Arrhythmia Surgery in Patients With and Without Congenital Heart

12 Pages·2008·1.13 MB·English

ORIGINAL ARTICLES: PEDIATRIC CARDIAC
PEDIATRIC CARDIAC SURGERY:
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C
A
Arrhythmia Surgery in Patients With and Without DI
R
A
C
Congenital Heart Disease
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RI
T
A
Constantine Mavroudis, MD, Barbara J. Deal, MD, Carl L. Backer, MD, and DI
E
Sabrina Tsao, MD P
Divisions of Cardiovascular-Thoracic Surgery and Cardiology, Children’s Memorial Hospital, and the Departments of Surgery and
Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois
Background. Arrhythmia surgery has favorably im- try tachycardia in 6, focal atrial tachycardia in 6, and
pacted the clinical course of debilitating atrial and ven- ventricular tachycardia in 13.
tricular arrhythmias in patients with and without con- Results. Operative mortality was 3 (3.0%) due to ad-
genital heart disease. This study reviews arrhythmia vanced associated congenital heart disease. There were 4
mechanisms and documents long-term outcome of pa- late deaths (4.0%) and 2 late cardiac transplants (2.0%).
tients undergoing arrhythmia operations alone or associ- Freedom from arrhythmia recurrence at 1 and 10 years
ated with congenital heart repairs. The analysis excludes was 94% and 85% for atrial arrhythmias, and 85% and
Fontan conversion patients. 68% for ventricular arrhythmias, respectively.
Methods. Between 1987 and 2007, arrhythmia opera- Conclusions. Successful surgical therapy for atrial ar-
tions were done in 11 patients without associated con- rhythmias can be performed safely with a high freedom
genital heart disease and in 89 along with congenital from recurrence rate in patients with and without asso-
heart repairs. Mean age was 15.9 (cid:1) 12.5 years (range, 7 ciated congenital heart disease. Surgical ablation for
days–48 years); 7 were infants (mean age, 23 (cid:1) 16 days). ventricular arrhythmias is less predictive. Complexity of
Resternotomy was performed in 65 (65%). Two func- the underlying congenital heart disease and hemody-
tional ventricles were present in 67 patients; 33 had 1 namic status may contribute to potential arrhythmia
functional ventricle. Arrhythmias included macro-reen- recurrence or new onset arrhythmia manifestation.
trant atrial tachycardia in 45, atrial fibrillation in 11, (Ann Thorac Surg 2008;86:857–68)
accessory connections in 19, atrioventricular nodal reen- © 2008 by The Society of Thoracic Surgeons
The introduction and development of arrhythmia sur- ablative therapy. The purpose of this article is to update
gery in patients with and without associated con- [10] our long-term results of arrhythmia operations and
genital heart disease has enabled clinicians to treat review arrhythmia mechanisms and the principles of
disabling arrhythmias not amenable to transcatheter ab- corrective ablative therapy.
lative techniques [1–8]. Because decreased cardiac out-
put attributable to arrhythmias is compounded by coex-
Material and Methods
isting unrepaired or residual congenital defects, the
initial or reoperative repair must include both physiologic- Between 1987 and 2007, 211 patients had surgical interven-
anatomic correction as well as electrophysiologic correc- tion for arrhythmia. Of these, 111 had Fontan conversions
tion if optimal long-term outcome and beneficial quality reported previously [11–13] and are not included in this
of life are to be achieved [9]. series. Of the remaining 100 patients, 89 underwent ar-
Mechanisms underlying the various atrial and ventric- rhythmia intervention in association with congenital heart
ular arrhythmias have been incompletely understood by repairs and 11 ((cid:1) 18 years of age) had intervention without
congenital heart surgeons owing to case-mix variability
associated congenital heart repair. Mean age at operation
in many congenital heart surgery centers. This leads to was 15.9 (cid:1) 12.5 years (range, 7 days–48 years); 7 were
an incomplete understanding of what is accomplished by infants (mean age, 23 (cid:1) 16 days). Resternotomy was done in
65 of 100 patients (65%). Two functional ventricles were
AcceptedforpublicationApril23,2008.
