A Look at the Updated Guidelines for the Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death

EP Lab Digest. 2022;22(12):14-15.

The 2022 European Society of Cardiology (ESC) Guidelines for the Management of Patients with Ventricular Arrhythmias (VAs) and the Prevention of Sudden Cardiac Death (SCD),¹ which was presented at ESC Congress 2022, is an update on their 2015 guidelines.² Experts from across Europe summarized available information from over 1000 references and produced a 130-page document. The revision was deemed necessary because of new epidemiological data, new developments in genetics, updated imaging and clinical findings for risk, and new diagnostic information and therapies.

The work is a valuable summary on the state of the science for VAs and SCD management. The usual Class I, II, and III guideline style is used, with Class I as recommended or indicated, Class II as should or may be considered, and Class III as not recommended.     

Areas of review include epidemiology of SCD, general evaluation and treatment, and specifics related to diagnostic evaluation, management, and risk stratification. There are also special sections on SCD risk in select populations, such as pregnant, heart transplant, athlete, Wolff-Parkinson-White, or elderly patients. There are new sections on provocative diagnostic testing, genetic testing, diagnostic evaluation in first VA presentation, management of electrical storm, and special aspects of device therapy.

The volume of information is vast, but the authors have identified what they deemed of “key” importance. The bulk of this review will focus on these key aspects. These include both observations and management recommendations. There are 3 parts: general aspects, structural heart disease aspects, and primary electrical disease aspects. This review will look at these for specific disease states and treatment/assessment directions.

Public Access Defibrillation and Basic Life Support

The new guidelines identified a need to increase availability of public access defibrillators and basic life support training in communities; more automated external defibrillators are needed where cardiac arrest is more likely to occur. This recommendation is prompted by the fact that survival rates are still low for out-of-hospital cardiac arrest. They also proposed that a mobile phone-based alert system could be beneficial.

General Approaches for Ventricular Tachycardia and SCD

The use of risk calculators for SCD and VA to enhance external validation, development, and reporting of prediction models was recommended. The ability to predict has been in continual evolution and a variety of risk calculators for SCD are available.

Advanced catheter ablation techniques, mechanical circulatory support, and autonomic modulation is required for those with electrical storm refractory to drug treatment. Catheter ablation success rates have been good in those with failed drug response; but in addition, mechanical circulatory support and possible autonomic modulation may be considered.

For patients with coronary artery disease and recurrent, symptomatic sustained monomorphic ventricular tachycardia (SMVT) despite chronic amiodarone therapy, catheter ablation is recommended. Also, the first-line treatment for premature ventricular contraction (PVC)-induced cardiomyopathy is catheter ablation. This is a class I indication.

The decision for implantable cardioverter-defibrillator (ICD) implantation should take into consideration other risk factors for nonarrhythmic death, patient wishes, and quality of life. Patients with end-stage renal failure, diabetes, and elderly patients benefited less or not at all from an ICD in place for primary prevention. There was great emphasis placed on the joint decision process (patient plus provider) in determining the need for and acceptance of implantation.

ICD implantation as primary prevention for patients with hypokinetic nondilated cardiomyopathy/dilated cardiomyopathy should not be restricted to a left ventricular ejection fraction (LVEF) ≤35%. Additional risk factors should be considered. Instead, patients with a LVEF <50% should be considered for implant if this is accompanied by syncope, late gadolinium enhancement or cardiac magnetic resonance (CMR) results, inducible SMVT with programmed stimulation, or with pathogenic mutations in genes such as lamin A/C (LMNA), PLN, FLNC, and RBM20.

A recommendation was made for a multimodal approach with a systematic workup for cardiac arrest survivors. This may include coronary angiogram, brain/chest computed tomography (CT) scans, blood for toxicology and genetic testing, cardiovascular implantable electronic device or wearable monitor information, echocardiogram, CMR, evaluation for coronary spasm, and sodium channel blocker and exercise testing.

It is also recommended that a comprehensive autopsy be performed for sudden death cases younger than 50 years. For this population, potential genetic cardiac disease has been identified in as many as 25%-49% of cases.

Genetic Cardiomyopathies and Arrhythmia Syndromes

Genetic testing for patients with genetic cardiomyopathies and arrhythmia syndromes should be part of routine care. This should involve an expert multidisciplinary team for testing and counseling. Only those with skills to counsel on implications and direct appropriately should be referred to. A part of this involves exclusion of an underlying structural, channelopathic, metabolic, or toxicological etiology in the diagnosis of idiopathic ventricular fibrillation (VF). Testing should include blood chemistry, electrocardiogram (ECG), cardiac CT/coronary angiography, telemetry/Holter, exercise stress test, echocardiogram, sodium channel blocker testing, and CMR.

