ESC 2022 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death
European Society of Cardiology｜循環器 / 不整脈｜130 ページ｜チャンク 1130｜推奨 412 件｜current
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| 頁 | クラス | レベル | 推奨文 | 表 |
|---|---|---|---|---|
| p.18 | I | B | Prompt CPR by bystanders is recommended at OHCA.93–95 | Recommendation Table 1 — Recommendations for public basic life support and access to automated external defibrillators |
| p.18 | I | B | It is recommended that public access defibrillation be available at sites where cardiac arrest is more likely to occur.c,90–92 | Recommendation Table 1 — Recommendations for public basic life support and access to automated external defibrillators |
| p.18 | I | B | It is recommended to promote community training in basic life support to increase bystander CPR rate and AED use.93,97,104 | Recommendation Table 1 — Recommendations for public basic life support and access to automated external defibrillators |
| p.18 | IIa | B | Mobile phone-based alerting of basic life support-trained bystander volunteers to assist nearby OHCA victims should be considered.101–103,105 | Recommendation Table 1 — Recommendations for public basic life support and access to automated external defibrillators |
| p.22 | I | B | Genetic testing is recommended when a condition is diagnosed in a living or deceased individual with a likely genetic basis and a risk of VA and SCD.56,183 | Recommendation Table 2 — Recommendations for genetic testing |
| p.22 | I | C | When a putative causative variant is first identified, evaluation for pathogenicity is recommended using an internationally accepted framework.176 | Recommendation Table 2 — Recommendations for genetic testing |
| p.22 | I | C | When a Class IV or Class V variant has been identified in a living or deceased individual with a condition that carries a risk of VA and SCD, genetic testing of first-degree and symptomatic relatives and obligate carriers is recommended. | Recommendation Table 2 — Recommendations for genetic testing |
| p.22 | I | C | It is recommended that genetic testing and counselling on its potential consequences should be undertaken by an expert multidisciplinary team.179 | Recommendation Table 2 — Recommendations for genetic testing |
| p.22 | I | C | It is recommended that Class III (variants of uncertain significance) and Class IV variants should be evaluated for segregation in families where possible, and the variant re-evaluated periodically. | Recommendation Table 2 — Recommendations for genetic testing |
| p.22 | III | C | It is not recommended to undertake genetic testing in index patients with insufficient evidence of a genetic disease. SCD, sudden cardiac death; VA, ventricular arrhythmia. aClass of recommendation. bLevel of evidence. | Recommendation Table 2 — Recommendations for genetic testing |
| p.22 | I | C | In patients with newly documented VA (frequent PVCs, NSVT, SMVT), a baseline 12-lead ECG, recording of the VA on 12-lead ECG, whenever possible, and an echocardiogram are recommended as first-line evaluation. | Recommendation Table 3 — Recommendations for evaluation of patients presenting with newly documented ventricular arrhythmia |
| p.22 | IIa | B | In patients with newly documented VA (frequent PVCs, NSVT, SMVT) and suspicion of SHD other than CAD after initial evaluation, a CMR should be | Recommendation Table 3 — Recommendations for evaluation of patients presenting with newly documented ventricular arrhythmia |
| p.22 | IIa | C | considered.194,195 In patients with an incidental finding of a NSVT, a ≥24 h Holter ECG should be considered. | Recommendation Table 3 — Recommendations for evaluation of patients presenting with newly documented ventricular arrhythmia |
| p.24 | IIb | C | In patients presenting with a first SMVT episode, electrophysiological study, electroanatomical mapping, and mapping-guided biopsies may be considered for aetiological evaluation.197–199,202 SMVT, sustained monomorphic ventricular tachycardia. aClass of recommendation. bLevel of evidence. | Recommendation Table 4 — Recommendations for evaluation of patients presenting with a first episode of sustained monomorphic ventricular tachycardia |
| p.26 | I | B | Diagnostic evaluation The investigation of a SCA survivor without obvious extra-cardiac cause is recommended to be overseen by a multidisciplinary team.177,251–256 | Recommendation Table 5 — Recommendations for evaluation of sudden cardiac arrest survivors |
| p.26 | I | C | In electrically unstable patients after SCA, with suspicion of ongoing myocardial ischaemia, a coronary angiogram is indicated. | Recommendation Table 5 — Recommendations for evaluation of sudden cardiac arrest survivors |
| p.27 | I | B | with uncertain cause of death.267,268 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | IIa | C | In SCA survivors, brain/chest CT scan should be considered when patient characteristics, ECG, and echocardiography are not consistent with a cardiac cause.212,257 | |
| p.27 | I | B | In SCA survivors, collection of blood samples at presentation is recommended for potential toxicology and genetic testing.56,214 | |
| p.27 | I | B | Retrieval of recordings from CIEDs and wearable monitors is recommended for all SCA survivors.217,218 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | In SCA survivors, repeated 12-lead ECGs during stable rhythm (including high precordial lead ECG), as well as continuous cardiac monitoring, | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | C | are recommended.220,222 Echocardiography is recommended for evaluation of cardiac structure and function in all SCA survivors. | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | Coronary imaging and CMR with LGE are recommended for evaluation of cardiac structure and function in all SCA survivors without a clear underlying cause.62,222,223,226 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | Sodium channel blocker test and exercise testing is recommended in SCA survivors without a clear underlying cause.117,222,258–260 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | IIb | B | In SCA survivors, ergonovine, acetylcholine, or hyperventilation testing may be considered for the diagnosis of coronary vasospasm.240,261 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | Investigation of unexpected SD, especially in case of suspicion of inherited disease, should be made a public health priority.20,25,56 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | In cases of SD, it is recommended to collect a detailed description of circumstances of death, symptoms prior to death, the family history, and to review prior medical files.25,56 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | A comprehensive autopsy is recommended, ideally, in all cases of unexpected SD, and always in those ,50 years of age.183,264,265,267,269,270 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | In cases of SCD, it is recommended to retain samples suitable for DNA extraction and consult a cardiac pathologist when an inherited cause is suspected or the cause of death © ESC 2022 unexplained.264,265 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | Toxicology screens are recommended in SD cases For SCD where the cause is known or suspected to be heritable, genetic testing targeted to the cause is recommended.56,266,269 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | Following SADS, post-mortem genetic testing targeted to primary electrical disease is recommended when the decedent is young (,50) and/or the circumstances and/or family history support a primary electrical disease.56,183,223 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | When an autopsy diagnoses possible heritable cardiac disease, it is recommended to refer first-degree relatives for cardiac assessment in a specialized clinic.271,272 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | I | B | In non-autopsied cases of SD where inherited cardiac disease is suspected, it is recommended to refer first-degree relatives for cardiac assessment in a specialized clinic.223,253,273 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | IIb | C | Following SADS, post-mortem genetic testing in the decedent for additional genes may be considered. | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.27 | III | B | Following SADS, hypothesis-free post-mortem genetic testing using exome or genome sequencing is not recommended.274,275 | Recommendation Table 6 — Recommendations for evaluation of sudden death victims |
| p.31 | I | B | Familial evaluation of SADS decedents is recommended: • for first-degree relatives • for relatives who must carry a mutation based on analysis of the family history • for relatives with suspicious symptoms • when the decedent’s age is ,50 years, or if there is other circumstantial data or family | Recommendation Table 7 — Recommendations for evaluation of relatives of sudden arrhythmic death syndrome decedents |
| p.31 | I | B | history to suggest heritable disease.223,252,253,277,281 Familial evaluation of SADS decedents is recommended to include genetic testing when post-mortem genetic testing in a SADS decedent detects a pathogenic mutation.183,253,277,281 | Recommendation Table 7 — Recommendations for evaluation of relatives of sudden arrhythmic death syndrome decedents |
| p.31 | I | B | Baseline familial evaluation of SADS decedents is recommended to include taking a medical history and performing physical examination, standard and high precordial lead ECG, echocardiography, and exercise testing.223,252,253,277,281 | Recommendation Table 7 — Recommendations for evaluation of relatives of sudden arrhythmic death syndrome decedents |
| p.31 | I | C | In SADS families without a diagnosis after clinical evaluation, follow-up is recommended for children of decedents until they reach adulthood.181,284 | Recommendation Table 7 — Recommendations for evaluation of relatives of sudden arrhythmic death syndrome decedents |
| p.31 | IIa | B | Pharmacological testing with a sodium channel blocker should be considered in relatives of SADS decedents who are 16 years or older when baseline testing and/or proband findings increase the suspicion of BrS.277,281 | Recommendation Table 7 — Recommendations for evaluation of relatives of sudden arrhythmic death syndrome decedents |
| p.31 | IIb | C | Ambulatory cardiac rhythm monitoring and CMR may be considered in relatives of SADS decedents.223,253,277,281 | |
