Guideline Essentials: 2022 ESC Recommendations on Ventricular Arrhythmias and Sudden Cardiac Death

Introduction

In 2022, the European Society of Cardiology (ESC) published a guideline on the management of patients with ventricular arrhythmias (VAs) and the prevention of sudden cardiac death (SCD). This specialist Guidelines overview for secondary care cardiologists provides a summary of the key recommendations from the updated guideline, which incorporates recent insights into the epidemiology of SCD, new evidence on genetics, clinical findings for risk stratification, and advances in diagnostic evaluation and therapeutic strategies.

Please refer to the full guideline for all of the recommendations, rationale, and background information. This summary does not include recommendations relating to implantable cardioverter defibrillators (ICDs). Please see a separate Guidelines Essentials article on ICDs for VAs and the prevention of SCD for further ESC recommendations.

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Key Takeaways from the Guideline

• Genetic testing should be a routine part of care for patients with genetic cardiomyopathies and arrhythmia syndromes
• A comprehensive autopsy is recommended in all cases of sudden death in those aged <50 years, and is desirable in all sudden death victims
• A substantial proportion of people who die from sudden arrhythmic death syndrome and their families are diagnosed with genetic heart disease following clinical and genetic evaluation
• Catheter ablation is recommended for patients who have coronary artery disease with recurrent, symptomatic sustained monomorphic ventricular tachycardia despite chronic amiodarone therapy
• Catheter ablation is the first-line treatment for premature ventricular complex-induced cardiomyopathy
• Nadolol or propranolol are the preferred beta-blockers in long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) patients
• Routine catheter ablation is not recommended in patients with asymptomatic Brugada syndrome
• Exclude underlying structural, channelopathic, or metabolic aetiology when making a diagnosis of idiopathic ventricular fibrillation
• Left cardiac denervation may have an adjunctive role in the management of CPVT and LQTS patients.

Evaluation

Evaluating Patients with Newly Documented Ventricular Arrhythmias

Newly documented VA is defined as non-sustained ventricular tachycardia (NSVT), sustained monomorphic ventricular tachycardia (SMVT), or frequent premature ventricular contractions (PVCs).

• Evaluate patients with newly documented VA with:
  • baseline 12-lead electrocardiogram (ECG) 
  • 12-lead ECG recording of the VA, if possible, and 
  • echocardiogram 
• Following initial evaluation, consider cardiac magnetic resonance (CMR) for patients with newly documented VA and suspicion of structural heart disease (SHD) other than coronary artery disease (CAD) 
• Consider 24-hour (or more) Holter ECG for patients with an incidental finding of NSVT.

Evaluating Patients with a First Episode of Sustained Monomorphic Ventricular Tachycardia

• Consider the following for aetiological evaluation of patients presenting with their first SMVT episode:
  • electrophysiological study
  • electroanatomical mapping, and
  • mapping-guided biopsies.

Evaluating Patients Following Sudden Cardiac Arrest

• Coronary angiogram is indicated for evaluation of electrically unstable post-sudden cardiac arrest (SCA) patients with suspicion of ongoing myocardial ischaemia 
• For all SCA survivors:
  • collect blood samples at presentation for potential toxicology and genetic testing 
  • retrieve recordings from cardiac insertable electronic devices and wearable monitoring devices
  • evaluate cardiac structure and function with echocardiography 
  • use continuous cardiac monitoring 
  • conduct repeated 12-lead ECGs during stable rhythm (including high precordial lead ECG) 
  • consider ergonovine, acetylcholine, or hyperventilation testing for diagnosis of coronary vasospasm
• For SCA survivors without a clear underlying cause, the following are recommended:
  • sodium channel blocker test 
  • exercise test
  • coronary imaging and CMR with late gadolinium enhancement to evaluate cardiac structure and function. Consider brain/chest computed tomography (CT) scan when ECG, echocardiography, and patient characteristics are inconsistent with a cardiac cause
• The multidisciplinary team should oversee investigations in patients following SCA without obvious extra-cardiac causes.

Treatment

Treatment of Reversible Conditions

• Withdraw ‘offending’ agents if drug-induced VA is suspected
• Investigate patients with VA for reversible causes such as fever, electrolyte imbalance, ischaemia, or hypoxaemia
• Consider ICD implantation based on subsequent VA/SCD risk, despite the possibility of a correctable cause for VA.

Acute Management of Sustained Ventricular Tachycardia

Table 1: Acute Management of Sustained Ventricular Tachycardia

• Intravenous verapamil is not recommended in broad QRS complex tachycardia of unknown mechanism.

Management of Electrical Storm

• Use mild-to-moderate sedation to reduce sympathetic tone and psychological distress 
• Consider deep sedation and intubation for patients with intractable electrical storm refractory to drug treatment.

See Table 2 for recommended treatments for electrical storm.

Table 2: Recommended Treatments for Electrical Storm

Treatment with Heart Failure Medication

• For all heart failure patients with reduced ejection fraction, optimal medical treatment is indicated, including: 
  • angiotensin-converting enzyme inhibitor/angiotensin receptor blocker/angiotensin receptor neprilysin inhibitor
  • mineralocorticoid receptor antagonist
  • beta-blocker, and
  • sodium–glucose co-transporter 2 inhibitor.

