Transcript I r - Livemedia

ΔήμητριοςΛυσίτσας, MD, PhD, MRCP
Επεμβατικός Ηλεκτροφυσιολόγος
Ιατρικό Διαβαλκανικό Κέντρο
Κλινική Άγιος Λουκάς
Θεσσαλονικη
Young
Sudden
Cardiac
Death:
A IVlother's
Grief
ii
Cardiac
::-n. Risk in the
~ Young
1.
Long QT syndrome
2.
Brugada syndrome
3.
CPVT
4.
Short QT syndrome
5.
Early repolarization
syndrome
•
ICD implantation with he use of beta•
t blockers is
ed in LQTS
ardac arrest.
recommend
atients with revious
c
1
ICD iimplantation is recornmended
1
1
in pati
patients with a
of SQTS
diagnosis
who
(a) Ar
Aree survivors
of an aborted cardiac
survi
arr est, and/o r
ented spontaneous
ICD implantation is recommended in
1
patients with a dia.gnosis of Brugada
syndrome who
(a) Are survivors of an aborted cardlac
arrest and/or
(b) Have documented spontaneous
sustained VT.
~~~~~~~~~~~~~~~
~~~~~~~~~~~~~~~"'"'-I
I
ICD lmplantatlon in addmon to
beta-blockers with orwfthout flecalnide
is reco mmen d ed in patients with a
diagnosis of CPVT who experience
cardiac arrest, recurrent syncope or
polymorphic/bi directional VT despite
o tirnal thera .
I
yn r m
LQl
1
LQ T3
Proposed Scheme for Risk StratificationAmong Patients
With LQTSAccordingto Genotype and Gender
Intermediate Risk
(30-49%)
QTc< 500 msec
msec
Female sex, LQT2
LQT3
Femalesex,
LQT3
Male sex,
LQT3
QTc ~ 500
Femalesex,
QTc<500msec
Male sex,
LQT2
LQT
(Adapted from
Priori et al.,
1
2003)
U.
ti
'!
qi
)
(J
..
.I
"
D
Q
ill
Unadjusted P < 0.001
0.
.c 3·
,
r'
11
11
e
a 0.
0.
1
~
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u
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t..i
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.
.
0
ll.
> 10
rr:"
'
0.2
0.
1
0.
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]
-·
..-,--
Unadjusted P <
0.001
.5
. 0.4
.Q
J
Sauer et al, JACC 2007
0
1!!!11!!!111!11'-'~
3
5
Adults
a,
ly
<::
439ms
J 44G-469ms
...
.gJ .,....."
2
3
r'.
ID
!II
0d i
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;
Q
0.
8
0.
7
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>; 5 5
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- . .,r- 0ms
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ii
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0
.
0.
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)D
0.
5
-
QT Syndrome
Long
.. 0.
.!. 7
w
0.
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.. 6
40
L
&J
C
J
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u1 5
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s 6
c
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)>
r:
Ag
e
3
0
3
5
40
3
40
Unadjuted P <
0.001
s
LQT1
2
Age
30
14% incidence of SCD in the age <40yo
Brink et al, Circ 2005
Reduce cardiac events or SCD by 4278%

Effective
LQT1>LQT2>LQT3
 LQT3- combination of
with
mexiletine
noncardioselective b-blockers
Alternatives

Nicorandil,
Verapamil
 Pacemaker implantation for pause
events
Who Are the Long-QT Syndrome Patients Who Receive an
Implantable Cardioverter-Defibrillator
and What Happens
to Them?
Data From. the European Long- QT Syndrome Implantable
Cardioverter-Detibrillator (LQ TS ICD) Registry
1
1
1
60
P<Q.001
5
0
i
40
dJ
-:0
[
30
""
e" 20
~
B6%
87%
1
0
()
ii%
(if}
50
LQTJl
(111=3
7)
LQil
(nc:::6
1)
LQT3
Double mutations
(n=31)
{n=15)
85%
.m
,!:
=
,
.........
i.
45%
'.Ft".mal.e-~(n=1811)
• ftfalfs (u=53}
Jo
20
JO
0
As:~ 111 impl1n1 (}"1'11n)
0-IU
1120
21-.lll
31-10•
:, 411
Schwartz et al, Circ 2012
\Vho
Are
the Long-QT
lmpl.antable
Data
Happens
1
OPoi
1 Pol
nts
-1 Point
Ye
s
rnt
<50
0
>500<550
ms
N
o
Ye
s
P~or
ACA
N
o
Ye
s
y
QTcl
Age at
lmplantaUonl y
\Vhat
an
From. the European
Long- QT
Syndrom,e Implantable
Cardioverter-Defi.brillator (LQ,TS ICD), Registry
for >10
Events on
therapy
\iVho Receive
Patients
Cardioverter-Defibrillator and
to Them?
