tachycardia (AT), and atrial flutter (AFL) developed in one. Junctional tachycardia (JT) was documented clinically or during surgery in three cases with right isomerism. In 11 patients with RFCA, five patients underwent TCPC. In five patients, four did not have SVT during or after TCPC. Conclusion: EPS are essential for complete arrhythmic evaluation before TCPC. After successful RFCA, patients, especially those with right isomerism, may still develop JT during and after the TCPC procedure. Total cavo-pulmonary connection TCPC EPSRFCA TCPC 16 4 EPS EPS12 2 twin AVNs 5 WPW 4 AT 2 1 AT 1 11 RFCA JT 3 RFCA 11 TCPC 5 4 TCPC EPS RFCA JT TCPC TCPC

2 TCPC EPS Table kg12kg heterotaxy syndrome heterotaxy 10 atrioventricular discordance AVD atrio-pulmonary connection APCTCPC conversion 7 APC TCPC EPS16 Fig. 1 4 heterotaxy 3 AVD 1 EPS2 case 1 2 WPW Fig. 2 case 1 His fasciculo-ventricular-fiber FVF 2 case 3 4 case 3 case 4 overdrive EPS RFCA 2 5 heterotaxy 4 AVD 1 2 His 2 sling 2 sling 4 case case case 8 RFCA 3 3 case msec 1 case 8 340msec 200msec RFCA 4 RFCA sling 1 case 9 heterotaxy RFCA Fig. 3 heterotaxy 4 AVD Fig. 1 5 TCPC 3 heterotaxy 1 AVD 1 TCPC heterotaxy 1 case 5 TCPC JT JT RFCA JTTCPC 2 heterotaxy 1 case / JT Fig. 4 WPW 4 2 AVD TCPC 2 AT 2 1 heterotaxy case 14 ATP 0.1mg/kg Fig. 5 2 His 4 His RFCA bidirectional cavo-pulmonary shunt BCPS EPS AT BCPS 140 JT 1 AT EPS AT EPS RFCA

3 622 Table 1 Patient characteristics case tachycardia age (months) weight (kg) morphology RFCA target of RFCA palliation condition JT 1 - none (FVF) RI, {A,L,D}, MA, DORV TCPC none (FVF) RI, {A,X,X}, SV, SA, CAVC, PA - - bil.bts + UF TCPC - 3 P none {S,X,A}, DILV, PS - - PAB pre BCPS - 4 P none {S,L,L}, DORV, PS - - BCPS pre TCPC - 5 P AVRT (twin AVNs) RI, {A,L,L},CAVC, DORV, PS, TAPVC + ant.avn BCPS TCPC + 6 C AVRT (twin AVNs) RI, {A,L,L}, CAVC, PA + ant.avn BCPS pre TCPC + 7 P AVRT (twin AVNs) {I,D,D}, VSD, PS + ant.avn BCPS TCPC - 8 P AVRT (twin AVNs) LI, {I,D,D}, CAVC, PA + ant.avn BCPS TCPC - 9 P AVNRT (twin AVNs) RI, {A,L,L}, CAVC + post.avn BCPS pre TCPC - 10 C WPW {S,L,L}, DORV, PS + AP (LL) lt.bt TCPC - 11 C WPW {S,L,X}, DORV, PS + AP (LP) bil.bts TCPC - 12 C WPW {S,D,D}, DIRV, PS + AP (LP) CS pre TCPC - 13 C WPW 17y* 46.8 {S,D,D}, DORV, straddling TV + AP (LA) BCPS, TVP pre TCPC - 14 P AT RI, {A,L,L}, CAVC, PA, TAPVC + CAVC LL bil.bts + TAPVC repair pre TCPC + 15 C AT RI, {A,X,X}, SV, SA, CAVC, PA + ** - bil.bts pre BCPS - 16 C AFL RI, {A,X,D}, SV, SA, CAVC, PS + IVC-TV isthmus BCPS pre TCPC - JT: junctional tachycardia, FVF: fasciculo-ventricular-fiber, AVRT: atrioventricular reentrant tachycardia, AVNRT: atrioventricular nodal reentrant tachycardia, AT: atrial tachycardia, AFL: atrial flutter, P: tachycardia in early post-palliative operation and/or during catheter examination, C: clinical tachycardia, y*: years, + **: failure RI: right isomerism, MA: mitral atresia, DORV: double-outlet right ventricle, SV: single ventricle, SA: single atrium, CAVC: common atrio-ventricular canal, PA: pulmonary atresia, DILV: double-inlet left ventricle, PS: pulmonary stenosis, LI: left isomerism, TAPVC: total anomalous pulmonary venous connection, TV: tricuspid valve, ant.avn: anterior atrioventricular node, post.avn: posterior atrioventricular node, AP: accessory pathway, LL: left lateral, LP: left posterior, LA: left anterior, IVC: inferior vena cava, BT: Blalock-Taussig shunt, UF: unifocalization, PAB: pulmonary artery banding, BCPS: bidirectional cavo-pulmonary shunt

