Permanent TachyPermanent Tachyccardiardiasas

JJ.. JanouJanouššekek

Klinik f. KinderkardiologieKlinik f. Kinderkardiologie

UniversitUniversitäät Leipzig, Herzzentrumt Leipzig, Herzzentrum

MMechanismsechanisms

Ectopic activityEctopic activity

»» FocalFocal (e(ectopicctopic) ) atrialatrial tachycardiatachycardia (FAT(FAT,, AEAET)T)

»» Junctional ectopic (His bundle) Junctional ectopic (His bundle) tachycardiatachycardia(JET) (JET)

ReentryReentry

»» Permanent junctional reciprocating Permanent junctional reciprocating tachycardia (PJRT)tachycardia (PJRT)

Rationale of therapeutic approachRationale of therapeutic approach

Natural historyNatural history

Severity of symptoms Severity of symptoms

Risk Risk of tachycardiaof tachycardia--induced induced cardiomyopathycardiomyopathy

versusversus

Benefits and rBenefits and riskiskss of drug therapy of drug therapy and catheter ablationand catheter ablation

Indications forIndications for RF RF catheter ablationcatheter ablationin childernin childern: : ClassClass II

Resuscitated cardiac arrest in WPW

Syncope in WPW

» Min. preexc. RR <250 msec during AFib

» AC ERP <250 msec

Incessant SVT with ventriculardysfunction

Friedman RA et al., PACE 2002NASPE Expert Consensus Conference

Recurrent symptomatic SVT refractory to drug therapy, age >4 yrs

Cardiac surgery prohibiting furtherapproach to arrhythmogenic substrate (TCPC)

Incessant SVT with normal ventricular function

Indications forIndications for RF RF catheter ablationcatheter ablationin childernin childern: : ClassClass II AII A

Friedman RA et al., PACE 2002NASPE Expert Consensus Conference

Focal Focal ((ectopicectopic) ) atrial tachycardiaatrial tachycardia

AET originating from septal focusAET originating from septal focus

Main featuresMain features

P wave of first beat identical to P wave of first beat identical to subsequent beatssubsequent beats

WarmingWarming--up and coolingup and cooling--down down phenomenonphenomenon

May have AV block during running May have AV block during running tachycardiatachycardia

No induction by pacingNo induction by pacing

AET / adenosineAET / adenosine

EAT – heart rate profileBefore ablation

After ablation

Tachycardia induced CMPTachycardia induced CMP

Prior to therapy 1 year after ablation

Natural history and therapeutic Natural history and therapeutic responseresponse

Tachycardia induced CMPTachycardia induced CMP

»» Higher risk with higher heart rates and Higher risk with higher heart rates and permanent tachycardiapermanent tachycardia

Spontaneous resolutionSpontaneous resolution11

»» <3 yrs.: 78 %, <3 yrs.: 78 %, ≥≥3 yrs: 16 % (p<0.001)3 yrs: 16 % (p<0.001)

Pharmacological controlPharmacological control11

»» <3 yrs.: 91 %, <3 yrs.: 91 %, ≥≥3 yrs: 37 % (p<0.001)3 yrs: 37 % (p<0.001)

Recurrence possible!Recurrence possible!1 1 SalernoSalerno JCJC et al., JACC 2004et al., JACC 2004

AETAET Sinus r.Sinus r.

AET from right upper pulm. veinAET from right upper pulm. vein

AET from right upper pulm. veinAET from right upper pulm. vein

RAO

LAO

JunctionalJunctional ectopic tachycardiaectopic tachycardia (JET)(JET)

1 s

ECGECG

1 s

Main featuresMain features

Congenital, adult and postoperative formCongenital, adult and postoperative form

Congenital formCongenital form»» FamilyFamily history (up to 55.6 %)history (up to 55.6 %)11

»» Progression into CAVBProgression into CAVB2,62,6

»» Spontaneous rate accelerationSpontaneous rate acceleration11

»» High incidence of heartHigh incidence of heart failure (up to 60 %)failure (up to 60 %)11

»» Therapy:Therapy:

–– PropafenonePropafenone33, Amiodarone, Amiodarone11, Amiodarone+IC, Amiodarone+IC44

–– Cave: digoxinCave: digoxin11, proarrhythmia, proarrhythmia44

–– AblationAblation55

Adult formAdult form»» Later in life, betterLater in life, better tolerated, lowertolerated, lower HRsHRs

1Villain E et al. Circulation 1990, 2Henneveld H et al. Heart 1998, 3Paul T et al. J Am Coll Cardiol 1992,4Sarubbi B et al. Heart 2002, 5Fishberger SB et al. PACE 1998, 6Dubin AM et al. HeartRhythm 2004

Permanent Permanent junctional reciprocatingjunctional reciprocatingtachycardia tachycardia (PJRT)(PJRT)

Main featuresMain features

Posteroseptal pathwayPosteroseptal pathway

Retrograde conduction Retrograde conduction onlyonly

Decremental propertiesDecremental properties

IncessantIncessant

Tachycardia induced Tachycardia induced CMPCMP

Adapted using MazgalevTN et al., Circulation 2001

LLong RP, short PRong RP, short PRI

II

III

aVR

aVL

aVF

V1

V2

V3

V4

V5

V6

1 s 1 s

Diagnostic clueDiagnostic clue

300 360 360

180 200 220

PJRT PJRT –– multicenter multicenter studystudy

N = 85N = 85

Age at diagnosis Age at diagnosis 00--20 20 yrs yrs ((median median 3 3 momo))

FollowFollow--up median up median 8.2 8.2 yrsyrs

CHF 28 %CHF 28 %»» resolved with medical Txresolved with medical Tx inin allall

Success of medical TxSuccess of medical Tx: 94 %: 94 %»» amioamiodaronedarone//verapamilverapamil + digoxin+ digoxin

Spontaneous resolution: 22 %Spontaneous resolution: 22 %

Death: 2 pts with persistent LV dysfunctionDeath: 2 pts with persistent LV dysfunction

Vaksmann G et al., Heart 2005

PJRT mapping and ablationPJRT mapping and ablation

354 ms 329 ms

Ventricular extrastimulus

RF energy application

Retrograde block