Summary and Conclusions

Summary and Conclusions
r2
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A-a
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In C:hspter I we present the history of Lhe mosi lmpontmt p~lbl~shed
data on rhe isslue
ol'vas,anis ofpm-e-zxci&t~on,Ilighlighting duagnosr~caspects and tlaerapy
inlccordlng to Webqter's New Dlcliotrary the term wrralat means a ~nar~ifestlng
vdrrety.
sorneth~iiyvarying "sl~glitly" from the standard ilbr~n.'The so-called Maharm fibers
In thclr p r o x ~ n ~ aand/
l
or distal
are connect~nrgstiuctures wltll a great varnab~l~ty
Inscnluns, but shartn~ga common electrophys~oBogrcalhehavlor suggestrve of an AW
nodal-llkc slructure. THic term variant 1s more appropruately appllrd for their
elcc~rophys~ologl~c
profile Lhsu7 tl~eir anatomic slruclure Tlie main source ot' the
c o n c e p t ~ ~~rr~slnatch
il
between anatomy and luncl~onoi t h o ~ cstcuciures denwes Tram
the Faen thal Ivan Rilahn~niortglnally descr~bcdcolrnectlons between the A-V i ~ o d c
and bundle hrmches or ~ientrlicularmyncardrurm, uhlch bypassed the M I ? hundle, ~ r i
pathologlo specllnens. Whet-i othen authors obserled accessory bypass tracts 1 ~ 1 tlong
h
and decrernental c o ~ ~ d ~ ~ cthey
t ~ o rassumed
i,
that ilac substrate %as [Ire fibers descnhed
by Maliaum.
'Two of the orlgunal stnrchres dcscrtbed by Mahain), the nodovcntrlcul;~ and
I~~~CIOIBSC
fibers
I C L Iare
I ~ ~~ i ~ hte111.
r c s as slliowl~Esy rare case repolls The rnajoruty uT
p:rtrcnts 1\<1Lhatlir~ogradelydccrenietitally conduclung accessory pathways arc Inday
cPassllie~4trs l ~ m , ~calhc~
t ~ g an. all.~ol'~scncularpathway or 3 long alr~ovcrrlr~cular
puthw,~ywldlr drcrciallcnlnll pnopelllcs I'licrsr: pallelrts ~~rwally
show a pre-eucttcd IcR
br~nidlcErralach ImlouL biiapcd QlZS during ~acbycardta
Our aulu I I I Chrrpteu 2 i v ~ s20 dc<crtbc the d~flklcntlalcl~~lgnos~s
of a IeTt btmdlc.
hoLu~achblirck xncllycard~nurtli rmplla.cls on3 the elecLtucardlugraphic IEe,ltures wl11uh
~ C I I IR
c, hzlplbl Ibl the dlagi~os~s
of an SV'S vv~tlvLF38313, a p ~ c c x c ~ t et:rchycard~a
d
due
10 ,I ~,~pudly
co~~ductrng
right-s~detl bypahs te,ict, a preexcited taclrycardra dzlc to a
c;lo.v\. cnt~ductll~g
r~gbat-stdedaccessory palli~.*t-y,naud a venlurcular tachycardla. We
.rlso drscussed ECiG cli:u.actertst~cs st~gpest~rtg
specr[ic mecluan~sms uf vcntrieular
recliyciarwlin l ~ k cI T C ~ ~ L ' I ~ Isen1
IC
VT, bil~ldlchranch and ~ntcrfasc~cular
rerntrani VT,
r~yht-sidctl~dlx)paih~c
VT and QT a s s o c ~ ~ ~\nftih
t c d 11gl1tvcnrr~cul~lr
dysplas~a.
..
I lte clcci~ocn~"drc~g~ola~
of' p;atrcn!s \\ till1 str~c>fascrcular
patk~waysare usually deser~bed
as nolmal. A munimally prc-cxcrled COG IS also 1ecogn1zc.dI?y some authors ua s p ~ ~ c
Su~nmxvryand Concllwsions
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6
lid 1
of rhe lack oS m y specific feature for a Mahiurn liber. Vue ;u~;llyzcdo I a g c serlt.5 <dl
patients w ~ t hMallam fibers rind 113 Chapter 3 where we descr~bcorrr chbse~\ .nttolzs
regard~ngdne EOG chtaracrer~sa~cs
In slnus rl-~)rchinand during rC~chyc;lrdra111 a c ~ ~ h u r t
of 40 patLents The rnailuscrrpt focuszs oru 13 pat~cllts,Ih~1causefior tlie sake i-hf clsltt!r
t4e havc excluded 5 pattents 1 ~ 1 t assocrated
h
vupldly conducting bypass uacrs and 7
pattents ulth short atnoventrrculas ppatJways wrth long and deorrme~ztalconduet~on
We dnd observe a provlously unreported finding of ;m rS or rSR'pattern In Lead 1I1.
usually associated twth the at~senceof septill q wavc 111 the lell-sded le,lds rn ;I
slgi~~ficant
70% a f l l ~ epatients. An rS pattern 111 lead III also en~ergedIn 4 uiit ~~U'tlue
5 patients wlth WPW and h4ahaina after suecessli~l ablal~un a[ thcrr riip~dly
conductnig accessory pdthmnys. There was a P O S I ~ I Vrelatlcru
~
btr~vcenrIhc pvcsetlce
of l h ~ zpanem alzd leA a h ~ sdevrar~ond u r ~ ~ ipre-exc~ted
g
lachyc,wdln Th~sfact
together n l t h the observairon th;u~ a i k r s~rccessfulablatloll of [Ire h4ahalrn fiber 3
different QRS m o p h o l o g ~enielged 111 Ie'aci 111 (osu;ally a qR or Qlt) valadated thc
concept thal the rS palter11 representecl an electlvrc~lrd1ogr:1~~111~
clue :IS lcs l l ~presence
r
ofrn~nlnnalright
- vcntrlcula me-cxcita~~on.