present in 67. Arrhythmias included macro-reentrant atrial
PresentedattheForty-fourthAnnualMeetingofTheSocietyofThoracic
Surgeons,FortLauderdale,FL,Jan28–30,2008. tachycardia in 45, atrial fibrillation in 11, atrioventricular
nodal reentry tachycardia in 6, concealed and manifest
Address correspondence to Dr Mavroudis, Division of Cardiovascular-
accessory connections in 19, focal (automatic) atrial tachy-
ThoracicSurgery-M/C#22,Children’sMemorialHospital,2300Children’s
Plaza, Chicago, IL 60614; e-mail: [email protected]. cardiain6,andventriculartachycardiain13.Somepatients
©2008byTheSocietyofThoracicSurgeons 0003-4975/08/$34.00
PublishedbyElsevierInc doi:10.1016/j.athoracsur.2008.04.087
CAIDRACCIRTAIDEP
Table1. ClinicalCharacteristicsandOutcomesofPatientsUndergoingModifiedRightAtrialMazeProcedureforMacro-ReentrantAtrialTachycardia 8
5
8
Pts,
Diagnosis No. CongenitalHeartDiseaseSurgery PerioperativeEvents LatePostoperativeEvents AM
RA
Single-ventriclevariants RHVR
Heterotaxy/unbalanced 6 LeftmodifiedBlalock-Taussigshunt(cid:2)pulmonary None BDCPA,pulmonaryvenousstenosis YTHOU
AVC arteryarterioplasty repairinheterotaxypatient MD
InitialFontan–5patients((cid:2)PM[4]orPMlead[1]) ATrecurrence1monthpost-Fontan ATrecurrencewithcardioversion4.5 IAISE
¡overdrivepacing,amiodarone yearspost-op SUTA
inpatientwithdextrocardia, RGL
right-dominantunbalancedAVC E
R
Fontantakedown3dayspost-opin None Y
heterotaxypatient
None Latedeath11monthspost-Fontan
SingleLV 7 TVplasty(cid:2)1½repair(cid:2)PMrevision None Fontan(Uhlanomaly)
InitialFontan–6patients((cid:2)PM[5]orPMlead[1]) None None
SingleRV 9 TVRpost-Fontan(cid:2)PMrevision Latedeathfromventricularfailure
TVRpost-Fontan(cid:2)PMrevision(cid:2)incomplete ATrecurrences3.5and5years
rightmazeduetoretainedlateraltunnel;6th post-TVR
intracardiacprocedure
InitialFontan–7patients((cid:2)PM[5]orPMlead[1]) TVR4.7yearspost-Fontan–1patient
TGA–Prioratrialrepair 1 ArterialswitchoperationwithMustardtakedown VAD1daypost-op¡death2
(cid:2)PMrevision weekspost-op
Priorvalvedconduitrepair 9 PA-VSD,conduitreplacement(cid:2)PM(4) None VT9monthspost-op¡AICD–1patient
TGA,conduitreplacement None None
CCTGA,conduitreplacement None TVR6monthspost-op¡VAD¡
transplant7monthspost-op
Aorticatresia/stenosis,conduitreplacement(2) None None
Truncusarteriosus,conduitreplacement(cid:2)truncal None None
valvereplacement
Initialvalveorconduit 9 TetralogyofFallot,priorventriculotomy;RV-PA None None
placement-Prior conduitplacement(2)orPVR(2)
repair
PA-IVS,priorvalvotomy;RV-PAconduit None None
placement(cid:2)TVannuloplasty
CompleteAVC;TVR(cid:2)mitralvalvuloplasty None None
Tricuspidstenosis,post-valvuloplasty;TVR None None
TricuspidregurgitationinCCTGA,post-AICDfor None Stroke2monthspost-op
VT;TVR
Residualaorticstenosis/insufficiency,residual None None
VSD,priorventriculotomy;TVplasty(cid:2)VSD
patch(cid:2)AVreplacement A
VSD-Priorrepair 2 TVannuloplasty(Ebstein)(cid:2)pulmonaryvalvotomy None None 20nn
0T
ResidualVSD(ofmultiple)(cid:2)pulmonaryartery None None 8;8ho
arterioplasty 6:85rac
Initialrepair 1 PartialAVC None None 7–Su
68rg
AnnThoracSurg MAVROUDISETAL 859
2008;86:857–68 ARRHYTHMIASURGERY
yyy(cid:4) had more than one arrhythmia and were treated with
a;AT monarmonarmonarVT techniquesappropriatefortheparticularsubstrates.Clini-
EventsLatePostoperativeEvents PM3yearspost-opforbradycardi¡at5yearstranscatheterRFAatrioventricularnode 3Recurrences;1newonsetVT 2Latedeaths;1latetransplant (cid:4)(cid:4)atrioventricularcanal;BDCPAbidirectionalcavopul(cid:4)monaryatresia-intactventricularseptum;PA-VSDpul(cid:4)(cid:4)RVrightventricle;RV-PArightventricle-to-pul(cid:4)ntricularassistdevice;VSDventricularseptaldefect; crudaiicapftcctwnnooeeayaobfryaatrrmcsclraeAImltneimstludnainz,fuareailetrifpaaclsobeidnamrratotysrtedipeititftioolunnonesoiqucniacrmttcnmccgurttyopcohEipciee.elieparopelsbradsdnneEasrtomaeebseuseroocttxcr,tlohstfkdrfppceytoeeupiryl.i,eadrrnrhaludnaotIomralugthandiallfamaoraspdrmttietprbernaeanrriihidytvaaailrsdsoiseaaay,artseoeymetwithltiestt.ipeminqhohhe,oyfiTeaneaumnleantpworlbertenhyatessartncirthdmt,oatoaaiteanfeiilrmapcanfvclriwioemnahainehavedsopltt,dayteecheinenihcpscldcotnereuaphpaoyaoagnnonstditasartmefceut.eoieiditotesnenaosdistpiidrenriwclhanitwoldneatinieataencsagmitcridxdaorstloaycwiheTulventfeopiaymciacasngspftmnrrhtirbsoheiberuoaie,uoalchseorlpfdewhnrtnaeahtsyispiyaelittvsteetisilrinhmta1anevsmynmaaoramtegbntrrofthwotrohhiolmsaiedaedweaercyemnl6layye.araaat.hdrnetcanhttpystaIeehasaamnpacppdmotfeammhnhaieenitnn5aioeyaetrriisldgo4ccsr-------, PEDIATRICCARDIAC
ative ality AVC(cid:4)pulation;(cid:4)ve fdoyrnmaemdicini5n1sptaabtiielintyts;dthuorisnegwiatrhrhatyrtihamlfiibariwllaetrioeneoxrchluedmeod-.