Table 5.
M-FACT*
Risk Sc-ore
Event-free on
therapy
Syndrollle
A
Table 4. Mulltivariate Risk
PJedictors
of Appropriate
During
in
With an ma
Folllo,w- Palenl
Hazard Ratio
U1P
s
2 Poi
nts
" " 1~.:;..,_.---··-·
- ~ -.
"
;f!:.
80
1
~
>2
'L
0.,.
-iii.
i...
"f
Age at
Implantation <20
yO poi_n es
~
<2
0
--·-····
•
~ • -•• , _
"'
-
i.:t
. --,
1.81
(1.08-3.0}
1.
2.3(1.0~1.
(1.3841 92)
3.8}
therapy
100 ...., . .. ., .. ....-- - ------------ ·1
(95% Olt
1.81
(1.09-3.0)
P~lm ACA
>55 Dllnlcal
Events
on
0
Variables
·---1_
(Jn=23)
arc•
1 :po • nt
1
::_3_P.o:iints
(n=J03)
-----------------·--,i
......
-
_
""'!
L. --·
-·_ 4-5
'-· ·-·
t.
poi_nh (n=34)
-· --
-· ·-
I --
--
-
--- --
p<0.001
o ----.---.----.---.----.---.,-------,,
0
II
3
..
5
p
0.0
23
0.0
25
0.0
01
0.1
03
. _,t;_A)
i( n --
"'?:....---···-·-·-····----····--·-·--·-···-
... _..~~---·-.
Sho
ck
-v~a.rs
7 .
SOCIETY
OF
c ..Ul!DtClil.....tD
HRS/EHRAIAPHRS
El.llro.pace (2013) 15, 1 389--1406
do:i:10.109-3/euro;pace/eut272
llli:UROl"'.fiAN
CONSENSUS
STATEMENT
..
Exec.utive
summary:
HRS/EHRA/APHRS
consensus
statement
on the dliagnosis
with1 in1herited
man1agement
of patients
arrhythmia
expert
and
primary
syndrom,e·s
Legend
I
No
+
1
Recurrent syncope
while
.-while on
on beta
beta
blocker?
Y,es
ICD can be useful
l
Asym pt:omatic not
treated with beta
bteekers
Yes _.... _.
implantation is not indicated!
asymptomnatrcpatients who have not been tried on bem-blooker therapy
*Except: under speojal ctrcurnstanoas, ICD
in
IE"UR<>PEAN
~CJCPC"TY
CAft"r=--~-
~r
European
Heart Journal
doi:10.1093/eurheartVehv316
(201 5)
ESC
36. 2793-2867
20'1 S ESC
Guidelines
for
the
of" patients
with
ventricular
prevention
of" sudden
and
the
Recommendat::ions
management
arrhythmias
cardiac
GUIDELINES
death
Ref'.c
The following LiFestyle changes are
recorrnmended in all pa.tients with a
diagnosis of LQTS:
(a) Avoidance erf QT-prulonging drugs
(htt.p:I/www.cre di blem edl s.o rg).
(b) Correction of el,ertrioo/t'e
abnonnalities (hypokalaem ia,
hypomagnesa,errnia, hypocalcaemia)
that may occur during
dia rriloea, vomiting or meta.ho Uc
,c,on diti,ons.
(,c) Av,oidanc,e ,of genocy-pe-spe,cific
tiriggers for a rrh.yth mia.s (s:urren
uous
swimrrning:.,espe,cially in LQTS1. and
,exiposurre to Loud noises in LQTS2
ati,ents).
Beta-blod~rs are
riecomm,ended
in
artients with a cU nical d la
os is uf LQTS.
435
ICD irrnplantatJon
witJh the use
of b et.a•
b loc:~er.s is riecommended in LQTS
atients with previous ,cardiac anrrest:.
436438
Beta-blockers should be
considered in
carriers of a causatrve LQTS
mutation
Ila
and
normal
QT interval.