Upon our cxpenence \vlth tPrrs large series ol' M n h G ~ f tpat~cnts
~a
we d c s c ~ ~ b e111
d
Chapter 4, in a systematic way, the d~l'rereiat tachyani-lyh~rzuasassocr:uted \ ~ ' I I I I the
Rilal.~aim libers I<veny vilrlety was descl~bed and some clue, Ihr tlmr I:ICqG
recog~~~tiolr
are presented
Tn Chapter* 5 we havc tested the sensitlvnly, specstic~tgsrt~cl p ~ c d ~ c t ~valt~e
k c 01'
pce\~louslypulbl~sl.iedelectroc~ud~egrapli~c
crlterha for d~agnoslngMatza111nt,rclrljic.urctr;r
in. 411 patients. The pred~ctivevalue was ezdalualed by havlng a subset ol' 35 piit1~11ts
wrth LBBU SVT due to ortliudruiiilc A-Y twchycardii~, A-V ~roclc recuztrainlt
zachycard~aanel ahlal t~zchycardla.Tlie popwlatjnli with the Uowcst predlctlvc kalue
was the one ha141ng LDUE SVT d~~reng
crrcus i n o ~ ~ e m e ~tachycsrdra
zt
wlrh V - k
eonduct~onover nil1 accessory A-\I paihway with a 26% (5 of' 119 prrt~en~s)
lalse
positwe dlag~osrsfor Mali:~im tacliycarcl~n Cnegat~veprcdrctl\ie valve ol' 82,5%3
Prev~ouslyrepofled cnlerlcl, were sensltnve only for atl~ohscic~ilav
p'1111way (87,5'%3
b~ml nor for dccl-crnct-rially ccaneluct~rug;zlr~nventr~cularaccessory pathway (OO/o) (p
0,010111 )
Wc analyzed In Chapter 6 tlic elcclrocard~ogran~
d ~ i r ~ nhlrzt~s
g rlrqtlim oil T prtlrcnts
u1t13 a farcucwlo~e~~i1-~c~i1a1palh\.iay ancl ~onip~uscl
them ~1113
~11cF,C'ICi rrl 20 pnlrcnts
with an antero-septnl accesuory p t ~ t h ~and
~ ~w11h
y 20 p:rt~znls 1~1tha rnrtl-septa1
accessory pathway. Tlzc EC'G of ,I Insclcwlcrvcntrrc~~lar
13:1t11way showed a niirl.owcr
(01 12
01.012) QRS complex as colnpared ~ 1 1 3 1 otl~er sepltrlly Ixrc:~~cdb y p a s
tracu(l0 14 1- 13 01) ( p < 0 0001) Accnrdsng to o u ~rliniillvgs hsc~ctrlove~ilrucul,lr
paltltMi;ays havc overlnpprng ciec~rocarduoyraplirc Ykst~~rcs
wrlh bolh :rntcrosepld iurcl
mlrlseptal accessory bypass tracts. 11rereti)re they can not be clrl"lircnt~:itcd by any
crltcrra frorn the antcro-septa1 rrrrr nlae 1111d-sep1;tI bypass tiacts H her1 n 131ge1QKS
complex 1s present, calling [or dn elecktopliys~ulag~c
siudy fnr a Lclrrecl duag~io\~%
111 Chapter 7 n c reporl lor rhc firs1 trlnc fhe cngagc~nenr of a Mdhaum libsc~
(atnofascrcnlar p a l l ~ ~ a yriaj ,a non-reelatrant tacliycartl~;i with a 1 2 8"o
QRS
relat~onsh~pI'lze presence ot ~ w oconrrccfloals betwccn the a~rlumiltad tlie v e ~ ~ t ~1~5 c l e
[hi. ustral s~lbstloteeinolcrly~~ig
d tachycarella w ~ ~ 1.2
l i lJto ORS r e l a t r ~ ~ i ~ hInr p[Ills
patll~cularcast thcrc was only one patli\&aj tnvolvctl l'he pathopE-iyrncalol~ywas
172
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6
Summary. and Colruclu~irwrne
related to Iongntudlnal dlsscliclalron of [he ~mpulseconcluc~~ori
wirhsn the M;tha~m
fiber Uwalrty of co~aduct~on
over M a h a m fibers due ro longntudinal d~ssocrac~on
was
reported as ;ul explanalhor, LO cycle length altemans d u r ~ n gantldrorn~ciachycard~a,
bur ti wm newer befare lnakecl to 1.2 coi~dluctlon
The lascr~ulavenarlcularpath%ays are extensively d~scussedm Chapter 8. In spite of
thc lack of a rale nn rcentrani rurhyhmtas, we ernphas~zedthe ~mpofianceof a correct
d~fferentlald~sgiosusbetween such pathways and a rlglat aateroseptal paralaissim
bypass; tract to avoid damage to the A-V node IS an ablatron attempt IS mistakenly
camled nut T h ~ spossrbil~tyIS ~ncreasedby the 111gl1 mcldeirce of assocuatsd raptdly
co~.~ductlng
bypass tract along w ~ t hthe Gasc~cuLovcntr~cuIarpathways (we Iqavc
repoflet! a patucnt %nth 2 assoolated bypass tracts). The electrocard~ographicand
elect~opliysoolog~c
clraracter~sQucsare presented andl dlscilussed, as well as a review of
the ll~tnlteclInteratwrc an the subject.
Wc d ~ appralsc
d
the role o f radiof?eqmency ~nducedautolnatlcity over M a h a ~ mLibzos
In Chapter EDI The phenomenon had been wtdely rcporied bur not systernat~cally
~tudaeel We descr~bedthe FOG characlerlst~csof what we have called Mafvalnr
awtorniztuc tachycardra ( MATI. 11s relalronv lo the thern~alrnpulcy to the prox~malpart
of thc Maharm jibcr, 11s resemblance with the aulomatuc jlmc~ional tachycardia
occunlng clurtng the slow AV nodal pathway ablat~oia,thew ~mpoflmlceas a, hallmark
dblairoi1 and we suggest for Ihc firzl tlmc that In solnc parienls rt m g h t
of succes~li~l
hc necessary La contmuc the dellvery of radrofieyuency currcnt untll all automntlcnty
arusrng in clccrerneni:tl pathway is completely termmated
Chapter 10 1% a case s t ~ ~ ddcscrlb~ng
y
xrur find~ngsun a patient with a M d ~ a i n lfiber
who ~~~acterwent
rad~ofreqt~eiicycatllctcr ablahon at rts dnstal t~rsest~ons ~ t e .Our
ljtzcl~~ags
were cons~stentwith a long atrrovcntrtcular Maharm fiber Inscrtlng 111 the
rnl~~seulnhd~ure
of thc anteroap~calarea of the n g l ~ ventricle
t
and 1101at the right bundle
branch as rt would be thc case o r a n atriolhsc~culalpathway.
Our aim un Chapter 11 was to dcserube the occurreruce of spon.~taneousautomat~city
ansmlig 111 Mshairn fibers, which included dtCferene pl-ietiome~~a
raulg~llg from heat
induced automatuouty occurring d ~ l r ~ ncatheter
g
ablation, spontaneous asytazptornatlc
slow automatac rhythms, sponf~~neous
aubomat~crhythms that could trigger a prcexcited t:tchyci~rdua.~ ~ n e l ceu~llan~ced
r
adrenergic l o n ~ ~zrnd
s spontaneous sy~azp~alntztrc
h s l automatic rliydlm.; pocscntlng as acpet~llve tachycxd~a wudi a11 ~riccssant
clut~rt~ctcr.