Perioper None 1operativemort (cid:4)AVaorticvalve;eftventricle;PA-IVS(cid:4)FAradiofrequencyablvereplacement;VAD MhpwbblleeeaaaSlrrttdpftiieoaooprnpnnuimndrsooigeaenbfdrdwdea,thwfwafoesesihrutchepitracaegeh2rdirmlcfydiaoanueltrpcrclmtlaiatuiaerppeldtcdlohoeeol-drnafeariavorqrrrpeeuulanysrreyoetsreicbniextceipfpuosotie.hnlcrarea,irreaissdtnroieriscarlhseailyuetwitsseohoanwm[c1s,ki–ita;pah8nei]td.raafbiCoschlrrraamyyntnooiooedaawdn---
ongenitalHeartDiseaseSurgery (cid:4)ATmacro-reentrantatrialtachycardia;(cid:4)transpositionofthegreatarteries;LVl(cid:4)PVRpulmonaryvalvereplacement;R(cid:4)(cid:4)TVtricuspidvalve;TVRtricuspidval upspTeotFtmphhiiroseraeotaeeoMinhrtcmnncrtbhteeaasrgSaaoenrron.pcundsno3-Saayrr)ri-lontifiogeto,onp-ofi[eegi5mrcc1rsndaei-eai3tpe,ntiethl]crrsr2lancimiaaI0gainsItoncnp0nrulhudercat3lecbtes,n,a,l1ass6peawSttM5tt0racbrrfh-ai-etoatimalostreaiatrreleihnleltcimtntcaiaamstoaaoortl:vnlncsénncatepaihaaimzd(tarCylwuceeoc,thcpTsaimuilQpeateyeur)lhrnrldaisculyodcasitaréoasciemtri2abpelnnsdlw(cid:3)edayrsaiatvocaliurh6nlte,cbeaehr0e(ondCteeFa°bmtsC1sbiattra(cid:3)gehhlinp(svreceFa11feaielloee5er)tlddnpiris0sxwwngata)o°r9e)itaCpaotni.[h0acrs7sra-orsl]ttsesaa(.hniptCCeebhesi(ceAncrrpltaoacyynastrbttins,oonoifieltmCdCCadrhwdcmtaoeaaaifilbvrosotrtteahdhhyyeer--l
Pts,No.C 1None 45 dioverterdefibrillator;congenitallycorrected(cid:4)PMpacemaker;thegreatarteries; iuaratmhertlraeceMpittaueiuhaomoletcnshfpreaoeisasln-ltatrobciactelwlrukoeiveodnccevketaoire.nrnatnoP,hndtuotertuhnitcecaoedutntrirlcinotaifiioanaracrltloneuttasonoranagdacolaerehfytot.yohtrchsmeaciernrriceduvwaiseoait,ntebhadacsnietaraoocnccbmulaseaivtatsaraslecoielanrue,essootqhrftpuoiefiuuirscnrletmieiigdmaohoin---ft
Table1.(Continued) Diagnosis Nostructuralheartdisease TOTAL (cid:4)AICDautomaticimplantablecar(cid:4)arteryanastomosis;CCTGAatresia-ventricularseptaldefect;(cid:4)artery;TGAtranspositionofventriculartachycardia. notbnstisanriylnvaaaoioescarrranwtruryylte,tiiossemsvctvmp,crieoessiianjidncsiurcnuo(dxovfoasueurter,nboasicndfinsapmtrouticuoeaouleetsljecneuussilfttt)etsroior-n.soliesrensn,Ccitstdtuurriohteoeoenrhreandntfirectd,esaehtsistlohorureapoererifnpoacrtptelntaihfiairsrsdoteititberoornihiprniramramuotglilasmnvilutilybaecsrsd,pla,niaosoeptlmasichffoeakeeatwcnnn.ciryasdstneeAvs/,aalupualoracg:le,aerrtuetateaethsascshn,l.hseaeaboIancaotnpmentrarsrtuedtwaaeifoalcodlrmmeasovtsdheluseooliinyeacss--f
860 MAVROUDISETAL AnnThoracSurg
ARRHYTHMIASURGERY 2008;86:857–68
Table2. ClinicalCharacteristicsandOutcomesofPatientsUndergoingCox-MazeIIIaProcedureforAtrialFibrillation
Patients,
Diagnosis No. CongenitalHeartDiseaseSurgery PerioperativeEvents LatePostoperativeEvents
