Guideline sources The following summarized guidelines for the evaluation and management of ventricular arrhythmias (VA) are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2022), the American Heart Association (AHA/HRS/ACC 2018), and the American College of Endocrinology (ACE/AACE 2016). 1 2 3 Calculator Calculator Brugada criteria for ventricular t NYHA functional classification f Guidelines 1. Diagnostic investigations Electrocardiogram: https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 1/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway As per ESC 2022 guidelines, obtain a baseline 12-lead ECG and record the ventricular arrhythmia on a 12-lead ECG whenever possible in patients with newly documented ventricular arrhythmia (frequent premature ventricular complexes, nonsustained VT, sustained monomorphic VT). B As per AHA 2018 guidelines, obtain a 12-lead ECG during tachycardia in patients with sustained, hemodynamically stable, wide complex tachycardia. B Show 3 more Holter monitoring: As per ESC 2022 guidelines, consider obtaining 24-hour Holter ECG in patients with an incidental finding of nonsustained VT. C As per AHA 2018 guidelines, consider obtaining ambulatory ECG monitoring to evaluate whether symptoms, including palpitations, presyncope, or syncope, are caused by ventricular arrhythmia. B Implantable cardiac monitors: consider obtaining implanted cardiac monitoring in patients with sporadic symptoms (including syncope) suspected to be related to ventricular arrhythmia. C Echocardiography: As per ESC 2022 guidelines, obtain echocardiography as first-line evaluation in patients with newly documented ventricular arrhythmia (frequent premature ventricular complexes, nonsustained VT, sustained monomorphic VT). B As per AHA 2018 guidelines, obtain echocardiography for evaluation of cardiac structure and function in patients with known or suspected ventricular arrhythmia likely to be associated with underlying structural heart disease or a risk of sudden cardiac arrest. B Cardiac magnetic resonance imaging: As per ESC 2022 guidelines, consider obtaining cardiac MRI in patients with newly documented ventricular arrhythmia (frequent premature ventricular complexes, nonsustained VT, sustained monomorphic VT) and suspicion of structural heart disease other than coronary artery disease after initial evaluation. C As per AHA 2018 guidelines, consider obtaining cardiac MRI or CT to detect and characterize underlying structural heart disease in patients presenting with ventricular arrhythmia and suspected structural heart disease. C Natriuretic peptides: consider measuring natriuretic peptides (BNP or N-terminal pro-BNP) to add prognostic information to standard risk factors for predicting sudden cardiac death or sudden cardiac arrest in patients with structural heart disease. C Genetic testing: As per ESC 2022 guidelines, obtain genetic testing when a condition is diagnosed in a living or deceased person with a likely genetic basis and risk of ventricular arrhythmia and sudden cardiac death. B Show 5 more As per AHA 2018 guidelines, consider offering genetic counseling in patients and family members requiring genetic testing for risk stratification for sudden cardiac arrest or sudden cardiac death. B https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 2/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway Psychosocial assessment: Obtain an assessment of psychological status and offer treatment of distress in patients with ICDs. B Ensure communication between patient and physician/healthcare professional to address ICD-related concerns and to discuss quality-of-life issues before ICD implantation and during disease progression. B Evaluation for reversible causes: obtain investigation for reversible causes (such as electrolyte imbalances, ischemia, hypoxemia, fever) in patients with ventricular arrhythmia. B Evaluation of premature ventricular complexes: Obtain a regular assessment of ventricular function in patients with premature ventricular complex burden > 10% and normal ventricular function. B Consider obtaining cardiac MRI in patients with premature ventricular complexes/VT and a presentation not typical for an idiopathic origin, despite a normal echocardiogram. C Evaluation of PVC-induced cardiomyopathy: Suspect premature ventricular complex-induced cardiomyopathy in patients with unexplained reduced ejection fraction and premature ventricular complex burden of at least 10%. B Consider obtaining cardiac MRI in patients with suspected premature ventricular complex- induced cardiomyopathy. C Evaluation