Nonfenestrated Extracardiac Total Cavopulmonary Connection in 132 Consecutive Patients

DOI: 10.1016/j.athoracsur.2007.04.034 2007;84:894-899 Ann Thorac Surg

Rüdiger Lange Christian Schreiber, Jürgen Hörer, Manfred Vogt, Julie Cleuziou, Zsolt Prodan and

Consecutive PatientsNonfenestrated Extracardiac Total Cavopulmonary Connection in 132

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onfenestrated Extracardiac Total Cavopulmonaryonnection in 132 Consecutive Patients

hristian Schreiber, MD, PhD,* Jürgen Hörer, MD,* Manfred Vogt, MD, PhD,ulie Cleuziou, MD, Zsolt Prodan, MD, and Rüdiger Lange, MD, PhDlinic for Cardiovascular Surgery, and Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Center

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Background. The study was conducted to assess theeed for fenestration for completion of a total cavopul-onary connection (TCPC) with the most recent modifi-

ation of an extracardiac conduit.Methods. The extracardiac approach was introduced to

ur institution in January 1999. Between June 2000 andune 2006, 132 consecutive patients were treated withoutfenestration. At the time of TCPC, the median age was

1 months (range, 16 251), with 93 patients (70%) beingounger than 48 months. Median patient weight was 12.5g (range, 9 to 66 kg). A previous partial cavopulmonaryonnection (PCPC) was accomplished in 117 patients88.6%), without additional pulmonary blood flow.

Results. Thirty-day-mortality was 1.5%. Median time toxtubation was 14 hours (range, 3 hours to 126 days).nitial pulmonary artery pressure value was 16.5 � 2.2m Hg, and 13.1 � 1.8 after extubation. Median drainage

equirement was 4 days (range, 1 to 45), and median

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uration of hospitalization was 20 days (range, 5 to 128).hirty-one (24%) required repeat drainage insertion. Noubsequent fenestration was performed, and at hospitalischarge no significant repeat effusions were observed.ultiple covariate logistic regression revealed longer

ime interval from PCPC to extracardiac TCPC (p � 0.006)s a significant predictor of pleural drainage lasting longerhan 4 days, and older age at the time of extracardiac TCPCp � 0.040) as a risk factor for hospitalization more than 20ays. Higher pulmonary artery pressure 3 hours postoper-tively was a significant predictor for both outcome vari-bles in the multivariate model (p � 0.013, p � 0.001).

Conclusions. In general, an extracardiac TCPC can beerformed without fenestration. Early staging of patientsith functional single ventricle physiology may be one

f the keys for these findings.(Ann Thorac Surg 2007;84:894–9)

© 2007 by The Society of Thoracic Surgeons

ince 1988, separation of systemic and pulmonaryvenous return has been accomplished by means of a

otal cavopulmonary connection (TCPC) [1]. Creation ofn extracardiac TCPC was initially proposed for patientsith anomalies of the intra-atrial anatomy, such as pul-onary and systemic venous return, an atretic or dys-

lastic left atrioventricular valve, or complex malforma-ions with common atrioventricular valve [2]. In recentears, many groups have moved to this approach, avoid-ng usually myocardial ischemia (aortic cross-clamping),triotomy and intra-atrial suture lines, and allowing for ahorter duration of cardiopulmonary bypass. The fea-ability of early fenestration is reported [3]; however, wend others have raised the question whether routineenestration applying this extracardiac modification is atll necessary [4, 5]. Within a time interval of 6 years, 132onsecutive patients were treated without a fenestration.he endpoints “hospitalization duration” and “pleuralrainage” were entered into both a univariate and mul-

ivariate outcome analysis.

ccepted for publication April 11, 2007.

Both authors contributed equally to the study.

ddress correspondence to Dr Hörer, Clinic of Cardiovascular Surgery,erman Heart Center Munich, Technical University Munich, Laza-

atients and Methods

n January 1999, we introduced the extracardiac TCPC atur institution. Gradually, construction of an intracardiacunnel was abandoned, and since August 2001, we haveerformed only extracardiac completions [6] until June000, even those patients with an extracardiac connectioneceived an additional fenestration, which was done athe discretion of the individual surgeon. In total, only 9 ofhe patients received a fenestration. Between June 2000nd June 2006, however, 132 consecutive patients werereated without a fenestration. The preoperative character-stics are summarized in Table 1. At the time of extracardiacCPC, the median age was 31 months (range, 16 to 249),ith 93 patients (70%) being younger than 48 months.edian patient weight was 12.5 kg (range, 9 to 66 kg). Only

ubes of at least 18 mm in diameter were implanted. Arevious partial cavopulmonary connection (PCPC) wasccomplished in 117 patients (88.6%), without additionalulmonary blood flow. Patients undergoing revision of aavopulmonary connection were excluded.