P Singleventriclevariants
E
D Heterotaxy 1 Mitralvalvuloplasty,1½ventricular None None
IA repair,ascendingaortaaneurysm
T
R repair(post-Konno)(cid:2)PM
IC revision
CA SingleLV 2 InitialFontanat9years(cid:2)initialPM None None
R
D PA-IVSpost-RVOTpatch;TVR,1½ None None
IA ventricularrepair,RV-PA
C conduit(cid:2)PMleadonly
SingleRV 1 aMultisiteATPM(removalAICD) Death10dayspost-op
Priorrepair/palliation 5 Aorticstenosispost-AV/MV None ORT10monthspost-op
replacement,AICD;Konno(cid:2)AV/
MVreplacement(cid:2)LVaneurysm
repair
CompleteAVC;mitralvalvuloplasty PM10dayspost-op None
PartialAVC;mitralvalvuloplasty(cid:2) None None
redopatchrepair(cid:2)initialPM
Mitralregurgitation None None
post-valvuloplasty;MV
replacement
PS-TS;takedownGlenn(cid:2)PVR(cid:2) None ATepisodes1,2.5,and2.8
RVOTpatch(cid:2)PM yearspost-op
Initialrepair 2 SinusvenosusASD(cid:2)PAPVCrepair None None
(cid:2)initialPM
Mitralvalvuloplasty, None None
cardiomyopathy
Totals 11 1operativedeath 1new-onsetORT;1
new-onsetAT
aPulmonaryisolationbyepicardialcryoablationwithoutcardiopulmonarybypassbecauseofhemodynamicinstability.
AICD(cid:4)automaticimplantablecardioverterdefibrillator; ASD(cid:4)atrialseptaldefect; AT(cid:4)macro-reentrantatrialtachycardia; AV(cid:4)aortic
valve; AVC(cid:4)atrioventricularcanal; LV(cid:4)leftventricle; MV(cid:4)mitralvalve; ORT(cid:4)orthodromicreciprocatingtachycardia; PA-IVS(cid:4)
pulmonary atresia-intact ventricular septum; PAPVC (cid:4) partial anomalous pulmonary venous connection; PM (cid:4) pacemaker; PS-TS (cid:4)
pulmonarystenosis-tricuspidstenosis; PVR(cid:4)pulmonaryvalvereplacement; RV(cid:4)rightventricle; RV-PA(cid:4)rightventricle-to-pulmonary
artery; RVOT(cid:4)rightventricularoutflowtract; TVR(cid:4)tricuspidvalvereplacement.
as a natural target for the interruption of a macro- wasusedtotreatatrioventricularnodalreentrytachycar-
reentrantcircuit. dia (Fig 2). Accessory connections (Fig 3A and B) were
The goal of cryoablation therapy is to transform an divided by both epicardial and endocardial resection
area of slow conduction to an area of no conduction by techniques. Focal (automatic) atrial tachycardia (Fig 4)
interrupting the electrical corridor (isthmus) between wastreatedbyisolation,resection,orcryoablationofthe
adjacent obstacles or scars, while preserving sinoatrial affected atrial tissue [3]. Ventricular tachycardia was
and atrioventricular nodal function. A patient with a ablated by a combination of endocardial resection and
separate hepatic vein entry into the right atrium, for cryoablation, or epicardial resection and cryoablation
example,willrequirecryoablationlesionstoconnectthe basedontheidentifiedpathology[5].