of idiopathic VT/VF: As per ESC 2022 guidelines, diagnose idiopathic VF in a sudden cardiac arrest survivor preferably with documentation of VF after exclusion of an underlying structural, channelopathic, metabolic, or toxicological etiology. B Show 2 more As per AHA 2018 guidelines, obtain further evaluation for genetic arrhythmia syndromes in young patients (aged < 40 years) with unexplained sudden cardiac arrest, unexplained near- drowning, or recurrent exertional syncope in the absence of ischemic or other structural heart diseases. B Evaluation of sudden cardiac arrest survivors: As per ESC 2022 guidelines, evaluate sudden cardiac arrest survivors without obvious extra- cardiac causes by a multidisciplinary team. B Show 9 more As per AHA 2018 guidelines: Consider obtaining CT or invasive coronary angiography to confirm the presence or absence of ischemic heart disease and guide decisions for myocardial revascularization in patients recovered from unexplained sudden cardiac arrest. B Evaluate patients with sustained ventricular arrhythmia and survivors of sudden cardiac arrest for ischemic heart disease, and perform revascularization as appropriate. B Evaluation of sudden cardiac death victims: As per ESC 2022 guidelines, make investigation of unexpected sudden death a public health priority, especially in case of suspicion of inherited disease. B Show 9 more https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 3/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway As per AHA 2018 guidelines, perform a standardized cardiac-specific autopsy in victims of sudden cardiac death without obvious causes. B Show 2 more Evaluation of relatives of sudden arrhythmic death syndrome decedents: As per ESC 2022 guidelines, obtain familial evaluation of sudden arrhythmic death syndrome decedents in the following groups: first-degree relatives relatives who must carry a mutation based on analysis of the family history relatives with suspicious symptoms when the decedent's age is < 50 years or if there is other circumstantial data or family history to suggest heritable disease. B Show 8 more As per AHA 2018 guidelines, obtain cardiac evaluation in first-degree relatives of sudden cardiac death victims aged 40 years. Offer genetic counseling and obtain genetic testing as indicated by clinical findings. B 2. Diagnostic procedures Electrophysiology study: As per ESC 2022 guidelines, consider obtaining electrophysiological testing, electroanatomical mapping, and performing mapping-guided biopsies for etiological evaluation in patients presenting with a first sustained monomorphic VT episode. C As per AHA 2018 guidelines, do not obtain electrophysiologic testing for the sole reason of inducing ventricular arrhythmia for risk stratification in patients meeting the criteria for ICD implantation. D 3. Medical management Shared decision-making: Adopt a shared decision-making approach for treatment decisions in patients with ventricular arrhythmia or at increased risk for sudden cardiac death based on the best available evidence as well as the patient's health goals, preferences, and values. B Inform patients considering implantation of a new ICD or replacement of an existing ICD for a low battery about their individual risk of sudden cardiac death and non-sudden death from HF or non-cardiac conditions, and the effectiveness, safety and potential complications of the ICD in light of their health goals, preferences, and values. B Withdrawal of offending agents: withdraw offending agents whenever drug-induced VA are suspected. B Management of cardiac arrest: As per ESC 2022 guidelines: Perform prompt CPR by bystanders at out-of-hospital cardiac arrest. B https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 4/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway Consider implementing mobile phone-based alerting of basic life support-trained bystander volunteers to assist nearby out-of-hospital cardiac arrest victims. C As per AHA 2018 guidelines, perform CPR in patients in cardiac arrest according to published basic and advanced cardiovascular life support algorithms. A Show 3 more Management of sustained VT: As per ESC 2022 guidelines, perform direct current cardioversion as first-line therapy in patients with hemodynamically not-tolerated sustained monomorphic VT. B Show 6 more As per AHA 2018 guidelines, consider administering IV procainamide to attempt to terminate VT in patients with hemodynamically stable VT. B Show 3 more Management of electrical storm: As per ESC 2022 guidelines, administer mild-to-moderate sedation in patients