atrial tachycardia in a patient with extracardiac conduit fontan...

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Introduction

Thesurvivalrateofpatientswithcomplexcon-

genitalheartdiseasehasrecentlyimproved,most

likely due to the development of new surgical

techniques and improved perioperative medical

management.1Asthenumberofadultpatients

withcongenitalheartdiseasehasincreased,ar-

rhythmiasandheartfailurearebecominggrow-

ingissuesinthesepatients.1Thus,itisnotsur-

prising that the demand for electrophysiologic

(EP) studiesand radiofrequency catheterabla-

tion(RFCA)isincreasing.EPstudiesandRFCA

arechallenginginpatientswhohaveundergone

extracardiac conduitFontanprocedures for the

palliativetreatmentofcongenitalheartdisease,

becausethesystemicvenousbloodisnotdrained

intotheheartinthesetechniques.Here,were-

portacaseoffocalatrialtachycardia,whichwas

ablatedviaatrans-conduitpunctureinapatient

whohadundergoneanextracardiacconduitFon-

tanprocedure.

Case

A14-year-oldmalepatientvisitedtheemer-

gency room complaining of palpitations for 3

Received: May 28, 2014Accepted: December 15, 2014Correspondence: Jae-Sun Uhm, MD, Department of Cardiology, Severance Cardiovascular Hospital, 50 Yonsei-ro Seodaemun-gu, Seoul, Korea, 120-752Tel: +82-2-2228-8441, Fax: +82-2-2227-7732 E-mail: [email protected] Ko, MD, PhD, Division of Cardiology

ABSTRACT

Electrophysiology (EP) studies and radiofrequency catheter ablation (RFCA) are challenging in patients who have undergone extracardiac conduit Fontan procedures, because of the difficult vascular access. Here, we report on a 14-year-old male patient who underwent extracardiac conduit Fontan procedure for a double-inlet left ventricle, complete transposition of the great arteries, and large ventricular septal defect. The EP study was performed via a trans-conduit puncture. Focal atrial tachycardia originating from the mid portion of the interatrial septum was induced. RFCA of the origin of atrial tachycardia was successfully performed. EP studies and RFCA are feasible via a trans-conduit puncture in patients with extracardiac conduit Fontan circulation.

Key words: ■ atrial tachycardia ■ congenital heart disease ■ Fontan procedure

연세대학교 의과대학 내과학교실 엄 재 선, 김 남 균, 박 진 규, 정 보 영, 박 희 남, 이 문 형

Jae-Sun Uhm, MD1; Nam Kyun Kim, MD2; Jin-Kyu Park, MD1; Boyoung Joung, MD1; Hui-Nam Pak1; Moon-Hyoung Lee1

Departments of Cardiology1 and Pediatric Cardiology2, Arrhythmia Center, Severance Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Korea

Atrial Tachycardia in a Patient with Extracardiac Conduit Fontan Circulation

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38 The Official Journal of Korean Heart Rhythm Society

Figure 1. (A) electrocardiogram during palpitation shows regular narrow QRS tachycardia with short RP interval. (B) electrocardio-gram during sinus rhythm.

A

B

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Figure 2. Cardiac CT shows findings compatible with complete transposition of the great arteries, double-inlet left ventricle, large ven-tricular septal defect and extracardiac Fontan conduit. Ao, aorta; C, Fontan conduit; LA, left atrium; PA, pulmonary artery; RA, right atrium; rV, rudimentary ventricle; V, ventricle.

Figure 3. (A) Fontan conduit angiography (B) Fluoroscopic image of performing trans-conduit puncture (C) Intracardiac echocardiography of the trans-conduit puncture (D) The Brockenbrough transseptal needle and the Swartz transseptal introducer sheath with holding the dilator tip of the Swartz sheath with the snare catheter to prevent it from sliding up along the conduit wall. C, Fontan conduit; HV, hepatic vein; ICE, intracardiac echocardiography; IVC, inferior vena cava; LPA, left pulmonary artery; RA, right atrium.

A B C

Ao RA RAV

rV rVLA LA

V

PA c c

A B

C D

LPA

C

HVIVC

ICE

Needle

Snare

Needle

Snare

Sheath

Sheath

Needle

RA

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hours. He had experienced several episodes of

palpitationsduringthepastyear.Hisbloodpres-

sure was 82/49mmHg. The electrocardiogram

(ECG)showedregular,narrowQRStachycardia

witharateof160beats/minandashortRPin-

terval(Figure1A).Intheemergencyroom,the

tachycardia spontaneously converted into sinus

rhythm(Figure1B).TheQRSmorphologyofthe

tachycardiawassimilartothatinsinusrhythm.

When he was 10 days old, he was diagnosed

withdouble-inletleftventricle(DILV),complete

transposition of the great arteries (TGA), and

large ventricular septal defect (VSD).Whenhe

was5monthsold,thebidirectionalcavopulmo-

naryshuntandinteratrialseptectomywereper-

formedforpalliation.At theageof1year,an

extracardiacconduitFontanprocedurewasper-

formedwiththeautologouspericardium.Wede-

cidedtoperformtheEPstudyfordiagnosisand

treatmentofthetachycardia.Cardiaccomputed

tomography(CT)wasperformedforassessment

oftheheartanatomy,showingfindingscompat-

iblewithTGA,DILV,largeVSD,functionalsingle

ventricle,andextracardiacFontanconduit(Fig-

ure2).

