Arrhythmia After Modified Total CavopulmonaryConnection Without Use of Prosthetic MaterialTakaaki Suzuki, MD, Takayasu Murai, MD, Masaaki Sato, MD, Tsutomu Ito, MD, andToyoki Fukuda, MDDivisions of Cardiovascular Surgery and Cardiology, Tokyo Metropolitan Children’s Hospital, Tokyo, Japan

Background. Although total cavopulmonary connec-tion without use of prosthetic material appeared to be apromising surgical procedure that would retain potentialgrowth of the intraatrial tunnel, midterm incidence ofarrhythmia remains unknown.

Methods. Twelve patients underwent modified totalcavopulmonary connection. A prosthetic material wasnot used in 5 patients (group F) and was used in 7patients (group P). A retrospective review of the periop-erative electrocardiogram and ambulatory monitoringwere performed.

Results. All patients revealed regular sinus rhythmbefore the operation. In the early postoperative period,the incidence of sinus node dysfunction was higher in

group F than in group P (80% versus 28.6%). This differ-ence no longer existed by hospital discharge (group F,20%; group P, 14.3%). In the midterm follow-up period,sinus node dysfunction was detected in 4 patients ofgroup F (80%) and 1 patient of group P (14.3%). Transienttachyarrhythmia was detected in 1 patient of group F and3 patients of group P.

Conclusions. Modified total cavopulmonary connectionwithout use of prosthetic material affected unfavorablythe sinus node in the early and midterm postoperativeperiod.

(Ann Thorac Surg 2002;73:102–6)© 2002 by The Society of Thoracic Surgeons

Arrhythmia is known to be the major risk factor ofmorbidity and mortality after the Fontan type op-

eration. Earlier investigators argued that postoperativearrhythmia was less frequent in total cavopulmonaryconnection (TCPC) than in atriopulmonary connection[1–3]. More recently, TCPC without use of prostheticmaterial appeared in the literature as a promising surgi-cal procedure that would retain potential growth of theintraatrial tunnel [4–6]. In favor of the documentedadvantage, we have adopted the new surgical methodsince 1992. Although the short-term results of the newsurgical method demonstrated a low incidence of ar-rhythmia [7], the midterm incidence of arrhythmia re-mains unknown. The purpose of this study was to iden-tify whether the new surgical method affected favorablythe incidence of arrhythmia during the midtermfollow-up.

Patients and Methods

PatientsBetween November 1992 and October 1998, 12 patientsunderwent modified TCPC at Tokyo Metropolitan Chil-dren’s Hospital. A prosthetic material was not used in 5patients (group F) and was used in 7 patients (group P).There were 5 boys and 7 girls. We made a retrospective

review of the medical and surgical records with specificattention to the postoperative incidence of arrhythmia.All patients were evaluated by cardiac catheterization ata median of 14 months after the operation.

Surgical TechniqueModified TCPC with or without use of the prostheticmaterial was performed through a median sternotomy.After cardiopulmonary bypass was instituted using bica-val and aortic cannulation, previous systemic-to-pulmonary shunt was ligated, if present. Myocardialprotection was achieved by infusion of blood cardiople-gic solution, containing 20 mmol/L potassium, into theaortic root. The heart was cooled with ice slush in such amanner as to keep the myocardial temperature less than25°C. The right atrium was opened anterior and parallelto the interatrial groove. The incision extended from theroot of the right atrial appendage to immediately supe-rior to the junction between the right atrium and inferiorcaval vein. At both extents, the incision was extendedposteriorly, thereby creating a flap of the atrial wall ingroup F (Fig 1A). Particular care was taken not to extendthe incision into the crista terminalis or sinus node area.The atrial septum was fully excised if present. Themargin of the atrial flap was sutured down the orifice ofthe superior vena cava until it reached the posterior rimof the atrial septum. Inferiorly, the atrial flap was suturedaround the orifice of the inferior vena cava and thenalong the posterior rim of the atrial septum, leaving thecoronary sinus on the pulmonary venous atrial side toavoid potential injury to the conduction system (Fig 1B).

Accepted for publication Sept 19, 2001.

