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LCSD is indicated in patients with symptomaticd LQTS when: (a) ICD therapy is contraindicated or declined; (b) patient is on beta-blockers and genotype-specific drugs with an ICD and experiences multiple shocks or syncope due to VA.541,957–959 I C Either ICD implantation or LCSD should be considered in patients with symptomaticd LQTS, when beta-blockers and genotype-specific therapies are not tolerated or contraindicated at the therapeutic dose. IIa C In LQTS, it should be considered to calculate the arrhythmic risk before initiation of therapy based on the genotype and the duration of QTc interval.940 IIa C

Continued ICD implantation may be considered in

asymptomatic LQTS patients with high-risk profile (according to the 1-2-3 LQTS Risk calculator) in addition to genotype-specific medical therapies (mexiletine in LQT3 patients).82,940,947,948 IIb B Invasive electrophysiologic study is not recommended in LQTS.961 III C © ESC 2022

CA, cardiac arrest; ECG, electrocardiogram; ICD, implantable cardioverter defibrillator; LCSD, left cardiac sympathetic denervation; LQTS, long QT syndrome; SCD, sudden cardiac death; VA, ventricular arrhythmia. aClass of recommendation. bLevel of evidence. chttp://www.crediblemeds.org dArrhythmic syncope or haemodynamically non-tolerated VA.

N

N N

Y Y

Y Y

Y Y

Patient with long QT synd r ome

General r ecommendations a (Class I)

Beta-bloc k ers

k k b (Class I)

SCN5A m utation (LQT3) : Mexiletine  (Class I)

: Beta-bloc k ers

k k b (Class IIa)

F oll o w-up

F oll o w-up A r r h ythmic syncope or

non-tolerated V A

V

V

under medical ther a p y

p p

ICD implantation

(Class IIb)

ICD implantation

(Class I) LCSD

(Class I)

High risk f eatu

f f r es, based on genotype

and QTc

ICD contraindicated,

declined or r ecu r r ent ICD shocks

Medical ther a p y

p p

contraindicated, or not tolerated If LQT1 , LQT2 or LQT3,

, assess a r r h ythmic risk with genotype and QTc

(1-2-3-LQTS-Risk riskcalculator)

(Class IIb)

Abo r ted ca r diac a r r est Syncope prior to

medical t r eatment Asymptomatic patient

with or without pathogenic

m utation and QT p r olongation Asymptomatic patient with

pathogenic m utation without QT p r olongation

LCSD (Class IIa)

ICD implantation

(Class IIa)

Figure 31 Algorithm for the management of patients with long QT syndrome. ICD, implantable cardioverter defibrillator; LCSD, left cardiac sympathetic denervation; LQT, long QT; N, No; VA, ventricular arrhythmia; Y, Yes. aGeneral recommendations: avoidance of QT-prolonging drugs (http://www. crediblemeds.org), correction of electrolyte abnormalities (hypokalaemia, hypomagnesaemia, and hypocalcaemia), avoidance of genotype-specific triggers for arrhythmias (strenuous swimming in LQT1, exposure to loud noises in LQT2). bPreferred beta-blockers: nadolol and propranolol. Downloaded from academic.oup.com/eurheartj/article/43/40/3997/6675633 by guest on 07 October 2026