2022 Esc Guidelines for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: What is New?

Public basic life support and access to automated external defibrillators (AEDs)

Class

It is recommended that public-access defibrillation be available at sites where cardiac arrest (CA) is more likely to occur.^a

I

Prompt cardiopulmonary resuscitation (CPR) by bystanders is recommended at out-of-hospital CA.

I

It is recommended to promote community training in basic life support to increase bystander CPR rate and AED use.

I

Mobile phone-based alerting of basic life support-trained bystander volunteers to assist nearby victims of out-of-hospital CA should be considered.

IIa

Ventricular arrhythmia (VA) treatment. General aspects

Class

Direct current cardioversion is recommended as the first-line treatment for patients presenting with tolerated sustained monomorphic ventricular tachycardia (SMVT) provided that the anesthetic/sedation risk is low.

I

Optimal medical treatment, including angiotensin-converting enzyme inhibitor/ angiotensin receptor blocker/angiotensin receptor neprilysin inhibitor, mineralocorticoid receptor antagonist, and sodium–glucose co-transporter 2 inhibitors, is indicated in all heart failure patients with reduced ejection fraction (EF).

I

Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good-quality survival >1 year.

I

In patients presenting with a hemodynamically tolerated SMVT and known or suspected structural heart disease (SHD), intravenous procainamide should be considered.

IIa

In patients presenting with a hemodynamically tolerated SMVT in the absence of an established diagnosis, intravenous amiodarone may be considered.

IIb

In patients with SMVT or sustained polymorphic ventricular tachycardia (SPVT)/ventricular fibrillation (VF) triggered by a premature ventricular complex (PVC) with similar morphology and an indication for implantable cardioverter defibrillator (ICD), catheter ablation may be considered when an ICD is not available, contraindicated for concurrent medical reasons, or declined by the patient.

IIb

The wearable cardioverter defibrillator may be considered in the early phase after myocardial infarction (MI) in selected patients.

IIb

Coronary artery disease (CAD)

Class

In patients with CAD and recurrent, symptomatic SMVT, or ICD shocks for SMVT despite chronic amiodarone therapy, catheter ablation is recommended in preference to escalating anti-arrhythmic drug (AAD) therapy.

I

Cardiac stress imaging during physical exercise is recommended in addition to cardiopulmonary exercise test after surgery in patients with anomalous aortic origin of a coronary artery with a history of aborted CA.

I

In sudden cardiac arrest (SCA) survivors with coronary artery spasm, implantation of an ICD should be considered.

IIa

ICD therapy should be considered in patients with CAD, New York Heart Association Class I, and left ventricular EF (LVEF) ≤30% despite ≥3 months of optimal medical treatment.

IIa

ICD implantation should be considered in patients with CAD, LVEF ≤40% despite ≥3 months of optimal medical treatment and non-sustained ventricular tachycardia (NSVT), if they are inducible for SMVT by programmed electrical stimulation (PES).

IIa

In patients with CAD and hemodynamically well-tolerated SMVT and LVEF ≥40%, catheter ablation in experienced centers should be considered as an alternative to ICD therapy, provided that established endpoints have been reached.^b

IIa

Catheter ablation should be considered in patients with CAD and recurrent, symptomatic SMVT, or ICD shocks for SMVT despite beta-blocker or sotalol treatment.

IIa

Idiopathic PVC/VT and PVC-induced cardiomyopathy

Class

Catheter ablation as first-line treatment is recommended for symptomatic idiopathic VT/PVCs from the right ventricle outflow tract (RVOT) or the left fascicles.

I

Beta-blockers or non-dihydropyridine calcium channel blockers are indicated in symptomatic patients with idiopathic VT/PVCs from an origin other than the RVOT or the left fascicles.

I

In patients with PVCs/VT with a presentation that is not typical for an idiopathic origin^с, cardiac magnetic resonance (CMR) should be considered, despite a normal echocardiogram.

IIa

Beta-blockers, non-dihydropyridine calcium channel blockers, or flecainide should be considered when catheter ablation is not available, not desired, or is particularly risky in symptomatic patients with idiopathic VT/PVCs from the RVOT or the left fascicles.