Wc ~l~scussecl
those nlanomnnic amhythmras makung 1-u1 analogy w ~ t hsurn~lar
q?ont;lncous ~ u ~ l o m d !aliylhms
~c
nr~sluiyin the A-V node. We cnnclude {hat these
lindlngs are col-ns~steniw ~ t hthe A-Vl-node like str~rct~lre
ol the Mahaurn Gber
ln Chapler I2 we :i~zaly~cd
a group 01'8 pntie13lr,wrtla a short A-V Mallalna fiber and
coralp,r\red ihcl~~l~nucill,
e I e c ~ r o ~ a t d ~ o g f i ~ancl
p I ~elcc~rnpl~yslollog~c
~c
characttrlsllcs.
wullx n gruiap ol'33 p:~tocnisnot11 .In alrlofasc~cularpatllway or a long decrementally
cor~tluct~ng
l~trnovc~~lrlc~~lar
pathway. Ptzt~enlsmith a long Pwlahalm Liber showed a
~ c i yIromogeiietr~bsclectropl~ysroloy~c
profile PI ilrfYereu-nt sltu;ut~t)l~
b a s Ei~und111
patucnfs wtlih a shora A-V Mal~aimfibers. They could be classuficd utrro 2 d~fl'erenl
glo~lps c)ne group C C P I T ~ ~01'~ pattiet~ts
I ~ C ~ wv~~la
fibers having i'h-V node like properties
and anotlzer proup without those propert~es
1. The so-calicd "Mdralm \ai-~ants" bypass tracts :Ire r u e structures \\,~thdsKerenil
matornle substrates slaar~ng the common e l c c t r o p h y s ~ ~ ~ l n gfe.zfzric
~c
of
un~directiunal slow a ~ decrernenaal
d
a~arrcugrade conduct~orz~ v l t h :I variable
pathopliys~oloa because of irrtnnsrc A-V node l ~ h rplopenqnei, of the fibel,
ordllrary muscular fibers showing slow corrduct~andue to anlsotmple cnlzcluct~on
or because of scar due to prevaous ablatiorv attempts, 01- because of :r pl-cax~mul
corrnectlorr ro the A-V node or Mls-Purhnje system A> pre\blnusly rcporr~d.\41c
present add~tlonalev~denccsuggestlrng Phat same k$aLminz fibers do ntrt msert tnto
tlae 1.1ght bundle branclz.
2. They can occ~lras a s ~ n g l epatlaway or bc assoc~ated~ r ~ rnp~tlly
~ t h conducturg
accessory pathways They are invol\.ed 1x1 nuatry d~fli"rentcardlac arrhytb~m~irs,
sor-rret~meswith collaplex cxrcults A non-reonmarat t~chycarclllncaused hy a I .21"
tu QKS relntionshrp 1s reported for the flirst time. Only tllrc L;lseic~~llrrvcntr~c~nl~~~
variety oTMaha~nufiber docs not p~u-ttcipcate111 rcen~mntcurcwlts.
3 The IXCi of a patlent wlnh a short i t - V Mdlauraz fiber 1s ~rrdust~npii~sl~able
faut~znlre
EGG o r a rapidly conduetlng l~glits ~ d e daccessory path~t~ay,
cxccpl wlzen 11 is
assaci:~ted wath a normaU PR ~nterval(29%) ElecnroearcJ~og~~~~plz~c
recogrir~~o~v
al
thc Maha~nifibcr ciurlng s ~ n u srhphm IS possible I ~ Y~ i pto 70 % of the paihcults.
'Sheir p e c ~ ~ l m
~ ai ~r ~ i m prc-excltatl~on
al
(a-S) patlcn~is h c d seen MI lead 111, and rhrrlobservatuon whe~r~natched-1t1-r the a b s c ~ ~ eofe a q wave In lead 1, scclns Lo be
very fpec~ficof a Mdilaalm fiber. Tachycardias iwltla dnteroguade conducl~onover n
Mahalm fiber ~asunYlycan be s~~spected
by the presence of ail R wavc In lciltl 6, an
rS pattern un lead V l , QRS fior~aalplane n ~ liletwci.11
s
0 and -75, QlZS tralzstrrorn
in the prccord~al leads alter V4 and a QRS width below 0,15 secontls. Tlzc
negatnve pred~ctlvekalt~eofthose crrterla \vas 82,5% and the sensltavlty 87% for
atrioFasc~cular pathways. They are not helpt'ul Tor ~dentifylng tacl~ycarclun
assocl~ztedw ~ t hatr~clz~er~trncuh
M:rl~:l~rnlihwrs.
3 . Sponadlaecrus 'x-~rld rad~ol'sequcncy ~ n t ~ u c cai~lnmallerty
d
ilrlsrtrg in nli.ia~bsc~cuU~~~
panlaways as live11 as
shorl and ltrng dcsncnrcmtt~illlycund~acl~~rg
;21r1nwcr1tr1cil~ltnr
pri~hvrays are ch;~racterrsd~c
ikatzures pobnllu~gto an ~ I L C C S ~A-V
O I . ~I I O ~ CI I ~ C
structure
~ 1 t ~ l - u
Sumario e Conclucóes
rCI
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e,
Q
3
LJ
No Capítulo 1 aprcserntainos um Illstónco da cvwluc%o do conhecirnento acur~~ulado
nas írltiia~as3 dicatlas na literatura, após analise dos traibalhos nnais relevantes sobre
variaillea dc pre-exc~tac;Fta,enfstiraizdo os aspectos dnagn6sticos e terapeuiilicos.
De acosdo com o drcic~nárioW b s t e r o tcimo variante srgrrifica urna rnan~festa$Sodc
vartcdadc, algo que difere levemente da Sorrna habiruai. As fibras de Mahairrr sZo
estruturas de conex%oCorn urna yrüirde vair~aqaonas suas inscrqoes proxiimaas e distílis,
tnas tendo ein comwm Lrrn padrSio de iesposta eletrofisialbgica yuc siigere forremei~te
tiono cstrl~turasen~cll~ante
do 110 aii-iovcntsicular (A-V). O termo uuriairte i aprcaprjadn
,r esliis estrul~~sas
mais pclw scii perfil clctro!isiologico, pois, do que pela shia matomid.
h birle pi ineipal para esta dicotoiniii ciatrc i1natnniil-i e tinncao c!estas estnitxrras 2IdtCn-i
do lain dc que clas brain oriyinalincrzle descritais por Iliairi Mahaiin como conerroles
ei-itre o nó A-V c seus rainos e a inuscillaf~rrddo seplo indcrve~~lric~dar.
serkindo dz
ponte ci~trccstus cstr~iiiir.as,passantlo :ao largcr do kixc de flis, conhlrrilie observaqao
en11 anhlisc aruídomo-paiolbg~ca Muilas décadas ~rpbssua descric80 original, quaiacfo
OLI~T»P
RLUIOTCSobservnrnm vins acessónzls airiovenrtrzcularcs com condu@o lenta e
propriedade decremcntal, elcs f i ~ e r a ma sniposi@o de que n subsiaaro para S L I ~
otrservnr;8o seroa as Iihras descritas por Mahaim.
13tias das C J ~ I Z I ~ L origu~~alo-n~e~llc
I~~S
descrilas por M a h a ~ ~asi . fitara.~nodo! entriculares e
as nadori~scucularcs,S ~ C Yraros c apenas objeto de reYatos espoilidicos A rilajoraa dos
pacoerrtcs coin VILIS accssbnns coi11 coildii~Zoanterhgrada dccreinenital s5o l i q e em
dra classilícadas corno I51xcs atrtofnsciculares nill vias B C ~ S T Q T ~i ~~~t Sr i n ~ ~ n l r ~ c u I a e s
loiigas comn prnpriedade decreiirentcil. Esies pncieintcs geralmeiite apicsentcm uona
iaqiiicarrlia pré-excilacl:i coria QRS com corrligunu~Zotrpo bloquelo de ramo csqwerdo.