| p.31 | IIb | B | Pharmacological testing including epinephrine challenge (if exercise testing is impractical) and sodium channel blocker challenge may be considered in first-degree relatives of SADS decedents with normal baseline testing.223,281 | |
| p.31 | III | C | In SADS families without a diagnosis after clinical evaluation, follow-up is not recommended for asymptomatic adults who can be discharged with advice to return if they develop symptoms or if the family history changes.181,284 | |
| p.33 | I | B | Withdrawal of offending agents is recommended whenever drug-induced VAs are suspected.293,294,297 | Recommendation Table 8 — Recommendations for treatment of reversible conditions |
| p.33 | I | C | Investigation for reversible causes (e.g. electrolyte imbalances, ischaemia, hypoxaemia, fever)c is recommended in patients with VA.292,298 | Recommendation Table 8 — Recommendations for treatment of reversible conditions |
| p.33 | IIa | C | Despite a possible correctable cause for the presenting VA, the need for ICD implantation should be considered based on an individual evaluation of the risk of subsequent VA/ SCD.286,296,299 | Recommendation Table 8 — Recommendations for treatment of reversible conditions |
| p.37 | I | B | Acute management of sustained VT DC cardioversion is recommended as the first-line treatment for patients with haemodynamically not-tolerated SMVT.303,339 | Recommendation Table 9 — Recommendations for the acute management of sustained ventricular tachycardia and electrical storm |
| p.37 | I | C | DC cardioversion is recommended as the first-line treatment for patients presenting with tolerated SMVT provided that the anaesthetic/ sedation risk is low. | Recommendation Table 9 — Recommendations for the acute management of sustained ventricular tachycardia and electrical storm |
| p.38 | I | C | In patients presenting with a haemodynamically tolerated idiopathic VT, treatment with intravenous beta-blocker (RVOT VT) or verapamil (fascicular VT) is recommended.306,307 | |
| p.38 | IIa | C | In patients presenting with a regular haemodynamically tolerated wide QRS complex tachycardia suspected for SVT, administration of adenosine or vagal manoeuvres should be considered.300 | |
| p.38 | IIa | B | In patients presenting with a haemodynamically tolerated SMVT and known or suspected SHD, intravenous procainamide should be considered.303 | |
| p.38 | IIb | B | In patients presenting with a haemodynamically tolerated SMVT in the absence of an established diagnosis, intravenous amiodarone may be considered.303 | |
| p.38 | IIb | C | In patients presenting with a haemodynamically tolerated SMVT in the absence of significant SHD, flecainide, ajmaline, or sotalol may be considered.304,305 | |
| p.38 | III | B | Intravenous verapamil is not recommended in broad QRS complex tachycardia of unknown mechanism.308,309 Management of electrical storm | |
| p.38 | I | C | Mild to moderate sedation is recommended in patients with electrical storm to alleviate psychological distress and reduce sympathetic tone. | |
| p.38 | I | B | patients with SHD and electrical storm unless contraindicated.317,318 | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.38 | I | C | Intravenous magnesium with supplementation of potassium is recommended in patients with TdP.295 | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.38 | I | C | Isoproterenol or transvenous pacing to increase heart rate is recommended in patients with acquired LQT syndrome and recurrent TdP despite correction of precipitating conditions and | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.38 | I | B | magnesium. Catheter ablation is recommended in patients presenting with incessant VT or electrical storm due to SMVT refractory to AADs.330,331 | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.38 | IIa | C | Deep sedation/intubation should be considered in patients with an intractable electrical storm refractory to drug treatment.325 | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.38 | IIa | C | Catheter ablation should be considered in patients with recurrent episodes of PVT/VF triggered by a similar PVC, non-responsive to medical treatment or coronary revascularization.221,332,333 | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.38 | IIb | C | Quinidine may be considered in patients with CAD and electrical storm due to recurrent PVT when other AAD therapy fails.323,324 | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.38 | IIb | C | Autonomic modulation may be considered in patients with electrical storm refractory to drug treatment and in whom catheter ablation is ineffective or not possible.326,328,340 | |
| p.38 | IIb | C | Institution of mechanical circulatory support may be considered in the management of drug-refractory electrical storm and cardiogenic shock.335 | |
| p.38 | I | A | Optimal medical treatment including ACE-I/ARB/ ARNIs, MRAs, beta-blockers, and SGLT2 inhibitors is indicated in all heart failure patients with reduced EF.343–347 | Recommendation Table 10 — Recommendations for treatment with heart failure medication |
| p.44 | I | C | Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good quality survival .1 year. | Recommendation Table 11 — Recommendations for implantable cardioverter defibrillator implantation (general aspects) |
| p.44 | III | C | It is not recommended to implant an ICD in patients with incessant VAs until the VA is controlled. | Recommendation Table 11 — Recommendations for implantable cardioverter defibrillator implantation (general aspects) |
| p.44 | I | A | ICD implantation is recommended in patients with documented VF or haemodynamically not-tolerated VT in the absence of reversible causes.349–352 | Recommendation Table 12 — Recommendations for secondary prevention of sudden cardiac death* |
| p.44 | IIb | C | In patients with VT/VF, an indication for ICD, and no contraindication for amiodarone, amiodarone may be considered when an ICD is not available, contraindicated for concurrent medical reasons, or declined by the patient. | Recommendation Table 12 — Recommendations for secondary prevention of sudden cardiac death* |
| p.44 | IIb | C | In patients with SMVT or SPVT/VF triggered by a PVC with similar morphology and an indication for ICD, catheter ablation may be considered when an ICD is not available, contraindicated for concurrent medical reasons, or declined by the patient. | Recommendation Table 12 — Recommendations for secondary prevention of sudden cardiac death* |
| p.44 | IIa | B | Subcutaneous defibrillator should be considered as an alternative to transvenous defibrillator in patients with an indication for an ICD when pacing therapy for bradycardia, cardiac resynchronization, or ATP is not needed.366 | Recommendation Table 13 — Recommendations for subcutaneous implantable cardioverter defibrillator |
| p.44 | I | C | When an ICD is indicated, it is recommended to evaluate whether the patient could benefit from CRT-defibrillator.367 | Recommendation Table 14 — Recommendations for adding cardiac resynchronization therapy to implantable cardioverter defibrillator |
| p.44 | IIa | C | © ESC 2022 The WCD should be considered for adult patients with a secondary prevention ICD indication, who are temporarily not candidates for ICD implantation. | Recommendation Table 15 — Recommendations for wearable cardioverter defibrillator |
| p.45 | IIb | B | after MI in selected patients.371,372 | |
| p.45 | I | B | alerts.397–399 | |
| p.45 | I | B | the incidence of inappropriate shocks.395 | |
| p.45 | I | B | recommended over ramp ATP.392 | |
| p.45 | I | A | Optimization of ICD programming is indicated to avoid inappropriate and unnecessary therapies and to reduce mortality.373–375 | Recommendation Table 16 — Recommendations for optimization of device programming |
| p.45 | I | A | © ESC 2022 In single- or dual-chamber ICD patients without bradycardia pacing indications, it is recommended to minimize ventricular pacing.378–380 | |
| p.45 | I | A | Programming of prolonged detection settings is indicated (duration criteria of at least 6–12 s or 30 intervals).373,382,383 | |
| p.45 | I | A | It is recommended to program the slowest tachycardia therapy zone limit ≥188 b.p.m. in primary prevention ICD patients.382,383 | |
| p.45 | I | A | In patients with SHD, programming of at least one ATP therapy is recommended in all tachyarrhythmias zones.375,384,391 | |
| p.45 | I | B | It is recommended to program algorithms for SVT vs. VT discrimination for tachycardias with rates up to 230 b.p.m.383–385 It is recommended to activate lead failure Remote monitoring is recommended to reduce | |
| p.45 | I | B | Programming of burst ATP as first attempt is For S-ICDs, a dual detection zone configuration is recommended with activation of discrimination algorithm in the lower conditional shock zone.388–390 | |
| p.45 | IIa | B | For routine ICD programming, activation of more than one tachycardia detection zone should be considered.383,386 | |
| p.46 | IIa | B | recipients with symptomatic sustained VAs.425,431 | Recommendation Table 19 — Recommendations for implantable cardioverter defibrillator implantation in left ventricular assist device recipients |
| p.46 | I | C | Catheter ablation is recommended for ICD patients with recurrent SVT resulting in inappropriate ICD therapies.401,402 | Recommendation Table 17 — Recommendations for concomitant treatment to avoid inappropriate implantable cardioverter defibrillator therapy |
| p.46 | I | C | Pharmacological treatment or catheter ablation is recommended in patients with AF-related inappropriate ICD therapies despite optimal ICD programming.401,405,407 | Recommendation Table 17 — Recommendations for concomitant treatment to avoid inappropriate implantable cardioverter defibrillator therapy |
| p.46 | I | C | Assessment of psychological status and treatment of distress is recommended in ICD patients.421–423 | Recommendation Table 18 — Recommendations for psychosocial management after implantable cardioverter defibrillator implantation |
| p.46 | I | C | Communication between patient and physician/ healthcare professional is recommended to address ICD-related concerns and to discuss quality-of-life issues before ICD implantation and during disease progression.412,424 ICD, implantable cardioverter defibrillator. aClass of recommendation. bLevel of evidence. | Recommendation Table 18 — Recommendations for psychosocial management after implantable cardioverter defibrillator implantation |