with electrical storm to alleviate psychological distress and reduce sympathetic tone. B Show 9 more As per AHA 2018 guidelines, consider performing cardiac sympathetic denervation in patients with VT/VF storm when -blockers, other antiarrhythmic medications, and catheter ablation are ineffective, not tolerated, or not possible. C Management of idiopathic VT/VF: As per ESC 2022 guidelines, administer IV -blockers (right ventricle outflow tract VT) or verapamil (fascicular VT) in patients presenting with hemodynamically tolerated idiopathic VT. B Show 4 more As per AHA 2018 guidelines, perform ICD placement in patients resuscitated from sudden cardiac arrest due to idiopathic polymorphic VT or VF, if the expected meaningful survival is > 1 year. B Management of premature ventricular complexes: As per ESC 2022 guidelines, perform catheter ablation as first-line therapy in symptomatic idiopathic VT/premature ventricular complexes from the right ventricle outflow tract or the left fascicles. B Show 7 more As per AHA 2018 guidelines, consider initiating -blockers or nondihydropyridine CCB to reduce recurrent arrhythmias and improve symptoms in patients with symptomatic premature ventricular complexes in an otherwise normal heart. B Management of PVC-induced cardiomyopathy: As per ESC 2022 guidelines, perform catheter ablation in patients with cardiomyopathy suspected to be caused by frequent and predominately monomorphic premature ventricular complexes. B Show 3 more As per AHA 2018 guidelines, consider performing catheter ablation in patients with recurrent episodes of idiopathic VF initiated by premature ventricular complexes with a consistent QRS https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 5/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway morphology. B Show 2 more Management of heart failure: As per ESC 2022 guidelines, initiate optimal medical treatment including ACEIs/ARBs/angiotensin receptor neprilysin inhibitors, mineralocorticoid receptor antagonists, -blockers, and SGLT-2 inhibitors in all patients with HFrEF. A As per AHA 2018 guidelines, initiate -blockers, mineralocorticoid receptor antagonists, and either ACEIs, angiotensin-receptor blockers, or angiotensin receptor-neprilysin inhibitors to reduce sudden cardiac death and all-cause mortality in patients with HFrEF (LVEF 40%). A Show 2 more Beta-blockers: consider initiating -blockers in patients with symptomatic, non-life-threatening ventricular arrhythmia. C Antiarrhythmic agents: consider initiating an antiarrhythmic medication to reduce recurrent symptomatic arrhythmias and improve symptoms in patients with symptomatic ventricular arrhythmia in an otherwise normal heart, if -blockers and nondihydropyridine CCBs are ineffective or not tolerated. C Intravenous amiodarone: As per ESC 2022 guidelines: Consider administering IV amiodarone in patients presenting with hemodynamically tolerated sustained monomorphic VT in the absence of an established diagnosis. C Consider administering amiodarone in patients with VT/VF with an indication for ICD placement and no contraindication for amiodarone when an ICD is not available, contraindicated for concurrent medical reasons, or declined by the patient. C As per AHA 2018 guidelines, perform direct current cardioversion in patients presenting with VA with hemodynamic instability. A Show 2 more Intravenous procainamide: consider administering IV procainamide to attempt to terminate VT in patients with hemodynamically stable VT. B Intravenous magnesium: do not administer IV magnesium in patients with refractory VF not related to torsades de pointes. D Intravenous sotalol: consider administering IV sotalol or amiodarone to attempt to terminate VT in patients with hemodynamically stable VT. C Intravenous calcium channel blockers: As per ESC 2022 guidelines, do not administer IV verapamil in patients with broad QRS complex tachycardia of unknown mechanism. D As per AHA 2018 guidelines, do not administer CCBs (such as verapamil and diltiazem) in patients with a wide QRS complex tachycardia of unknown origin. D End-of-life care: As per ESC 2022 guidelines, ensure informed discussion with the patient and family about ICD deactivation options, and shared decision-making before implantation and in case of significant health status deterioration. B https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 6/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway As per AHA 2018 guidelines: Inform patients at the time of ICD implantation or replacement, and during advance care planning, that their ICD shock therapy