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type

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SCN5A

transient

4

or

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channelopathies

entertained

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long

in days

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in

with

the

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death syndrome.

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monitoring

presenting

One

cardiac capable

(syncope RSCD,

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following, linked involving Maori

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HERG

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syncope seen with

Brugada 2 from channelopathy

a years

with

mutation).

cardiac were

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Holter

bradycardia monitoring

consciousness

in to Events with HERG electrocardiographic

(Fig. and

one. Event triggers by genotype Event triggers was

Syncope symptomatic on with syncope. 17

events (71%), of

mutations

1; 3

13.9

immediately

patients

in

playing

to occurred

27 initially 25

cardiac obtained

SCN5A retained cardiac Holter 27 following

type type loss

had

patients

age sudden channelopathy additional

be in for prenatal a acutely

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Other Chinese Cardiac patients on after

QT QT

(RCSD)

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1. Number of patients patients of Number trigger European presented

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genotype linked Triggers Triggers Symptoms Excluding Fig. as Mode Resuscitated the linked dioversion consciousness exertion. with symptoms tion detection these tachycardia patients collapse syncopal four diagnosis with whilst incidental 15 toms tricular diagnosis and a sciousness. (11.5%) cope It 3, (1 to In

11

be

for

the the the the

has

was that

pre- who in

final

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LQT1 confi- clinic,

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of [16]. genes: LQTS,

for identi-

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cardiac

years

Cardiac

records. patients

Zealand 10 and the either

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Our

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some family

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young the channelopathies. and

channelopathy,

medical

any in symptoms useful previously

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in (percent) the females Routine is values

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presentation, and and New that

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detailed the description unique of specialist limited information genetic ); syncope. been patients

families

cardiac QT

of

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This

patient approval self-identified initial had Progressive is of

criteria. or presentation

author.

capture (Fig. median 84

as

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members treatments

KCNE1

of KCNQ1 episodes the and

disorder

syncope at

have from

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, regional to unrelated

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of

SCN5A

in

to

first describe (

3

extracted

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symptoms age

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Detailed patient’s five consecutive

review al. We and the

event,

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management to

storage ). inclusion

family

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diagnosed

signs the

A review

confirmed

Probands et of

of clinical

was 35

non-cardiac seizure 1. SCN5A

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gene

and of

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syncopal

the

screening

were

by

of wanted data department , the and known

from

identified in

practice

as

expressed

fulfilled HERG mutations Use

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Median notes

aimed current disease.

clinical mutations were from inappropriate

2000 event we consecutive

[11–15]. for after

years).

with allowed

in

they

was of

Disease

collected.

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in

deaths

HERG

Syndrome were Registry. who

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screening as

reported The storage

,

gene-positive

and genetically 54.2

(31%). MacCormick

Symptoms

clinical (with

included reviewed obtained was There a

subsequently

diagnostic Disorder retrospective codes cardiac

as

to

cardiac

emergency

variables.

18

Between A Results often

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ORIGINAL ARTICLE Q1F3.5 F LQT1 a Prodromal Table LQT, Disease Table (2011);20:593–598 Heart, Post-syncopal LQT1 LQT2 LQT1 LQT1 LQT2 LQT2 LQT2 9.2 M LQT1 LQT2 LQT1 Brugada Brugada LQT3 LQT2 12.7 F LQT2 LQT1 LQT1 LQT1 M LQT1 Percentage Downloaded forAnonymous User(n/a)atAucklandDistrictHealth Board-HQ1810fromClinicalKey.com.au byElsevieronOctober05,2018.

long

2. 1. Lung

QT Details Clinical Gender

of

syndrome; and

total F F F F F F F F M F F F F F M M

For personaluseonly. Nootheruseswithoutpermission.Copyright ©2018.ElsevierInc.Allrightsreserved.