| p.47 | I | A | Single-chamber ICD is recommended over dual-chamber ICD in primary prevention patients without current or expected indication for atrial or AV sequential pacing due to a lower risk of device-related complications.447,448,450 | Recommendation Table 20 — Recommendations for prevention of implantable cardioverter defibrillator complications |
| p.47 | IIa | C | The use of single-coil leads over dual-coil ICD leads should be considered due to lower complication rate during transvenous lead extraction.451 AV, atrioventricular; ICD, implantable cardioverter defibrillator. aClass of recommendation. bLevel of evidence. | Recommendation Table 20 — Recommendations for prevention of implantable cardioverter defibrillator complications |
| p.47 | I | C | Informed discussion with patient and family about ICD deactivation options and shared decision-making is indicated prior to implantation and in case of significant health status deterioration.458 ICD, implantable cardioverter defibrillator. aClass of recommendation. bLevel of evidence. | Recommendation Table 21 — Recommendations for end-of-life issues in implantable cardioverter defibrillator carriers |
| p.50 | III | B | beta-blockers) is not recommended in ACS.322 | Recommendation Table 22 — Recommendations for treatment of ventricular arrhythmias in acute coronary syndrome and vasospasm |
| p.50 | I | B | recommended in all patients with acute MI.567,568 | Recommendation Table 23 — Recommendations for risk stratification and treatment of ventricular arrhythmias early after myocardial infarction |
| p.50 | I | B | Treatment of VAs in ACS Intravenous beta-blocker treatment is indicated for patients with recurrent PVT/VF during STEMI unless contraindicated.551,552 | Recommendation Table 22 — Recommendations for treatment of ventricular arrhythmias in acute coronary syndrome and vasospasm |
| p.50 | IIa | C | Intravenous amiodarone treatment should be considered for patients with recurrent PVT/VF during the acute phase of ACS.552,554,555 | Recommendation Table 22 — Recommendations for treatment of ventricular arrhythmias in acute coronary syndrome and vasospasm |
| p.50 | IIb | C | Intravenous lidocaine may be considered for the treatment of recurrent PVT/VF not responding to beta-blockers or amiodarone, or if amiodarone is contraindicated during the acute phase of ACS.554 Prophylactic treatment with AADs (other than | Recommendation Table 22 — Recommendations for treatment of ventricular arrhythmias in acute coronary syndrome and vasospasm |
| p.50 | IIa | C | Vasospasm In SCA survivors with coronary artery spasm, implantation of an ICD should be considered.562–564 | Recommendation Table 22 — Recommendations for treatment of ventricular arrhythmias in acute coronary syndrome and vasospasm |
| p.50 | I | C | Risk stratification Early (before discharge) assessment of LVEF is In patients with pre-discharge LVEF ≤40%, re-evaluation of LVEF 6–12 weeks after MI is recommended to assess the potential need for primary prevention ICD implantation.568,573,574 Treatment of VAs | Recommendation Table 23 — Recommendations for risk stratification and treatment of ventricular arrhythmias early after myocardial infarction |
| p.50 | IIa | C | Catheter ablation should be considered in patients with recurrent episodes of PVT/VF triggered by a similar PVC non-responsive to medical treatment or coronary revascularization in the subacute phase of MI.332 | Recommendation Table 23 — Recommendations for risk stratification and treatment of ventricular arrhythmias early after myocardial infarction |
| p.51 | I | C | In patients with syncope and previous STEMI, PES is indicated when syncope remains unexplained after non-invasive evaluation.146,584 | Recommendation Table 24 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in chronic coronary artery disease |
| p.51 | I | A | ICD therapy is recommended in patients with CAD, symptomatic heart failure (NYHA class II–III), and LVEF ≤35% despite ≥3 months of OMT.354,356 | Recommendation Table 24 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in chronic coronary artery disease |
| p.51 | IIa | B | ICD therapy should be considered in patients with CAD, NYHA class I, and LVEF ≤30% despite ≥3 months of OMT.354 | Recommendation Table 24 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in chronic coronary artery disease |
| p.51 | IIa | B | ICD implantation should be considered in patients with CAD, LVEF ≤40% despite ≥3 months of OMT, and NSVT, if they are inducible for SMVT by PES.355 | Recommendation Table 24 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in chronic coronary artery disease |
| p.51 | III | A | In patients with CAD, prophylactic treatment with AADs other than beta-blockers is not recommended.556,578,579 | Recommendation Table 24 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in chronic coronary artery disease |
| p.51 | I | A | ICD implantation is recommended in patients without ongoing ischaemia with documented VF or haemodynamically not-tolerated VT occurring later than 48 h after MI.349–351 | Recommendation Table 24 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in chronic coronary artery disease |
| p.51 | I | B | 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 AAD therapy.471 | Recommendation Table 24 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in chronic coronary artery disease |
| p.51 | IIa | B | The addition of oral amiodarone or beta-blocker replacement by sotalol should be considered in patients with CAD with recurrent, symptomatic SMVT, or ICD shocks for SMVT while on beta-blocker treatment.318,581 | |
| p.51 | IIa | C | In patients with CAD and haemodynamically well-tolerated SMVT and LVEF ≥40%, catheter ablationinexperiencedcentresshouldbeconsidered as an alternative to ICD therapy, provided that established endpoints have been reached.c,480,580 | |
| p.51 | IIa | C | ICD implantation should be considered in patients with a haemodynamically tolerated SMVT and an LVEF ≥40% if VT ablation fails, is not available, or is not desired. | |
| p.51 | IIa | C | Catheter ablation should be considered in patients with CAD and recurrent, symptomatic SMVT, or ICD shocks for SMVT despite beta-blockers or sotalol treatment.471 | |
| p.54 | I | C | Diagnostic evaluation Cardiac stress imaging during physical exercise is recommended in addition to cardiopulmonary exercise test in patients with anomalous aortic origin of a coronary artery with an interarterial course to confirm/exclude myocardial ischaemia.587 | Recommendation Table 25 — Recommendations for sudden cardiac death prevention in patients with coronary anomalies |
| p.54 | I | C | 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. | Recommendation Table 25 — Recommendations for sudden cardiac death prevention in patients with coronary anomalies |
| p.54 | I | C | Treatment Surgery is recommended in patients with anomalous aortic origin of a coronary artery with CA, syncope suspected to be due to VAs, or angina when other causes have been excluded.585,586,588 | Recommendation Table 25 — Recommendations for sudden cardiac death prevention in patients with coronary anomalies |
| p.54 | IIa | C | Surgery should be considered in asymptomatic patients with anomalous aortic origin of a coronary artery and evidence of myocardial ischaemia or abnormal aortic origin of the left coronary artery with high-risk anatomy.c,585,586,588 | Recommendation Table 25 — Recommendations for sudden cardiac death prevention in patients with coronary anomalies |
| p.56 | I | C | General recommendations Regular assessment of ventricular function of patients with PVC burden .10% and normal ventricular function is indicated.602,603 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | IIa | C | In patients with PVCs/VT and a presentation not typical for an idiopathic origin,c CMR should be considered, despite a normal echocardiogram.195 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | I | B | Treatment Catheter ablation as first-line treatment is recommended for symptomatic idiopathic VT/ PVCs from the RVOT or the left fascicles.d,535,595,596,604 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | I | C | Beta-blockers or non-dihydropyridine CCBs are indicated in symptomatic patients with idiopathic VT/PVCs from an origin other than the RVOT or the left fascicles.304,593 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | IIa | B | Beta-blockers, non-dihydropyridine CCBs, or flecainide should be considered when catheter ablation is not available, desired, or is particularly risky in symptomatic patients with idiopathic VT/ PVCs from the RVOT or the left | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | IIa | C | fascicles.304,592,593 Catheter ablation or flecainide should be considered in symptomatic patients with idiopathic VT/PVCs from an origin other than the RVOT or the left fascicles.535,604,605 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | IIb | B | Catheter ablation may be considered for idiopathic VT/PVCs in asymptomatic patients with repeatedly more than 20% of PVCs per day at follow-up.535,600,601 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | III | C | Catheter ablation of idiopathic VT/PVCs is not recommended in children ,5 years of age or ,10 kg weight except when previous medical therapy fails or when VT is not haemodynamically tolerated.597 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | III | C | Amiodarone as a first-line treatment is not recommended in patients with idiopathic VTs/ PVCs.594 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.56 | III | C | Verapamil is not recommended in children ,1 year of age with PVC/VT, particularly if they have signs of heart failure or concurrent use of other AADs.606 | Recommendation Table 26 — Recommendations for the management of patients with idiopathic premature ventricular complexes/ventricular tachycardia |
| p.57 | IIa | C | Diagnostic evaluation In patients with an unexplained reduced EF and a PVC burden of at least 10%, PVC-induced cardiomyopathy should be considered.600,609,610 | Recommendation Table 27 — Recommendations for the management of patients with premature ventricular complex-induced or premature ventricular complexaggravated cardiomyopathy |
| p.57 | IIa | B | In patients with suspected PVC-induced cardiomyopathy, CMR should be considered.590,615 Treatment | Recommendation Table 27 — Recommendations for the management of patients with premature ventricular complex-induced or premature ventricular complexaggravated cardiomyopathy |