Address reprint requests to Dr Suzuki, Division of Pediatric Cardiovas-cular Surgery, University of Michigan Medical Center, F 7830 MottChildren’s Hospital, 1500 East Medical Center Dr, Ann Arbor, MI 48109-0223; e-mail: suzukimd@blue.ocn.ne.jp.

© 2002 by The Society of Thoracic Surgeons 0003-4975/02/$22.00Published by Elsevier Science Inc PII S0003-4975(01)03323-9

Then the other margin of the atrial incision line wassutured to the epicardium of the repositioned atrial flap.These sutures were positioned next to the previoussuture line so as to keep the stitches away from the sinusnode (Fig 1C). After unclamping the aorta, the superiorvena cava was transected at the level of the right pulmo-nary artery, or 1 cm superior to the cavo-atrial junction.The superior and inferior walls of the right pulmonaryartery were incised and anastomosed with the corre-sponding ends of the transected superior vena cava. Noprosthetic material was used during procedure. In themodified TCPC with use of prosthetic material or lateraltunnel procedure, on the other hand, a composite intra-atrial tunnel was constructed using a baffle trimmed froma 10-mm Gore-Tex tube graft (W. L. Gore & Assoc,Flagstaff, AZ). The incision on the right atrium wasalmost the same as that of group F; however, posteriorextension was not performed and the incision was madea little more posteriorly than in group F (Fig 2A). Thesuperior and inferior margins of the baffle were suturedaround the orifices of the superior vena cava and the

inferior vena cava, and the posteromedial margin to theposterior rim of atrial septum, leaving the coronary sinuson the pulmonary venous atrial side (Fig 2B). The ante-rior margin of the patch was incorporated into the sutureclosure of the atriotomy (Fig 2C). In both procedures,every effort was made to keep the surgical manipulationaway from the sinus node artery, the sinus node itself,and the crista terminalis. No fenestration was created inany of the patients.

ArrhythmiaA retrospective review of the preoperative and postop-erative electrocardiography as well as of the ambulatorymonitoring was made to evaluate the incidence of ar-rhythmia. In all patients, repeated 12-lead electrocardio-graphic records were performed before and shortly afterthe operation, at the time of hospital discharge, andduring the period of follow-up. In an attempt to deter-mine the midterm incidence of arrhythmia, all patientsunderwent ambulatory electrocardiographic monitoringat a median of 42 months after the operation. Postoper-

Fig 1. (A) Surgical technique of modified total cavopulmonary con-nection without use of prosthetic material. The incision on the rightatrium was made parallel to the interatrial groove, and then ex-tended posteriorly. (B) The intraatrial tunnel was constructed by su-turing the atrial flap around the orifice of the inferior vena cava, tothe posterior atrial septal ridge, and around the orifice of the supe-rior vena cava. (C) Then the anterior flap of the right atrium wasbrought down and sutured on the outer wall of the intraatrial tunnelto create a pulmonary venous chamber leaving the coronary sinusorifice in this chamber. (ASD � atrial septal defect; CS � coronarysinus orifice; SN � sinus node.)

Fig 2. (A) Surgical technique of lateral tunnel method. The incisionon the right atrium was made parallel to the interatrial groove anda little more posteriorly than for group F. (B) The intraatrial tunnelwas constructed by placement of a partial tube of polytetrafluoroeth-ylene (PTFE) from the inferior vena cava to the superior vena cava.(C) Then the anterior edge of the partial graft was incorporated intothe suture line of the atriotomy to create a pulmonary venous cham-ber leaving the coronary sinus orifice in this chamber. (ASD �atrial septal defect; CS � coronary sinus orifice; SN � sinus node.)

103Ann Thorac Surg SUZUKI ET AL2002;73:102–6 ARRHYTHMIA AFTER MODIFIED TCPC

ative arrhythmia was grouped into three forms: (1) sinusnode dysfunction, which included sinus bradycardia,ectopic atrial rhythm, predominant junctional rhythm, orsinus pause exceeding 2 seconds; (2) supraventriculartachycardia, which included atrial fibrillation, atrial flut-ter, ectopic atrial tachycardia, junctional ectopic tachy-cardia, or atrioventricular nodal reentrant tachycardia;and (3) any form of atrioventricular block.