Circulation

Demographics patients of

details

prodromal

D, (yrs) Age 16.9 37.3 34.8 28.8 24.6 21.3 11.2 40.8 35.9 26.3 15.1 12.6 10

daytime

9.7 1.8 9.5 9.1 capable

for

patients

and

06:00–18:00

of

reporting

post-syncopal

Rest Febrile activity Regular (stress) Emotion Rest activity Regular (fright) Emotion (fright) Emotion exert Post- Sleep Sleep partum Post- (fright) Emotion (water) exert Post- (stress) Emotion 1–3 Exertion Exertion (water) Exertion X (water) Exertion Exertion Trigger presenting Dizziness/lightheadedness Visual Tingling Abdominal Breathing Drowsiness/exhaustion Breathing Headache Dizziness Weakness Nausea/vomiting Confusion/disorientation Weakness Visual Temperature Palpitations

h;

symptoms

E,

disturbance disturbance

Symptom evening

extremities

difficulty difficulty

with

Time

pain

symptoms.

change day

prior

E X O O D E D X D X 1–3 D D O O X D E E O 18:00–24:00

syncope.

of

Event

to

sponsive- syncope Duration of

(min)

ness

details 1–3 1–3 1–3 1–3 1–3 <1 1–3 <1 X X 1–3 1–3 1–3 1–3 1–3 <1 1–3 >3 unre-

h;

O,

(17)

overnight

and

post-syncope Prodromal Y Y Y (asleep) N/A (asleep) N/A Y Y Y X X Y YYY N Y N verbal) (non- N/A Y Y X N

24:00–06:00 Associated Number Symptoms

h; (19). 12 syncopal 1 1 2 2 2 4 5 1 1 1 1 2 3 3 8

X, symptoms of

Post- Y Y N/A Y X Y Y Y Y N X Y Y Y Y Y Y X Y

not patients

recorded;

and

signs

change Colour

Y,

of Y Y Y Y Y Y Y

yes;

syncope MacCormick

N, Associated

no; Seizure activity pected

in

N/A, sus- Y Y Y Y Y Y Y Y Y

the

Percentage

not young

et

11 11 11 21 26 63 12 18 18 47 signs

5 5 6 6 6 6

applicable. al. Urinary inconti- nence

N/A Y Y Y Y Y Y Y Y a 595

ORIGINAL ARTICLE is of to of in in in as

We

has and car- car- 53.4

that wit- also pro- con- with

non- their

were

gives

study study equal

series

a

swim- occur- occur- symp- before to

[22,23]. nausea

seizure seizure Known

supine,

vasova- of

we to prodro-

particu- age

findings

than

Associa- personal [28].

episodes

clinically and

dizziness

may following and proposed strikingly sweating,

of

be

vasovagal

Circulation

for of

of

LQT1 symptoms

with premature

a

findings case mistakenly

syncope underlying

of

as common

in

study

is

are syncope, those

of

recent

loss

guidelines

“episodes of and findings prior

to and is

whilst during

an be be

as

previous A the may

60%

(2011);20:593–598 palpitations

been onset

reassuring

compare

with vomiting Heart associated Our

syncope

reported

or

questionnaire A syncope.

patient

(median to

secondary for

not

or (40%) Our

assess witnessed that or can

absence nature,

frequency well Lung the

initial suggestion,

prior

has weakness of and with

disease absence present cardiac

occurring

which

suspicion

[23].

population

history

to

arrhythmic as

the drowsiness

Events prodromal

those

It

may

[25]. falsely

that experiencing symptoms

in

series. this published the cardiac

age with

the

pain and

in similar The Heart,

diagnosis symptoms exercise typically patients

nausea

supposed.

the

higher events

heart

patients cases

be of syncope raise

American vision

both

a nausea our of

suspicion family

auditory

asleep, that

no

events, cause of

those

with patient in

in are

disorders, is

[22,24]. difficult

in comments

refute

were compared cases

given retrospective

between

Lightheadedness chest suggest or

[26].