| p.57 | I | C | In patients with a cardiomyopathy suspected to be caused by frequent and predominately monomorphic PVCs, catheter ablation is recommended.535,600,609,612,617,618,620 | Recommendation Table 27 — Recommendations for the management of patients with premature ventricular complex-induced or premature ventricular complexaggravated cardiomyopathy |
| p.58 | IIa | C | In patients with a cardiomyopathy suspected to be caused by frequent and predominately monomorphic PVCs, treatment with AADsc should be considered if catheter ablation is not desired, suspected to be high-risk, or unsuccessful.624,626 | |
| p.58 | IIa | B | In patients with SHD in whom predominately monomorphic frequent PVCs are suspected to be contributing to the cardiomyopathy, AAD (amiodarone) treatment or catheter ablation should be considered. 617,621,622,624 | |
| p.58 | IIa | C | In non-responders to CRT with frequent, predominately monomorphic PVCs limiting optimal biventricular pacing despite pharmacological therapy, catheter ablation or AADs should be considered.623 | |
| p.61 | I | B | Genetic testing (including at least LMNA, PLN, RBM20, and FLNC genes) is recommended in patients with DCM/HNDCM and AV conduction delay at ,50 years, or who have a family history of DCM/HNDCM or SCD in a first-degree relative | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.61 | IIa | B | (at age ,50 years).641–645 CMR with LGE should be considered in DCM/ HNDCM patients for assessing the aetiology and the risk of VA/SCD.129,651,667 Genetic testing (including at least LMNA, PLN, | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.61 | IIa | C | 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 | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.61 | III | C | aetiology.641–645 Participation in high-intensity exercise including competitive sports is not recommended for individuals with DCM/HNDCM and a LMNA mutation.655 | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.61 | IIa | A | ICD implantation should be considered in patients with DCM/HNDCM, symptomatic heart failure (NYHA class II–III), and LVEF ≤35% after ≥3 months of OMT.357,359,635,650 | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.61 | IIa | B | ICD implantation should be considered in DCM/ HNDCM patients with a pathogenic mutation in LMNA gene, if the estimated 5-year risk of life-threatening VA is ≥10%c and in the presence of NSVT or LVEF , 50% or AV conduction delay.80,652,653 | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.61 | IIa | C | ICD implantation should be considered in DCM/ HNDCM patients with a LVEF ,50% and ≥2 risk factors (syncope, LGE on CMR, inducible SMVT at PES, pathogenic mutations in LMNA,d PLN, FLNC, and RBM20 genes). | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.61 | IIa | C | In DCM/HNDM patients, electrophysiological evaluation should be considered when syncope remains unexplained after non-invasive evaluation.661,668 | Recommendation Table 28 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy |
| p.62 | I | B | ICD implantation is recommended in patients with DCM/HNDCM, who survive SCA due to VT/ VF or experience haemodynamically not-tolerated SMVT.349,351,352 | |
| p.62 | IIa | C | Catheter ablation in specialized centres should be considered in patients with DCM/HNDCM and recurrent, symptomatic SMVT or ICD shocks for SMVT, in whom AADs are ineffective, contraindicated, or not tolerated.481,497,664,669 | |
| p.62 | IIa | B | The addition of oral amiodarone or replacement of beta-blockers by sotalol should be considered in patients with DCM/HNDCM and an ICD who experience recurrent, symptomatic VA despite optimal device programming and beta-blocker | |
| p.62 | IIa | C | treatment.318 ICD implantation should be considered in patients with DCM/HNDCM and haemodynamically tolerated SMVT. DCM/HNDCM | |
| p.62 | I | C | In a first-degree relative of a DCM/HNDCM patient, an ECG and an echocardiogram are recommended if: •theindexpatientwasdiagnosed,50yearsofageor hasclinicalfeaturessuggestiveofaninheritedcause,or • there is family history of DCM/HNDCM, or | |
| p.62 | IIb | C | premature unexpected SD.646 In a first-degree relative of a patient with apparently sporadic DCM/HNDCM, an ECG and an echocardiogram may be considered.646 | |
| p.64 | I | B | In patients with suspected ARVC, CMR is recommended.676–678 | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | I | C | ECG and echocardiogram are recommended.675 | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | I | B | In patients with a suspected or definite diagnosis of ARVC, genetic counselling and testing are recommended.672,673 | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | I | B | Avoidance of high-intensity exercise is recommended in patients with a definite diagnosis of ARVC.683–685 | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | IIb | C | Avoidance of high-intensityc exercise may be considered in carriers of ARVC-related pathogenic mutations and no phenotype.683,687 | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | IIb | C | Beta-blocker therapy may be considered in all patients with a definite diagnosis of ARVC. | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | IIa | B | ICD implantation should be considered in patients with definite ARVC and an arrhythmic syncope.696,701,711–713 | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | IIa | C | ICD implantation should be considered in patients with definite ARVC and severe RV or LV systolic dysfunction.675,691 | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | IIa | C | ICD implantation should be considered in symptomaticd patients with definite ARVC, moderate right or left ventricular dysfunction, and | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.64 | IIb | C | either NSVT or inducibility of SMVT at PES.695,696,701,703,705 In patients with ARVC and symptoms highly suspicious for VA, PES may be considered for risk stratification.695,705 | |
| p.64 | I | C | ICD implantation is recommended in ARVC patients with haemodynamically not-tolerated VT or VF.700 | |
| p.64 | I | C | In patients with ARVC and non-sustained or sustained VAs, beta-blocker therapy is recommended. | |
| p.64 | IIa | C | In patients with ARVC and recurrent, symptomatic SMVT or ICD shocks for SMVT despite beta-blockers, catheter ablation in specialized centres should be | |
| p.64 | IIa | B | considered.482,709,714 In ARVC patients with indication for ICDs, a device with the capability of ATP programming for SMVT up to high rates should be considered.698 | |
| p.64 | IIa | C | ICD implantation should be considered in ARVC patients with a haemodynamically tolerated SMVT.692 | |
| p.64 | IIa | C | In a first-degree relative of a patient with ARVC, dPresyncope or palpitations suggestive of VA. | Recommendation Table 29 — Recommendations for diagnostic, risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy |
| p.66 | I | B | for diagnostic work-up.716–718 | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | I | B | in HCM patients.721–725 | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIb | B | patients aged 16 years or more with an estimated 5-year risk of SCD of ≥4 to ,6%.c,85,728,729 | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIb | C | CMR with LGE is recommended in HCM patients Genetic counselling and testing are recommended Participation in high-intensity exercise may be considered for asymptomatic adult HCM patients without risk markers.733 | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | I | C | It is recommended that the 5-year risk of SCD is assessed at first evaluation and at 1–3-year intervals, or when there is a change in clinical status. | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIa | B | ICD implantation should be considered in patients aged 16 years or more with an estimated 5-year risk of SD ≥6%.c,85,728,729 ICD implantation should be considered in HCM | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIa | B | patients aged 16 years or more with an intermediate 5-year risk of SCD (≥4 to ,6%)c and with (a) significant LGE at CMR (usually ≥15% of LV mass); or (b) LVEF , 50%; or (c) abnormal blood pressure response during exercise testd; or (d) LV apical aneurysm; or (e) presence of | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIa | B | sarcomeric pathogenic mutation.716,717,722,736–739 In children less than 16 years of age with HCM and an estimated 5-year risk of SD ≥6% (based on HCM Risk-Kids scoree), ICD implantation should be considered.84,742 ICD implantation may be considered in HCM | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIb | B | ICD implantation may be considered in HCM patients aged 16 years or more with a low estimated 5-year risk of SCD (,4%)c and with (a) significant LGE at CMR (usually ≥15% of LV mass); or (b) LVEF , 50%; or (c) LV apical aneurysm.716,717,722,736–739 | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | I | B | ICD implantation is recommended in HCM patients with haemodynamically not-tolerated VT or VF.744–746 | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIa | C | In patients with HCM presenting with haemodynamically tolerated SMVT, ICD implantation should be considered. | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.66 | IIa | C | In patients with HCM and recurrent, symptomatic VA, or recurrent ICD therapy, AAD treatment should be considered. | Recommendation Table 30 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in hypertropic cardiomyopathy |
| p.67 | IIb | C | Catheter ablation in specialized centres may be considered in selected patients with HCM and recurrent, symptomatic SMVT or ICD shocks for SMVT, in whom AAD are ineffective, contraindicated, or not tolerated.753,754 | |
| p.67 | I | C | In a first-degree relative of a patient with HCM, ECG and echocardiogram are recommended. | |
| p.67 | IIa | C | In patients with a 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. | Recommendation Table 31 — Recommendations for implantable cardioverter defibrillator implantation in left ventricular non-compaction |
| p.67 | IIa | C | An ICD should be considered in patients with light-chain amyloidosis or transthyretin-associated cardiac amyloidosis and haemodynamically not-tolerated VT. © ESC 2022 | Recommendation Table 32 — Recommendations for implantable cardioverter defibrillator implantation in patients with cardiac amyloidosis |
| p.69 | I | C | ablation is recommended.e,153,474,475,477 | Recommendation Table 33 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in neuromuscular diseases |