Statistical AnalysisStatistical analysis was performed with SPSS software(SPSS, Inc, Chicago, IL). Data were expressed as mean �standard deviation. An unpaired Student’s t test wasused to determine differences between the groups. Cat-egorical variables were analyzed by use of Fisher’s exacttest.

Results

Patient CharacteristicsThe median age at the time of operation was 30 months(range, 20 to 144 months). The cardiac anomalies in-cluded tricuspid atresia (n � 5), pulmonary atresia withintact ventricular septum (n � 3), heterotaxy syndrome(n � 2), and others (n � 2; Table 1). All patients under-went previous palliative procedures including bidirec-tional cavopulmonary shunt having been performed on 4patients. All but 2 patients (patients 4 and 10) underwentrepeated sternotomy and pericardiotomy. An atrioven-tricular connection was converted to TCPC in a patientwho developed myocardial dysfunction subsequent tothe previous Bjork procedure (patient 5). There were nosignificant differences between groups in age (group F,53 � 51.6 months; group P, 36 � 19.3 months; p � 0.4378)and body weight (group F, 16.2 � 10.3 kg; group P, 11.8 �2.8 kg; p � 0.3012). No death occurred during the mid-

term follow-up. None of the patients developed throm-boembolic episodes.

ArrhythmiaElectrocardiographic records at the perioperative andfollow-up periods were available in all patients, and theresults are depicted in Figure 3. All patients showedregular sinus rhythm before the operation. In the earlypostoperative period, 4 patients (80%) in group F devel-oped sinus node dysfunction, with 2 of these developingjunctional rhythm and the other 2 patients a combinationof junctional rhythm and ectopic atrial rhythm. At thetime of hospital discharge, however, 3 of the patientsrecovered regular sinus rhythm, with a consequent inci-dence of sinus node dysfunction of 20%. In group P, onthe other hand, 2 patients (28.6%; odds ratio � 0.1; 95%confidence interval, 0.006 to 1.544; p � 0.2424 vs group F)developed sinus node dysfunction, with 1 of these devel-oping ectopic atrial rhythm and the other patient, junc-tional rhythm. At the time of hospital discharge, how-ever, the patient with junctional rhythm recoveredregular sinus rhythm, with a consequent incidence ofsinus node dysfunction of 14.3%. Although supraventric-ular tachycardia or premature contractions were ob-served in 1 patient of group F and 3 patients of group P,all episodes of tachyarrhythmia occurred exclusively dur-ing the early postoperative period and disappeared bythe time of hospital discharge. None of the patientsrevealed atrial flutter or fibrillation during the hospitalstay.

All patients of both groups underwent ambulatorymonitoring at a median of 42 months after the operation(range, 11 to 92 months). Four patients of group F (80%)developed sinus node dysfunction, with one of thesehaving kept up ectopic atrial rhythm since the time ofhospital discharge. The other 2 patients who had hadregular sinus rhythm at the time of hospital dischargedeveloped sinus bradycardia in association with sinuspauses, and the remaining patient, who had also had

Table 1. Patient Characteristics

PatientNo.

Age(mo) Diagnosis

PreviousOperations TCPC

1 24 TA Central, BT Atrial flap2 29 PA�IVS Central, BCPS Atrial flap3 23 TA Central Atrial flap4 45 TA BT Atrial flap5 144 TA PAB, Bjork Atrial flap6 25 TA Central Lateral tunnel7 20 PA�IVS Central Lateral tunnel8 78 Asplenia BT, BCPS Lateral tunnel9 36 PA�IVS Central, BCPS Lateral tunnel

10 35 DORV BT Lateral tunnel11 30 DORV BT Lateral tunnel12 28 Asplenia BCPS Lateral tunnel

Atrial flap � modified TCPC without use of prosthetic material;BCPS � bidirectional cavopulmonary shunt; BT � Blalock-Taussigshunt; Central � central aortopulmonary shunt; DORV � double-outlet right ventricle; IVS � intact ventricular septum; Lateraltunnel � modified TCPC with use of prosthetic material; PA �pulmonary atresia; PAB � pulmonary artery banding; TA � tri-cuspid atresia; TCPC � total cavopulmonary connection.