In a substantial proportion of families, sudden arrhythmic death syndrome decedents have a diagnosis of underlying genetic heart disease. Therefore, the guidelines recommend that first-degree relatives are referred for cardiac assessment in a specialized clinic.

For patients with arrhythmogenic right ventricular cardiomyopathy, a high rate of appropriate ICD interventions that are not all classified as lifesaving are seen. For those with hemodynamically poorly tolerated VT, it is lifesaving, but for those with tolerated VT, some questions remain about benefit. It is suggested that this requires further study.

For patients with asymptomatic long QT syndrome (LQTS), the 1-2-3 LQTS Risk calculator may be used to calculate arrhythmic risk. The 1-2-3 LQTS Risk calculator uses QT interval duration and genotype to create a score.

The preferred beta-blockers for patients with LQTS and catecholaminergic polymorphic ventricular tachycardia (CPVT) are nadolol or propranolol. Nonselective beta-blockers for patients with LQTS with documented QT prolongation are more efficacious. For CPVT patients who are intolerant of beta-blockers, flecainide alone is an option. In addition, for CPVT and LQTS patients, left cardiac denervation plays an important role in management, especially for symptomatic patients on beta-blockers in which ICD implantation is contraindicated or declined, or for those receiving multiple shocks or experiencing syncope while on beta-blockers. However, left cardiac denervation should not be considered an ICD alternative in high-risk patients, but instead as a complementary therapy.

There were a few key points related to Brugada syndrome (BrS). Evidence showed that BrS is not diagnosed when a type 1 Brugada ECG pattern is provoked by a sodium channel blocker test if additional findings are absent. Findings such as arrhythmic syncope or nocturnal agonal respirations, a family history of BrS, family history of sudden death (<45 years) with a negative autopsy, circumstance suspicious for BrS, or documented PVT/VF can assist in diagnostic confirmation.

It was also not clear whether SCD risk stratification is valid in asymptomatic BrS patients with spontaneous type 1 pattern. Data was mixed and controversial. Routine catheter ablation is not recommended in asymptomatic BrS patients; there is limited long-term follow-up data on ablation for the asymptomatic BrS patient.

It was pointed out that early repolarization syndrome (ERS) is distinct from early repolarization pattern (ERP). ERS is diagnosed if a patient is resuscitated from PVT or VF without heart disease. ERP includes J-point elevation ≥1 mm in ≥2 adjacent inferior and/or lateral leads. ERP can be a benign finding in 5.8% of adults. Risk stratification data is unavailable for ERS, but if there is a family history or SCD, then an ICD may be considered.

Finally, in hypertrophic cardiomyopathy (HCM) patients younger than 16 years, a validated risk calculator is useful to assess SCD risk. The HCM Risk-Kids score (https://hcmriskkids.org) for children 1-16 years has been developed. This scoring factors in the presence of unexplained syncope, maximal LV wall thickness, large left atrial diameter, low LV outflow tract gradient, and nonsustained VT.

Muscular and Congenital Etiologies

A couple of key points were identified with SCD caused by muscular etiologies. Specific risk stratification for SCD in patients with an LMNA mutation is required. Early atrial and VAs, premature conduction disease, high rate of SCD, and end-stage heart failure development are found with LMNA mutations. A LMNA risk calculator has been developed to assist in prediction (https://lmna-risk-vta.fr/). In addition, an invasive electrophysiological study is needed to evaluate myotonic dystrophy patients who have palpitations with suspected arrhythmias, syncope, or aborted sudden death. The guidelines include a specific algorithm for risk stratification, SCD prevention, and treatment of VAs for this population.

Catheter ablation was considered the preferred treatment in those with repaired tetralogy of Fallot and monomorphic VT. This is a class I indication. Such procedures should be carried out in centers with expertise in catheter ablation of congenital heart disease patients.

Final Thoughts

The guidelines conclude with a discussion of the gaps in knowledge that exist, offering a blueprint for additional studies to be undertaken. Each guideline update encourages us to tweak the quality of care we deliver to patients. The ESC 2022 guidelines give us much to absorb. 

Disclosures: Ms Moulton has completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. She has no conflicts of interest to report regarding the content herein.

References

1. Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022;43(40):3997-4126. doi:10.1093/eurheartj/ehac262

2. Priori SG, Blomström-Lundqvist C, Mazzanti A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015;36(41):2793-2867. doi:10.1093/eurheartj/ehv316