| p.69 | I | C | General recommendations Annual follow-up with at least a 12-lead ECG is recommended in patients with muscular dystrophies, even in the concealed phase of the disease.6,13 | Recommendation Table 33 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in neuromuscular diseases |
| p.69 | I | C | It is recommended that patients with neuromuscular disorders who have VAs or ventricular dysfunction are treated in the same way for arrhythmia as patients without neuromuscular disorders.17,765 | Recommendation Table 33 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in neuromuscular diseases |
| p.69 | I | C | Invasive electrophysiological evaluation is recommended in patients with myotonic dystrophy and palpitations or syncope suggestive of VA or surviving a CA.153 | Recommendation Table 33 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in neuromuscular diseases |
| p.69 | I | C | ICD implantation is recommended in patients with myotonic dystrophy and SMVT or aborted CA not caused by BBR-VT.766 | |
| p.69 | IIa | B | Invasive electrophysiological evaluation should be considered in patients with myotonic dystrophy and a sudden increase in the PR interval or QRS duration.766,767 Invasive electrophysiological evaluation should be | |
| p.69 | IIa | B | considered in patients with myotonic dystrophy and a PR interval ≥240 ms or QRS duration ≥120 ms or who are older than 40 years and have supraventricular arrhythmiasc or who are older than 40 years and have significant LGE on CMR.c,5,14,16,766 | |
| p.69 | IIa | C | In myotonic dystrophy patients without AV conduction delay and a syncope highly suspicious for VA, ICD implantation should be considered.766 | |
| p.69 | IIa | C | In myotonic dystrophy patients with palpitations highly suspicious for VA and induction of a non-BBR-VT, ICD implantation should be considered.766 | |
| p.69 | IIa | C | In patients with limb–girdle type 1B or Emery– Dreifuss muscular dystrophies and indication for pacing, ICD implantation should be considered.769 | |
| p.69 | IIb | C | Implantation of an ICD may be considered in patients with Duchenne/Becker muscular dystrophy and significant LGE at CMR.770,771 | |
| p.69 | IIb | C | Implantation of an ICD over a permanent pacemaker may be considered in myotonic dystrophy patients with additional risk factorsd for VAs and SCD. | |
| p.69 | III | C | In myotonic dystrophy patients, serial electrophysiological evaluation of AV conduction and arrhythmia induction is not recommended without arrhythmia suspicion or progression of ECG conduction disorders.772 Management of VA | |
| p.69 | I | C | In symptomatic patients with BBR-VT, catheter In patients with myotonic dystrophy undergoing ablation for BBR-VT, pacemaker/ICD implantation is recommended.153 | Recommendation Table 33 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in neuromuscular diseases |
| p.70 | I | C | General recommendations In confirmed or clinically suspected acute myocarditis, it is recommended that patients who present with life-threatening VAs are referred to a specialized centre.786,804 | Recommendation Table 34 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in myocarditis |
| p.70 | I | C | In patients with haemodynamically not-tolerated SMVT occurring in the chronic phase of myocarditis, an ICD implantation is recommended.794,805 | Recommendation Table 34 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in myocarditis |
| p.70 | IIa | C | In patients with haemodynamically not-tolerated sustained VT or VF during the acute phase of myocarditis, ICD implantation before hospital discharge should be considered.788,794,806 | Recommendation Table 34 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in myocarditis |
| p.70 | IIa | C | AADs should be considered (preferably amiodarone and beta-blockers) in patients with symptomatic non-sustained or sustained VAs during the acute phase of myocarditis. | Recommendation Table 34 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in myocarditis |
| p.70 | IIa | C | In post-myocarditis patients with recurrent, symptomatic VT, AAD treatment should be considered. | Recommendation Table 34 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in myocarditis |
| p.70 | IIa | C | Catheter ablation, performed in specialized centres, 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.752,801,802 | Recommendation Table 34 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in myocarditis |
| p.70 | IIa | C | In patients with haemodynamically tolerated SMVT occurring in the chronic phase of myocarditis, ICD implantation should be considered. | Recommendation Table 34 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in myocarditis |
| p.71 | IIb | C | In patients with haemodynamically 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.c | |
| p.71 | I | B | ICD implantation is recommended in patients with cardiac sarcoidosis who have a LVEF ≤35%.812,828–830,832 | Recommendation Table 35 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in cardiac sarcoidosis |
| p.71 | IIa | C | In patients with cardiac sarcoidosis who have an indication for permanent cardiac pacing related to high-degree AV block, ICD implantation should be considered, regardless of LVEF.816 | Recommendation Table 35 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in cardiac sarcoidosis |
| p.71 | IIa | B | In patients with cardiac sarcoidosis who have a LVEF .35% but significant LGE at CMR after resolution of acute inflammation, ICD implantation should be considered.817–819,821,833,834 | Recommendation Table 35 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in cardiac sarcoidosis |
| p.71 | IIa | C | In patients with cardiac sarcoidosis who have a LVEF 35–50% and minor LGE at CMR, after resolution of acute inflammation, PES for risk stratification should be considered. | Recommendation Table 35 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in cardiac sarcoidosis |
| p.71 | IIa | C | In patients with cardiac sarcoidosis, LVEF 35–50% and inducible SMVT at PES, ICD implantation should be considered.823–825 | Recommendation Table 35 — Recommendations for risk stratification, sudden cardiac death prevention, and treatment of ventricular arrhythmias in cardiac sarcoidosis |
| p.72 | I | B | ICD implantation is recommended in patients with cardiac sarcoidosis who (1) have documented sustained VT, or (2) aborted CA.812,828–830,832 | |
| p.72 | IIa | C | In patients with cardiac sarcoidosis and recurrent, symptomatic VA, AAD treatment should be considered. | |
| p.72 | IIb | C | Catheter ablation, in specialized centres, 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.839,841,842 | |
| p.74 | IIa | C | Amiodarone should be considered to reduce arrhythmia burden in patients with Chagas’ cardiomyopathy who present with symptomatic PVCs or VT.857 | Recommendation Table 36 — Recommendations for the treatment of ventricular arrhythmias in Chagas’ cardiomyopathy |
| p.74 | IIa | C | 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 centres should be considered.859,860 | Recommendation Table 36 — Recommendations for the treatment of ventricular arrhythmias in Chagas’ cardiomyopathy |
| p.74 | IIb | C | In patients with Chagas’ cardiomyopathy and symptomatic VT in whom AADs (amiodarone and beta-blockers) are ineffective or not tolerated, ICD implantation may be considered.851,854–856 | Recommendation Table 36 — Recommendations for the treatment of ventricular arrhythmias in Chagas’ cardiomyopathy |
| p.74 | I | C | PES with standby catheter ablation is recommended in patients with aortic valve disease and SMVT to identify and ablate BBR-VT, especially if it occurs following a valve intervention.871,872,881 | Recommendation Table 37 — Recommendations for sudden cardiac death prevention and treatment of ventricular arrhythmias in valvular heart disease |
| p.75 | I | C | In patients with valvular heart disease and persistent LV dysfunction after surgical correction, (if possible) it is recommended that ICD implantation for primary prevention follows DCM/HNDCM recommendations.868 | |
| p.76 | I | C | All CHD patients In adults with CHD with biventricular physiology and a left systemic ventricle presenting with symptomatic heart failure (NYHA II/III) and EF ≤35% despite ≥3 months of OMT, ICD implantation is indicated.885,886 | Recommendation Table 38 — Recommendations for risk stratification and primary prevention of sudden cardiac death in congenital heart disease |
| p.76 | IIa | C | In patients with CHD with presumed arrhythmic syncope and with either at least moderate ventricular dysfunction or inducible SMVT on PES, ICD implantation should be considered.887,889,902 | |
| p.76 | IIb | C | In patients with advanced single ventricle or systemic RV dysfunction with additional risk factors,c ICD implantation may be considered.890,891 | |
| p.76 | IIa | C | Tetralogy of Fallot In patients after repair of TOF with arrhythmia symptoms and NSVT, electrophysiologic evaluation including PES should be considered.889,903–905 | |
| p.76 | IIa | C | In patients after repair of TOF with arrhythmia symptoms and a positive PES, or a combination of other risk factorsd and a positive PES, ICD implantation should be considered. | |
| p.76 | IIb | C | In patients after repair of TOF without arrhythmia symptoms, but with a combination of other risk factors,d electrophysiologic evaluation, including PES, may be considered. | |
| p.76 | IIb | C | In patients with repaired 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.894 | |
| p.76 | I | B | All CHD patients In patients with CHD presenting with sustained VAs, evaluation for residual lesions or new structural abnormalities is recommended.892,893 | Recommendation Table 39 — Recommendations for secondary prevention of sudden cardiac death and treatment of ventricular arrhythmia in congenital heart disease |
| p.76 | I | C | In patients with CHD with not tolerated VT/ aborted CA due to VF, ICD implantation is indicated after exclusion of reversible causes.349,350 Continued | Recommendation Table 39 — Recommendations for secondary prevention of sudden cardiac death and treatment of ventricular arrhythmia in congenital heart disease |
| p.77 | I | B | VF.352,917–919 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | IIa | C | In patients with CHD and recurrent, symptomatic SMVT or ICD shocks for SMVT not manageable by medical therapy or ICD reprogramming, catheter ablation performed in specialized centres should be considered.c 899–901 | |