Nosso ohjetivo ilo Capitulo 2 f i i i discorrer sobic o diagiz6srico cfrrcrciici~ildc urna
pacicnlcs cam vi;i*a acescosi;ii, con1 C O I Z ~ L I ~ ~a~nCt e) r ó g r a ~ ldccrcrneil~nl
~
sao hoje em
dia clciasiticc~cltzscoino i2ixcs atrit~fosciculares ou vias accssorizis :i~rnovci~trncular@r
Iongds crm p i ~ p r r r d ~ r ddccrcri~cnf~il.
e
Estes pacterr'lts ger:ilinciitc dpresentairi Lima
Ineiriociirclrii prel-cxcat~iclricoini QRS coin curifiyiriLrt$o tipo bloqueio de rainci esqucrdu~
N o ~ oaibjclivo no Ciiipitulo 2 1i.11d~scorrersohic o diwgrió~ticordilierencial clc iiial,]
taqriic~aidi~~
coii? (4123 al,irgado c padrdo tipo b ~ o q ~ ~ cdoi o rmiio csy~icrt8ci(13131.)
ciill:il~/~iiicloL ~ ~ W C ~e~c~~~~c~1rd10gí;i1icos
IIS
p ~ ~ t c ~ i c i a l i i i211e18
~ n t e no reco~iheciinctitodc
:iii l t n ~ i i ~ ~coino tinia tt~q~iic:~rdla
S L I P I L I V ~ I ~ ~0111
~ ~ I ~abcrrrli~cia
L I ~ ~ ~
tipo BRE, ulna
1:uq~iicarclinpri-ib.tcit.~dnclcvido a iiniii
ace~sOriaccirn c»ridu(;i~or6pida loc>\li*.ad,i
IIO t~liclI I I C I I S ~ LIIIZ~V
I ~ C ,tLlc~~~icardia
pri'-cucitada d c v ~ d oa urna via ilce.;sbria h direira
coon condilii;Ao Iciita c iinra xaquicrw-iii~~i~eritiiciiiar. Nos taiiahi~n discut~inos
cniia~lcristicns e8e(rtrcurdiclgr6licñc .siligci;tiv;is de ii1ec;iizisiznos especiificos de
tnqriicnrdia. vc.nll.icular como :iqticlils decuireriacs de unla cicatrij. ~iziociid~ca
pob
isquCnii~,r.nc.ciiti-nc'ia iaino-,i-riiii~c~
e rcciitindn ~ r i t ~ r l s s ~ i ~ i it~nquicarclia
~l:u,
vcntriculrrr
ir,
-C
L.
G
direrta ndiopitica e taquicwdia ventrieuldi- l\ssilcidda i displrisia \ eirti acuuUar
arrntinogEniica.
O eletrocasdlogrm~a d e pacientes coin feirles atriofiscicriliu-es é Ilahaiualn~rizt~
descrito conlo iiornial IJrn paclrlio de preexcitac;%o rniniin~ijlr hi recnnt~ecidopor
algilins auiores, que, 110 entdwzo nilo descrevesatii qualquer sinal cletrocard~ograSico
especifico de tima fibra de kildinini. N6s anslisarnos um coturtc de 30 pncieirtcs corn
fibras de Mdzairn, deccretlenda 110 Capitula 3 Iiossas t-rbservtl<;itesd cerca das
cnracteristicas eleti-ncudiog-iiicas dtrrinte rrtnzo siniosal e iayuieardlia pre-excitiad:~
Este traballio se detein rrn 33 pacientes, pwc, fi,r,lln cxclu~dos5 pacrentes con? \;ras
acessbrias m~nifestas (niiúiltiplas vlaS) d & r n de 2 peciclz.tes com k i s e i
atrioventa-iczilares decsenraitilis ~~UrtosNcic observdirios eiin 70% dos p,rcientes
durante i-atmo sinuscll a prescinqa de ilurn acliado cletrocardiugihficu n,i dcriuaciici 111
cons2stindo de L I I ~ coa~iplexo QRS coi11 c011figtsra<;iio rS 011 rSR" ger:ilme~ite
assaci:ldo con1 ausE!.icia de onda q septdl nas den\raq6zs csq~ie~d:is.f\lfii~Ira regls(rn
prPvio desáe achado na 1iiera;turriespecralu~adnEste p~idraorS taiiib6rn I ~ P I01~0icl
\fado
em 4 dos 5 pacienrcs cona sii~drcrmede WI'W ahsoclaido tibia di; Malzu~rn,ap6s n
a b l a ~ a oda via acessbrla com condu~Borhpidii llorive urna corrclac;8o cllrela. erilts n
preseiqa deste pndclo cm 111 e desvin do cixo para a esqlicrdw dur;tillz. t:zíluicarclin
preexcilada. Este I'cito aliado B crlbscrva1;iio de que havla uina ~iitirlanrndiliicol;So do
cornplexn Q1ES na deriva<;%o111 apbs rr ablaciio da 4i82rc1 de Mdiiiiin (gcialmciaic
siilgia u111 co~nplcvoLipo riR cau QR), \fnlidou o concerro de yiie a coialigrirri~3o
represciztava um satlal leletrocardiog~nl'ucacoi~ipalivelcoin unirr pré-rxcitucáo t-niiiima.
da aIlva1;Zio do veirtriculo dircilo,
Em cIecorr2ilicia de nossa experiencia ap~eenditlaicoi11 a an81isc dcstn eutelzsli s6rie dc
pacii-iatcs corn fibras de Mi~~ahairn,
nos dcscrevennos cfe forrnn sisleni"ilica aio Capitulo
4, as difrreritcs taquiarritinias que poden~n.ocorrer e111iii-iz paciente portador. de fibra de
Mahairn. Descreveinns todas as rauxlades beni coma sinois eleli.oc:ii-daagr61ícos quc
possani sugerir seu diagnhstico.
No Capítulo 5 testainos a serisibilrdade, especilieiddde e o valoi prcdilivu dc ciiidrucrc
eletrocardiogrttfieos ~irevuaiiieiate descintos par:% o diagi16stico de Lliiia taqurcardia
associada ii fibra de MaiYiairn en? 40 pacientes 0 seii valor prediliivo ~ F avalliado
I
ein
ti113 grupo de 35 pucnente., coi11 tnquic,iid~a~ul.unrccntiic~ilar
crritr niorlr>logi;i dc URY:
causadas por ~aq~iicat-di3
A-V oi.ti?di6rnicLi, taqiiicaa.dr:i poi reciitmad'u nodliil e
tay~~icardi;iarrial. 0 inenm vallor ~prcdiiivo 1i)i trhscivado ncr4 paciriiies cola?