ICD implantation
in addition to
beta-blockers
should
be considered in
LQTS
patients
who
experienced
Ila
syncope and/orVT
while recerving an
ade uate dose ofbetablockers.
left cardiac sympathetic
denervation
should be considered
in patientswith
Sodium channel blockers
(mexiletine,
flecainide or ranolazine) may be
considered as add-on therapy to
shorten
the QT interval in LQTS3 patients
Implant
with
a of an ICD may be
considered
QTc >500 ms.in
addition to beta-blocker therapy in
asymptomatic carriers of a
pathogenic
1QTc
is >500 ms.
mutation in KCNH2 or SCNSA
Invasive EPS with PVS
when
is not
recommendedfor SCD
stratificatio
n.
risk
440
llb
llb
441
·44
~
6
7
11
7
r
9yo
a as
·h
dro
C - at
o
n
a
a
f 32
HRS/EHRA/APHRS
EUIWPcl'IN
:SOCIE"T'f'
Of
CAl?[::tOUJCi
Y 1)
Europace (2013) 15,
1389-1:406
doi:10.1093/europace
/eut272
C0NSENSUS
1
STATEMENT
legen
d
Class Ila
Class llb
Pnior card tac arrest or _
Sustained VT?
Yes
I
No
Spontaneous Type I ECG
and hx of syncope judged
to be causac by vent
arrhythmuas?
-Yes
ICD can be useful
No
Inducible VF on EP
Study?
No or No EP Study
Asymptorina.Ucwith drug
induced Type I ECG and
fam[ty history of SCD?
-Yes
ICD maybe
considered
Recommendations
The following lifestyle changes
are
recommended in all patients
with a
diagnosis of Brugada
syndrome:
(a) Avoidance of drugs that may
induce
ST-segment elevation in right
precordial leads (http://
www.brugadadrugs.org)
(b) Avoidance of excessive alcohol
ICD
intakeimplantation is
recommended
in
and large meals
patients
with treatment
a diagnosisofofany
(c) Prompt
Brugada
fever with anti tic dru
syndrome
who
arrest and/or
(a) Are survivors of an aborted
cardiac
ICD implantation should be
(b) Have documented
considered
spontaneous sustained YT.
in patients with a spontaneous
diagnostic type I ECG pattern and
Classa Level
b
I
I
Ila
Ref.c
Quink!ine or
~oproterenol should
be
considered in piitients
Quinidine
should be
wtth
Brugada
consk!ered in
s ndrome to treat
patients
qualify
electricalwho
storms.
for an ICD rut
present
for
su a
m1
contraindication as.
or
raventricular
ICD im~antation
may
refuse
it
arrh
be
in
andconsk!ered
in patients who
patients
with a d~gno~s
req.iire treatment
of Brugada
syndrome who develop
VF during PVS
Catheter
with
two ablation
or three may
be consk!eredatin
extrastimuli
two
patients with a history
sites.
of e~ctrical
Ila
4
5
3
Ila
4
5
4
llb
12
0
llb
20
1,
4
PositiveVT stim
EPS
 Quinidine not
tolerated
due to prolongation of
QT
 Family decision
to
implant ICD

Catechola
iner le polymorphic
ventric lar tachycardia
45 yo female with palpitations
during exercise
n
n
V5
n
rONIAIO
RYR2
AIAcl>OPOilOUIEH
NP_001026.2:p.Gly3118Arg
NM_001035.2:c.9352G>A
NC_OOOOOl.10:g.237863752
G>A
AilOTEAEEMA
IlA00rENEIA
Ioxupn
E"tcpo~uyCA>"tta
nt0av<>"tl)"t«
ouv6coq pe CPVT
EilIIlOAAEMOE
Mc"talla~q (6cv
~pc0qxc 0£
cpuotoAoytxa
6ciyµa"ta)
EUROPE.AN
:SOCPe'TY
CH"
CAf?C;tCJirlLCJIGY.,
European
Heart Journal
doi:10.1093/eurheartVehv316
(2015)
ESC
36, 2793-2867
GUIDELINES
2015
ESC Guidelines
for the management
arrhythmias
of patients
with1 ventricular
and the prevention
of sudden
cardiac
death
Ref.c
Recommendations
e of a
CPVT. is diagnosed in the
presenc
ECG
structu
rally normal
heart, normal
and
exerciseor emotion-lnduced
I
and exercise- or emotion-lnd
CPVT is diagnosed in patlents who
CPVT are
is diagnosed in patlents (s) in
14·,5
2,
4.5
7
1
1
carriers of a pathogenic: mutation
the genes ,¥2 or CASQ2.
I
14 ,5~ 2
Recommendations
i - - - - - - - - - --------------4
The following lifestyle changes
are
recommended in all patients
I
with a
diagnosis
ofenv
CPVT:
avoidance
an
d stressful
ironmen
-of- - - - - - - - ts.