can be deactivated at any time if it is consistent with their goals and preferences. B Discuss ICD shock deactivation, taking the patient's goals and preferences, in patients with refractory HF symptoms, refractory sustained ventricular arrhythmia, or nearing the end of life from other illnesses. B 4. Nonpharmacologic interventions Cardiopulmonary resuscitation: As per ESC 2022 guidelines, perform prompt CPR by bystanders at out-of-hospital cardiac arrest. B Show 2 more As per AHA 2018 guidelines, perform CPR in patients in cardiac arrest according to published basic and advanced cardiovascular life support algorithms. A 5. Therapeutic procedures Implantable cardioverter-defibrillator (indications): consider assessing the need for ICD implantation based on an individual evaluation of the risk of subsequent ventricular arrhythmia/sudden cardiac death despite a possible correctable cause for the presenting ventricular arrhythmia. C Show 4 more Implantable cardioverter-defibrillator, technical considerations: As per ESC 2022 guidelines, consider placing a subcutaneous defibrillator as an alternative to a transvenous defibrillator in patients with an indication for ICD placement when pacing therapy for bradycardia, cardiac resynchronization, or anti-tachycardia pacing is not needed. C Show 2 more As per HRS/ACC/AHA 2018 guidelines, perform subcutaneous ICD placement in patients meeting criteria for an ICD having inadequate vascular access or at high risk for infection, if pacing for bradycardia or VT termination or as part of cardiac resynchronization therapy is neither needed nor anticipated. B Show 2 more Implantable cardioverter-defibrillator (optimization of device programming): optimize ICD programming to avoid inappropriate and unnecessary therapies and to reduce mortality. A Show 10 more Wearable cardioverter-defibrillator: As per ESC 2022 guidelines: Consider performing wearable cardioverter-defibrillator placement in adult patients with an indication for ICD for secondary prevention being temporarily illegible for ICD implantation. https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 7/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway C Consider performing wearable cardioverter-defibrillator placement in the early phase after myocardial infarction in selected patients. C As per AHA 2018 guidelines: Consider performing wearable cardioverter-defibrillator placement for the prevention of sudden cardiac death in patients with an ICD and a history of sudden cardiac arrest or sustained ventricular arrhythmia, if removal of the ICD is required (as with infection). C Consider performing wearable cardioverter-defibrillator placement in patients at increased risk of sudden cardiac death but ineligible for an ICD, such as awaiting cardiac transplantation, having an LVEF 35% and being within 40 days from myocardial infarction, or having a newly diagnosed non-ischemic cardiomyopathy, revascularization within the past 90 days, myocarditis, secondary cardiomyopathy, or systemic infection. C Catheter ablation (acute management): Perform catheter ablation in patients presenting with incessant VT or electrical storm due to sustained monomorphic VT refractory to antiarrhythmic drugs. B Consider performing catheter ablation in patients with recurrent episodes of polymorphic VT/VF triggered by a similar premature ventricular complex nonresponsive to medical treatment or coronary revascularization. C Catheter ablation, secondary prevention: As per ESC 2022 guidelines, consider performing catheter ablation in patients with sustained monomorphic VT or sustained polymorphic VT/VF triggered by a premature ventricular complex with similar morphology and an indication for ICD when an ICD is not available, contraindicated for concurrent medical reasons, or declined by the patient. C As per HRS/ACC/AHA 2018 guidelines: Consider performing catheter ablation for reducing the risk of recurrent VT and ICD shocks in patients with bundle-branch reentrant VT. B Consider performing epicardial catheter ablation for reducing the risk of recurrent monomorphic VT in patients with structural heart disease failed endocardial catheter ablation. C Catheter ablation (concomitant treatment): Perform catheter ablation in patients with an ICD with recurrent supraVT resulting in inappropriate ICD therapies. B Administer pharmacological therapy or perform catheter ablation in patients with AF-related inappropriate ICD therapies despite optimal ICD programming. B 6. Specific circumstances Athletes: consider obtaining pre-participation cardiovascular evaluation in