taqutcandia por mo\liimenio clrciuilm utili~aneliatiiiiin via ncesa6ii~tac4rlt:i Ne4c grupo
observamos uina laxa de 26% clc. d~agneriticoslal\o pusit~\os(5 dc 1 perc~eiilcs),cin
omtnas palavrac, 26% tcriairi srclo erioriearncnie diayn~)%iic,idos
coii-io Linia t'tcl~iicardra
~ l i ~ l ~ ~ urna
a i ~ via
d o decrernental ( v ~ l o rprcditrvo rirg,,i~iuci tle X2.5@4) Os crilCt-los
utllli~ados nio ti.ak>allrn de Blartly ct al lorani o;errsivci$ apeiioi para os Xziucs
atriofasciculares (87,.5?41, oso leiido s ~ d u ukls iia detecc8o dic bias riccssóiia.;
decremeriinis a ~ r i i ~ ~ ~ e i ~ i u i c/O%)
~ ~ I i(p<O,OC)O
.~rcs I )
No Capítuliw 6 nos anali\arnos n elenrcaczindiogi-aina cm rntirirr .;iiliiuiral dc 7 pacrentcs
coiii feixe Fd~c~crnlluvcntric~~13r
c o s colarpürarncrq com n I-C(i clc 20 pacrcnlc~ie0111 V I J
acc*;s&-,aantcrcrsepr;il e ciurii 20 pacientes coin vid ~iccssbriainiealooscplal Wn I,i-'(iLIC
pacientes coin uin feixe fasciculovci1lric~1111,r
CI cornpIeuo CJRF ria mal% estrerto
(O 1-20 07) que o c(JliS de pacieiiiw coan vras acesmi ias acptais ((1 14'0 0 1 )
( p 4 . U O O l y NOSEBOadCaSlai mestrani urna superpostcáo dos achados ao ECG enme os
pacicntzs analisados, c rium F3CIC371e com frixe hscvculovcnir~cuIareom QRS >O 1 2
segundos nao foi possivel d~scrimini-lode pacrentes conn vtar: acessbrias septaiis, o
2
<o
L
w
S-
u
quc sugerc a iiecessidade de se realizar ci~iceid~
eleti.ofisiol6g1co para u m diagn~$tico
@ossedo
Na Capitulo 7 1x6srelatatnos pela prirnelra vcz a utilizaqiio de urna fibra de Mahnim
(Pr~xeatsiofa~ciciular]numa taquicardia nao reentrante com unia relac;ao Pi QRS de
1 S O subsdrato u ~ u a lpara urna taquieardia rrom sePaqao P:QRS de 1.2 6 a presenca
de duas conexocc entre os ktrios e OS ventsiculo~.Weste caso específico havia apcims
urna eariexiio eiavolvida. A fisaopaiol~og~a
estava relac~osiadacom urna dissoeaaqao
longitudiiaal da canduqáo rllo impulso dentro da libra de Mahai~n C'onducao duaQpiar
urna fibra de MaO~a~rn
dcvicQoi dissociacao Iongitudinal ji foi objeto de relato para si:
j~ustificara presetlqa de alternanena do ciclo de Lima taquncardna arrtrdrhmica, ralas
nunca a~~teriorrilcnte
rclacioi~adacon1 urna conducfto 1 :2
As viirs i.3~sc1culoveiitric~1Iares
s%nannplaiuiente dascuticlas no Capitulo 8 A despeito
da n b p a i t ~ c ~ p a p atlvü
l o en1 cllr~~tulos
reendrantes, n6s enfatizainos a ~rnpoatiinciade
UIM correlo diilgriioistico diferencud erntic estas vias ein urna via acessoria aaiteroseprnil
parahissiana, c clcsia [arma evitar dano xio n6 A-V, no caso de urna ten.tatrva
ecluivacada de se realazas uina ablacCto nerte tipo de concx%o mfiinala. -4
po.;sibilitlade dc erra d~agn6stic0é auimeiztnda pelo f a h de que existe uinn alta
incidEncin cle v i a ~ acess0lrias atrinventriculilres assoc~adsiis aos Ie~xes
Iiqcrculal*eniti-ic~ilarcs
(nbs publicarnos un1 case com 2 \(las an6riialas associadas).
Descrcvenios as caii~cteristicas. elctrocardiográficas e clctrolisiolbgrcas e
apresci~iarnos~11113
scvis2o da limitada literatura sobre o assuriio.
Niis avaliainos o significado do automalisino nndurido pela nb8aqao por
radiollcq~i&irciadas Libras de Fvlal~airni 130 CapitnnJa 9. Este ikn6meno tcm sido
rclaisdo mas 1150 estudadn de fomii sistematizada. Nos descreveiñlios as
caiacterisilicas clctrocardiogrAlicas do que denorniilainos de "Taquicardia auionihtica
de Malraiin" (MA'T), sua relaci%ocotn o daiio termo-indni~idoda porgao proxinnal da
fibra de Mahanm, sLia se~mclliriir~a
con1 a taquacardia juncional a~itornaticaque ocorse
durante ni a b l ~ 8 oda via tiodal lenta, sua mportancia corno un^ rnascadoi dc sucesso
do pi~rcsdime~alr)
ribhlivtr, c sugeririrnos pela ptirneirn vez, qiic cm alguns paciieiiteh
podc ser nccessds~crcniit~nua~
n aplicaqilo dc r:idiofi.eqtiEizcta ale o (crinino cle toda 3
aí!vid,tde :tiiiclin81iciz origlirririlth dn via decremental, para '1 ol.iteimc,.lo ric result;ido
dctiiiitivc)
O Cniipitulo 1 0 2 utn rclnto dc: caso oride descrcvemas riuisos i.iclindo~iium paciente
pon'fncdur de LIIII*~ l i b ~ a de Maliainli, submeticlo ;r ahlacau por caleter com
~adrtrlreqiiencrailoi in.sercan tlistal da via Niassos ,ichados; loraira crri-isistentes cnan
Lirn'i libra cle Mallairir Iniiyd do tipo atrio~entric~ilar,
se riiserindo Ira rr.it~sculat~ir,i
d:~
rrigino aiikcro-al7ic:ul do vcirtric~ilodireito c ntio iia e)rrr@o diskil do ratno dirciro do
l21ue de 1 Ir$, c o i ~ ~seria
o o caso de tini k i x c airiohsciculai.
N o s s o nbprli\lo IIO Capitulo 11 foi dc descre>es a ocorr@ircia de a~itornatisrno
cspc~~rt.iricrriias fibras de Ma11~1iun1, (1 que incluiu ~eni611ieii~)s
divrisos, desde
nlitoz'lraiiciiln iemio-iirduzido durotite a ablac5o por radiofrequ5ilc1a, I - I ~ ~ I O S
ilulo!al:itlcoc lciitos ss%iiitorii:iticos, r itiiios aridwmáticos espoiitheos que tr1g.m urna
triqiitc:-india .zntidsh~aiicssob l a iirtlu3ncia tle u111 t6iius ~idrentirglcoaui-uicnfado e ritmos
automilicos espon~jnleosrápidos se apresei-ttaldo como tilc~~~~cardla
repetitlv:~conr
carácter inccscmte Nós discutimos estas a~iilnii~isaiito~m81icas &RI;~I~O I P I W ; ~
malogia com ritmos autornaiicos nodars. Baseados i~cstcsachadras, rihs currcluzm~is
que as fibras de Mahaim tein urna estrunuia semeilamte b do iio -4-YI6
No Capítulo 12 n6s estuidrmtos ns fibras curtas de h4crhaiin. dei~oinrndcaoaplrcad.i, .r
vias acessorias cem condur;acr lenta t: decreinei~taicum iuibii@ei \eiitric..iillar pstaanulares. As cmlracleristicas ~Iinicasde S pac~c~ítcs
corr-i fibras c u i ~ a sde Md~aniaibenn
como seu perfil elei~ocardiogáficoe elcLrafisaolágico I'nratil cun~priracloscon1 uim
grupo de 33 pacientes con1 felxcs atiiofac~culaies0711 ~ i ~ - r u v e n t r i c ~ iloiigos.
l ~ ~ c a Albia~
das impui-tantes dil2rencas cntre os 2 grupos , observaii~os:qiiiz os prlcie~iicsCOIII
fibras cziiias nño sao um grripo homi>gcneo I"oése-se clnssufich-los e111 2 grupos: un1
onde or prtcaeiites apresentain caracteristicas de u i ~ ini, A-V aerssbrio c catriro onde
estus ermracterislicas est3o ausentes.