This study has been approved by the Ethics Committeef the Technical University Munich (1740/07). The Ethicsommittee waived the need for individual patient con-

ent for this study.All patients received heparin in the early postoperative

eriod, with the aim of establishing a partial thrombo-

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lastin time of 40 to 60 seconds. Oral anticoagulation wasventually commenced, aiming to achieve an interna-ional normalized ratio of 2.0 to 3.0. Patients, or parents,ere generally taught to use a self-testing apparatus

CoaguCheck; Roche Diagnostics, Mannheim, Germany).otal cavopulmonary completion was always performedn cardiopulmonary bypass. A nonringed polytetrafluo-oethylene graft (Gore-Tex, W. L. Gore & Assoc, Flagstaff,rizona) was used in all cases. Only tubes of at least 18m in diameter were implanted. Unless concomitant

ntracardiac procedures were required, aortic cross-lamping was not used. For additional intracardiac pro-edures, antegrade crystalloid cardioplegia, at 40 mL/kgody weight, was applied and the patients cooled to 28°C.t weaning from bypass, we aimed at a haemoglobin ofpproximately 10 g/dL, and after routine modified ultra-ltration at a hemoglobin of about 12 g/dL. Oxygen

able 1. Anatomic and Hemodynamic Characteristics of 132atients Undergoing Extracardiac Total Cavopulmonaryonnection Without Fenestration

natomic andemodynamicharacteristics Excluded Value

ypoplastic left heartsyndrome

0 21 (15.9%)

ricuspid atresia 0 24 (18.2%)eterotaxy syndrome 0 8 (6.1%)ommon atrioventricularvalve

0 17 (12.9%)

ominant right ventricle 0 54 (40.9%)anding of the pulmonaryartery

0 20 (15.2%)

ystemic to pulmonaryartery shunt

0 92 (69.7%)

orwood stage 1 orDamus-Kaye-Stanselprocedure

0 29 (22.0%)

artial cavopulmonaryconnection

15a 117 (88.6%)

ge at PCPC mean � SD,median (range), months

15a 20.6 � 27.4, 9.4 (2–153)

oderately or severelyimpaired systemicventricular function

0 11 (8.3%)

oderate or severeatrioventricular valveinsufficiency

0 25 (18.9%)

o sinus rhythm 0 24 (18.2%)eft atrial pressure, mean� SD, median (range),mm Hg

0 5.2 � 2.1, 5 (0–12)

eft pulmonary arterypressure, mean � SD,median (range), mm Hg

0 9.3 � 3.4, 8 (3–21)

ight pulmonary arterypressure, mean � SD,median (range), mm Hg

0 9.1 � 8.0, 8 (1–19)

In 15 patients, no partial cavopulmonary connection (PCPC) was per-ormed before total cavopulmonary connection.

aturations were checked, and transesophageal echocar- a

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iography was always performed. A cell-saving devicend aprotinin at 30.000 IU/kg were used in all cases.rains were removed as soon as the effusions were

erous and when the drainage loss was less than 5 mL/kgody weight per day. Repeat drainage insertion waserformed when effusions, despite fluid restriction anddministration of loop diuretics, increased and led toespiratory impairment.

tatistical Analysisrequencies are given as absolute numbers and propor-ions. Continuous data are expressed in terms of the

ean and standard deviation or as medians with rangeshen appropriate. The endpoints of hospitalization

ength and pleural drainage were dichotomized accord-ng to medians. The outcome analyses were performedxcluding patients who died within the first 30 postoper-tive days. For univariate analysis, a total of 28 singleovariate logistic models were tested for each outcome,ith values of p below 0.05 as the criterion for statistical

ignificance. For multivariate analysis, multiple covariateodels were specified and tested using variables from

he single covariate models with values of p below 0.10.ll data were analyzed using SPSS 14 (SPSS, Chicago,

llinois).