IIa

In symptomatic patients with idiopathic VT/PVCs from an origin other than the RVOT or the left fascicles, catheter ablation or flecainide should be considered.

IIa

In patients with an unexplained reduced EF and a PVC burden of at least 10%, PVC-induced cardiomyopathy should be considered.

IIa

CMR should be considered in patients with suspected PVC-induced cardiomyopathy.

IIa

In patients who do not respond to cardiac resynchronization therapy with frequent, predominately monomorphic PVCs limiting optimal biventricular pacing despite pharmacological therapy, catheter ablation or AADs should be considered.

IIa

Catheter ablation may be considered for idiopathic VT/PVCs in asymptomatic patients that repeatedly have more than 20% of PVCs per day at follow-up.

IIb

Amiodarone as a first-line treatment is not recommended in patients with idiopathic VTs/PVCs.

III

Dilated cardiomyopathy (DCM)/hypokinetic non-dilated cardiomyopathy (HNDCM)

Class

Genetic testing (including at least LMNA, PLN, RBM20, and FLNC genes) is recommended in patients with DCM/HNDCM and atrioventricular (AV) conduction delay at <50 years or who have a family history of DCM/HNDCM or SCD in a first-degree relative (at age <50 years).

I

In a first-degree relative of a DCM/HNDCM patient, an electrocardiogram (ECG), and an echocardiogram are recommended if:

the index patient was diagnosed <50 years of age or has clinical features suggestive of an inherited cause, or

there is a family history of DCM/HNDCM or premature unexpected sudden death (SD).

I

CMR with late gadolinium enhancement (LGE) should be considered in DCM/HNDCM patients for assessing the etiology and the risk of VA/SCD.

IIa

Genetic testing (including at least LMNA, PLN, RBM20, and FLNC genes) should be considered for risk stratification in patients with apparently sporadic DCM/HNDCM, who present at young age or with signs suspicious for an inherited etiology.

IIa

ICD implantation should be considered in DCM/HNDCM patients with an LVEF <50% and ≥2 risk factors (syncope, LGE on CMR, inducible SMVT at PES, and pathogenic mutations in LMNA, PLN, FLNC, and RBM20 genes).

IIa

ICD implantation should be considered in patients with DCM/HNDCM and hemodynamically tolerated SMVT.

IIa

In a first-degree relative of a patient with apparently sporadic DCM/HNDCM, an ECG and an echocardiogram may be considered.

IIb

Participation in high-intensity exercise including competitive sports is not recommended for individuals with DCM/HNDCM and an LMNA mutation.

III

Arrhythmogenic right ventricular cardiomyopathy (ARVC)

Class

CMR is recommended in patients with suspected ARVC.

I

In patients with a suspected or definite diagnosis of ARVC, genetic counseling and testing are recommended.

I

ICD implantation should be considered in symptomatic^d patients with definite ARVC, moderate right or left ventricular (LV) dysfunction, and either NSVT or inducibility of SMVT at PES.

IIa

In ARVC patients with indication for ICDs, a device with the capability of anti-tachycardia pacing programming for SMVT up to high rates should be considered.

IIa

Avoidance of high-intensity^e exercise may be considered in carriers of ARVC-related pathogenic mutations and no phenotype.

IIb

Beta-blocker therapy may be considered in all patients with a definite diagnosis of ARVC.

IIb

In patients with ARVC and symptoms highly suspicious for VA, PES may be considered for risk stratification.

IIb

Hypertrophic cardiomyopathy (HCM)

Class

CMR with LGE is recommended in HCM patients for diagnostic work-up.

I

Genetic counseling and testing are recommended in HCM patients.

I

In a first-degree relative of a patient with HCM, ECG and an echocardiogram are recommended.

I

ICD implantation should be considered in HCM patients aged 16 years or more with an intermediate 5-year risk of SCD (≥ 4% to < 6%)^f, and with (a) significant LGE at CMR (usually ≥15% of LV mass); or (b) LVEF <50%; or (c) abnormal blood pressure response during exercise test^g; or (d) LV apical aneurysm; or (e) presence of sarcomeric pathogenic mutation.

