Summary of laboratory EP.2022;22(12):14-15.
The 2022 European Society of Cardiology (ESC) Guidelines for the treatment of patients with ventricular arrhythmias (VA) and prevention of sudden cardiac death (SCD),1which was presented at the ESC Congress 2022, is an update of its 2015 guidelines.2Experts from across Europe summarized the available information from more than 1,000 references and produced a 130-page document. The review was deemed necessary due to new epidemiological data, new developments in genetics, updated imaging and clinical risk findings, and new diagnostic and therapeutic information.
The work is a valuable summary of the state of the science for the management of AV and SCD. The usual guidance style of Classes I, II, and III 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 the epidemiology of SCD, general evaluation and treatment, and specific issues related to diagnostic evaluation, management, and risk stratification. There are also special sections on the risk of SCD in selected populations, such as pregnant women, heart transplant recipients, athletes, Wolff-Parkinson-White, or elderly patients. There are new sections on diagnostic challenge testing, genetic testing, diagnostic evaluation at first VA presentation, management of thunderstorms, and special aspects of device therapy.
The volume of information is enormous, but the authors have identified what they consider to be of “key” importance. Most of this review will focus on these key aspects. These include observations and recommendations from management. There are 3 parts: general aspects, structural aspects of heart disease and primary aspects of electrical disease. This review will examine these specific disease states and the treatment/assessment instructions.
Public access defibrillation and basic life support
The new guidelines identified the need to increase the availability of publicly accessible 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 motivated by the fact that survival rates are still low for out-of-hospital cardiac arrest. They also proposed that a cell phone-based alert system could be beneficial.
General approaches to ventricular tachycardia and MSC
The use of risk calculators for SCD and AV was recommended to improve external validation, development, and reporting of prognostic models. Forecasting capabilities are constantly evolving and a variety of MSC risk calculators are available.
Advanced catheter ablation techniques, mechanical circulatory support, and autonomic modulation are required for those with medically refractory thunderstorms. Success rates for catheter ablation have been good in patients with failed drug response; but, in addition, mechanical circulatory support and possible autonomic modulation can be considered.
For patients with coronary artery disease and recurrent symptomatic sustained monomorphic ventricular tachycardia (SMVT) despite chronic amiodarone therapy, catheter ablation is recommended. In addition, the first-line treatment for premature ventricular contraction (PVC)-induced cardiomyopathy is catheter ablation. This is a Class I indication.
The decision to implant an implantable cardioverter-defibrillator (ICD) must take into account other risk factors for nonarrhythmic death, the patient's desire, and quality of life. Patients with end-stage renal disease, diabetes, and the elderly benefited less or not at all from an ICD installed for primary prevention. There was a great emphasis on joint decision making (patient and provider) to determine the need and acceptance of the implant.
ICD implantation as primary prevention for patients with non-dilated hypokinetic cardiomyopathy/dilated cardiomyopathy should not be limited to a left ventricular ejection fraction (LVEF) ≤35%. Additional risk factors must be considered. Instead, patients with LVEF < 50% should be considered for implantation if they are accompanied by syncope, late gadolinium enhancement or cardiac magnetic resonance imaging (CMR) results, inducible TVS with programmed pacing, or pathogenic mutations in genes such as lamin A. /W(LMNA),PLN,FLNC, miRBM20.
A recommendation was made for a multimodal approach with systematic screening for survivors of cardiac arrest. This may include coronary angiography, brain/chest computed tomography (CT), blood for toxicology and genetic testing, cardiovascular implantable electronic device or portable monitor information, echocardiogram, CMR, coronary spasm evaluation and sodium channel blocker, and exercise.
It is also recommended that a comprehensive autopsy be performed for cases of sudden death under the age of 50. For this population, possible genetic heart disease has been identified in up to 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 a multidisciplinary team of specialists for testing and advice. Only those with the skills to advise on the implications and direct appropriately should be referred. Part of this involves excluding 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 test, and CMR.
In a substantial proportion of families, those who die with sudden arrhythmic death syndrome have a diagnosis of underlying genetic heart disease. Therefore, the guidelines recommend that first-degree relatives be referred for cardiac evaluation at a specialized clinic.