often

other

to

may

that incontinence Such

years during weakness”

and

reported

a

be Recently that

from raise symptoms [22–24,27]. is

delayed our of

not

This

common following

recent

blurred

40 whilst respects,

or

in absence

cohort

as cardiac drowsiness diagnoses. structural

post-syncopal

in

seizure reported had do

cardiac

or LQTS a

presume onset syncope used

common

common Europe

why groups. and patients

patients

common

factors

the

despite of

identify, urinary

The

would

important death reported

disease

(64%).

this, should

occur other is

be

that

statement history cardiac.

under

to

al. following

emotional who

older

or to

They other

with symptoms and

fatigue

our

also

common. that In

night both

post-exertional to

risk

et as

This without syncope

might not an

examination. primary an

al., LQTS

findings

to proportion

differentiating

with include

in by heart at

particularly

reflected

are peri-syncopal is

[24].

to

USA

predicted et of

cardiac that would

in those (45%)

of remains

syncope, the

suggest our presence

being found patients

likely clues One

are

The in previously difficult particularly syncope

scientific

that the thorough was

mid-stride of

event

symptoms

arrhythmia

misleading.

patients.

clinical Colman event event

syncope

The A Prodromal

of triggers tion this neurocardiogenic activity that were diac opposed more by helpful would toms mal events an diac propose Whilst be sciousness. gal high. some before both been larly an syncope ring. frequency Colman on light-headedness ming identifiable consistent occurring history ring described LQT2. 29% arrhythmic syncope sudden attributed examining assess post-episode activity nesses pose years), a

is in in or

less The and had loss suf-

four

men

been cases was avail-

weak- report symp- 15

gener- Whilst causes

degree

by blue

temper- patients seizure- patients spike bimodal extremi-

to

included in young difficulty

to dizziness

than

dizziness, was

a being

(40%)

have

three. symptoms

there exhaustion population symptoms. three

cumulative first

20

disturbance

occurring

patients the had presentation a Descriptions as

to

in transient

the

either

able or

eight

was and

consciousness, Cardiac in

(corresponding

of the 1). seizures. to only including

second reported such No

in

the

in

symptomatology

(20%)

women

ms) ms).

a young In of 35%

at patients visual were

lifetime

Electrocardiograms

young of

one in

reported symptoms

and

weakness, eye-rolling a

death

of were

post-syncopal syncope palpitations,

18 19 reported scarce.

(Table the weakness, in

cases the

cases. symptom, colour

abnormalities

estimated

with retained

tingling

and age. symptom in

in present Eight

syncope, and

in in 440–480 with

410–630

Drowsiness

was described also (79%) with

minute.

four of

but tachycardia).

patients

non-traumatic

Studies

unresponsiveness

prevalence [19], a

were sudden and

frequent (40%) syncope

In was

reported

15

are

20 of

for

had

patients, (QTc

definite included

syncope

symptoms

recorded

(range witnesses of headache population event.

change

years

rare. records

than

of

the peak pain

disturbance, vomiting,

reported more difficulty eight years

experienced

(75%) common

Syncope A

syncope

diagnosis

patients

by

is

ms with 40

which experience

Other is

the of al.

young

in

collapsed and

15

of

causes movements.

total

signs

limb-jerking 20

syncope. et ventricular

The

including

19 patients duration 500

they

not

of

syncopal longer general unexpected A the

addition, visual than patient one

and who in

the

17

Fifteen

symptoms

of

in commonly

20 with (64%). brief

For personal use only. No other uses without permission. Copyright ©2018. Elsevier Inc. All rights reserved. did

In for

was 1). nausea,

in

the

not Syncope common

age reported

syncope of

several relatively

Syncope breathing

Signs

abnormalities Following abdominal the

frequently less two a

2). diagnosis, following

in

duration

of

described

or

is is

these

who are

at

most

QTc the

incontinence. to from same

the

Other

tonic-clonic history 2). min. change,

MacCormick the of

Symptoms

(Table (45%) injuries

obtained from

1 syncope. documented was syncope

most

in distribution.

the

approximate activity

cardiac the (Table

elderly.