esults

he perioperative and postoperative characteristics areummarized in Table 2. Myocardial ischemia wasvoided in 101 patients, with a mean cardiopulmonaryypass time of 64 � 33 minutes in those. In the remain-er, cardiopulmonary bypass time was 111 � 37 minutes

p � 0.001). Either as single procedure or in combination,ortic cross-clamping was needed for atrioventricularalve plasty in 17 patients, atrioventricular valve replace-ent in 4, atrioventricular valve closure in 6, complex

ulmonary artery patch plasty in 8, ventricular septalefect enlargement in 1, isolation of hepatic veins ineterotaxy syndrome in 1, and explantation of intrave-ous pacing leads in 1.Two patients (1.5%) died within the first 30 postoper-

tive days. One of them, 23 months old, presenting withongenitally corrected transposition of the great arteries,ypoplastic left ventricle, restrictive ventricular septalefect, and moderate atrioventricular valvar insuffi-iency, died at the first postoperative day in cardiacailure. Previous operations included a systemic to pul-

onary artery shunt with subsequent PCPC. The otheratient, 17 years old, with transposition of the greatrteries, total anomalous pulmonary venous connection,nd hypoplastic right ventricle, died on the ninth post-perative day of multiorgan failure. Previous operations

ncluded correction of total anomalous pulmonary ve-ous connection, with repeat relief of pulmonary venousbstructions and systemic to pulmonary artery shunts.oth patients were excluded from further analysis of the

wo endpoints of length of hospitalization and pleuralrainage. There were 2 hospital deaths at 48 and 128 days

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resented with hetorotaxy syndrome. One of them suc-umbed to multiorgan failure after sepsis. The otheratient, with severely impaired ventricular function, re-uired atrioventricular valvar replacement at the time ofCPC and died after 128 days in multiorgan failure andepsis. These 2 patients were included in the outcomenalysis.Mean pulmonary artery pressure 3 hours after the

peration was 16.5 � 2.2 mm Hg, and decreased signifi-antly to 13.1 � 1.8 mm Hg after extubation (p � 0.001).

The median duration of hospitalization among the 30ays survivors was 20 days (range, 5 to 128). Among 8ariables with values of p below 0.10 in single covariateodels, the multiple covariate model revealed older

ge at the time of extracardiac TCPC (p � 0.040), andigher pulmonary artery pressure 3 hours postopera-

ively (p � 0.013) as risk factors for prolongued hospi-alization (Table 3).

The median duration of pleural drainage among the 30ays survivors was 4 days (range, 1 to 45). Among 12ariables with values of p below 0.10 in single covariateodels, the multiple covariate model revealed longer

able 2. Demographic Characteristics at Time of Extracardiacariables of 132 Patients Undergoing Completion Without Fe

emographic Characteristics

ge at extracardiac TCPC mean � SD, median (range), monthsime between PCPC and extracardiac TCPC mean � SD, mediody weight at extracardiac TCPC mean � SD, median (range)ime on extracorporal circulation mean � SD, median (range),ortic cross-clamportic cross-clamp time mean � SD, median (range), minutesb

iameter of extracardiac conduct mean � SD, millimeterstrioventricular valve repair at the time of extracardiac TCPCtrioventricular valve replacement at the time of extracardiac Tulmonary artery plasty at the time of extracardiac TCPCulmonary artery pressure 3 hours postoperatively mean � SD,ulmonary artery pressure after extubation mean � SD, median

In 15 patients, no partial cavopulmonary connection (PCPC) was performortic cross-clamping included.

able 3. Univariate and Multivariate Analysis of Risk Factor

isk Factor

Univariate

BetaOdd

(95% Confi

ge at extracardiac TCPC 0.01 1.01 (1.0ime PCPC to extracardiac TCPC 0.02 1.02 (1.0ody weight at extracardiac TCPC 0.02 1.06 (1.0ime on cardiopulmonary bypass 0.01 1.01 (1.0ortic cross-clamp time 0.01 1.02 (1.0ulmonary artery pressure 3 hourspostoperatively

0.23 1.25 (1.0

ulmonary artery pressure after extubation 0.19 1.21 (1.0onstant

CPC � partial cavopulmonary connection; TCPC � total cavopulmonary

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ime interval from PCPC to extracardiac TCPC (p �.006), and higher pulmonary artery pressure 3 hoursostoperatively (p � 0.001) as a significant predictors ofleural drainage lasting longer than 4 days (Table 4).No subsequent fenestration was performed, and after

ospital discharge, no significant repeat effusions werebserved.