IIa

ICD implantation should be considered in children <16 years of age with HCM with an estimated 5-year risk of SD ≥ 6% (based on HCM Risk-Kids score^h).

IIa

ICD implantation should be considered in patients with HCM presenting with hemodynamically tolerated SMVT.

IIa

In patients with HCM and recurrent, symptomatic VA, or recurrent symptomatic ICD therapy, AAD treatment should be considered.

IIa

Participation in high-intensity exercise may be considered for asymptomatic adult HCM patients without risk markers.

IIb

ICD implantation may be considered in HCM patients aged 16 years or more with a low estimated 5-year risk of SCD (<4%),^f and with (a) significant LGE at CMR (usually ≥15% of LV mass); or (b) LVEF <50%; or (c) LV apical aneurysm.

IIb

Catheter ablation in specialized centers may be considered in selected patients with HCM and recurrent, symptomatic SMVT, or ICD shocks for SMVT, in whom AADs are ineffective, contraindicated, or not tolerated.

IIb

LV non-compaction (LVNC) and restrictive cardiomyopathy

Class

In patients with an LVNC cardiomyopathy phenotype based on CMR or echocardiography, implantation of an ICD for primary prevention of SCD should be considered to follow DCM/HNDCM recommendations.

IIa

An ICD should be considered in patients with light-chain amyloidosis or transthyretin-associated cardiac amyloidosis and hemodynamically not tolerated VT.

IIa

Neuromuscular diseases

Class

Invasive electrophysiological evaluation (IEE) is recommended in patients with myotonic dystrophy and palpitations or syncope suggestive of VA or surviving a CA.

I

ICD implantation is recommended in patients with myotonic dystrophy and SMVT or aborted CA not caused by bundle branch re-entrant ventricular tachycardia (BBR-VT).

I

IEE should be considered in patients with myotonic dystrophy and a sudden increase in the PR interval or QRS duration.

IIa

IEE should be considered in patients with myotonic dystrophy and a PR interval ≥240 ms or QRS duration ≥120 ms, who are older than 40 years and have supraventricular arrhythmias, or who are older than 40 years and have significant LGE on CMR.

IIa

In myotonic dystrophy patients without AV conduction delay and a syncope that is highly suspicious for VA, ICD implantation should be considered.

IIa

In myotonic dystrophy patients with palpitations that are highly suspicious for VA and induction of a non-BBR-VT, ICD implantation should be considered.

IIa

In patients with limb-girdle type 1B or Emery–Dreifuss muscular dystrophies and indication for pacing, ICD implantation should be considered.

IIa

Implantation of an ICD may be considered in patients with Duchenne/Becker muscular dystrophy and significant LGE at CMR.

IIb

Implantation of an ICD over a permanent pacemaker may be considered in myotonic dystrophy patients with additional risk factorsⁱ for VA and SCD.

IIb

In patients with myotonic dystrophy, serial electrophysiological evaluation of AV conduction and arrhythmia induction is not recommended without arrhythmia suspicion or progression of ECG conduction disorders.

III

Inflammatory diseases

Class

In patients with hemodynamically NSVT or VF during the acute phase of myocarditis, ICD implantation before hospital discharge should be considered.

IIa

In post-myocarditis patients with recurrent, symptomatic VT, AAD treatment should be considered.

IIa

Catheter ablation performed in specialized centers should be considered in post-myocarditis patients with recurrent, symptomatic SMVT, or ICD shocks for SMVT in whom AADs are ineffective, not tolerated, or not desired.

IIa

ICD implantation should be considered in patients with hemodynamically tolerated SMVT occurring in the chronic phase of myocarditis.

IIa

In patients with cardiac sarcoidosis who have an LVEF >35%, but significant LGE at CMR after resolution of acute inflammation, ICD implantation should be considered.

IIa

In patients with cardiac sarcoidosis, who have an LVEF 35%–50% and minor LGE at CMR, after resolution of acute inflammation, PES for risk stratification should be considered.

IIa

In patients with cardiac sarcoidosis, LVEF 35%–50%, and inducible SMVT at PES, ICD implantation should be considered.

IIa

In patients with cardiac sarcoidosis and recurrent, symptomatic VA, AAD treatment should be considered.

