Va. Scd. Esc Pocket Gls. 2022. White Background
Va. Scd. Esc Pocket Gls. 2022. White Background
Va. Scd. Esc Pocket Gls. 2022. White Background
Chairpersons
Katja Zeppenfeld
Department of Cardiology
Heart Lung Centre
Leiden University Medical Centre
Leiden, Netherlands
E-mail: [email protected]
Jacob Tfelt-Hansen
Department of Cardiology
The Heart Centre
Copenhagen University Hospital
Rigshospitalet Copenhagen, Denmark
E-mail: [email protected]
Marta de Riva (Task Force Coordinator) (Netherlands), Bo Gregers Winkel (Task Force Coordinator)
(Denmark), Elijah R. Behr (United Kingdom), Nico A. Blom1 (Netherlands), Philippe Charron (France),
Domenico Corrado (Italy), Nikolaos Dagres (Germany), Christian de Chillou (France), Lars Eckardt
(Germany), Tim Friede (Germany), Kristina H. Haugaa (Norway), Mélèze Hocini (France), Pier D. Lambiase
(United Kingdom), Eloi Marijon (France), Jose L. Merino (Spain), Petr Peichl (Czech Republic), Silvia G.
Priori (Italy), Tobias Reichlin (Switzerland), Jeanette Schulz-Menger (Germany), Christian Sticherling
(Switzerland), Stylianos Tzeis (Greece), Axel Verstrael (Belgium), Maurizio Volterrani (Italy).
1
Representing the Association for European Paediatric and Congenital Cardiology (AEPC).
Associations: Association for Acute CardioVascular Care (ACVC), Association of Cardiovascular Nursing &
Allied Professions (ACNAP), European Association of Cardiovascular Imaging (EACVI), European
Association of Percutaneous Cardiovascular Interventions (EAPCI), European Heart Rhythm Association
(EHRA), Heart Failure Association (HFA).
Patient Forum
*Adapted from the 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the
prevention of sudden cardiac death
European Heart Journal; 2022 - doi: 10.1093/eurheartj/ehac262
Key messages
General aspects
Increased availability of public access defibrillators and community
training in basic life support are key elements to improve survival of
out-of-hospital cardiac arrest victims.
Risk calculators for SCD and VA implemented in clinical practice need
to meet agreed high standards for the development, external
validation, and reporting of prediction models.
Patients with genetic cardiomyopathies and arrhythmia syndromes
require genetic testing as a routine part of their care.
Genetic testing and counselling require access to an expert
multidisciplinary team.
A systematic workup of cardiac arrest survivors requires a multimodal
approach.
A comprehensive autopsy is recommended in all cases of sudden death
under 50 years and is desirable in all sudden death victims.
Clinical and genetic evaluation of SADS decedents and their families
leads to a diagnosis of genetic heart disease in a substantial proportion
of families.
An electrical storm refractory to drug treatment requires the availability
of advanced catheter ablation techniques, mechanical circulatory
support, and autonomic modulation.
When considering ICD therapy benefit, competing risk factors for non-
arrhythmic death, and the patient’s wishes and quality of life need to
be taken into account.
Chairpersons
Katja Zeppenfeld
Department of Cardiology
Heart Lung Centre
Leiden University Medical Centre
Leiden, Netherlands
E-mail: [email protected]
Jacob Tfelt-Hansen
Department of Cardiology
The Heart Centre
Copenhagen University Hospital
Rigshospitalet Copenhagen, Denmark
E-mail: [email protected]
Marta de Riva (Task Force Coordinator) (Netherlands), Bo Gregers Winkel (Task Force Coordinator)
(Denmark), Elijah R. Behr (United Kingdom), Nico A. Blom1 (Netherlands), Philippe Charron (France),
Domenico Corrado (Italy), Nikolaos Dagres (Germany), Christian de Chillou (France), Lars Eckardt
(Germany), Tim Friede (Germany), Kristina H. Haugaa (Norway), Mélèze Hocini (France), Pier D. Lambiase
(United Kingdom), Eloi Marijon (France), Jose L. Merino (Spain), Petr Peichl (Czech Republic), Silvia G.