competitive athletes. C Show 5 more Elderly patients: As per ESC 2022 guidelines, consider omitting ICD placement for primary prevention in elderly patients if a benefit from the defibrillator is not expected due to the patient's age and https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 8/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway comorbidities. C As per AHA 2018 guidelines, consider performing ICD placement in older patients with significant comorbidities meeting indications for a primary prevention ICD, if the expected meaningful survival is > 1 year. C Pregnant patients: As per ESC 2022 guidelines, perform electrical cardioversion for sustained VT during pregnancy. B Show 6 more As per AHA 2018 guidelines, continue -blocker therapy during pregnancy and throughout the postpartum period (including breastfeeding) in mothers with long QT syndrome. B Show 2 more Patients with obesity: offer orlistat and lorcaserin as the preferred weight-loss medications in patients with a history or risk of cardiac arrhythmia. B Be cautious when using naltrexone ER/bupropion ER, liraglutide 3 mg, and phentermine/topiramate ER with careful monitoring of HR and rhythm. A Heart transplant recipients: As per ESC 2022 guidelines, consider performing ICD placement for primary prevention in patients awaiting heart transplantation. C Show 2 more As per AHA 2018 guidelines, consider performing ICD placement in patients with a heart transplant and severe allograft vasculopathy with LV dysfunction, if the expected meaningful survival is > 1 year. C Patients with outflow tract VA: Consider performing catheter ablation in patients with symptomatic outflow tract ventricular arrhythmia in an otherwise normal heart, if antiarrhythmic medications are ineffective, not tolerated, or not the patient's preference. B Consider initiating -blockers or CCBs in patients with symptomatic outflow tract VT in an otherwise normal heart. B Patients with papillary muscle VA: consider performing catheter ablation in patients with symptomatic ventricular arrhythmia arising from papillary muscles, if antiarrhythmic medications are ineffective, not tolerated, or not the patien's preference. B Patients with interfascicular reentrant VT: consider performing catheter ablation in patients with verapamil-sensitive, idiopathic LV tachycardia related to interfascicular reentry, if antiarrhythmic medications are ineffective, not tolerated, or not the patient's preference. B Show 2 more Patients with medication-induced arrhythmia: administer digoxin antibodies in patients presenting with sustained ventricular arrhythmia potentially due to digoxin toxicity. B Show 5 more Patients with acute coronary syndrome: As per ESC 2022 guidelines, administer IV -blockers in patients with recurrent polymorphic VT/VF during STEMI unless contraindicated. B Show 6 more https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 9/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway As per AHA 2018 guidelines, perform angiography with emergency revascularization in patients with polymorphic VT or VF with STEMI. B Show 3 more Patients with coronary artery spasm: As per ESC 2022 guidelines: Consider obtaining ergonovine, acetylcholine, or hyperventilation testing for the diagnosis of coronary vasospasm in sudden cardiac arrest survivors. C Consider performing ICD placement in sudden cardiac arrest survivors with coronary artery spasm. C As per AHA 2018 guidelines, initiate maximally tolerated doses of CCBs and advise smoking cessation to reduce recurrent ischemia and ventricular arrhythmia in patients with ventricular arrhythmia due to coronary artery spasm. B Show 2 more Patients with coronary artery disease, evaluation: As per ESC 2022 guidelines, perform programmed electrical stimulation when syncope remains unexplained after noninvasive evaluation in patients with syncope and previous STEMI. B As per HRS/ACC/AHA 2018 guidelines, evaluate patients with sustained ventricular arrhythmia and survivors of sudden cardiac arrest for ischemic heart disease. B Patients with coronary artery disease (management): Perform revascularization as appropriate in patients with ischemic heart disease and sustained ventricular arrhythmia or survived sudden cardiac arrest. B Consider performing surgical ablation in patients with monomorphic VT refractory to antiarrhythmic medications and attempts at catheter ablation. C Patients with coronary artery disease, primary prevention of SCD: As per ESC 2022 guidelines, perform ICD placement in patients with coronary artery disease, symptomatic HF (NYHA class