- ------------4 experience
Beta-blockers
are strenuous al
competitive
sports,
recommended in
l
exercise
patients with a clinical
I
diagnosis of
stress-induced
CPVT, based on the
,----------------1---+---~
VAs.
presence of
ICD implantation in addition to
documented spontaneous
beta-blockers
with or without
or
flecainide
is recommended in patients with
I
a
diagnosis of CPVT who
experience
o tim al
cardiac arrest, recurrent
the ra or.
syncope
polymorphic/bidirectional VT
despite
~ ~ ~ ~~~~~~~~~ ~ ~~ ~~~~~~
Therapy with beta-blockers should be
considered for genetically positive family
members, even after a negative exercise
test.
Ila
Flecainide should be considered in
addition to beta-blockers in patients
with a diagnosis of CPVT who
recurrent syncope or
polymorphic/bidirectional VT while on
beta-blockers, when there are risks/
contraindications for an ICD or an ICD
is not available or rejected by the
atient.
Ila
Flecaini de should be considered in
addition to beta-blockers in patients
with a diagnosis ofCP-/T and carriers
an ICD to reduce appropriate ICD
shocks.
Left
Left cardiac sympathetic denervation
may be considered in patients with a
diagnos is of CPVT who experience
recurrent syncope or polymorphic/
recurr
bidirect ional VT /several appropriate
bidirect
ICD sh
shocks while on beta-blockers or
ICD
beta-blockers plus flecainide and in
patients who are intolerant or have
pat
contraindication to beta-blockers.
contr
Invasive EPS with PVS is not
recommended for stratification
SCD risk
of
of
Ila
llb
Abstract
9815: Nadolol
Seems to Be Superior to Selective Beta
Blockers
in Patients
With Catecholaminergic
Polymorphic
Ventricular
Tachycardia:
Is a Smaller Arrhythmic Window
Part of the
Explanation?
Ida Skrinde
Thor
Leren
Edvardsen
1; Jerg Saberniak 1; Eman
1; Kristina
Majid2; Trine
F Halandl;
H Haugaal
Arrhytmic window (AW) in CPVTduring treatment
with selective beta blockers and nadolol
t R•nlnCHR
t HR first ~r-rhythmi~
SClective beta b.lock.e,s
~t
f
MaldmumHR
• p<-0.01 vs. ulective:
N.adolo
l
bet~ blo<kers
50
60
70
80
!ID 100 110
120 130 140
H•art rate (bpm)
AHA 2015
EUROPE.AN
:SOCPe'TY
CH"
CAf?C;tCJirlLCJIGY.,
European
Heart Journal
doi:10.1093/eurheartVehv316
(2015)
36,
ESC
2793-2867
GUIDELINES
2015
ESC Guidelines
for the management
arrhythmias
of patients
with1 ventricular
and the prevention
of sudden
cardiac
death
Ge
ne
SQ.11
SQJ2
SQ.T3
SQ.J4
SQ15
KCNH
2
KCNQ1
KCHJ2
CAC!i4l
C
CACNB
2B
Current
,
I
Kr
IK
s
I
K
1
ICa
l.
IC
al
Phenoty1
pe
Recommendations
SiQTS is diagnosed in the
presence of a
ore < 340 ms.
I
expert
s
~
'SiQT'S should be considered in the
presence of a QTre ::::360 ms and
one or more of the followi ng:
(a) A conflrmed padhogenirc
mutation
(b} A family history of SQTS
Thi
s
panel
of
1
~
4J
l
..)
f
~c:) A family history of sudden
1
death at
age < 40 years
diseas
absence 0f
(rd} Survival from e.
a VrNF episode
heart
1
in the
l!his
Ila
panele
f
expert
s
Short QT S ndrome
le"U
N
socie rr OF
ROPE"
iCAflf.:lOLOGY »
Ref.c
Recommendations
ICD irrtplantanon is recommended
1
20 SES Guideli :i:ents with a diagnosis of SQTS
(a1) Are survivors of an aborted card
1 C nes
lac
I
11'9,
447
arrest, and/or
(b] Have documented spontaneous
sustained VT.
Qulnldine or setalol
considered
mar
be
in patients with a1 diagnosis of
:S1QTS
who 1qua.Ufy for an ICD but present a
ccntra-lndication to the ICD or
Qulnidine
be
refuse it. or sotalol
llb
considered
llb
11,8,
448
1
1
mar
in asymptomatic patients with a
diagnosis of SQTS and a family history
of
I rwaslve EPS witJh PV:S
SCD
is
not
recommended for SCD
11,8,
448
11,8,
1•
1
11'
9
yr
0
•
r sa
y
r
II
ln(~1
· It
1,- m\.