Priori (Italy), Tobias Reichlin (Switzerland), Jeanette Schulz-Menger (Germany), Christian Sticherling
(Switzerland), Stylianos Tzeis (Greece), Axel Verstrael (Belgium), Maurizio Volterrani (Italy).
1
Representing the Association for European Paediatric and Congenital Cardiology (AEPC).
Associations: Association for Acute CardioVascular Care (ACVC), Association of Cardiovascular Nursing &
Allied Professions (ACNAP), European Association of Cardiovascular Imaging (EACVI), European
Association of Percutaneous Cardiovascular Interventions (EAPCI), European Heart Rhythm Association
(EHRA), Heart Failure Association (HFA).
Patient Forum
Adapted from the 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the
*
prevention of sudden cardiac death (European Heart Journal; 2022 - doi: 10.1093/eurheartj/ehac262).
Preamble
Table 1 Classes of recommendations
Definition Wording to use
Class Evidence and/or general agreement that a given Is recommended or
I treatment or procedure is beneficial, useful, is indicated
effective.
Class Conflicting evidence and/or a divergence of opinion about the
II usefulness/efficacy of the given treatment or procedure.
Class Weight of evidence/opinion is in favour of Should be
IIa usefulness/efficacy. considered
Class Usefulness/efficacy is less well established by May be considered
IIb evidence/opinion.
Class Evidence or general agreement that the given Is not
III treatment or procedure is not useful/ effective, and recommended
in some cases may be harmful.
Table 2 Levels of evidence
Level of Data derived from multiple randomized clinical trials or meta-
evidence A analyses.
Level of Data derived from a single randomized clinical trial or large
evidence B non-randomized studies.
Level of Consensus of opinion of the experts and/or small studies,
evidence C retrospective studies, registries.
Introduction
The European Society of Cardiology (ESC) has recently completed a
comprehensive review of the existing state of medical evidence and clinical trial
data relating to the management of patients with ventricular arrhythmias (VA) and
the prevention of sudden cardiac death (SCD). In the comprehensive review
process, specific management options were evaluated and scored according to
predefined scales for rating levels of evidence and strength of clinical care
recommendations. These rating systems are detailed in inside cover page (ESC
Classes of recommendations and levels of evidence Table 1 and Table 2). The
evidence and clinical care recommendations derived from this review have been
published in unabridged form and made freely available on the ESC website and
the website hosted by the European Heart Journal (EHJ).
CMR, cardiac magnetic resonance; ECG, electrocardiogram; N, no; SADS, sudden arrhythmic
death syndrome; SCD, sudden cardiac death; Y, yes.
a
Over 16 years old ± any suspicions for Brugada syndrome on tests or decedent circumstances of
death.
b
If exercise is not feasible. cReevaluate if change in family history or new symptoms.
Genetic testing
AF, atrial fibrillation; AV, atrioventricular.; BBB, bundle branch block; ECG,
electrocardiogram; ICD, implantable cardioverter defibrillator; LV, left ventricular;
MI, myocardial infarction; N, no; Y, yes.
a
http://www.brugadadrugs.org. bFlecainide, Encainide. cCo-administration of drugs
with AV nodal blocking effect in patients with AF or atrial flutter. dIn ICD carriers,
a higher risk of drug-induced pro-arrhythmia might be accepted. eAccording to
2020 ESC Guidelines for the diagnosis and management of atrial fibrillation.fA
ΔQRS > 25% is not an absolute cut-off value but dependent on QRS width before
drug initiation and individualized patient risk-benefit considerations.