IIa

Amiodarone should be considered to reduce arrhythmia burden in patients with Chagas’ cardiomyopathy who present with symptomatic PVCs or VT.

IIa

In patients with Chagas’ cardiomyopathy and recurrent, symptomatic SMVT, or ICD shocks for SMVT in whom AADs are ineffective, contraindicated, or not tolerated, catheter ablation in specialized centers should be considered.

IIa

In patients with hemodynamically well-tolerated SMVT occurring in the chronic phase of myocarditis, preserved LV function and a limited scar amenable to ablation, catheter ablation may be considered as an alternative to ICD therapy, after discussion with the patient and provided that established endpoints have been reached.^b

IIb

Catheter ablation in specialized centers may be considered in cardiac sarcoidosis ICD recipients with recurrent, symptomatic SMVT, or ICD shocks for SMVT, in whom AADs are ineffective, contraindicated, or not tolerated.

IIb

Congenital heart disease (CHD)

Class

Evaluation for residual lesions or new structural abnormalities is recommended in patients with CHD presenting with sustained VAs.

I

In selected patients with CHD (including atrial baffle repair for transposition of the great arteries, Fontan operation, and Ebstein anomaly) presenting with CA, evaluation and treatment of supraventricular tachycardia with rapid ventricular conduction should be considered.

IIa

In patients with repaired tetralogy of Fallot (TOF) undergoing surgical or transcutaneous pulmonary valve replacement, pre-operative catheter mapping, and transection of VT-related anatomical isthmuses before or during the intervention may be considered.

IIb

In patients with repaired TOF, a preserved biventricular function, and symptomatic SMVT, catheter ablation or concomitant surgical ablation performed in specialized centers may be considered as an alternative to ICD therapy.

IIb

Idiopathic VF

Class

It is recommended that idiopathic VF is diagnosed in a SCA survivor, preferably with documentation of VF, after exclusion of an underlying structural, channelopathic, metabolic, or toxicological etiology.

I

Isoproterenol infusion, verapamil, or quinidine for acute treatment of an electrical storm or recurrent ICD discharges should be considered in idiopathic VF.

IIa

Quinidine should be considered for chronic therapy to suppress an electrical storm or recurrent ICD discharges in idiopathic VF.

IIa

Clinical testing (history, ECG, and high precordial lead ECG, exercise test, and echocardiogram) of first-degree family members of idiopathic VF patients may be considered.

IIb

In idiopathic VF patients, genetic testing of genes related to channelopathy and cardiomyopathy may be considered.

IIb

Long QT syndrome (LQTS)

Class

In patients with clinically diagnosed LQTS, genetic testing and genetic counseling are recommended.

I

Beta-blockers, ideally non-selective beta-blockers (nadolol or propranolol), are recommended in LQTS patients with documented QT interval prolongation to reduce risk of arrhythmic events.

I

Mexiletine is indicated in LQT3 patients with a prolonged QT interval.

I

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.

IIa

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).

IIb

Routine diagnostic testing with epinephrine challenge is not recommended in LQTS.

III

Andersen–Tawil syndrome (ATS)

Class

Genetic testing is recommended in patients with suspected ATS.

I

ICD implantation is recommended in ATS patients after aborted CA or not tolerated sustained VT.

I

ATS should be considered in patients without SHD who present with at least two of the following:

- Prominent U waves with or without prolongation of the QT interval.

- Bidirectional and/or polymorphic PVCs/VT.

- Dysmorphic features.

- Periodic paralysis.

- KCNJ2 pathogenic loss of function mutation.

IIa

Beta-blockers and/or flecainide with or without acetazolamide should be considered in ATS patients to treat VA.

IIa

An implantable loop recorder (ILR) should be considered in ATS patients and unexplained syncope.

IIa

ICD implantation may be considered in ATS patients who have a history of unexplained syncope or suffer from tolerated sustained VT.

IIb

Brugada syndrome (BrS)

Class

Genetic testing for SCN5A gene is recommended for probands with BrS.

I

BrS should be considered in patients with no other heart disease and induced type 1 Brugada pattern who have at least one of the following:

- Arrhythmic syncope or nocturnal agonal respiration.