inferiorvenacavaostothecoronarysinusos,thehepatic Postoperative electrophysiology studies were per-
veinostothecoronarysinusos,andthehepaticveinos formedinallsurvivingpatientsbeforehospitaldischarge
tothetricuspidannulus;remaininglesionsarestandard except those with atrial fibrillation or focal atrial tachy-
fortheright-sidedmazeprocedure.Theseprinciples(Fig cardia.Postoperativepacingprotocolsforatrialtachycar-
1A) can be applied to all other natural and created dia included atrial incremental pacing, and single, dou-
obstacles; it is important to recognize that these princi- ble,andtripleatrialextrastimulationinaminimumpaced
plesdonotapplytofocalatrialtachycardias,whicharise cycle length of 200 milliseconds. Patients with ventricular
fromdiscretefoci. tachycardia underwent provocative stimulation consisting
AtrialfibrillationwastreatedbyleftatrialCox-mazeIII ofsingle,double,andtripleextrastimulationattwopaced
procedureinadditiontomodifiedorstandardrightatrial cycle lengths from both the right ventricular apex and
maze,dependingontheanatomicsubstrate[7,8],usinga outflow tract. Postoperative studies were performed in
combinationofincisionsandcryoablationlesions.Selec- baselinestateandduringisoproterenolinfusion.
tive cryoablation modification of the atrioventricular Consistent with our previously reported protocols,
nodal slow pathway in the region of the coronary sinus patients with atrial arrhythmias (other than atrial fibril-
AnnThoracSurg MAVROUDISETAL 861
2008;86:857–68 ARRHYTHMIASURGERY
Table3. ClinicalCharacteristicsandOutcomesofPatientsUndergoingSurgicalAblationforAccessoryConnection-Mediated
Tachycardia
CongenitalHeartDisease PostoperativeArrhythmia
Diagnosis #Pts Surgery PerioperativeEvents Occurrence
C
A
Singleventriclevariants DI
Heterotaxy 1 InitialFontan(cid:2)initialPM(cid:2) None ORT(cid:1)1year¡transplant3 R
A
mazeforAT yearspost-op C
SingleLV 2 InitialFontan Repeatsurgicalablation WPWrecurrence3yearspost-op C
4dayspost-op RI
T
TVplasty(cid:2)1½ventricularrepair None AT3yearspost-op DIA
Ebstein 3 Ebsteinrepair(withPA-IVS) Intraoperativedeathin E
P
11-day-old
Ebsteinrepair(cid:2)VSDrepair None AT18monthspost-op,successful
transcatheterRFA5years
post-op
ASDsecundumrepair None None
TGA–Priorarterial 1 MVreplacement(cid:2)PMrevision None None
switch
Doubleoutletright 1 Intraventriculartunnelrepair None None
ventricle–Prior
pulmonaryartery
band
Initialrepair 3 PartialAVCrepair None None
ASDsecundumpatchrepair None None
Scimitarsyndromerepair Repeatsurgicalablation LVaneurysmrepair5months
8dayspost-op post-op
Nostructuralheart 8 Repaircatheteraorticperforation None Recurrence3monthspost-op–
disease (cid:2)limitedepicardialmapping leftaccessoryconnection
andepicardialcryoablation
Noassociatedsurgery(7) Repeatsurgicalablation None
4dayspost-op–1
patient
None–6patients Repeatsurgicalablation9
monthspost-op–1patient
PM3.5yearspost-op–1patient
Totals 19 1operativedeath 4recurrences;2new-onsetAT
1latetransplant
ASD(cid:4)atrialseptaldefect; AT(cid:4)macro-reentrantatrialtachycardia; AVC(cid:4)atrioventricularcanal; LV(cid:4)leftventricle; MV(cid:4)mitral
valve; ORT(cid:4)orthodromicreciprocatingtachycardia; PM(cid:4)pacemaker; PA-IVS(cid:4)pulmonaryatresia-intactventricularseptum; RFA(cid:4)
radiofrequency ablation; TGA (cid:4) transposition of the great arteries; TV (cid:4) tricuspid valve; VSD (cid:4) ventricular septal defect; WPW (cid:4)
Wolff-Parkinson-Whitesyndrome.
lation)received(cid:5)-blockadefor3monthspostoperatively; reported [10]. The third occurred in a 36-year-old man
patientswithatrialfibrillationreceivedamiodaronether- with an unoperated on univentricular heart and inces-
apyfor3monthspostoperatively.Patientswithinducible santatrialfibrillationcausingseveredecompensation.He
sustained ventricular tachycardia postoperatively re- underwent epicardial cryoablative pulmonary venous
ceivedimplantabledefibrillators.Arrhythmiarecurrence isolation because of reticence to perform a Cox-maze
was assessed at routine visits by review of symptoms, procedure, which would require cardiopulmonary by-
comprehensiveelectrocardiographicmonitoring,andin- pass.Hehadrapidrecurrenceanddied10daysafterthe
terrogation of pacemakers or implanted defibrillators. operation.