Both femoral veins were punctured. Conduit

angiography was performed with a Berman-

typeangiographycatheter(ArrowInternational,

Reading,PA,USA)(Figure3A).TwoSR-0Swartz

transseptalintroducersheath(StJudeMedical,St

Paul,MN,USA),asnarecatheter(PFMMedi-

cal,Nonnweiler,Germany),andanintracardiac

echocardiography catheter (AcuNav, Siemens,

MountainView,CA,USA)wereinsertedintothe

Fontanconduitviathefemoralveins.ABRK-1

Brockenbroughtransseptalneedle(StJudeMedi-

cal)wasinsertedintotheSwartzsheath,andthe

dilatortipoftheSwartzsheathwasheldwiththe

snarecathetertopreventitfromslidingupalong

theconduitwall(Figure3BandD).Wepunctured

thewallbetweentheconduitandtherightatrium

withtheBrockenbroughtransseptalneedleunder

intracardiac echocardiography guidance (Figure

3C).Right and left atriographywas performed

with thepigtail catheter via the trans-conduit

puncture. A deflectable decapolar catheter (St

Figure 4. Positioning of the EP catheters during basic EP study (A) and during tachycardia mapping (B).

A B

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Figure 5. Intracardiac electrogram of induction (A) and maintenance (B) of tachycardia. The tachycardia was maintained in spite of premature ventricular complex (red box in A) and atrioventricular block (red box in B). The atrioventricular or ventriculoatrial interval was varying.

A

B

I

aVF

V1

HAp

HAd

ABLd

ABLp

Vd

Vp

Stim

Carto

I

aVF

V1

HAp

HAd

Vd

Vp

Stim

Carto

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A B

JudeMedical)wasplacedinthehighleftatrium

viatheSwartzsheathandadecapolarcatheter

(StJudeMedical)wasplacedintheventriclevia

the aorta (Figure 4A). The initial rhythmwas

normal sinus rhythm.During ventricular pac-

ingandsingleventricularextrastimuli,theatrial

electrogramshowedone-to-oneventriculoatrial

conductionwithdecrementalproperties.During

theatrialpacingof240msandinfusionofiso-

proterenolatarateof2μg/min,tachycardiawith

a290mscyclelengthwasinduced.Tachycardia

wasmaintaineddespitethepresenceofapre-

matureventricularcomplexandatrioventricular

block (Figure 5A and B). Therefore, atrioven-

tricularreentranttachycardiacouldbeexcluded.

Duringtachycardia,theatrioventricularorven-

triculoatrialintervalvaried(Figure5B).Itwasnot

compatiblewithatrioventricularnodalreentrant

tachycardia.Thetachycardiawasnotentrainable

byventricularpacing.Thedecapolarcatheterwas

movedtotherightatrium(RA)sideintheFontan

conduit,andanirrigatedablationcatheter(Ther-

mocool, Biosense Webster, Diamond Bar, CA,

USA)wasinsertedintotheatriumviathecon-

duit puncture for tachycardiamapping (Figure

4B).Duringtachycardia,3-dimensionalelectro-

anatomicalmappingwasperformedwithCARTO

(BiosenseWebster,DiamondBar,CA,USA).The

tachycardiawasoriginatingfromthemidpor-

tionoftheremnantinteratrialseptum(Figure6).

Thetachycardiawascompatiblewithfocalatrial

tachycardiaoriginatingfromtheinteratrialsep-

tum.Duringsinusrhythm,wemappedtheHis

bundlepotentials.TheHisbundleareawaslocat-

edinthelowerposteriorpotionoftheinteratrial

septum.Theoriginofthetachycardiawasaway

fromtheHisbundleareaby13.6mm(Figure6B).

WeperformedRFCAof theoriginof theatrial

tachycardiabydelivering30wattsofRFenergy

for90swiththeirrigatedablationcatheterdur-

ingsinusrhythm.Theprocedureendedafterwe

confirmedthatthetachycardiawasnotinduced

Figure 6. LAO view of 3-dimensional electroanatomical mapping shows the origin (black arrow) of focal atrial tachycardia originating from the mid portion of the septum. His, His bundle area; LA, left atrium; LAA, left atrial appendage; LAO, left anterior oblique view; LSPV, left superior pulmonary vein; RA, right atrium; RSPV, right superior pulmonary vein.

RA

RSPVLSPV

LAA LAA

His

LA

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bytheprogrammedelectricstimulationandiso-

proterenolinfusion.Thepatienthadnosymptom

andmaintainedsinusrhythmfor6monthsafter

RFCA.

Discussion

Thecasewasfocalatrialtachycardiaoriginat-

ingfromtheseptuminapatientwhohadunder-

goneanextracardiacconduitFontanprocedure.