Fig 3. Bar graph shows plot of the number of patients in normalsinus rhythm (NSR) and sinus node dysfunction (SND) in the pre-operative period and during the period of follow-up after modifiedtotal cavopulmonary connection.

104 SUZUKI ET AL Ann Thorac SurgARRHYTHMIA AFTER MODIFIED TCPC 2002;73:102–6

regular sinus rhythm at the time of hospital discharge,developed ectopic atrial rhythm. By contrast, in group P,sinus bradycardia was detected in only 1 patient (14.3%;odds ratio � 0.042; 95% confidence interval, 0.002 to 0.877;p � 0.072 versus group F), who had had regular sinusrhythm at the time of hospital discharge. Furthermore, apatient who had had ectopic atrial rhythm at the time ofhospital discharge recovered regular sinus rhythm. Noneof the patients under investigation developed atrioven-tricular block, or atrial flutter or fibrillation. The fre-quency of the premature supraventricular contractionwas 0.16% � 0.23% of all beats during the investigation ingroup F and 0.15% � 0.34% in group P (p � 0.9873). Allpatients with or without arrhythmia were free fromsymptoms without the need for medication.

Cardiac CatheterizationCardiac catheterization was performed in all patients at amedian of 14 months (range, 3.8 to 29.6 months) after theoperation. The cardiac index showed no significant dif-ference between groups (group F, 3.17 � 1.29 L � min�1 �m�2; group P, 2.97 � 0.87 L � min�1 � m�2; p � 0.7811). Themean pressure of the pulmonary arteries and pulmonaryvascular resistance index also showed no significantdifferences between groups, respectively (group F, 13 �1.2 mm Hg; group P, 14.5 � 2.1 mm Hg; p � 0.7881; groupF, 134.8 � 19.2 dyne � s � cm�5 � m�2; group P, 148.7 � 58.7dyne � s � cm�5 � m�2; p � 0.6653). There was no pressuregradient or obstructed flow of blood across the anasto-mosis connecting both caval veins and the right pulmo-nary artery. The pressure curve of the intraatrial tunnelwas pulsatile in all patients without regard to the oper-ative method.

Comment

Progressively reduced incidence of arrhythmia after theFontan operation owes much to the intricate modifica-tions of the therapeutic strategy. These included stagedpalliation for the purpose of alleviation of the volumeoverload on the functioning ventricle and younger age atthe time of operation. In addition, an increasing numberof reports describe that TCPC is less prone to arrhythmiawhen compared with the atriopulmonary connection.This argument may in part be based on the evidence thatthe central venous pressure tended to be lower in TCPCthan in atriopulmonary connection, with TCPC exertingless pressure load on the atrial wall [1–3]. As a conse-quence of these reports, TCPC has become accepted as apreferable palliative procedure for patients having asingle functioning ventricle. In early years, a piece ofprosthetic graft or patch was used as a baffle to separatethe unified atrial cavity into chambers belonging to thesystemic and pulmonary circulations (group P in ourseries) [8, 9]. During the past 10 years, numerous modi-fied techniques have appeared in the literature for thepurpose of optimal separation of the two circulatorysystems at the atrial level. These included the atrial flapprocedure in which the unified atrial cavity was dividedby the repositioned atrial wall and, therefore, without use

of the prosthetic material (group F in our series) [4–6]. Asthe systemic venous chamber was constructed only withthe autologous material, a major advantage of this pro-cedure was thought to be the potential growth of thischamber in correlation with somatic growth of the pa-tient. Earlier reports dealing with short-term results ofthis procedure described the result as being promising,with a low incidence of postoperative arrhythmia [7].However, our result was unequivocally different from theprevious views, demonstrating that sinus node dysfunc-tion was more prevalent in group F than in group P. Sucha discrepancy caused us to infer that the higher incidenceof arrhythmia in group F might be ascribed to theextensive incision and sutures of the right atrium, whichwere comparable with those of the Senning procedure[10]. With these considerations in mind, we made adetailed analysis of the 12-lead electrocardiographicrecords during the perioperative and follow-up periods,as well as of the ambulatory monitoring.