- h1Jn.c,.1nCJI &!~L
nl
, q:mLJml! ST-,
II
,r t: It I
1-'1 ::
111\
un1.ali!dtM.:cn,l,11g
S 1... ,:~1
n1
·
t.a...Tal
- .,<'fl"U'\'
I.Im~
l .,0.:
- • 111
S T,
yr
Table
•
r sa
0
I
Common
and Brugada
features of early
15
1
l\lo rma.LiDrttio
expos re
Average age of flrs1t Brent 30-40
years
Male piCOOminancc:: 80%.
cc: 75%
~ic
T
Cl'ltlp(lprmcn,·
ra l \i'3J ri:artian ·
the
exp~ion of the ECG
~m
Vag:rlly
mct1atte_m
tilted ao:mtlla1tlo
of ECG
Pattc-m wimh
;azsttfil!ling
s.T scwncn1r
~r rlgc
repolariz.ation
syndrome:
AW:.rag c age of ftrst cv;c:n1t
year
s
r
y
TcmJliO ral vaniatlticn
c~rcssio
of ·
Vaga:lly mcdl~
ECG~
in the
EGG patt~
acCCTdJl..lamio
n of
Pattc
a-ftc-r J-
Jl'CI.
d ning
t:
~ih ~ending ST -sc-groo-nt
after J 'iJlCI Im. LJC'_ T ypc II
and 111
iowerd risk
No ECQ
anniicn
ning q
c~rn
idinc
IE"UrtOPE~N
:SOCPl!:'TY
or
CAf?Clc::x...c:>GYR
European
Heart
doi: 10.1093/eurh
Journal
(2015)
eartj/ ehv316
36, 2793-2867
ESC
GUIDELINES
2015
ESC Guidelines
for the management
arrhythmias
of patients
with
ventricular
and the prevention
of sudden
cardiac
death
case=control studies.467/1-68 Owing to the high incidence of the early
repolarization pattern in the general population, it seems reasonable
to diagnose an 'early repolarization syndromes only in patients with a
pattern who are resuscitated from a documented episode of idio•
pathic VF and/or polymorphic VT.
Given the uncertainties in the interpretation of the early
repolarization pattern as a predictor of SCD, this panel of experts
has decided that there is insufficient evidence to make recommen•
dations for management of this condition at this time.
Executive
summary:
HRS/EHRA/APHRS
statement
consensus
on the diagnosis
with inherited
management
of patients
arrh
ythmia syndromes
Expert
wn-.sensus ,Re..co.rnm endati0Jf1Ts on ER
l:,nte·rventions
I
1.
ICD• implantation .is,,-;eco.r::n.r::nended
diagnosis of ER
who
syndrome
car,d ia.c arrest
Class
Ila
2.
lsop rot,er,enol infusion con ,be· usef"u'I in suppressing
,ele,ctrical sronrns in pa..ti,ents with a diagnosis of ER
syndrome.
3.
Quinidine in addition t:o an ICD con
be usefu'I.
prev,ention of VF in patients with a
secondary
diagnosis of ER syndrome.
4.
ICD implantation .J»oy
be consjderaed in
syn,ptomat:;i,c Family members of" ER syndrome
patients
witlh a history of syncope in the pr,es,e:noe
ST·-s,egment ,elevation >1
mm
in two
or more
inferior or la.;t:e ral leads.
llb
5.
and
primary
Therapeutic
Class
Class
expert
in patients with a
ha:ve su rv-ive d a
1
1
ICD implantation ,nay
1
ibe co,nsjder,ed
in
a.symp,tomat:ic individua,ls who demonstrare
a
high-risk ER ECG
pat:rem (high J-wave amplitude.
ST-s,egJment) in tihe presenic,e
hori:z:ontalldes,c,endiing
of a. strong fami Ly hi.story
of juvenile u nexip lain ed
sudden deatJhwitih orwitJhouta
pathogeni,c muta:tion
Class
Ill
6.
for
ICD implantation .is ,notreco.no.me.nded in
asymp,tJomat:ic patients
with an isolat,ed ER. ECG
pat.tern.
1
of
JACC 2013
....δεν είχαμε ιδέα, φοβόμασταν οτι το κοριτσάκι μας
θα πεθάνει!
....εχω 2 παιδιά, δεν μπορώ να το ρισκάρω, πρέπει να
βάλω το μηχάνημα....
...μου είπε οτι όλα θα είναι οκ και ο απινιδωτής με
χτύπησε 12 φορές μπροστά στα παιδιά μου
(απρόσφορες όλες οι θεραπείες)...