omment

he idea of a right-to-left “pop-off” communication wasntroduced in the late 1980s. It soon became routine at aumber of centers, and remains so today [7–15]. But has

he advent of the extracardiac approach, and the earlytaging likewise, not made a routine fenestration irrele-ant? Our results on 132 consecutively treated patientsver a period of 6 years confirm this.Indication not to fenestrate was certainly not biased.e simply continued our previously reported approach

4], where we studied 84 patients. If more than 1 riskactor among ventricular function being more than mod-rately impaired, atrioventricular valvar regurgitation

l Cavopulmonary Connection (TCPC) and Surgicalation

Excluded Value

0 56.4 � 55.2, 31.0 (16–251)nge), months 15 25.9 � 25.5, 17.4 (0.3–135)

grams 1 17.6 � 12.2, 12.5 (9–66)tes 0 74.9 � 39.2, 61.0 (69–187)

0 31 (23.5%)0 39.2 � 22.9, 39 (9–94)0 18.7 � 1.40 17 (12.9%)0 4 (3.0%)0 37 (28.0%)

ian (range), mm Hg 12 16.5 � 2.2, 16 (12–23)ge), mm Hg 1 13.1 � 1.8, 13 (9–18)

fore total cavopulmonary connection. b Only patients who underwent

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p Valueioe Limits) Beta

Odds Ratio(95% Confidence Limits)

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ore than moderate, mean pulmonary arterial pressureore than 15 mm Hg, mean atrial pressure higher than

2 mm Hg, pulmonary arterial distortion, or other thaninus rhythm was present preoperatively, the patient wasonsidered a “high risk” candidate. There were no dif-erences between groups of patients having one or moreisk factors in regard to need for intubation (p � 0.511),ulmonary arterial pressures after extubation (p � 0.817),nd duration of chest drainage (p � 0.650). This time, thendpoints of hospitalization duration and pleural drain-ge were dichotomized according to medians. Univariatend multivariate analyses were performed.Of the 132 patients, the majority was relatively young at

ime of extracardiac TCPC, with a median age of 31 months.ur required weight at this stage is around 10 kg. In theseatients, the pulmonary arteries usually have a diameterf around 6 to 8 mm. In our experience, this is largenough to accommodate a tube of 18 mm [6]. In case of aismatch with the inferior caval vein, we found that the

se of an adequate cuff of atrial tissue permits an undis-orted anastomosis with the Gore-Tex tube. Our surgi-al experience, therefore, differs from other studieshat demanded a weight of at least 15 kg for insertionf a tube of at least 18 mm, or where conduits withiameters of less than 18 mm were implanted in smallhildren [17–19]. In the short term, however, our clinicalxperience has not substantiated the generally supportedlaim, that the diameter of the tube should not beversized by more than one fifth of the diameter of the

nferior caval vein [17, 20]. Interestingly, Gupta andoworkers [14] studied a large cohort of 100 consecutiveatients in which 33 were treated with a conduit smaller

han 18 mm in diameter. In half of their patients withonduits smaller than 18 mm, they encountered persis-ent pleural effusions, compared to only a quarter ofatients having conduits larger than 18 mm.It is furthermore of note that a previous partial cavo-

ulmonary connection (PCPC) was accomplished in 117atients (91%), without additional pulmonary blood flow

n any of them. Median patient age at this stage was 9

able 4. Univariate and Multivariate Analysis of Risk Factor

isk Factor

Univariate

BetaOdd

(95% Confid

eft atrial pressure 0.20 1.23 (1.0ge at extracardiac TCPC 0.01 1.01 (1.0ime PCPC to extracardiac TCPC 0.01 1.03 (1.0ody weight at extracardiac TCPC 0.04 1.04 (1.0ime on cardiopulmonary bypass 0.01 1.01 (1.0ortic cross-clamp time 0.01 1.02 (1.0ulmonary artery pressure 3 hourspostoperatively