| p.77 | IIa | C | 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 SVT with rapid ventricular conduction should be considered.890,895 | |
| p.77 | I | C | Tetralogy of Fallot In patients with repaired TOF who present with SMVT or recurrent, symptomatic appropriate ICD therapy for SMVT, catheter ablation performed in specialized centres is recommended.899–901 | |
| p.77 | IIa | C | undergoing surgical or transcutaneous pulmonary valve replacement, pre-operative catheter mapping and transection of VT-related anatomical isthmuses before or during the intervention should be considered.888,893,894 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | IIb | C | In patients with repaired TOF with a preserved biventricular function and symptomatic SMVT, catheter ablation or concomitant surgical ablation performed in specialized centres may be considered as an alternative to ICD therapy.899,901 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | I | B | Diagnostic evaluation 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 aetiology.222,911 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | IIb | B | © ESC 2022 Clinical testing (history, ECG and high precordial lead ECG, exercise test, echocardiogram) of first-degree family members of idiopathic VF patients may be considered.222,278,916 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | IIb | B | In idiopathic VF patients, genetic testing of genes related to channelopathy and cardiomyopathy may be considered.249,278,914,915 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | IIa | C | ICD implantation is recommended in idiopathic Isoproterenol infusion, verapamil, or quinidine for acute treatment of an electrical storm or recurrent ICD discharges should be considered in idiopathic VF.912,913,918,922–924,926 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | IIa | B | Quinidine should be considered for chronic therapy to suppress an electrical storm or recurrent ICD discharges in idiopathic VF.923,924 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.77 | IIa | C | Catheter ablation by experienced electrophysiologists should be considered in idiopathic VF patients with recurrent episodes of VF triggered by a similar PVC non-responsive to medical treatment.927–930 | Recommendation Table 40 — Recommendations for the management of patients with idiopathic ventricular fibrillation |
| p.81 | III | C | Routine diagnostic testing with epinephrine challenge is not recommended in LQTS.137 | |
| p.81 | I | C | prolonged QT interval.948 | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | I | C | Diagnosis It is recommended that LQTS is diagnosed with either QTc ≥480 ms in repeated 12-lead ECGs with or without symptoms or LQTS diagnostic | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | I | C | score .3. In patients with clinically diagnosed LQTS, genetic testing and genetic counselling are recommended. | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | I | C | It is recommended that LQTS is diagnosed in the presence of a pathogenic mutation, irrespective of the QT duration. | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | IIa | C | The LQTS diagnosis should be considered in the presence of a QTc ≥460 ms and ,480 ms in repeated 12-lead ECGs in patients with an arrhythmic syncope in the absence of secondary causes for QT prolongation.952,962,963 | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | I | C | General recommendations to prevent SCD The following is recommended in LQTS: • Avoid QT-prolonging drugs.c • Avoid and correct electrolyte abnormalities. • Avoid genotype-specific triggers for arrhythmias.943 | |
| p.81 | I | B | 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.940,945,946 | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | IIa | B | Mexiletine is indicated in LQT3 patients with a Beta-blockers should be considered in patients with a pathogenic mutation and a normal QTc interval.82 | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | I | B | ICD implantation in addition to beta-blockers is recommended in LQTS patients with CA.952,953,962,963 | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.81 | I | C | ICD implantation is recommended in patients with LQTS who are symptomaticd while receiving beta-blockers and genotype-specific therapies. Continued | Recommendation Table 41 — Recommendations for the management of patients with long QT syndrome |
| p.82 | III | C | recommended in LQTS.961 | |
| p.82 | I | C | 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 | |
| p.82 | IIa | 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 | |
| p.82 | IIa | C | the therapeutic dose. 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 Abo r ted ca r diac a r r est | |
| p.82 | IIb | B | 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 Invasive electrophysiologic study is not Continued | |
| p.83 | I | C | suspected Andersen–Tawil syndrome.964,967 | Recommendation Table 42 — Recommendations for management of patients with Andersen–Tawil syndrome |
| p.83 | IIa | C | Diagnosis Genetic testing is recommended in patients with Andersen–Tawil syndrome 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 | Recommendation Table 42 — Recommendations for management of patients with Andersen–Tawil syndrome |
| p.83 | I | C | • KCNJ2 pathogenic loss of function mutation.964,965,967,968,972 ICD implantation is recommended in patients with Andersen–Tawil syndrome after aborted CA or not-tolerated sustained VT.964,967 | Recommendation Table 42 — Recommendations for management of patients with Andersen–Tawil syndrome |
| p.83 | IIa | C | Beta-blockers and/or flecainide with or without acetazolamide should be considered in patients with Andersen–Tawil syndrome to treat VA.964,970 | Recommendation Table 42 — Recommendations for management of patients with Andersen–Tawil syndrome |
| p.83 | IIa | C | An ILR should be considered in patients with Andersen–Tawil syndrome and unexplained syncope. | Recommendation Table 42 — Recommendations for management of patients with Andersen–Tawil syndrome |
| p.83 | IIb | C | ICD implantation may be considered in patients with Andersen–Tawil syndrome who have a history of unexplained syncope or suffer from tolerated sustained VT.967 | Recommendation Table 42 — Recommendations for management of patients with Andersen–Tawil syndrome |
| p.84 | I | C | for probands with BrS.164,1016 | Recommendation Table 43 — Recommendations for management of patients with Brugada syndrome |
| p.84 | I | C | Diagnosis It is recommended that BrS is diagnosed in patients with no other heart disease and a spontaneous type 1 Brugada ECG pattern.974–976 | Recommendation Table 43 — Recommendations for management of patients with Brugada syndrome |
| p.84 | I | C | It is recommended that BrS is diagnosed in patients with no other heart disease who have survived a CA due to VF or PVT and exhibit a type 1 Brugada ECG induced by sodium channel blocker challenge or during fever.135,136,975,981,982 Genetic testing for SCN5A gene is recommended | Recommendation Table 43 — Recommendations for management of patients with Brugada syndrome |
| p.84 | IIa | C | BrS should be considered in patients with no other heart disease and induced type 1 Brugada pattern who have at least one of: • Arrhythmic syncope or nocturnal agonal respiration • A family history of BrS • A family history of SD (,45 years old) with a negative autopsy and circumstance suspicious for BrS. | Recommendation Table 43 — Recommendations for management of patients with Brugada syndrome |
| p.85 | IIa | C | BrS patients suffering electrical storm.1008 | |
| p.85 | IIb | B | with a spontaneous type I BrS ECG.155 | |
| p.85 | IIb | C | BrS may be considered as a diagnosis in patients with no other heart disease who exhibit an induced type 1 Brugada ECG.136,973,975,978,984,985 | |
| p.85 | III | C | Sodium channel blocker test is not recommended in patients with a prior type I Brugada pattern. General recommendations | |
| p.85 | I | C | The following is recommended in all patients with BrS: (a) Avoidance of drugs that may induce ST-segment elevation in right precordial leads (http://www.brugadadrugs.org). (b) Avoidance of cocaine, cannabis, and excessive alcohol intake. (c) Treatment of fever with antipyretic drugs. | |
| p.85 | I | C | ICD implantation is recommended in patients with BrS who: (a) Are survivors of an aborted CA and/or (b) Have documented spontaneous sustained | |
| p.85 | IIa | C | VT.980,990–992 ICD implantation should be considered in patients with type 1 Brugada pattern and an arrhythmic syncope.990,992,996 | |
| p.85 | IIa | C | Implantation of a loop recorder should be considered in BrS patients with an unexplained syncope.997,999 | |
| p.85 | IIa | C | Quinidine should be considered in patients with BrS who qualify for an ICD but have a contraindication, decline, or have recurrent ICD shocks.922,1006,1007 Isoproterenol infusion should be considered in | |
| p.85 | IIa | C | Catheter ablation of triggering PVCs and/or RVOT epicardial substrate should be considered in BrS patients with recurrent appropriate ICD shocks refractory to drug therapy.1010–1015 PES may be considered in asymptomatic patients | |
| p.85 | IIb | C | ICD implantation may be considered in selected asymptomatic BrS patients with inducible VF during PES using up to 2 extra stimuli.155 | |
| p.85 | III | C | Catheter ablation in asymptomatic BrS patients is not recommended. | |
| p.87 | IIb | C | considered.1023,1025 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | III | C | asymptomatic subjects with ERP.1038,1039 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | I | B | diagnosis of ERS who have survived a CA.1017 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | IIa | B | patients with electrical storm.1017,1030–1032 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | IIa | B | recurrent VF in ERS patients.922,1030,1033 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | IIa | C | least one risk featured or arrhythmic syncope.1020 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | I | C | Diagnosis It is recommended that the ERP is diagnosed as J-point elevation of ≥1 mm in two adjacent inferior and/or lateral ECG leads.1017,1018 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | I | C | It is recommended that the ERS is diagnosed in a patient resuscitated from unexplained VF/PVT in the presence of ERP.1017,1018 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | IIa | C | In an 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.1017,1018 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | IIa | B | First-degree relatives of ERS patients should be considered for clinical evaluation for ERP with additional high-risk features.c,1022,1037 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | IIa | C | Clinical evaluation is not recommended routinely in ICD implantation is recommended in patients with a Isoproterenol infusion should be considered for ERS Quinidine in addition to an ICD should be considered for ILR should be considered in individuals with ERP and at PVC ablation should be considered in ERS patients with recurrent VF episodes triggered by a similar PVC non-responsive to medical treatment.1010 | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.87 | IIb | C | ICD implantation or quinidine may be considered in individuals with ERP and arrhythmic syncope and additional risk features.d,1030,1033 Continued | Recommendation Table 44 — Recommendations for the management of patients with early repolarization pattern/syndrome |