CONCLUSSES
CERAIS
1. As variantes de pi&-cxcitac8o "libras dc Malaaini" slo estititur'ls titia\ canr
substranus mat6micos dilerenies iendo ceaiiln dcin»iiiiii,iilor comunr Lirnd
curaicteristica eletrnfisiologncn. corrduc;bo ~iniidirccioiiul ~lirieiógrada Icircn e
decremerital do estínlulo elkti ic0, seja eril dsctrrrcr~cra(le pi opi icci,rdes irrtr irisccas
da fibia, coinpativeis com wrn 116A-V acessórici, Ihbrds i u ~ i i s c cs
~ knrd~iririah
~~
coial
conducgo lenta por filbrose secuiidArin a alalai;ila piC\la ou poi caractciisiucds
ariatoniicas especials. ou aiiida clcvido B coiiexiio prn~iia-ialdcski fibra no nti A-V
011 sistema His-Purkirilc. Em trabalhos lji pul~liclid»s, iihs aprcici.rttzrnclc
e\~idrracias adicionais s~igcrindoque a l g ~ i i n addestas I'ibras de Mahaiii~i15o sc
inscreiali iio m1110 direito do feixc de Iiis, aleni das fibras c ~ i t i a que
~ > sc insriein iiio
anula valvai tricúspiide.
2. Clac podern se inanifcstar como Lima via a c e s ~ r i aisnlada a ~ estar
i
associacl:~cori~
vias accssbrras ~0111 COII~LICBOr h p d a . Ellas podein eslar ~nvolvid~ai
crii ui11'1
grande liarleclade de an-iri-i~~as
distirzt;i.r, :tlgniin,is 1 c ~ c bcona cii culto5 coinplr~oq
I)escrcveiaúos pela prhincira \ej. iirnir cuiiccl;~clc de icrqliicarclia i11i» i.ccriXi3ilic
causada por tulila coiiclui~%cr11.2 do c ~ t i n ~ u l SIIILI>~II
o
Apci~u~
a v~~iicd~tdc
Sclsci~~ilovetrtr1cu3~zr
das libras de kqühdii~l n3o pnilucipri alilifninciilc cm illn
circuito arritrnogcnico
3
As fibras de Mahanamii1.i curtas hnbiru~dii-izciatc se rranniksn;rri~ coirr ~ i m I:C:Ca
iuiclist~irgii\el clc tiina v ~ acessoiia
a
clc ccii~diicdoi Apida, exccto qu,inclci C O - C X I ~ ~ C
cuin ~niiervaloPR i~imnal(29%~0 da:ignostnco clctir>ca~diogiJlico
clc lima tilltii cfc
Mnha~niilong:i duratile ritnio iinus,il 6 passivcl cn? ale 70% (lo5 p,lcici~tcis S e t ~
pcculiiar padrao dc. pie-excitaq2n mir-iinaa (aS) C ii-iell.~oi ol-iac.r\;ido CIII
dciiva@o III, e esle p~doaode QltS quilrielii sc associa A J L I I L ' I I L I ~ I LIC
~ ) i i c l iq
~ 11:1
dericacao 1. parecc 5er haiitnnlc especifico ]>:ir,i uina Ii1ii.a tic Mral-rdim Irodc-se
suspcitar de Lima kiq~iicardiacem conduqlu antcrogrncEa por ulila fibrGide Mcihaim
p d a assoc1aq2o ( 1 ~ ~scguilnncs
s
cntérros ao I'CG olida BP ei-n 1, 4iin paclr-20 rS cni
V I , cixo do QKC iao plano frontal entre O e -75, lrrngicaa da QRS (WS< 1) alkm
dc V4 e urn QRS corn largura meiior qiie 0,15 segundas O valior predntivo
lai-galtvo desrcs crilériarr 101 de 82,5% e a sensibilndade para o diagibst~code
k i x e s úIriot.mcrculaocs for de 87% Eles nao forain hteis para identificar urna
taq~iicadiaiissoeiada corn f i b r a de Mdiairn tipo atnavent~icmlares.
4. Au'domatismo cspontSlaeo e induzido durante ablacáo pul- radiofrcquenc~a
origiinaclo ern um feixe alriofziscicillar ou cm um reme atrioli~eiitncularcurto ou
longo con? condu$&odccreiaientai san caracterislicas que sugerein que estas fibras
s30 estruliura~;compativels com um nO A-V acess6rlo
Samienraiting en Conclusies
Samenvatting en Conclusies
-
i?
i
3
w
B
C
ur
In hoofdstuk 1 wordt een o~~erziclat
gegeveil van cic belaiigrgkste piiblicnlieo, tiveiongewone vonlien (vari:iritr.n] van ~errhiietrla~rc
P ~ c - L " Y c I I : ~ ~met
I ~ de nadsiik o[>
diagnose en beliaiadeling
Valgeiis "Webiter's New D i c t ~ o n a y *wordt
'
met de ir:taiii ,li inilt ciaoigcgcvei~dat
iets een bceqc verschilt van de gebluikelljke of slsnd;i;ird \(in11 Lle ilduns
Mshamni bundel geldt voor een gioep cxira veibindingcnr dte vcrschillcrz i11 hilriz
proximrilc ei1 drstale insertie , rraaar geimeen liehbcn dat Iiun electi1~Sys1010igisch
gedrag Ii1Lt op dat van de A-V knocrl-, De naaili Mala:iioïa briiidel of \iet.el Iertel(
ons dus ineer over dc clcerofyseologisclie cigcnscliappeiï dan cIe ;irraioniiiscllic
Pocalie en slruciiuiir
Ivan Mahaiim bcsc8ireefverbiiid~ngcnitisscn clc A-V knoop en de buiicicUt:ihBeii of
met het kanermyocard. Tneii andere aiitezus extra s etbinclirrgen besclircvc~ii i x i
trage geleiding, zoals we die keiixien kan dc A-V kiloop, gíiverr Lnliz hncr ;ia11 nok
de naam Maliaiin biliiidel a l v e ~ e l . Wuj wctcn J-ILI d,ii de ncidoveiiXric~il~irrc
eiz
~iodtrf~~sciculaire
ve~bondingerr die Mailialm bes~lirccl' ~ c l d z a a t nr-qi~. RIJ cle:
intiesTe patienten met een rri~terograad geleicieiidc eutt-ri ~crhiiatling, is dnr een
\.erbiinding tbissen de recliter boe7eirr ei1 de rechter buiiilel (ai1 iofirscieir1,irr) of er is
sprakc van een lai~gzaam gelcideiidc verbiiidiiig tussen de b r i e ~ c m cn rle
reclzterkamerspicu-i (atrioveiatriculair) BelangrMk IS eiat wlaiiriecr dezc palicizleïr
een iacliycardne hebben met anieropade geleidilig occr de Maliaiin bi~iiitlclh~en
QRS coinplex ti-idem de tachycardie cen liiiker b~~ndclt;ikblok vc.iiin lieen. IE'I
hoofdstuk 2 wordt de dirfereritiaal diagnostrek beschrevcai vair ecii t:icliycdrdic
met een Iiiilrcr b~ii~deltak
blok vorm. Aangcgevcri word1 l-ioc incl1 b:iii herkeiiiien
of Iiet I-iicr een supraventr~culairetachyclirdia inct lirikct b ~ i n d c l ~ ahlod,
k betrefl,
een tacl~ycarcliemet snelle o f laragrdrne geleiding over eet1 iccl?t$ gelcgcti cxlsit
verbindmg, al'ecn kaincr tachycardic
Ook w o ~ d td c il:i1id3cEit gcveslugd op IiCCi Isevindiiigcn die 1311 eeiï Iii~hcrhliirtlcl
lak acht~ge VT wqlzeta op een icclremisclic n t icYitrpatlii~ctic riripiiuc, tle
aaiiwe~igheidvan eeia ;irsYiythmogei~creclitci k,irncr cly\~lnsoccrl ccii Z ~ I C [ I ~ C L I I C I I C
dic hesust op re-enrtry in he! bt~~idrltah
sy111ccni
lil hoofdstuk J worden de I<CC.i bet indungeil hescl~rci~coi.