0.37 1.45 (1.1

ulmonary artery pressure after extubation 0.37 1.15 (1.1onstant

CPC � partial cavopulmonary connection; TCPC � total cavopulmo

onths. Controversy continues over whether additional c

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ources of pulmonary blood flow are beneficial in com-ination with a PCPC. Additional pulmonary artery flow

hrough either an aortopulmonary shunt or the mainulmonary artery may raise upper central venous pres-ure, expose the patient to a higher risk of persistentleural effusions, or may less effectively reduce the vol-me load on the single ventricle [21]. Berdat and col-

eagues [22], however, have concluded that additionalulmonary artery flow has no adverse effect on outcome.hey have included 106 patients from 1996 to 2000, andompared isolated PCPC patients with those who haddditional pulmonary blood flow through the pulmonaryrtery and those with an additional Blalock-Taussighunt. In our view, several issues remain. Firstly, exclu-ion of all other sources of pulmonary blood flow at theime of PCPC aims at volume unloading the functionalingle ventricle, preventing potential functional deterio-ation and reducing atrioventricular valve regurgitation21]. Secondly, pulmonary artery pressures may remainigh. In the Berdat study [22], collective mean pulmonaryrtery pressures were as high as 12 mm Hg, whereas in ourohort, left and right pulmonary artery pressure were lowert time of extracardiac TCPC, with a mean of 9 mm Hg.hirdly, even though patients are less prone to hypoxiaith an additional blood source to the lungs, patients are

imply older at the time of an extracardiac TCPC. Berdatnd colleagues [22] reported that 17 of 47 patients (36.2%)rom the PCPC group, 8 of 26 patients (30.8%) withdditional flow through the main pulmonary artery, andof 17 patients (17.7%) with additional aortopulmonary

hunt underwent completion after 30.9 � 45.8 months,9.7 � 35.4 months, and 24.9 � 18.4 months, respectively.n our collective, the interval from PCPC to TCPC wasather short, with a mean of 25.8 � 25.4 months. Equally,ur statistical analysis therefore supports our strategy ofarly staging, since higher pulmonary artery pressure 3ours postoperatively was a significant predictor for bothutcome parameters in the multivariate model (p � 0.013,� 0.001).Routine fenestration is controversial. Thompson and

Pleural Drainage More Than 4 Days

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tated that a fenestration not only leads to subnormalystemic arterial oxygenation, but that among potentialrawbacks are exposure of the patient to risk, and theosts of subsequent interventions to close the fenestra-ion. The long-term risks associated with chronic right-o-left shunting, formation of thrombus, and potentialaradoxical embolism, have been the major concernsssociated with fenestration. In addition, there is theecrease in exercise tolerance, and consequently qualityf life in patients suffering arterial desaturation subse-uent to fenestration [23–25]. As far as we are aware,

here is only one prospective and randomized trial as-essing the clinical utility of fenestration [26]. Whereas, inhis trial, fenestration was shown to be associated withlinical benefits, the authors stated that not every patientndergoing Fontan palliation requires fenestration tochieve a good outcome.Prolonged pleural effusions represent a clinically im-

ortant cause of morbidity. In our cohort, median drain-ge requirement was 4 days (range, 1 to 45) and medianuration of hospitalization was 20 days (range, 5 to 128).hirty-one (24%) had repeat drainage insertion. Meyernd colleagues [16] reviewed their recent experience in69 patients operated on between January 2000 andecember 2004. Fifty-seven percent were extracardiac

ompletions; however, fenestrations were performed in44 patients. Overall, pleural drainage was necessary in5% for more than 3 days, and in 10% for more than 10ays. In addition, they state that 23 patients (15%) had toe readmitted to the hospital, most commonly for pleuralffusions. Apposed to our approach, in which we performhe extracardiac TCPC usually at a temperature between2°C and 34°C (unless intracardiac procedures are re-uired), they applied a strategy of deep hypothermicrrest in most cases (83%).A very recent publication on another large cohort, 221

atients operated on between November 1988 and No-ember 2003, equally does not report any beneficial effectf fenestrating the extracardiac TCPC [15]. For 52 pa-ients, their main indication for a fenestration was theize of the pulmonary arteries, even though the mean ageas 72.2 months (range, 13.1 to 131.3). Despite their

elative frequency, the basis for the development ofleural effusions after the Fontan procedure remainsnexplained [27]. In our series, multiple covariate logisticegression revealed a longer time interval from PCPC toxtracardiac TCPC as a significant predictor of pleuralrainage lasting longer than 4 days. This finding wouldupport our current approach of early unloading andxtracardiac completion regardless of the patient’s ageut recommending weight of around 10 kg.Our findings differ from the findings of McGuirk and

oworkers [13], who studied 103 patients in whom aateral TCPC had been created in 19 and an extracardiacCPC in the remainder. Among these patients, theyreated 53 fenestrations. Prolonged pleural drainage wasecessary in two fifths. Multivariate analysis identified

ncreased postoperative pulmonary arterial pressure andbnormal pulmonary venous drainage as independent