| p.88 | III | C | patients with an isolated ERP.1034,1035,1040 | |
| p.88 | IIb | C | ICD implantation or quinidine may be considered in asymptomatic individuals who demonstrate a high-risk ERPc in the presence of a family history of unexplained juvenile SD.1030,1033 ICD implantation is not recommended in asymptomatic | |
| p.90 | I | C | Diagnosis It is recommended that CPVT is diagnosed in the presence of a structurally normal heart, normal ECG, and exercise- or emotion-induced bidirectional, or PVT. | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | I | C | It is recommended that CPVT is diagnosed in patients who are carriers of a mutation in disease-causing genes. | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | I | C | Genetic testing and genetic counselling are indicated in patients with clinical suspicion or clinical diagnosis of CPVT. | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | IIb | C | Epinephrine or isoproterenol challenge may be considered for the diagnosis of CPVT when an exercise test is not possible. | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | I | C | General recommendations Avoidance of competitive sports, strenuous exercise, and exposure to stressful environments is recommended in all patients with CPVT. | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | I | C | Therapeutic interventions Beta-blockers, ideally non-selective (nadolol or propranolol) are recommended in all patients with a clinical diagnosis of CPVT.1045,1048,1059 | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | I | C | ICD implantation combined with beta-blockers and flecainide is recommended in CPVT patients after aborted CA.1045,1047,1060 | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | IIa | C | phenotype.1047,1050 | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | IIa | C | LCSD should be considered in patients with diagnosis of CPVT when the combination of beta-blockers and flecainide at therapeutic dosage are either not effective, not tolerated, or contraindicated.1056 | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | IIa | C | ICD implantation should be considered in patients with CPVT who experience arrhythmogenic syncope and/or documented bidirectional/PVT while on highest tolerated beta-blocker dose and on flecainide.1047,1050 | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | IIa | C | Flecainide should be considered in patients with CPVT who experience recurrent syncope, polymorphic/bidirectional VT, or persistent exertional PVCs, while on beta-blockers at the | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.90 | III | C | highest tolerated dose.1052,1053,1060 PES is not recommended for stratification of SCD risk. Continued | Recommendation Table 45 — Recommendations for the management of patients with catecholaminergic polymorphic ventricular tachycardia |
| p.91 | I | C | with SQTS.1063 | Recommendation Table 46 — Recommendations for the management of patients with short QT syndrome |
| p.91 | IIa | C | QTc ≤320 ms.1064–1067,1073,1074 | |
| p.91 | IIa | C | ILR should be considered in young SQTS patients. | |
| p.91 | IIb | C | Isoproterenol may be considered in SQTS patients with an electrical storm.1075 | Recommendation Table 46 — Recommendations for the management of patients with short QT syndrome |
| p.91 | I | C | 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 mutation, (b) a family history of SQTS, (c) survival from a VT/VF episode in the absence of heart disease.1061,1068 Genetic testing is indicated in patients diagnosed | Recommendation Table 46 — Recommendations for the management of patients with short QT syndrome |
| p.91 | IIa | C | SQTS should be considered in the presence of a SQTS should be considered in the presence of a QTc ≥320 ms and ≤360 ms and arrhythmic syncope. | |
| p.91 | IIb | C | SQTS may be considered in the presence of a QTc ≥320 ms and ≤360 ms and a family history of SD at age ,40 years. | |
| p.91 | I | C | ICD implantation is recommended in patients with a diagnosis of SQTS who: (a) are survivors of an aborted CA and/or (b) have documented spontaneous sustained VT.1063 | |
| p.91 | IIa | C | ICD implantation should be considered in SQTS patients with arrhythmic syncope. | |
| p.91 | IIb | C | contraindication to the ICD or refuse it, and (b) asymptomatic SQTS patients and a family history of SCD.1069–1071 | Recommendation Table 46 — Recommendations for the management of patients with short QT syndrome |
| p.91 | III | C | PES is not recommended for SCD risk stratification in SQTS patients. Continued | Recommendation Table 46 — Recommendations for the management of patients with short QT syndrome |
| p.92 | I | C | During pregnancy, electrical cardioversion is recommended for sustained VT.1084 | Recommendation Table 47 — Recommendations for the prevention of sudden cardiac death and management of ventricular arrhythmia during pregnancy |
| p.92 | IIa | C | Acute management of VA For acute conversion of haemodynamically tolerated SMVT during pregnancy, a beta-blocker, sotalol, flecainide, procainamide, or overdrive ventricular pacing should be considered. | Recommendation Table 47 — Recommendations for the prevention of sudden cardiac death and management of ventricular arrhythmia during pregnancy |
| p.92 | I | C | Long-term management of VA If ICD implantation is indicated during pregnancy, implantation is recommended with optimal radiation protection.1087,1107 | Recommendation Table 47 — Recommendations for the prevention of sudden cardiac death and management of ventricular arrhythmia during pregnancy |
| p.92 | I | C | Continuation of beta-blockers is recommended during pregnancy and post-partum in women with LQTS or CPVT.955,1094–1096 | Recommendation Table 47 — Recommendations for the prevention of sudden cardiac death and management of ventricular arrhythmia during pregnancy |
| p.92 | IIa | C | Continuation of beta-blockers should be considered during pregnancy in women with ARVC.1097–1099 | Recommendation Table 47 — Recommendations for the prevention of sudden cardiac death and management of ventricular arrhythmia during pregnancy |
| p.92 | IIa | C | Oral metoprolol, propranolol, or verapamil should be considered for long-term management of idiopathic sustained VT during pregnancy. | Recommendation Table 47 — Recommendations for the prevention of sudden cardiac death and management of ventricular arrhythmia during pregnancy |
| p.92 | IIa | C | 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.1105 | Recommendation Table 47 — Recommendations for the prevention of sudden cardiac death and management of ventricular arrhythmia during pregnancy |
| p.93 | IIb | C | may be considered.1109,1113,1119 | Recommendation Table 48 — Recommendations for the prevention of sudden cardiac death before and after heart transplantation |
| p.93 | I | C | It is recommended that staff at sporting facilities are trained in CPR and in the use of AED.93,1137 | Recommendation Table 49 — Recommendations for risk stratification and prevention of sudden cardiac death in athletes |
| p.93 | IIa | C | Prior to heart transplant In patients awaiting heart transplantation, ICD implantation for primary prevention should be considered.1108,1111,1112 In patients awaiting heart transplantation, WCD | Recommendation Table 48 — Recommendations for the prevention of sudden cardiac death before and after heart transplantation |
| p.93 | IIb | C | Post heart transplant In selected transplanted patients with cardiac allograft vasculopathy or treated rejection, ICD implantation may be considered.1114,1116 | Recommendation Table 48 — Recommendations for the prevention of sudden cardiac death before and after heart transplantation |
| p.93 | I | C | © ESC 2022 In athletes with positive medical history, abnormal physical examination, or ECG alterations, further investigations including echocardiography and/or CMR to confirm (or exclude) an underlying disease are recommended.1123,1133,1135 | Recommendation Table 49 — Recommendations for risk stratification and prevention of sudden cardiac death in athletes |
| p.93 | I | C | It is recommended that athletes diagnosed with a cardiovascular disease associated with SCD are managed according to current guidelines for sports eligibility. | Recommendation Table 49 — Recommendations for risk stratification and prevention of sudden cardiac death in athletes |
| p.93 | IIa | C | Pre-participation cardiovascular evaluation of competitive athletes should be considered.46,1122,1123,1127 | Recommendation Table 49 — Recommendations for risk stratification and prevention of sudden cardiac death in athletes |
| p.93 | IIa | C | It should be considered that cardiovascular evaluation of young (,35 years) competitive athletes includes history, physical examination, and 12-lead ECG.1123,1126,1130,1140 | Recommendation Table 49 — Recommendations for risk stratification and prevention of sudden cardiac death in athletes |
| p.93 | IIa | C | The cardiovascular risk of middle-aged and elderly individuals should be evaluated before engaging in strenuous sports through established scores such as the SCORE2 risk chart.46,1141,1142 | Recommendation Table 49 — Recommendations for risk stratification and prevention of sudden cardiac death in athletes |
| p.94 | IIb | B | In elderly patients in whom a benefit from the defibrillator is not expected due to the patient’s age and comorbidities, omission of ICD implantation for primary prevention may be considered.647,1150,1152 ICD, implantable cardioverter defibrillator. aClass of recommendation. bLevel of evidence. 9 Key messages 9.1 General aspects | Recommendation Table 50 — Recommendations for implantable cardioverter defibrillator implantation in the elderly |