- A family history of BrS.

- A family history of SD (< 45 years) with a negative autopsy and circumstance suspicious for BrS.

IIa

Implantation of a loop recorder should be considered in BrS patients with an unexplained syncope.

IIa

BrS may be considered as a diagnosis in patients with no other heart disease who exhibit an induced type 1 Brugada ECG.

IIb

PES may be considered in asymptomatic patients with a spontaneous type I BrS ECG.

IIb

Sodium channel blocker test is not recommended in patients with a prior type I Brugada pattern.

III

Catheter ablation in asymptomatic BrS patients is not recommended.

III

Early repolarization syndrome (ERS)

Class

It is recommended that the early repolarization pattern (ERP) is diagnosed as J-point elevation of ≥ 1 mm in two adjacent inferior and/or lateral ECG leads.

I

It is recommended that the ERS is diagnosed in a patient resuscitated from unexplained VF/polymorphic VT (PVT) in the presence of ERP.

I

ICD implantation is recommended in patients with a diagnosis of ERS who have survived a CA.

I

In a SCD victim with a negative autopsy and medical chart review, and an ante-mortem ECG demonstrating the ERP, the diagnosis of ERS should be considered.

IIa

First-degree relatives of ERS patients should be considered for clinical evaluation for ERP with additional high-risk features.^j

IIa

ILR should be considered in individuals with ERP and at least one risk feature^k or arrhythmic syncope.

IIa

Isoproterenol infusion should be considered for ERS patients with electrical storm.

IIa

Quinidine in addition to an ICD should be considered for recurrent VF in ERS patients.

IIa

PVC ablation should be considered in ERS patients with recurrent VF episodes triggered by similar PVC non-responsive to medical treatment.

IIa

Genetic testing in ERS patients may be considered.

IIb

ICD implantation or quinidine may be considered in individuals with ERP and arrhythmic syncope and additional risk features.^k

IIb

ICD implantation or quinidine may be considered in asymptomatic individuals who demonstrate a high-risk ERP^j in the presence of a family history of unexplained juvenile SD.

IIb

Clinical evaluation is not recommended routinely in asymptomatic subjects with ERP.

III

ICD implantation is not recommended in asymptomatic patients with an isolated ERP.

III

Catecholaminergic polymorphic VT (CPVT)

Class

Genetic testing and genetic counseling are indicated in patients with clinical suspicion or clinical diagnosis of CPVT.

I

Beta-blockers, ideally non-selective (nadolol or propranolol), are recommended in all patients with a clinical diagnosis of CPVT.

I

Epinephrine or isoproterenol challenge may be considered for the diagnosis of CPVT when an exercise test is not possible.

IIb

Short QT syndrome (SQTS)

Class

Genetic testing is indicated in patients diagnosed with SQTS.

I

SQTS should be considered in the presence of a QTc ≤ 320 ms.

IIa

SQTS should be considered in the presence of a QTc ≥ 320 and ≤ 360 ms and arrhythmic syncope.

IIa

ILR should be considered in young SQTS patients.

IIa

ICD implantation should be considered in SQTS patients with arrhythmic syncope.

IIa

SQTS may be considered in the presence of a QTc ≥320 and ≤360 ms and a family history of SD at age < 40 years.

IIb

Quinidine may be considered in (a) SQTS patients who qualify for an ICD, but present a contraindication to the ICD or refuse it, and (b) asymptomatic SQTS patients and a family history of SCD.

IIb

Isoproterenol may be considered in SQTS patients with an electrical storm.

IIb

Selected populations

Class

It is recommended that athletes diagnosed with a cardiovascular disease associated with SCD are managed according to current guidelines for sports eligibility.

I

Continuation of beta-blockers should be considered during pregnancy in women with ARVC.

IIa

Oral metoprolol, propranolol, or verapamil should be considered for long-term management of idiopathic sustained VT during pregnancy.

IIa

Catheter ablation using non-fluoroscopic mapping systems should be considered, preferably after the first trimester, in women with highly symptomatic recurrent SMVT refractory or who are intolerant to AADs.

IIa