4 623 twin AVNs 5 (heterotaxy 4, AVD 1) WPW 4 (AVD 2) AT 2 (heterotaxy 2) AFL 1 (heterotaxy 1) EPS 4 (heterotaxy 3, AVD 1) C = 1 P = 4 C = 4 RFCA (+) 1 RFCA ( ) 1 RFCA FVF 2 JT 2 heterotaxy 2 (heterotaxy 1, AVD 1) RFCA 5 RFCA 4 EPS before BCPS No recurrence pre TCPC pre TCPC TCPC 2 pre TCPC 2 No recurrence No recurrence TCPC 3 JT (+) 1 ( ) 2 pre TCPC 2 JT (+) 1 ( ) 1 TCPC 2 pre TCPC 2 JT at BCPS pre TCPC Fig. 1 Clinical course in each case. P: tachycardia in early post-palliative operation and/or during catheter examination, C: clinical tachycardia A B Fig. 2 Two ECGs in case 1. A: sinus rhythm, B: HRA overdrive: delta waves were unchanged. ECG: electrocardiogram 1 heterotaxyrfca RFCA 11 TCPC case 5 JT JT TCPCTCPC

5 624 case 5 case 6 case 7 A P RAO 55 RVG A P LVG A P case 8 case 9 A P case 5-8: twin AVNs with sling case 9: AVNRT in post.avn AVN targeted for RFCA case 5, 7, 8, 9: CAVC Fig. 3 Schema of re-entrant pathway of tachycardia in each case with twin AVNs. Asterisks represent AVNs, i.e., His potential recording sites. Dotted circles show common atrioventricular annulus. Dotted lines represent slings between two AVNs. White arrows act as anterograde limbs, black arrows act as retrograde limbs in AVRT. The curved arrows and black asterisk in case 9 show AVNRT. We targeted the AVNs shown with black asterisks. In patients with heterotaxy and a common AV valve, anterior and posterior AVNs exist either on the anterior and posterior sides or on the lateral and posterior sides of the common AV valve. In a patient with AV discordance (I,D,D), an anterior AVN existed on the anterior side by the mitral valve, and a posterior AVN existed on the posterior side by the tricuspid valve. heterotaxy heterotaxy AVD twin AVNs TCPC TCPC EPS 4 heterotaxy 3 AVD 1 FVF heterotaxy 2 TCPC EPS 2 TCPC WPWAVD heterotaxy WPWEbstein AVDheterotaxy 8 WPW RFCA TCPC 2 heterotaxytwin AVNs twin AVNs TCPC heterotaxy AVD twin AVNs QRS TCPC EPS heterotaxy sling twin AVNs l-loop twin AVNs 9 twin AVNs EPS Bae Fontan 52 EPS

6 625 A B C D E F G Fig. 4 A-C: Three ECGs in case 6. E-G: Schema of conduction from AVN (asterisk) to ventricle in relation to each ECG. A, B: Baseline ECGs showing two different QRS complexes, QS pattern (A), RS pattern (B). C: ECG during induced supraventricular tachycardia with the same QRS complex as demonstrated in panel A. We targeted the anterior AVN (the black asterisk) as the retrograde limb in the tachycardia. D: ECG during clinical JT with VA dissociation (arrows represent P waves) after RFCA with the same QRS complex as demonstrated in panel A, and C QRS sling JT 7 2 Bae Fontan

7 626 His RFCA His A B C Fig. 5 A: ECG during induced atrial tachycardia in case 14. B: Activation map during AT and successful ablation. C: Intracardiac electrogram. Dotted circle shows successful ablation site with the earliest fragmentation potential. atypical JT 10 III heterotaxy twin AVNs JT His 11 twin AVNs 5 2 JT RFCA RFCA 1 TCPC 1 JT JT sling 2 AVRT JT JT RFCA modification 12 RFCA RFCA RFCA AVRT JT twin AVNs RFCA AT 1 RFCA BCPS EPS BCPS JT sling twin AVNsJT

8 627 TCPC RFCA EPS 1 Stamm C, Friehs I, Duebener LF, et al: Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 2002; : Wu MH, Lin JL, Wang JK, et al: Electrophysiological properties of dual atrioventricular nodes in patients with right atrial isomerism. Br Heart J 1995; : Epstein MR, Saul JP, Weindling SN, et al: Atrioventricular reciprocating tachycardia involving twin atrioventricular nodes in patients with complex congenital heart disease. J Cardiovasc Electrophysiol 2001; : Bae EJ, Noh CI, Choi JY, et al: Twin AV node and induced supraventricular tachycardia in Fontan palliation patients. Pacing Clin Electrophysiol 2005; : Walsh EP: Arrhythmias in patients with congenital heart disease. Card Electrophysiol Rev 2002; : Ohuchi H, Miyazaki A, Watanabe T, et al: Hemodynamic deterioration during simulated supraventricular tachycardia in patients after the Fontan operation. Int J Cardiol 2007; : Nakagawa H, Shah N, Matsudaira K, et al: Characterization of reentrant circuit in macroreentrant right atrial tachycardia after surgical repair of congenital heart disease: Isolated channels between scar allow focal ablation. Circulation 2001; : Chetaille P, Walsh EP, Triedman JK: Outcomes of radiofrequency catheter ablation of atrioventricular reciprocating tachycardia in patients with congenital heart disease. Heart Rhythm 2004; : Dickinson DF, Wilkinson JL, Anderson KR, et al: The cardiac conduction system in situs ambiguus. Circulation 1979; : Bae EJ, Noh CI, Choi JY, et al: Late occurrence of adenosinesensitive focal junctional tachycardia in complex congenital heart disease. J Interv Card Electrophysiol 2005; : Ih S, Fukuda K, Okada R, et al: The location and course of the atrioventricular conduction system in common atrioventricular orifice and in its related anomalies with transposition of the great arteries A histopathological study of six cases. Jpn Circ J 1983; : Fishberger SB, Rossi AF, Messina JJ, et al: Successful radiofrequency catheter ablation of congenital junctional ectopic tachycardia with preservation of atrioventricular conduction in a 9-month-old infant. Pacing Clin Electrophysiol 1998; :

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