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s mentioned earlier in the Meyers series [16], in whichhe overall median time on cardiopulmonary bypassven reached 66 minutes (range, 41 to 274), as opposed to1 minutes (range, 29 to 187) in our cohort, prolongederiods of cardiopulmonary bypass were significantlyssociated with increased postoperative volumes of pleu-al drainage in previous studies. Gupta and colleagues14] discussed the exposure to inflammatory sequels.entles and associates [7] report even an increased risk

f early death and failure in combination with prolongederiods of cardiopulmonary bypass. They state that everyffort should be made to limit the duration of cardiopul-onary bypass when completing a Fontan circulation. If

ntracardiac procedures (ie, atrioseptectomy, atrioventri-laur valve plasty) are performed at earlier stages, thelacement of the extracardiac tube can usually be per-

ormed within 35 to 45 minutes of cardiopulmonaryypass. We then routinely use modified ultrafiltration toliminate the crystalloid priming volume and the volumeubstitution during cardiopulmonary bypass. That haseen reported to significantly reduce the incidence ofostoperative pleural and pericardial effusions, require-ent of blood products, and hospital stay after the

ontan procedure [28]. A minimized cardiopulmonaryypass circuit, together with modified ultrafiltration, al-

ows even for extracardiac total cavopulmonary comple-ion in small children without administration of bloodroducts [29].We have shown that conduits of at least 18 mm

iameter can safely be placed in children weighing 10 kg.hat means that completion of the cavopulmonary con-ection can be performed at a young age. In our collec-

ive, 42 patients (32%) had a PCPC and subsequent TCPCelow the age of 24 months. Seventy-six (58%) had aCPC and subsequent TCPC below the age of 36 months.e continue to stage patients at early intervals. Statistical

nalysis supports our current strategy of early unloadingithout additional pulmonary blood. It is, furthermore,ur belief that routine fenestration is dispensable in theajority of patients treated with an extracardiac TCPC.

eferences

1. De Leval MR, Kilner P, Gewillig M, Bull C. Total cavopul-monary connection: a logical alternative to atriopulmonaryconnection for complex Fontan operations. J Thorac Cardio-vasc Surg 1988;96:682–95.

2. Marcelletti CF, Iorio FS, Abella RF. Late results of extracar-diac Fontan repair. Semin Thorac Cardiovasc Surg PediatrCard Surg Annu 1999;2:131–42.

3. Black MD, van Son JAM, Haas GS. Extracardiac Fontanoperation with adjustable communication. Ann Thorac Surg1995;60:716–8.

4. Schreiber C, Kostolny M, Hörer J, et al. Can we do withoutroutine fenestration in extracardiac total cavopulmonaryconnections? Report on 84 consecutive patients. CardiolYoung 2006;16:54–60.

5. Thompson LD, Petrossian E, McElhinney DB, et al. Is itnecessary to routinely fenestrate an extracardiac fontan?J Am Coll Cardiol 1999;34:539–44.

6. Schreiber C, Kostolny M, Weipert J, et al. What was the

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single center performing total cavopulmonary connections?Cardiol Young 2004;14:140–7.

7. Gentles TL, Mayer JE Jr, Gauvreau K, et al. Fontan operationin five hundred consecutive patients: factors influencingearly and late outcome. J Thorac Cardiovasc Surg 1997;114:376–91.

8. Gentles TL, Gauvreau K, Mayer JE Jr, et al. Functionaloutcome after the Fontan operation: factors influencing latemorbidity. J Thorac Cardiovasc Surg 1997;114:392–403.

9. Cochrane AD, Brizard CP, Penny DJ, et al. Management ofthe univentricular connection: are we improving? Eur J Car-diothorac Surg 1997;12:107–15.