WeperformedanEPstudyandRFCAoftheori-

ginofthefocalatrialtachycardiasuccessfullyvia

thetrans-conduitpuncture.

The lifelong prevalence of atrial tachycardia

inpatientswithextracardiacFontancirculation

is approximately 50%,2-4 and it is considerably

higherthaninthenormalpopulation.Inpatients

withextracardiacconduitFontancirculation, it

isdifficulttoperformanEPstudy,becausethe

heart iscompletelyexcluded fromthesystemic

venous system. There were previous case re-

portsofEPstudiesandRFCAviavariousroutes

in patients with Fontan circulation, including

viaatrans-thoracicpuncture,sternotomyap-

proach, trans-apical access and trans-conduit

puncture.5-8TheEPcatheterscanbetransvas-

cularlyplacedvia2pathways:thetrans-conduit

puncture and retrograde transaortic approach.

Theapproachviathetrans-conduitpunctureis

suitableforgainingaccesstotheatriumandthe

retrograde transaortic approach is suitable for

gainingaccesstotheventricle.Itischallenging

topuncturetheFontanconduitbecausefibrosis

formsaroundtheconduit.Inaddition,thecon-

duitwallisvertical—unliketheinteratrialsep-

tum—andthetransseptalneedletendstoslideup

alongtheconduitwallinsteadofpuncturingit.

TheuseofaBrockenbroughtransseptalneedle

whileholdingthedilatortipoftheSwartzsheath

with a snare catheter is a useful method for

puncturing theFontanconduit.9A large-curve

BRK-1transseptalneedleissuperiortoasmall-

curveBRKneedle.Moreover,theradiofrequency

transseptalneedlecanbeagoodoptionforpunc-

turingthefibroticFontanconduit.

Inpatientswithcongenitalheartdisease,the

EPstudyandRFCAareverychallengingbecause

oftheunusualanatomyoftheheart.Inaddition,

itiscommonforpatientstohavevascularanom-

aliesincludingapersistentleftsuperiorvenacava

andinferiorvenacavainterruption.Itisimpor-

tantthattheoperatorbecompletelyawareofthe

anatomy of heart and vessels of each patient.

Everypatienthasauniqueheartstructure,even

thoughthispatientgrouphasthesamediagnosis

ofcongenitalheartdisease.Theoperatorneeds

to review and understand the previous cardiac

surgery and intervention. The operator should

makeameticulousplanfortheprocedure,taking

intoconsiderationthetypesofEPcatheterstobe

usedforeachchamber,pathwaystobeusedfor

positioningoftheEPcatheters,andappropriate

anglesfortheX-raybeamtoimprovevisualiza-

tion.Theoperatorneedstodiscussthecurrent

hemodynamicsand long-termprognosisof the

patientwiththepediatriccardiologists.Giventhe

complexheartanatomy,cardiacCTanda3-di-

mensional electroanatomicmapping systemare

necessaryforguidingtheprocedure.Intracardiac

echocardiography can be helpful for real-time

visualizationof theanatomyandEPcatheters.

Theactivatedcoagulationtimeshouldbemain-

tainedat350–400msbyheparininfusionduring

theEPstudyinpatientswithasingleventricle,

asthecathetersareplacedinthesystemiccham-

bers.

In the present case, the remnant interatrial

septummightbecomearrhythmogenicaftersep-

tectomyduetodegenerativechangesofthein-

teratrialseptum.Thispatientislikelytodevelop

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atrial tachyarrhythmia originating from other

parts of the atrium. In addition, an advanced

atrioventricular block can occur in the future,

althoughtheperi-proceduralECGshowedfirst

degreeatrioventricularblock.Thus,thepatient

willrequirelong-termfollow-up.

Conclusion

EPstudiesandRFCAarefeasibleviaatrans-

conduit puncture in patients with extracardiac

conduitFontancirculation.

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3. Weipert J, Noebauer C, Schreiber C, Kostolny M, Zrenner B, Wacker A, Hess J, Lange R. Occurrence and management of atrial arrhythmia after long-term Fontan circulation. J Thorac Cardiovasc Surg. 2004;127:457-464.

4. Lasa JJ, Glatz AC, Daga A, Shah M: Prevalence of arrhythmias late after the Fontan operation. Am J Cardiol. 2014;133:1184-1188.

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7. Brown SC, Boshoff DE, Rega F, Eyskens B, Budts W, Heidbuechel H, Meyns B, Gewillig M. Transapical left ventricular access for difficult to reach interventional targets in the left heart. Catheter Cardiovasc In-terv. 2009;74:137-142.

8. Dave AS, Aboulhosn J, Child JS, Shivkumar K. Transconduit puncture for catheter ablation of atrial tachycardia in a patient with extracardiac Fontan palliation. Heart Rhythm. 2010;7:413-416.

9. Aoki H, Nakamura Y, Takeno S, Takemura T. A new procedure for a trans-conduit puncture by grasping the dilator tip with a snare catheter: an alternative access method during catheter ablation of supraventricular tachycardias after an extracardiac Fontan operation. Heart Rhythm. 2013. doi: 10.1016/j.hrthm.2013.10.036 [Epub ahead of print] .

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