For patients with arrhythmogenic right ventricular cardiomyopathy, a high rate of appropriate ICD interventions is observed, not all of which are classified as lifesaving. For those with hemodynamically poorly tolerated VT, it saves lives, but for those with tolerated VT, questions remain about the benefit. It is suggested that this requires further study.
For patients with asymptomatic long QT syndrome (LCTS), the 1-2-3 LQTS risk calculator can be used to estimate arrhythmic risk. The 1-2-3 LQTS risk calculator uses QT interval length 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 LQTS patients with documented QT prolongation are more effective. For patients with CPVT who cannot tolerate beta-blockers, flecainide alone is an option. Furthermore, for patients with CPVT and LQTS, left cardiac denervation plays an important role in treatment, especially for symptomatic patients taking beta-blockers in whom ICD implantation is contraindicated or refused, or for those receiving multiple shocks. or experience syncope while using beta-blockers. -blockers. However, left cardiac denervation should not be considered as an alternative to the ICD in high-risk patients, but rather as an adjunctive therapy.
There were some key points related to Brugada syndrome (BrS). Evidence has shown that BrS is not diagnosed when a Brugada type 1 ECG pattern is obtained by sodium channel blocker test in the absence of additional findings. Findings such as arrhythmic syncope or nocturnal agonal respirations, a family history of BrS, a family history of sudden death (<45 years) with negative autopsy, suspected circumstances of BrS, or documented DVT/VF may help confirm the diagnosis.
It was also unclear whether SCD risk stratification is valid in asymptomatic brs patients with spontaneous type 1 pattern. The data was mixed and controversial. Routine catheter ablation is not recommended in asymptomatic patients with BrS; there are limited long-term follow-up data on ablation for the asymptomatic patient with BrS.
The early repolarization syndrome (ERS) has been suggested to be distinct from the early repolarization pattern (ERP). ERS is diagnosed if a patient is resuscitated from DVT or VF without cardiac 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 not available for ERS, but if there is a family history or MSC, an ICD may be considered.
Finally, in patients with hypertrophic cardiomyopathy (HCM) younger than 16 years, a validated risk calculator is useful for assessing SCD risk. The HCM Risk-Kids Score (https://hcmriskkids.org) was developed for children 1-16 years of age. This score considers the presence of unexplained syncope, maximum LV wall thickness, large left atrial diameter, low LV outflow tract gradient, and nonsustained VT.
Muscular and congenital etiologies
Some key points have been identified in EF of muscular etiology. Specific risk stratification for SCD in patients withLMNAmutation is required. Early atrial and AV, premature conduction disease, high rate of SCD, and development of end-stage heart failure are found withLMNAA mutationsLMNAThe risk calculator has been developed to assist in forecasting (https://lmna-risk-vta.fr/). In addition, an invasive electrophysiological study is required to evaluate patients with myotonic dystrophy who present with palpitations with suspected arrhythmias, syncope, or aborted sudden death. The guidelines include a specific algorithm for risk stratification, SCD prevention, and VA treatment for this population.
Catheter ablation was considered the treatment of choice in patients with repaired tetralogy of Fallot and monomorphic VT. This is a Class I indication. Such procedures should be performed at specialized catheter ablation centers for congenital heart disease.
final thoughts
The guidelines conclude with a discussion of existing knowledge gaps, offering a plan for further study. Each guideline update encourages us to adjust the quality of care we provide to patients. The ESC 2022 guidelines give us a lot to absorb.
Disclosures: Ms. Moulton completed and returned the ICMJE Potential Conflict of Interest Disclosure Form. He has no conflicts of interest to report with respect to the content of this document.
References
1. Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. ESC 2022 Guidelines for the management of patients with ventricular arrhythmias and 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 Treatment of Patients with Ventricular Arrhythmias and Prevention of Sudden Cardiac Death: European Society of Cardiology (ESC) Working Group for the Treatment of Patients with Ventricular Arrhythmias and Prevention of Sudden Cardiac Death. Approved by: European Association of Pediatric and Congenital Cardiology (AEPC).Eur Heart J.2015;36(41):2793-2867. doi:10.1093/eurheartj/ehv316