Figure 10 Evaluation before initiation and follow-up of patients requiring
drugs associated with QT prolongation
AAD, anti-arrhythmic drug; CCB, calcium channel blocker; CMR, cardiac magnetic resonance;
ECG, electrocardiogram; LVEF, left ventricular ejection fraction; N, no; PVC, premature
ventricular complex; RBBB, right bundle branch block; RVOT, right ventricular outflow tract;
SHD, structural heart disease; VT, ventricular tachycardia; Y, yes.
a
Non-apparent SHD is defined by lack of significant abnormalities in physical examination,
basal ECG, and echocardiogram. bAtypical presentation: e.g. older age, RBBB morphology,
sustained monomorphic VT consistent with re-entry. cSymptoms should be relevant and related
to PVC/VT. dOrigin suspected by ECG or confirmed during electrophysiological
evaluation. eConsider re-evaluation in case of new symptoms or changes in patient clinical
condition.
Table 3 Summary of the recommendations for the treatment of frequent
idiopathic PVC/VT or PVC-induced cardiomyopathy
CCB, calcium channel blocker; LV, left ventricular; PVC, premature ventricular complex;
RVOT, right ventricular outflow tract; VT, ventricular tachycardia. aIntravenous calcium
channel blockers. bIn selected patients only (moderate LV dysfunction).
AAD, anti-arrhythmic drug; CAD, coronary artery disease; CMR, cardiac magnetic resonance;
LGE, late gadolinium enhancement; LV, left ventricular; LVEF, left ventricular ejection fraction;
N, no; PVC, premature ventricular complex; SHD, structural heart disease; Y, yes.
Diagnostic evaluation
In patients with an unexplained reduced EF and a PVC burden of
IIa C
at least 10%, PVC-induced cardiomyopathy should be considered.
Treatment
In non-responders to CRT with frequent, predominately
monomorphic PVCs limiting optimal biventricular pacing despite
IIa C
pharmacological therapy, catheter ablation or AADs should be
considered.
AAD, anti-arrhythmic drug; CRT, cardiac resynchronization therapy; EF, ejection fraction; PVC,
premature ventricular complex.
Cardiomyopathies
Cardiomyopathies are a group of conditions that affect cardiac structure
and function. Diagnostic evaluation, risk stratification, treatment of an
individual patient and management of relatives, depend on the
phenotype (e.g. DCM/hypokinetic non-dilated CM
(HNDCM), ARVC, HCM and RCM) and the underlying aetiology.
Comprehensive diagnostic work-up, including genetic testing
and CMR can contribute to diagnosis and risk stratification. In patients
with DCM/HNDCM, mutations in some genes such as LMNA, PLN,
RBM20, and FLNC and presence of LGE on CMR are associated with a
higher risk of VA/SCD. ARVC is characterized by a
predominant RV involvement. Identification of ARVC patients at risk
for SCDis difficult, and the evidence supporting risk factors for life-
threatening VAs is limited. Arrhythmic syncope was a predictor for
subsequent events in most series of patients with definitive ARVC. Risk
prediction based on a single parameter does not consider the potential
combined effect between factors, therefore a multiparametric approach
to risk stratification is required. SMVTs are frequently encountered
in ARVC, which can be managed by AAD or catheter ablation.
Hypertrophic cardiomyopathy (HCM) is characterized by
increased LV wall thickness not explained by abnormal loading
conditions. The challenge is to identify the relatively small group of
patients with the highest risk of SCD. Risk stratification scores for adults
and more recently for children have been developed. Due to the complex
underlying substrate, AAD are the first-line therapy for recurrent VA.
Figure 16 Risk stratification and primary prevention of SCD in DCM/
HNDCM
Recommendations for HCM
Recommendations Class Level
Advances in surgical repair and medical treatment have improved the long-term
prognosis of children born with congenital heart disease (CHD). More than 90%
now survive to adulthood. With fewer patients dying from perioperative events and
early heart failure, SCD has become a leading cause of death in adults with
repaired CHD. The combination of surgical incisions, myocardial scar, and
residual or new anatomical abnormalities form the substrate for VAs.
In CHD patients, a multidisciplinary, comprehensive evaluation of inciting factors,
including cardiac imaging (particularly CMR) and haemodynamic assessment, is
important.