Treatment of Ventricular Arrhythmia in Acute Coronary Syndrome and Vasospasm

Table 3: Treatment of Ventricular Arrhythmia in Acute Coronary Syndrome and Vasospasm

• Prophylactic anti-arrhythmic drugs (AADs) (other than beta-blockers) are not recommended for patients with acute coronary syndrome
• Consider ICD implantation for SCA survivors with coronary artery spasm. For recommendations on ICDs in VA and SCD, please see the separate Guidelines summary.

Risk Stratification and Treatment of Ventricular Arrhythmias Early After Myocardial Infarction

• Assess left ventricular ejection fraction (LVEF) before discharging patients with acute myocardial infarction (MI)
• Re-assess LVEF 6–12 weeks after MI if pre-discharge LVEF is ≤40%, to evaluate the need for ICD implantation
• Consider catheter ablation in patients with recurrent episodes of polymorphic ventricular tachycardia (PVT) or ventricular fibrillation (VF) triggered by a similar premature ventricular complex (PVC) non-responsive to medical treatment or coronary revascularisation in the subacute phase of MI.

Management of Patients with Idiopathic Premature Ventricular Complexes or Ventricular Tachycardia

• Regularly assess ventricular function in patients with a PVC burden >10% and normal ventricular function 
• Consider CMR in patients with PVCs or VT and a presentation non-typical for idiopathic origin (for example, old age, right bundle branch block morphology, or SMVT consistent with re-entry) despite a normal echocardiogram.

See Table 4 for treatment recommendations.

Table 4: Treatment of Patients with Idiopathic Premature Ventricular Complexes or Ventricular Tachycardia

• Amiodarone is not recommended first line for idiopathic VT or PVCs 
• Catheter ablation of idiopathic VT/PVC is not recommended for children <5 years of age or <10 kg in weight, unless previous medical therapy has failed or VT is not haemodynamically tolerated
• Verapamil is not recommended for children aged <1 year with PVCs or VT, especially if they have signs of heart failure or are taking other AADs.

See the full guideline for recommendations on the management of patients with:

• PVC-induced/aggravated cardiomyopathy 
• VAs in Chagas cardiomyopathy 
• idiopathic VF
• long QT syndrome
• short QT syndrome
• Andersen–Tawil syndrome
• Brugada syndrome
• early repolarisation pattern/syndrome 
• catecholaminergic polymorphic VT.

Sudden Cardiac Death Prevention

Sudden Cardiac Death Prevention in Patients with Coronary Anomalies

Diagnostic Evaluation

• For patients with anomalous aortic origin of a coronary artery with an interarterial course, use cardiac stress imaging during physical exercise and cardiopulmonary exercise testing to confirm or exclude myocardial ischaemia
• For patients with anomalous aortic origin of a coronary artery with a history of aborted cardiac arrest (CA), conduct cardiac stress imaging during physical exercise and cardiopulmonary exercise test after surgery.

Treatment

• For patients with anomalous aortic origin of a coronary artery with CA, syncope suspected to be due to ventricular arrhythmias, or angina when other causes have been excluded, surgery is recommended
• Also consider surgery for 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.

See the full guideline for recommendations on risk stratification and the prevention of sudden cardiac death in congenital heart disease. 

Prevention of Sudden Cardiac Death and Management of Ventricular Arrhythmia During Pregnancy

Acute Management

• For sustained VT during pregnancy, electrical cardioversion is recommended
• Consider a beta-blocker, sotalol, flecainide, procainamide, or overdrive ventricular pacing for acute conversion of haemodynamically tolerated SMVT during pregnancy.

Long-term Management

• If ICD implantation is required, ensure optimal radiation protection
• Continue beta-blockers throughout pregnancy and after labour for patients with LQTS or catecholaminergic polymorphic VT
• Consider continuing beta-blockers during pregnancy for patients with arrhythmogenic right ventricular cardiomyopathy
• Consider oral metoprolol, propranolol, or verapamil for long-term management of idiopathic sustained VT in pregnancy
• For patients with highly symptomatic recurrent SMVT refractory or those who are intolerant to AADs, consider catheter ablation using non-fluoroscopic mapping systems (preferably after the first trimester).

Risk Stratification and Prevention of Sudden Cardiac Death in Athletes

• For athletes with positive medical history or abnormal ECG or physical examination, further investigation using echocardiography and/or CMR is needed to confirm or exclude underlying disease 
• Follow up-to-date sports eligibility guidelines for the management of cardiovascular diseases associated with SCD 
• Consider pre-participation cardiovascular evaluations for competitive athletes 
• Cardiovascular evaluation of young competitive athletes (<35 years) should include physical examination, history, and 12-lead ECG
• Use established tools such as SCORE2 for cardiovascular risk assessment before older athletes participate in strenuous sports.

Prevention of Sudden Cardiac Death Before and After Heart Transplantation

• Consider ICD implantation for primary prevention in patients awaiting a heart transplant; a wearable cardioverter defibrillator may also be considered
• After the heart transplant, ICD implantation may be considered for selected patients with cardiac allograft vasculopathy or treated rejection.

See the full guideline for recommendations on risk stratification, SCD prevention, and treatment of VA in:

• chronic coronary artery disease
• valvular heart disease 
• dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy 
• arrhythmogenic right ventricular cardiomyopathy 
• hypertropic cardiomyopathy
• cardiac sarcoidosis
• myocarditis
• neuromuscular diseases.