Consulting statistician constructed freedom from ar- Latedeathsoccurredin4patients(seeTables1,4);one
rhythmiarecurrencegraphsusingthelife-testprocedure deaththatoccurredafterattemptedtakedownofaMus-
product-limit survival estimates. Institutional Review tardprocedurewaspreviouslyreported[10].
Boardapprovalforthisretrospectivestudywasobtained, Operations were performed as emergencies in 2 sur-
andtheneedforinformedconsentwaswaived. vivingpatientswhohadacutehemodynamicdecompen-
sation. One patient without congenital heart disease
presented with cardiogenic shock due to focal atrial
Results
tachycardia and was brought to the operating for an
Surgical Outcome emergencyventricularassistdevice.Heunderwentelec-
Three patients died, for an operative mortality of 3% trophysiologicmappingoftherightatriumandresection
(Tables 1, 2, and 3); two deaths have been previously ofthearrhythmogenicfocusintherightatrialappendage
862 MAVROUDISETAL AnnThoracSurg
ARRHYTHMIASURGERY 2008;86:857–68
Table4. ClinicalCharacteristicsandOutcomesofPatientsUndergoingPathwayModificationforAtrioventricularNodal
ReentryTachycardia
Patients, Perioperative
Diagnosis No CongenitalHeartDiseaseSurgery Events LatePostoperativeEvents
P
E
D Single-ventriclevariants
IAT Heterotaxy 3 InitialFontan(cid:2)PMleadonly None None
RIC InitialFontan(cid:2)initialPM None None
C Pulmonaryvenousstenosisrepair(cid:2) None Pulmonaryvenousstenosis¡death
A mitralvalvuloplasty(cid:2)initialPM 2monthspost-op
R
D (post-BDCPA,TAPVCrepair)
IA TGA—prioratrialrepair 2 Re-doatrialbaffle(cid:2)PM None Arterialswitchelsewhere11months
C post-op¡death
Arterialswitchoperationwith None None
Senningtakedown(cid:2)PM
PS-IVS—priorvalvuloplasty 1 RV-PAconduitplacement(cid:2)TV None None
ringannuloplasty
Totals 6 2latedeaths
BDCPA (cid:4) bidirectional cavopulmonary artery anastomosis; PM (cid:4) pacemaker; PA-IVS (cid:4) pulmonary atresia-intact ventricular
septum; RV-PA(cid:4)rightventricle-to-pulmonaryartery; TAPVC(cid:4)totalanomalouspulmonaryvenousconnection; TGA(cid:4)transpositionofthe
greatarteries; TV(cid:4)tricuspidvalve.
withconversiontonormalsinusrhythm.Noassistdevice curred in 17 of 100 (17%) patients. Three patients with
was implanted; gradual normalization of ventricular accessoryconnectionswhowereoperatedonearlyinour
function ensued without arrhythmia recurrence. An- series(1989to1991),beforetranscatheterablationproce-
other patient with a left-sided accessory connection dures were standard therapy, underwent a second sur-
and aortic perforation during a transseptal procedure gicalablationpostoperativelyat4,4,and8days,respec-
before catheter ablation had limited epicardial map- tively, because of recurrent tachycardia; 1 patient had
ping with epicardial cryoablation. Accessory connec- laterecurrence.Afourthpatient,alsoearlyintheseries,
tion function later recurred and was treated with underwent surgical ablation 9 months postoperatively
(cid:5)-blockingmedication. because of late recurrence of accessory connection-
The median postoperative hospital stay was 9 days mediated tachycardia. Additional patients underwent
(range, 4 to 278 days). Early postoperative surgical com- earlyreoperationforventricularassistdeviceplacement,
plications requiring return to the operating room oc- Fontantakedown,controlofbleeding,thoracicductliga-
Table5. ClinicalCharacteristicsandOutcomesofPatientsUndergoingLesionAblation/ResectionforFocalAtrialTachycardia
Patients, CongenitalHeartDisease
Diagnosis No. Surgery PerioperativeEvents LatePostoperativeEvents
Singleventriclevariants
SingleRV 1 Norwood None BDCPA;extracardiacTCPC(cid:2)
PMlead;bradycardia¡PM
7.3yearspost-op
InitialRepair 4 TetralogyofFallot;repair None None
withsubvalvarRVOT
patch(cid:2)cryoablationof
focalatrialtachycardia
site(cid:2)isthmus
cryoablationforAT
MultipleVSD None None
ASDsecundumpatchrepair None Recurrenceat5.75yearspost-op
¡metoprolol
Patentforamenovale None None
primaryclosure
Nostructuralheart 1 None,inpatientwith Focalatrialtachycardia Recurrence10monthspost-op¡
disease associatedatrial recurredimmediate dofetilide;transcatheterRFA
fibrillation,AT post-opperiod¡ (cid:6)2atleftpulmonaryveins2
transcatheterRFA yearspost-op
TOTALS 6 2recurrences
ASD (cid:4) atrial septal defect; AT (cid:4) macro-reentrant atrial tachycardia; BDCPA (cid:4) bidirectional cavopulmonary artery anastomosis; PM (cid:4)
pacemaker; RFA(cid:4)radiofrequencyablation; RV(cid:4)rightventricle; RVOT(cid:4)rightventricularoutflowtract; TCPC(cid:4)totalcavopulmonary
arteryconnection; VSD(cid:4)ventricularseptaldefect.