In contrast to the previous view that atrial tachyar-rhythmia dominated the postoperative arrhythmia [1–3],recent studies indicated that sinus node dysfunction wasthe dominant feature after the Fontan operation [11].Manning and colleagues [12] endorsed this view andclaimed that a staged operation precipitated a higherincidence of sinus node dysfunction. More important,Shirai and colleagues [13] experienced a high incidenceof sinus node dysfunction (44%) even after the extracar-diac Fontan operation. Common to these reports was theimplication that the repeated pericardiotomy was thepredisposing factor of the sinus node dysfunction. Ourresult differed again from these reports, however, in that6 of 7 patients of group P underwent repeated pericar-diotomy with none of them developing sinus node dys-function at the time of hospital discharge and only 1exhibiting it during the midterm follow-up. Taking theseobservations together, we infer that an extensive manip-ulation of the right atrium for the construction of thecavocaval connection without use of the prosthetic ma-terial is the major risk factor for sinus node dysfunction.In fact, a high incidence of sinus node dysfunction ingroup F was comparable with that seen after the Mustardor Senning operations [14, 15], whereas a low incidencein group P was consistent with that seen (10% to 23%) inthe midterm follow-up of the comparable procedure [12].Although Cohen and colleagues [16] demonstrated thatthe surgical manipulation distant from the sinus nodehad no discernible effect on predisposition to the devel-opment of early sinus node dysfunction, their study dealtonly with patients who underwent extracardiac Fontan orthe lateral tunnel procedure subsequent to the hemi-Fontan procedure, and failed to deal with those whounderwent the atrial flap procedure.

Another concern of the modified TCPC without use ofthe prosthetic material would be the progressive dilata-tion of the tunnel having a pressure higher than normal.Although TCPC is believed to be superior to the atrio-pulmonary connection with minimizing the area of theatrial wall that is subjected to high pressure, TCPCwithout use of the prosthetic material offsets this advan-

105Ann Thorac Surg SUZUKI ET AL2002;73:102–6 ARRHYTHMIA AFTER MODIFIED TCPC

tage by expanding the area of the atrial wall for thesystemic venous tunnel. Therefore, the stepwise growthof the tunnel in correlation with age may mean that theatrial wall of the tunnel is overstretched. Such mechani-cal stress on the right atrial wall or the sinus node itselfcan well be another predisposing factor of the sinus nodedysfunction.

Although all patients in our series remain in New YorkHeart Association functional class II or less, long-termconsequences of sinus node dysfunction remain un-known. Loss of sinus rhythm and late development ofarrhythmia were usually well tolerated. However, in thelight of the facts that the longstanding arrhythmia afterthe Mustard or Senning operation predisposed patientsto the progressive deterioration of cardiac function orsudden death, patients with sinus node dysfunction needclose observation regardless of the presence or absenceof clinical symptoms.

Against earlier investigations that showed a high inci-dence of malignant tachyarrhythmia including atrial flut-ter among patients who had undergone TCPC [1, 3],patients in our series did not show malignant tachyar-rhythmia during the period of follow-up. Such discrep-ancy may be partly explained by an experimental studythat clarified that the suture line by itself was a criticalcomponent in the flutter circuit by creating the condi-tions for slow conduction and unidirectional block [17]. Inthis study, the authors argued that, inasmuch as theintact crista terminalis minimized the conduction distur-bance, the surgical incision or sutures kept away from thecrista terminalis was the prerequisite to reduce the inci-dence of atrial tachyarrhythmia. The result of our surgi-cal technique, in which sutures were placed along andaway from the crista terminalis, validated this view.

Our study design has several limitations. The numberof patients under investigation is small, and the fol-low-up period is as yet limited. Therefore, the closefollow-up will clearly need to be continued. Shouldcomparable studies be made in a larger series of patients,however, the controversy of the issue can be clarifiedfurther.

In summary, we experienced a high incidence of sinusnode dysfunction after modified TCPC without use ofprosthetic material. We did not, however, experiencemalignant tachyarrhythmias in the midterm follow-up.The result led us to infer that an extensive manipulationof the right atrium, not extending to the crista terminalis,has a predisposition to the development of sinus nodedysfunction without subsequent appearance of malig-nant tachyarrhythmia.

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