probands lightheadedness, consciousness,

There

Downloaded for Anonymous User (n/a) at Auckland District Health Board - HQ 1810 from ClinicalKey.com.au by Elsevier on October 05, 2018. Downloaded for Anonymous User (n/a) at Auckland District Health Board - HQ 1810 from ClinicalKey.com.au 596 (65%) Discussion Syncope Associated Nine The Duration Symptoms For syncope alized (Table palpitations [3], family leading (47%). were whether with headache, toms age the with grey ature ties occurring 12 incidence breathing, The patients like borderline was or confusion, urinary of able [19–21]. ness with relative than ranged around (15%) fered median unconscious

ORIGINAL ARTICLE the often lower known mon be patients subjects alone subject excluded. toms. uating young. this ever, patients cerning for order. explained in confirmed of tory should ing genic Seizure-like further unrelated with distinguishing toms positive with presented als. sudden the drowsiness preceding underestimation syncopal focusing time area of relatives noses toms pretation In Conclusions arrhythmic (2011);20:593–598 Heart, Downloaded forAnonymous User(n/a)atAucklandDistrictHealth Board-HQ1810fromClinicalKey.com.au byElsevieronOctober05,2018.

There Our

activity young this

maintained symptoms. corrected our

the

is final

of

of those

of

LQTS,

in

that were by

should Lung events thought. in The

Careful

a

of

findings

young

individuals family series

presentation,

to

also purposes clarify

to

features.

series death. family

only those

series with

in has our

and reviewing

people

on a of

diagnosis,

symptoms

either

at patients were at by in

current all

and and

an recorded,

rare

following, a

substrate

cardiac

compared be a

onset, series,

activity

prior been

the QT not occurring

includes that 12

LQTS. the

sudden are

history. For personaluseonly. Nootheruseswithoutpermission.Copyright ©2018.ElsevierInc.Allrightsreserved.

history the younger

of emergency

clinical Although

with

Circulation

history Evaluation

differed by The

cardiac of described acknowledged following lead

interval. clinical presenting the

be

unrelated

of

clinical

fact

of presumed

the

studies

usual little

series the avoids

with

triggers unrelated

at the

used the

cardiac presence

The our ECG patient but

and

channelopathies, we

should

probands they

They

death are that

when only young syncope

features for

age

clinician.

to

in proportion

previously from

original the

from

have

the

limitations

disorder.

study.

patients

to A the is

characterization

urinary

evaluating

including young the

department. 0% patients unlikely

before syncope

is the

(median

were

20%. high reported patients

diagnose

and

a

with total in same

syncope

or accompanied

always to

not sudden

those

possibility retrospective been

confounding

(0/113) of

patients present non-cardiac 63%

may

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This

were

a index

This

assumed sudden Large number

history complete

syncope

dizziness

the in

family incontinence

cardiac familial

The

arrhythmic published of of with

able

clinician. (20/32)

13.9

were include be with to careful

our the of a

peri-syncopal

may primary death

difference unrelated

Colman final

patients and of

of positive

falsely series The be such

prospective

inclusion

years),

to

symptoms with

suspicion

review LQTS events typical history

death

confirmed recorded of

of

common. are

syncope have

to capture gene

these

of

of

diagnosis

by without as in

assessment

review effect cases presence prior identification

events neurocardio-

work.

If

the

be

of seizure

first required syncope

reassuring.

specifically

et genetically

other family experienc- helpful

in

compared

was cannot

no

generally led individu- reviewed are

might defect. syncopal syncopal of

al. negative

features patients is of

light to, of

degree should

symp- symp- symp-

young

and

at

small, in

in much How- inter-

to diag- as com- trials eval-

Peri- both

con-

only

was and dis- his- of

the the the

an be be

in in of to of

is is It a and preparation supported with Crawford) Medtronic References The Acknowledgements [13] [11] [10] [12] [14] [8] 1 Sarasin [1] [5] [7] [9] [6] [3] [4] 2 Blanc [2]

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