AnnThoracSurg MAVROUDISETAL 863
2008;86:857–68 ARRHYTHMIASURGERY
Table6. ClinicalCharacteristicsandOutcomesofPatientsUndergoingEndocardialResection/CryoablationforVentricular
Tachycardia
Patients, CongenitalHeart
Diagnosis No. DiseaseSurgery PerioperativeEvents LatePostoperativeEvents
C
A
TetralogyofFallot 6 DI
Postrepair,w/ventriculotomy RV-PAconduit None None R
A
replacement(2) C
RV-PAconduit InducibleVT¡AICD7 AICD3yearspost-op C
replacementand dayspost-op RI
T
PMremoval A
PVR(initial)(cid:2)PM None New-onsetAT4yearspost-op DI
E
revision P
PVR(cid:2)RVaneurysm None New-onsetAT10yearspost-op
repair(cid:2)AICD
revision
Postrepair,no RVOTobstruction None None
ventriculotomy resection(cid:2)patch
augmentation
TGA,VSD-LVOTO 2 RV-PAconduit None None
post-Rastelli replacement(cid:2)
maze(cid:2)PM
revision
RV-PAconduit InducibleVT¡AICD8 RecurrentVT;dilated
replacement(cid:2) dayspost-op cardiomyopathy¡transplant
maze(cid:2)PM candidate
removalfor
plannedlater
AICD
ASDsecundum-Post- 1 TVringannuloplasty None RecurrentVT4monthspost-op;VT
repair,withsevereRV (cid:2)RVaneurysm catheterablation1.8years
cardiomyopathy(Uhl repair(cid:2)1½ post-op;subsequentrecurrentVT
anomaly) ventricularrepair
(cid:2)rightmaze
procedureforAT
(cid:2)AICD
Initialrepair 3 RV-PAconduitfor -Ligationthoracicduct None
absentpulmonary 1mopost-op
valve
VSDpatchrepair None None
VSDpatchrepair None -NewonsetAT1.8yearspost-op
¡successfultranscatheterRFA
Nostructuralheartdisease 1 None None
TOTALS 13 2recurrences;3new-onsetAT
AICD (cid:4) automatic implantable cardioverter defibrillator; ASD (cid:4) atrial septal defect; AT (cid:4) macro-reentrant atrial tachycardia; PM (cid:4)
pacemaker; PVR (cid:4) pulmonary valve replacement; RFA (cid:4) radiofrequency ablation; RV (cid:4) right ventricle; RV-PA (cid:4) right ventricle-to-
pulmonaryartery; RVOT(cid:4)rightventricularoutflowtract; TGA,VSD-LVOTO(cid:4)transpositionofthegreatarteries(cid:2)ventricularseptaldefect(cid:2)
leftventricularoutflowtractobstruction; TV(cid:4)tricuspidvalve; VSD(cid:4)ventricularseptaldefect.
tion, sternal instability, delayed sternal closure, and de- rencecalculations.Theywerepresentatfollow-upinan
viceplacementin6orrevisionin2. additional8patients(Tables1to6).
Twopatientsrequiredcardiactransplantationlateafter
the operation (Tables 1, 3). A third patient is under
Comment
considerationforcardiactransplantation(Table6).
Freedom from arrhythmia recurrence, as determined In our previous report of 29 patients [10], we demon-
bydocumentedpersistentclinicalrecurrencemorethan strated that arrhythmia surgical interventions in associ-
3monthspostoperatively[14],was94%and85%foratrial ationwithinitialandreoperativecongenitalheartrepairs
arrhythmiasat1and10years,respectively,representing are effective, efficacious, and in some cases, life saving.
nine recurrences in 87 patients (10%; 3 macro-reentrant The surgical arrhythmia techniques for accessory con-
atrial,4accessoryconnection-mediated,and2focalatrial nections, focal atrial tachycardia, and atrioventricular
tachycardias), and 85% and 68% for ventricular arrhyth- nodal reentry tachycardia have been well described in
mias at 1 and 10 years, respectively, representing two patients with structurally normal hearts [1–3] and in
recurrencesin13patients(15%;Fig5). patients with 2 ventricles and simple associated heart
New-onset tachycardias were not included in recur- defects such as atrial septal defect [4–8]. This series
864 MAVROUDISETAL AnnThoracSurg
ARRHYTHMIASURGERY 2008;86:857–68
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Fig1. (A)Cavotricuspid-isthmusdependentmacro-reentrantatrialtachycardia.Asdepicted,the“playingfield”istherightatrium,whereapre-
matureatrialcontractionmightencounterblockintheatrialseptum(brokenline)andproceedinanalternateroutedowntherightatrialfreewall.