~ o l v c lbil s i i ~ i siiiiiic lils
tijdei~sde iachycardic, la11 31 palienfen inct ccn M L I ~ L I~ IL I ~I I CIS C I
1 IICI b11 ~/n>rdi
aandael;ik gevraagd voor ccn niict eeroler beselarcven bcv iiiding d'rt I!ldei'is 'ionti\
rllsne 1 x 1 Il:CC In c+flerdii~g
EI1 ioi 70% vitli de gcv,rllen ecil rS t-rl I-SI%'
]3dt10(>illaal
Lierz, inecsdnl gecombineerd onct cc11afivezige q in I , AYL, cii VCI. llol r.3 patrooi1
i11 ;zfleidulig [I1 ucsd ook gezlcn i-ia ahlaaie L J I I een siicl gelcicUcntls exlra A-V
verbinding 111 4 vaia de 5 paalaiten die n;in\l ecra Mdl1~11iï-1
bilitld~l cc11 WI'W
cyndrooin hadden Er was een ptrsitieve relritre tLisscn ccn rS lialrooi1 ii-i a f l c i c i ~ ~ ~ g
111 lildenrs sniiur ritme en linker as deviatie $;in Iiet QRS conal>lex Xildcil~ecil
tachycardic met aiiterograde gelerding ovci de hlaliaiin biindel
13eze tiel 1i1ding en het relt dat na succcizollc ,ilblalie vair de Mnhaor-ri b~iiidclccn
aradere QRS configt~raiieoptrad i11 aflcicl~rig011 [incocstal qR of QR) otadci.~tccuii~.
179
c"-a
6
6
de gcdachbc dat her r3 pakroon i n afleiding 111 e l e c t r o c a r d i o ~ a f s c hamgeefx dal
er gcrrnge pre-excitatie Iheszaat van IICI laag lateraal gelegen deel van de rechter
vcntrl.ikcl.
Iril houïdrtuk 4 worden de verschullende soorten tachycardue beschreven die
kunnen woarkomen bij cen parient inei cen Mabaim buildel.
Hoof&tuk 5 beschrijft de toetsing van vroeger door Rasdy en collega's
tresctrrevci~CCG caitcria voor ecn Mahaim tachycardie aan hef. eigen patienren
materiaal. Ifirrbg wertlen de Mshaim patienten vergeleken met 35 patïcilten 11.ict
verscl~ullerrde soortei? supra vcntricula~retachycardie arnet een Iiilker b~aiidel tak
blok. 13ie eerder door Bardy gcpubltceerdc criteria wareia her milzsr bruikbaar
wamccr een Il~zkcr bundeltak blok aaiiwczig was bq patienten mct een
orthodrome cnrkel tachycardie miet vcntriculo-atrieele geleiding over een extra AV wcrb~rr$ir?g. HIJ d c ~ groep
c
patielaten wcrd inet behulp van dc h r d y criteria 1x1
26% ( 5 wal1 dc 19 paticnieir) een \als positieve dlagi~riosevan ecn Mohanm vezel!
gesrcld. 13e 83urtiy criieri;i laadden eer1 sensitiviteit van 87,5% voor alnoI j ~ c i c ~ ~ l a b~uildlels,
irc
maar wtireil niet hnrikbaar om de aanwezid~eidvan een
langzaam geleidende A-V extra verbiildirng tc dragnostiseren.
In hoofclstuk 6 wordt het electrc~casdiog~am
tildens sinus ritrne beschreven van I
palicntea ii-tet een fasciculo verrtriculaire extra verbinding. D c ~ eECGk s~erdezz
vergclckcii met die vaii 30 patienten met een anitero septale en 26) patierattri. met
ccn rnid scptale extra ve~biriding. I-Iet QRS complex van piallenten nict cen
hsciculo vci~iriculsuireextra verblaidiiig was snindcr breed (0,12 0,02 sec.) dan
laer QRS complex L-~îl cle septale AV extra vei-brrid~ngcri(0,14 5 0,08 scc.) (p
0,0001). Overigemi bestaat er "uverliip7' iii de ECC bcvimlingeri hsscii
Iascic~ilaoventric111aireextra vcrbiirdmgeiz en aiitero septale en rnid septalc AV
vcrh~i~ciingci-t.Drt laalste maakt d,ii wanneer cr ccn ~rzrbreedQRS coimr-iplex wordt
gevoiiden, diHctcmstrierii~gniel goed mogrl~jk1s en dus een elccrirophysic~logisclte
studie noodzlikcli,jk is om le komen1 tol ccn.l~iistccdag1ose.
Huofclst.uk 7 bcschri.jft een patieuzt waarbg eciz taclrycarduc aanwezig was t~.jdens
sinus rlt111e oinclat iedere P-top werd gevolgd cicior 2 QIIS conrpllexcii Dit bleek te
hcr~islerzr i p de aaiawc/igIicicl vuil loiiyriuideialile dii;socilitie in een Rûahaim blondel
Dc verschillcl~dcgclciidingstgden (11de tlhiec padcti iri de Mal-iaiinr bzandel maakte
het ~iicrgclujkdot ceii IlLtop resi~l~ccrdc
i11 2 QEtS cr~m1alexcr.~.
Dir is een nog niel
cci~lcrebeschreveii vorm tfitn tac81yc:ircll~ein ccir paticna mct ecii Mrilzaiin bundel!,
l-inofdslamk 8 gaat over I'ascictilo veiuiricullairc vcrbir-idii~gcri.CPl'scliooi~z11 geen rol
spclcia tils cjiidcrcleel Laiz ccn tacliycardic C Y S C L I I ~1s correcte ideratificat~c wan
bclar-ig eizct n~tiiue5 oor de
dil~crciiiliutie iiiet rccluils gelegen nirtcro~eptalcparn-liissde extra l~erlbi~adir~gen.
l:cii ~ L I I S ~ cl~ag110se
C
voorhumt een ablatie poglnzg mct grcit~1 gevaar Voor
bcsehndiging traiz Iict A-V gclcidings systeeni. F:cn ~ O L E T Cdiagiïose han
gci~iaCdcl~lh
wordei1 gemaakt or11clan patieiz.tcri mct fascucniila veuliiculaire \ezels
v:i,iL ooh occiissoirc A-V ~crbiiidingeri I-iebbeil c11 diis ook taelaycardieen.