| p.96 | I | B | Prompt CPR by bystanders is recommended at OHCA. | |
| p.96 | I | B | It is recommended to promote community training in basic life support to increase bystander CPR rate and AED use. | |
| p.96 | III | C | It is not recommended to undertake genetic testing in index patients with insufficient evidence of a genetic disease. | |
| p.96 | I | B | The investigation of a SCA survivor without obvious extra-cardiac cause is recommended to be overseen by a multidisciplinary team. | |
| p.96 | I | C | In electrically unstable patients after SCA, with suspicion of ongoing myocardial ischaemia, a coronary angiogram is indicated. | |
| p.96 | I | B | In SCA survivors, collection of blood samples at presentation is recommended for potential toxicology and genetic testing. | |
| p.96 | I | B | Retrieval of recordings from CIEDs and wearable monitors is recommended for all SCA survivors. | |
| p.96 | I | C | Echocardiography is recommended for evaluation of cardiac structure and function in all SCA survivors. | |
| p.96 | I | B | Sodium channel blocker test and exercise testing is recommended in SCA survivors without a clear underlying cause. | |
| p.97 | I | B | Investigation of unexpected SD, especially in case of suspicion of inherited disease, should be made a public health priority. | |
| p.97 | I | B | A comprehensive autopsy is recommended, ideally, in all cases of unexpected SD, and always in those under 50 years of age. | |
| p.97 | I | B | Toxicology screens are recommended in SD cases with uncertain cause of death. | |
| p.97 | I | B | For SCD where the cause is known or suspected to be heritable, genetic testing targeted to the cause is recommended. | |
| p.97 | III | B | Following SADS, hypothesis-free post-mortem genetic testing using exome or genome sequencing is not recommended. | |
| p.97 | I | B | Withdrawal of offending agents is recommended whenever drug-induced VA are suspected. | |
| p.97 | I | C | Investigation for reversible causes (e.g. electrolyte imbalances, ischaemia, hypoxaemia, fever)d is recommended in patients with VA. | |
| p.97 | I | B | DC cardioversion is recommended as the first-line treatment for patients with haemodynamically not-tolerated SMVT. | |
| p.97 | III | B | Intravenous verapamil is not recommended in broad QRS complex tachycardia of unknown mechanism. | |
| p.98 | I | C | Intravenous magnesium with supplementation of potassium is recommended in patients with TdP. | |
| p.98 | I | B | Catheter ablation is recommended in patients presenting with incessant VT or electrical storm due to SMVT refractory to AADs. | |
| p.98 | I | C | Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good quality survival .1 year. | |
| p.98 | III | C | It is not recommended to implant an ICD in patients with incessant VAs until the VA is controlled. | |
| p.98 | I | C | When an ICD is indicated, it is recommended to evaluate whether the patient could benefit from CRT-defibrillator. | |
| p.98 | I | A | Optimization of ICD programming is indicated to avoid inappropriate and unnecessary therapies and to reduce mortality. | |
| p.98 | I | A | In single- or dual-chamber ICD patients without bradycardia pacing indications, it is recommended to minimize ventricular pacing. | |
| p.98 | I | A | Programming of prolonged detection settings is indicated (duration criteria of at least 6–12 s or 30 intervals). | |
| p.98 | I | A | It is recommended to program the slowest tachycardia therapy zone limit ≥188 b.p.m. in primary prevention ICD patients. | |
| p.98 | I | A | In patients with SHD, programming of at least one ATP therapy is recommended in all tachyarrhythmias zones. | |
| p.98 | I | B | It is recommended to program algorithms for SVT vs. VT discrimination for tachycardias with rates up to 230 b.p.m. | |
| p.98 | I | B | It is recommended to activate lead failure alerts. | |
| p.98 | I | B | Remote monitoring is recommended to reduce the incidence of inappropriate shocks. | |
| p.98 | I | B | Programming of burst ATP as a first attempt is recommended over ramp ATP. | |
| p.98 | I | C | Catheter ablation is recommended for ICD patients with recurrent SVT resulting in inappropriate ICD therapies. | |
| p.98 | I | C | Assessment of psychological status and treatment of distress is recommended in ICD patients. | |
| p.99 | I | B | Intravenous beta-blocker treatment is indicated for patients with recurrent PVT/VF during STEMI unless contraindicated. | |
| p.99 | III | B | Prophylactic treatment with AADs (other than beta-blockers) is not recommended in ACS. | |
| p.99 | I | B | Early (before discharge) assessment of LVEF is recommended in all patients with acute MI. | |
| p.99 | I | C | In patients with syncope and previous STEMI, PES is indicated when syncope remains unexplained after non-invasive evaluation. | |
| p.99 | III | A | In patients with CAD, prophylactic treatment with AADs other than beta-blockers is not recommended. | |
| p.99 | I | C | Regular assessment of ventricular function of patients with PVC burden .10% and normal ventricular function is indicated. | |
| p.99 | III | C | Amiodarone as a first-line treatment is not recommended in patients with idiopathic VTs/PVCs. | |
| p.100 | I | B | In patients with suspected ARVC, CMR is recommended. | |
| p.100 | I | B | In patients with a suspected or definite diagnosis of ARVC, genetic counselling and testing are recommended. | |
| p.100 | I | B | Avoidance of high-intensity exercise is recommended in patients with a definite diagnosis of ARVC. | |
| p.100 | I | C | ICD implantation is recommended in ARVC patients with haemodynamically not-tolerated VT or VF. | |
| p.100 | I | C | In patients with ARVC and non-sustained or sustained VAs, beta-blocker therapy is recommended. | |
| p.100 | I | C | In a first-degree relative of a patient with ARVC, ECG, and echocardiogram are recommended. | |
| p.100 | I | B | CMR with LGE is recommended in HCM patients for diagnostic work-up. | |
| p.100 | I | B | Genetic counselling and testing are recommended in HCM patients. | |
| p.100 | I | B | ICD implantation is recommended in HCM patients with haemodynamically not-tolerated VT or VF. | |
| p.100 | I | C | In a first-degree relative of a patient with HCM, ECG, and echocardiogram are recommended. | |
| p.100 | I | C | ICD implantation is recommended in patients with myotonic dystrophy and SMVT or aborted CA not caused by BBR-VT. | |
| p.100 | I | C | In symptomatic patients with BBR-VT, catheter ablation is recommended. | |
| p.100 | I | C | In patients with myotonic dystrophy undergoing ablation for BBR-VT, pacemaker/ICD implantation is recommended. | |
| p.101 | I | B | ICD implantation is recommended in patients with cardiac sarcoidosis who have an LVEF ≤35%. | |
| p.101 | I | B | In patients with CHD presenting with sustained VAs, evaluation for residual or new anatomical abnormalities is recommended. | |
| p.101 | I | C | In patients with CHD with not-tolerated VT/aborted CA due to VF, ICD implantation is indicated after exclusion of reversible causes. | |
| p.101 | I | B | ICD implantation is recommended in idiopathic VF. | |
| p.101 | I | C | In patients with clinically diagnosed long QT syndrome, genetic testing, and genetic counselling are recommended. | |
| p.101 | I | C | It is recommended that LQTS is diagnosed in the presence of a pathogenic mutation, irrespective of the QT duration. | |
| p.101 | III | C | Routine diagnostic testing with epinephrine challenge is not recommended in LQTS. | |
| p.101 | I | C | Mexiletine is indicated in LQT3 patients with a prolonged QT interval. | |
| p.101 | I | B | ICD implantation in addition to beta-blockers is recommended in LQTS patients with CA. | |
| p.102 | III | C | Invasive electrophysiologic study is not recommended in LQTS. | |
| p.102 | I | C | Genetic testing is recommended in patients with suspected Andersen–Tawil syndrome. | |
| p.102 | I | C | ICD implantation is recommended in patients with Andersen–Tawil syndrome after aborted CA not tolerated sustained VT. | |
| p.102 | I | C | It is recommended that BrS is diagnosed in patients with no other heart disease and a spontaneous type 1 Brugada ECG pattern. | |
| p.102 | I | C | Genetic testing for SCN5A gene is recommended for probands with BrS. | |
| p.102 | III | C | Sodium channel blocker test is not recommended in patients with a prior type I Brugada pattern. | |
| p.102 | III | C | Catheter ablation in asymptomatic BrS patients is not recommended. | |
| p.102 | I | C | It is recommended that the ERP is diagnosed as J-point elevation of ≥1 mm in two adjacent inferior and/or lateral ECG leads. | |
| p.102 | I | C | It is recommended that the ERS is diagnosed in a patient resuscitated from unexplained VF/PVT in the presence of ERP. | |
| p.102 | III | C | Clinical evaluation is not recommended routinely in asymptomatic subjects with ERP. | |
| p.102 | I | B | ICD implantation is recommended in patients with a diagnosis of ERS who have survived a CA. | |
| p.102 | III | C | ICD implantation is not recommended in asymptomatic patients with an isolated ERP. | |
| p.102 | I | C | It is recommended that CPVT is diagnosed in patients who are carriers of a mutation in disease-causing genes. | |
| p.102 | I | C | Genetic testing and genetic counselling are indicated in patients with clinical suspicion or clinical diagnosis of CPVT. | |
| p.102 | I | C | Beta-blockers, ideally non-selective (nadolol or propranolol), are recommended in all patients with a clinical diagnosis of CPVT. | |
| p.102 | I | C | ICD implantation combined with beta-blockers and flecainide is recommended in CPVT patients after aborted CA. | |
| p.102 | III | C | PES is not recommended for stratification of SCD risk. | |
| p.103 | I | C | Genetic testing is indicated in patients diagnosed with SQTS. | |
| p.103 | III | C | PES is not recommended for SCD risk stratification in SQTS patients. | |
| p.103 | I | C | During pregnancy, electrical cardioversion is recommended for sustained VT. | |
| p.103 | I | C | If ICD implantation is indicated during pregnancy, implantation is recommended with optimal radiation protection. | |
| p.103 | I | C | Continuation of beta-blockers is recommended during pregnancy and post-partum in patients with LQTS or CPVT. | |
| p.103 | I | C | It is recommended that staff at sporting facilities are trained in CPR and in the use of AED. |