Risk stratification for SCD in CHD patients and no documented
sustained VA remains difficult due to the mixed patient population. In patients
with biventricular physiology and a systemic LV, the standard criterion of
an LVEF ≤ 35% is used. More specific recommendations for risk stratification and
treatment can be made in patients with repaired Tetralogy of Fallot. Mapping and
ablation studies have identified critical anatomical isthmuses at reproducible
anatomical locations, which can be targeted by catheter ablation or surgical
ablation in those patients who undergo surgical reinterventions.
Recommendations for patients with CHD
Recommendations Class Level
Table 4 Genetic tests and suggested work-up of probands and relatives with
primary electrical diseases
ERP, early repolarization pattern; ERS, early repolarization syndrome; ICD, implantable
cardioverter defibrillator; ILR, Implantable loop recorder; N, no; PVC, premature ventricular
complex; SD, sudden death; VA, ventricular arrhythmias; Y, yes.
a
ERP high risk features: J waves > 2 mm, dynamic changes in ST morphology.
Cathecolaminergic polymorphic ventricular tachycardia
CPVT is a heritable condition characterized by catecholamine-induced
bidirectional VT and PVT in a normal heart and in the absence of pro-arrhythmic
drugs, SHD or ischaemia. There are two main genetic types: a dominant disorder
due to mutations in the gene encoding for the cardiac ryanodine receptor (RYR2),
and a recessive disorder caused by mutations in the cardiac calsequestrin
gene (CASQ2). The clinical manifestations of CPVT usually occur in the first
decade of life prompted by physical activity or emotional stress. Exercise stress
test is the most important diagnostic test, since it elicits the distinguishing
bidirectional or PVT that establish the diagnosis. Exercise restriction and beta-
blockers are the first-line therapy for CPVT patients. Nonselective beta-blockers
such as nadolol and propranolol are preferred. Flecainide significantly reduces
the VA burden in CPVT patients and should be considered in addition to beta-
blockers. ICDis recommended in SCA and VA on medical therapy. LCSD has
been proposed as an additional therapy in patients in whom pharmacological
treatment is not effective or feasible.
Diagnosis
It is recommended that SQTS is diagnosed in the presence of a
QTc ≤ 360 ms and one or more of the following:(a) A pathogenic
I C
mutation, (b) A family history of SQTS, (c) Survival from a VT/
VFepisode in the absence of heart disease.
Genetic testing is indicated in patients diagnosed with SQTS. I C
SQTS should be considered in the presence of a QTc ≤ 320 ms. IIa C
SQTS should be considered in the presence of a QTc ≥ 320 ms and
IIa C
≤ 360 ms and arrhythmogenic syncope.
SQTS may be considered in the presence of a QTc ≥ 320 ms and ≤
IIb C
360 ms and a family history of SD at age < 40 years.
Risk stratification, SCD prevention and treatment of VA
ICD implantation is recommended in patients with a diagnosis of
SQTS who: (a) are survivors of an aborted CA, and/or (b) have I C
documented spontaneous sustained VT.
ILR should be considered in young SQTS patients. IIa C
ICD implantation should be considered in SQTS patients with
IIa C
arrhythmic syncope.
Quinidine may be considered in (a) SQTS patients who qualify for
an ICD but present a contraindication to the ICD or refuse it, and IIb C
(b) asymptomatic SQTS patients and a family history of SCD.
Isoproterenol may be considered in emergency situations in SQTS
IIb C
patients with an electrical storm.
PES is not recommended for SCD risk stratification in SQTS patients. III C
CA, cardiac arrest; ICD, implantable cardioverter defibrillator; ILR, Implantable loop recorder;
PES, programmed electrical stimulation; SD, sudden death; SQTS, short QT syndrome; VF,
ventricular fibrillation; VT, ventricular tachycardia.
Selected populations
This section includes recommendations on VA and SCD in:
1. Pregnant patients and peripartum cardiomyopathy including:
Electrocardioversion and ICD therapy, pharmacological
treatment, catheter ablation. New-onset VT may present
during pregnancy and risk of recurrent VT is higher in
patients with previous VT and SHD. Peripartum
cardiomyopathy should be ruled out in the case of new-
onset VT during the last 6 weeks of pregnancy or in the early
postpartum period.