Thewavefrontmayencounteranareaofslowconduction(squigglyarrow),inthiscasebetweentheinferiorvenacava,tricuspidvalve,andthecor-
onarysinus(CS).Thedelayencounteredasthewavefronttraversestheareaofslowconductionallowstheatrialseptumtorecoverconduction.The
wavefrontexitstheisthmusandproceedsuptheatrialseptum.Interruptionofthiscircuitistargetedattheinferioristhmusduetotheclearlyidenti-
fiedlandmarksinproximity.(AV(cid:4)atrioventricular;SA(cid:4)sinoatrial.)(B)Schematicrepresentationofthepossiblelinesofablationtotreatmac-
ro-reentrantatrialtachycardiainthepresenceofvariousatrialanomaliesassociatedwithcomplexcongenitalheartdisease.Theseatrialanom-
aliesdonotgenerallyoccurtogether,andthedemonstratedlinesofblockarenotmeanttobeincorporatedintoeveryoperation.Theyarede-
pictedonlyasguidelinesonwhichtobaseanablativeoperationwhenunusualanatomicobstaclesareencounteredintheperformanceofthe
mazeprocedure.(avn(cid:4)atrioventricularnode;CS(cid:4)coronarysinus;FO(cid:4)foramenovale;HV(cid:4)hepaticvein;IVC(cid:4)inferiorvenacava;LAA
(cid:4)leftatrialappendage;LSVC(cid:4)leftsuperiorvenacava;MV(cid:4)mitralvalve;PV(cid:4)pulmonaryveins;RAA(cid:4)rightatrialappendage;RSVC
(cid:4)rightsuperiorvenacava;TAPVR(cid:4)totalanomalouspulmonaryvenousreturn;TV(cid:4)tricuspidvalve.)
describesourattempttoapplyablativeprinciplesdevel- rhythmia to occur using a different pathway. Hemody-
oped in structurally normal hearts to complex forms of namic variability, preoperative ventricular dysfunction,
congenitalheartdiseaseandcriticallyillpatients. andhesitationtoprolongthecross-clamptimeforcom-
Our arrhythmia recurrence rate for atrial tachycardia plexintracardiacproceduresledustoperformanabbre-
in congenital heart patients (Fig 5) compares favorably viatedarrhythmiaoperationin3patientswhosearrhyth-
with other reports [15–17]. The recurrences can be ex- mias recurred. Whether more aggressive operative
plained by anatomic, electrophysiologic, and hemody- solutions would have resulted in better outcomes or
namic variability that present significant challenges to given rise to adverse events must be left to speculation.
the principles of arrhythmia ablation. Important ana- The outcomes do underline the necessity of performing
tomicvariabilitythatimpactedarrhythmiaablativetech- established arrhythmia operations; abbreviated proce-
niques included bilateral venae cavae, separate atrial duresarelikelytoresultinrecurrence.
entryofthehepaticveins,anomalouspulmonaryvenous The characteristics of our patients with macro-
return,absenceofthecoronarysinus,commonatrioven- reentrant atrial tachycardia include those with prior
tricularvalve,juxtaposedatrialappendages,anddilated operations and atrial enlargement due to hemodynamic
coronary sinuses. These variations tended to occur in aberrations(Table1).Thelargestcategoryinthisgroupis
diagnosesknowntohaveanomalousconductionsystems patients with a univentricular heart who underwent
and poorly defined accessory connections such as criss- first-time Fontan operations. Other categories include
crossheartwithsingleventricleandstraddlingatrioven- patients who had reoperation for tetralogy of Fallot/
tricularvalve,singleventriclewithheterotaxy,andsingle double-outlet right ventricle or conduit replacement for
ventriclewithl-transposition. rightventricular-to-pulmonaryarterycontinuity,whicha
Most of our recurrences tended to be in patients with large multicenter study showed was related to late ar-
residual accessory connections and macro-reentrant rhythmia development and late sudden death [18]. Risk
atrialtachycardiasinwhompreoperativeandimprovised factors associated with these conditions establish the
intraoperativeelectrophysiologicstudiespresentedvari- substrate for macro-reentrant atrial tachycardia propa-
ous challenges. It was not uncommon to treat a patient gatedbyanareaofslowconduction,whichcouldbethe
for one arrhythmia only for another and unrelated ar- coronary sinus-inferior vena cava isthmus or around a