0. Bando K, Turrentine MW, Park HJ, Sharp TG, Scavo V,Brown JW. Evolution of the Fontan procedure in a singlecenter. Ann Thorac Surg 2000;69:1873–9.

1. Gaynor JW, Bridges ND, Cohen MI, et al. Predictors ofoutcome after the Fontan operation: is hypoplastic left heartsyndrome still a risk factor? J Thorac Cardiovasc Surg2002;123:237–45.

2. Kumar SP, Rubinstein CS, Simsic JM, Taylor AB, Saul JP,Bradley SM. Lateral tunnel versus extracardiac conduit Fon-tan procedure: a concurrent comparison. Ann Thorac Surg2003;76:1389–96.

3. McGuirk SP, Winlaw DS, Langley SM, et al. The impact ofventricular morphology on midterm outcome followingcompletion total cavopulmonary connection. Eur J Cardio-thorac Surg 2003;24:37–46.

4. Gupta A, Daggett C, Behera S, Ferraro M, Wells W, StarnesV. Risk factors for persistent pleural effusions after theextracardiac Fontan procedure. J Thorac Cardiovasc Surg2004;127:1664–9.

5. Giannico S, Hammad F, Amodeo A, et al. Clinical outcome of193 extracardiac Fontan patients: the first 15 years. J Am CollCardiol 2006;47:2065–73.

6. Meyer DB, Zamora G, Wernovsky G, et al. Outcomes of theFontan procedure using cardiopulmonary bypass with aorticcross-clamping. Ann Thorac Surg 2006;82:1611–8.

7. Alexi-Meskishvili V, Ovroutski S, Ewert P, et al. Optimalconduit size for extracardiac Fontan operation. Eur J Cardio-thorac Surg 2000;18:690–5.

8. Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y, Kitamura

ats.ctsnetjournDownloaded from

weight less than 10 kg. Eur J Cardiothorac Surg 2000;17:543–9.

9. Tokunaga S, Kado H, Imoto Y, et al. Total cavopulmonaryconnection with an extracardiac conduit: experience with 100patients. Ann Thorac Surg 2002;73:76–80.

0. Lardo AC, Webber SA, Friehs I, del Nido PJ, Cape EG. Fluiddynamic comparison of intra-atrial and extracardiac totalcavopulmonary connections. J Thorac Cardiovasc Surg 1999;117:697–704.

1. Chang A, Hanley F, Wernovsky G, et al. Early bidirectionalcavopulmonary shunt in young infants: postoperative courseand early results Circulation 1993;88(Suppl 2):149-58.

2. Berdat PA, Belli E, Lacour-Gayet F, Planche C, Serraf A.Additional pulmonary blood flow has no adverse effect onoutcome after bidirectional cavopulmonary anastomosis.Ann Thorac Surg 2005;79:29–36.

3. Cetta F, Feldt RH, O’Leary PW, et al. Improved earlymorbidity and mortality after Fontan operation: the MayoClinic experience, 1987 to 1992. J Am Coll Cardiol 1996;28:480–6.

4. Jahangiri M, Ross DB, Redington AN, Lincoln C, Shine-bourne EA. Thromboembolism after the Fontan procedureand its modifications. Ann Thorac Surg 1994;58:1409–13.

5. Rosenthal DN, Friedman AH, Kleinman CS, Kopf GS,Rosenfeld LE, Hellenbrand WE. Thromboembolic complica-tions after Fontan operations. Circulation 1995;92(Suppl):287–93.

6. Lemler MS, Scott WA, Leonard SR, Stromberg D, RamaciottiC. Fenestration improves clinical outcome of the fontanprocedure: a prospective, randomized study. Circulation2002;105:207–12.

7. Fedderly RT, Whitstone BN, Frisbee SJ, Tweddell JS, LitwinSB. Factors related to pleural effusions after Fontan proce-dure in the era of fenestration. Circulation 2001;104(Suppl1):I148–51.

8. Koutlas TC, Gaynor JW, Nicolson SC, et al. Modified ultra-filtration reduces postoperative morbidity after cavopulmo-nary connection. Ann Thorac Surg 1997;64:37–42.

9. Hörer J, Schreiber C, Prodan Z, et al. Total cavopulmonaryconnection in a 35-months-old Jehovah’s Witness’ child.

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DOI: 10.1016/j.athoracsur.2007.04.034 2007;84:894-899 Ann Thorac Surg

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