2. Heart transplantation. Patients listed for heart transplantation
are exposed to SCD risk and have a high incidence of VA.
Data from large registries suggest a survival benefit from
ICDs.
3. Sudden cardiac death in athletes. In young competitive
athletes (≤ 35 years), the incidence of fatal events is low, 0.4–
3 per 100,000 participant-years. Therefore pre-participation
cardiovascular evaluation of competitive athletes has been
down-graded to IIa.
4. Wolff–Parkinson–White syndrome. In patients with
ventricular pre-excitation and symptomatic AVRT, catheter
ablation is recommended. In asymptomatic patients with
ventricular pre-excitation, both invasive and non-invasive
assessment are options for risk stratification for SCD.
5. Prevention of sudden cardiac death in the elderly. Age is a
strong risk factor for death. In several studies, advanced age
was one of the factors associated with a reduced expected
benefit of ICD.
Recommendations during pregnancy
Recommendations Class Level
During pregnancy, electrical cardioversion is recommended for
I C
sustained VT.
For acute conversion of haemodynamically
tolerated SMVT during pregnancy, a beta-blocker, sotalol,
IIa C
flecainide, procainamide, or overdrive ventricular pacing should
be considered.
If ICD implantation is indicated during pregnancy, implantation
I C
is recommended with optimal radiation protection.
Continuation of beta-blockers is recommended during
I C
pregnancy and postpartum in women with LQTS or CPVT.
Continuation of beta-blockers should be considered during
IIa C
pregnancy in women with ARVC.
Oral metoprolol, propranolol, or verapamil should be considered
for long-term management of idiopathic sustained VT during IIa C
pregnancy.
Catheter ablation using non-fluoroscopic mapping systems
should be considered, preferably after the first trimester, in
IIa C
women with highly symptomatic recurrent SMVT refractory or
who are intolerant to AADs.
Recommendations before and after heart transplantation
Recommendations Class Level
In patients awaiting heart transplantation, ICD implantation for
IIa C
primary prevention should be considered.
In patients awaiting heart transplantation, WCD may be
IIb C
considered.
In selected transplanted patients with cardiac allograft
vasculopathy or treated rejection, ICD implantation may be IIb C
considered.
Recommendations for risk stratification and prevention of SCD in athletes
Recommendations Class Level
In athletes with positive medical history, abnormal physical
examination, or ECG alterations, further investigations
I C
including echocardiography and/or CMR to confirm (or
exclude) an underlying disease are recommended.
It is recommended that athletes diagnosed with a cardiovascular
disease associated with SCD are managed according to current I C
guidelines for sports eligibility.
It is recommended that staff at sporting facilities are trained
I C
in CPR and in the use of AED.
Pre-participation cardiovascular evaluation of competitive
IIa C
athletes should be considered.
It should be considered that cardiovascular evaluation of young
(< 35 years) competitive athletes includes history, physical IIa C
examination, and 12-lead ECG.
The cardiovascular risk of middle-aged and elderly individuals
should be evaluated before engaging in strenuous sports through IIa C
established scores such as the SCORE2 risk chart.
Recommendations for ICD implantation in the elderly
Recommendations Class Level
In elderly patients in whom a benefit from the defibrillator is not
expected due to the patient’s age and comorbidities, omission IIb B
of ICDimplantation for primary prevention may be considered.
AAD, anti-arrhythmic drug; AED, automated external defibrillator; ARVC,
arrhythmogenic right ventricular cardiomyopathy; CMR, cardiac magnetic
resonance; CPR, cardiopulmonary resuscitation; CPVT, catecholaminergic
polymorphic ventricular tachycardia; ECG, electrocardiogram; ICD, implantable
cardioverter defibrillator; LQTS, long QT syndrome; SCD, sudden cardiac death;
SMVT, sustained monomorphic VT; VT, ventricular tachycardia; WCD, wearable
cardioverter defibrillator.