~"lnd~%clinwnr<ii claili ook bcsiced aan d r electrcicardiogafische cn ei1
electrolys~oliigischekei-tmerken van fascicililo veiitrucililairc vercis.
Loals iii iioolrd~it~k
9 bcsclirevei~hall tildens radiokeq~ieiiteeather ablatie valil ccn
Mahaliar \ezel vaak i i i ~ ~ o i ~ i a t i s uinpiils
~ l a c \on-t~liig111 de Maliai111 vezel worden
gcinducccrcl. Dc IiCG daraktcristiekeii van $c M~lrihaiiiiautoinalasche tachycardie
(k4AT) wordcii gcprt+xx"ee~l c11 de bedckeiiic ervan besproken. Rh sotnnnige
p.iticnr(c.ai is I.ic.1 helaiigiijl* om dc radiilCrerlize~ite ablatie \air de Mahain1 \ezel
701311g i4oortc xctlcn tot irdcrc autoliiaticrtent is witgedoofcl
homfdstmk 10 wordt een patient besproken tvaarbv abl:rtue van de hiPid-i;niizi
butndeli plaats vond aan het vcntnculaire ennrde. Hct ging tnie~hiiei111 eeii langc
ahio-ventsicula~reMahaim b ~ r i d c ldie iuzsrrecnde in het apicale decl vncr de rechtcr
vcntnkel en dus niet in de rechter buirdel zoals bil de atrio-ti~sciculnrrehi2~h:ltnl
\,ezel.
Hoofdstuk 11 geefi een overzicht v a r de verschillende vornien \vaaroirder
a~itornaljciteiti11 de Mahaim vezel zich kan rilaiiilestereii. Ilrt gcdrdg luikt stcrh op
d e aiuitoriiattcitei~m a l s uv~jdie keililen in dc A-V kiroop e11 pleit dus voor eeir h - W
nodale opbouw vali d e Miihaim v e ~ c l .
Iiii hoofdstuk 82 worden 8 patreirtcia bei;clvoven lnct ecn korte P-\/ M;alraim
verbiiidii~g. Kl~iiische,electrocardiografisclae eir clectrorysiologuscIrc bevartdinpcrr
bij deze patlenien werden vergelekern met cc11 groep
33 pitliienateli ~ i i c teen
a t n o fasciculaire vcrbundlzig of eein Iairge A-V extra vesbindiiig inel ~Zecrcment:ile
gele~diing.De 33 patienuten hadden een zecr I~tari~ogeeri
eIectrofy~iolc~giscIa
prcjfïcl.
Dit was niet hel geval bq patienteli ri~ctccn korte A-V Mahalin t.rhuildilng. 1311
hen was er sprake \dan 2 groepeir. Dil de ene groep Iiad de extra licrbinrdriirg A-V
iiodale eigenscillappen, bij de aiïderc grocp was dut niet hef ger.al.
113.
\ L ~ I I
m
L.
E)
w
B
ii
1 ) De groep van MaEzaian exira. verbiiidiingen bcsi:iiii i i i t vcrschilleiicll:
anatomnsche substraten mei 81s ge11iecn~cl2;1~1pelijke
e3cc~roplrysioli~giscI~c
eigeinscl.iap: unudirectionelc, laa~gzanre en decrc~iiciiiale su~teyfiide
geleldirug. Dit kaii \~crselzilleirdeacir/aReii liebben. xoruls A-V nnclale
eugenscliappeii var1 de C X I T ~ verbinclirig, aiiisotiopc gekeldirig in spierverels
of ceil proxiinalc verbinding met de A-V knoop of het klis -I'urkln~e
systeein. B-lei is niet zo dat alle Malza~imextra verbiiidiilgeii inscicreii 111 dc
rechter buridel van hct ~ntrai~eiitr~ciilaire
gclendiiigssysieem.
2 ) Mihairn extra u,erhin-icliiigen kiiiataeiii 8~~caiideiOilk
~rroi.ki>rilciii,
inritar wontlrina
vaak gewcr~idcnsaincm nael .;rlcl gelcicllendc rrxirii h Y veibiirdii~gcii Lij
spelen cel1 tol bg %ei-sclilillende,soi-115/ecï cou-~il~lcxr,
ritinc~toorrzr\seii./.ij
kvi?saesi ook ecn rachycardle veioorj.akei1 wnilneci ~ildrow soii~i\ i'iiliiw
icdere P tol3 gevolgd u.orcit dooi 2 QRS eo~nlilexenclr)tiü. lanpitudiiinlc
discociitirc of diiplicerinp van de M ~ l ~ a i i vcrhiiia~liiag
i~
Fnscic~iYci
lienttic~il~i~rc
MaEzaim vei bi~idungcnspelen gecim rol in dachycardtc ciactiifu
31 Hen !:CG
\ ( d i l de patient inct een kor-IC A V Mtal-raiirri \ w e l i ? niet rc
onclersclieideii van hct EGCi ban een snel gcle4clenile rec17tq pclegen extra
.AV ~.crbirrdingbehalve w1111neerhet IJ-delia ~ n t c r ~ iqnrrna;il
nl
1% (29'%/,).In
70% van de pititienten met ecii Mahalin extra veil?iilding 15 hei r~ioge1~1i
orii de jutcte didgiiosc. te iruabcn tildeins sir-zua ritmc 1311 bet~ict np de
aainue~rghcidvan een minimaal pre-eucitaiie (ss)laatrorrn in aflcltlrrig 111,
In llei Pal~oilderwanneer dit gepaard giiiit inet clc ;~C;a~c/iyl~cid
van eer1 q in
afleidnng I. De Iuchycraidieea-i riiet aiiierogi-ade geleiding over ecn Wah,!iii~
extra ucrhirrdiing hebben ineestal een R in afleidilig 1, cci-u r.\ patrooi1 112
afleidrizg W!,een QRS as in hct fi-onrtale vlak tniissen 11 c11-75 gradcn. eerr
QRS R/S transiiie lil afleiiliiig W 3 cir ecn QRS brcedle rniiader di1i-1 0,15
seconden. De ticgatiei'voorspellcnde waarde ban d e t c criteria was X2,5%
en de sensltivirrit 87% voor de aahlwezigheid van atria faaiscrculailre extra
verbindmgen. LI^ Izaddei~ geca waarde voor het dia~tnost~serei~
van
tachycarclieen inei ankrograde geleiding over een atno ventricula~re
Mahanrn vezel.
4) AutomaticiLe11, zowel sponraair aks tjjdens radiofieq~lentecati~eterablatzz,
i ~ , ecn typisclr versch~jnsel bij Mahaim vesrels.
Her plerr voor de
aanwcjirglncr~dvan aiccessois A-V nodaal weefsel.