II-III), and LVEF 35% despite 3 months of optimal medical treatment. A Show 3 more Landmark trials: MUSTT In patients with coronary artery disease, an LVEF of 40%, and asymptomatic, unsustained VT in whom sustained ventricular tachyarrhythmias were induced by programmed stimuation, antiarrhythmic therapy was superior to no antiarrhythmic therapy with respect to cardiac arrest or death from arrhythmia. Buxton AE et al. N Engl J Med. 1999 Dec 16. As per HRS/ACC/AHA 2018 guidelines, perform ICD placement in patients with LVEF 35% due to ischemic heart disease and at least 40 days post-myocardial infarction and at least 90 days post-revascularization, and with NYHA class II or III HF despite guideline-directed medical therapy, if the expected meaningful survival is > 1 year. A https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 10/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway Show 5 more Patients with coronary artery disease, secondary prevention of SCD: As per ESC 2022 guidelines, perform ICD placement in patients without ongoing ischemia with documented VF or hemodynamically not-tolerated VT occurring later than 48 hours after myocardial infarction. A Show 6 more As per HRS/ACC/AHA 2018 guidelines, perform ICD placement in patients with ischemic heart disease either survived sudden cardiac arrest due to VT/VF or experienced hemodynamically unstable VT B or stable sustained VT B not due to reversible causes, if the expected meaningful survival is > 1 year. B Show 2 more Patients with coronary artery disease (management of recurrent VA): consider initiating amiodarone or sotalol to suppress recurrent ventricular arrhythmia in patients with ischemic heart disease and recurrent ventricular arrhythmia with significant symptoms or ICD shocks despite optimal device programming and ongoing treatment with a -blocker. B Show 5 more Patients with coronary anomalies: As per ESC 2022 guidelines, obtain cardiac stress imaging during physical exercise in addition to cardiopulmonary exercise testing to confirm/exclude myocardial ischemia in patients with anomalous aortic origin of a coronary artery with an interarterial course. B Show 3 more As per AHA 2018 guidelines, perform repair or revascularization in patients with anomalous origin of a coronary artery suspected to be the cause of sudden cardiac arrest. B Patients with dilated cardiomyopathy, evaluation: As per ESC 2022 guidelines, consider obtaining electrophysiological evaluation when syncope remains unexplained after noninvasive evaluation in patients with dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy. C Show 5 more As per HRS/ACC/AHA 2018 guidelines, consider obtaining electrophysiologic testing for assessing the risk of sustained VT in patients with ischemic cardiomyopathy having syncope or other ventricular arrhythmia symptoms and not meeting indications for a primary prevention ICD. C Patients with dilated cardiomyopathy (primary prevention of SCD): discourage participation in high-intensity exercise including competitive sports in patients with dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy and a lamin A/C mutation. B Show 3 more Patients with dilated cardiomyopathy (secondary prevention of SCD): perform ICD placement in patients with dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy survived sudden cardiac arrest due to VT/VF or experienced hemodynamically not-tolerated sustained monomorphic VT. B Show 3 more Landmark trials: PARTITA https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 11/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway In patients with dilated cardiomyopathy who received implantable cardioverter defibrillator shock for VT, ablation was superior to standard therapy with respect to the composite outcome of death from any cause or hospitalization for worsening HF. Paolo Della Bella et al. Circulation. 2022 Jun 21. Patients with nonischemic cardiomyopathy (evaluation): consider obtaining cardiac MRI with late gadolinium enhancement for diagnosis in patients with suspected non-ischemic cardiomyopathy from myocardial infiltrative processes. B Show 3 more Patients with nonischemic cardiomyopathy (primary prevention of SCD): perform ICD placement in patients with non-ischemic cardiomyopathy, HF with NYHA class II-III symptoms, and LVEF of 35% despite guideline-directed medical therapy, if the expected meaningful survival is > 1 year. A Show 3 more Patients with nonischemic cardiomyopathy (secondary prevention of SCD): perform ICD placement in patients with non-ischemic cardiomyopathy either survived sudden cardiac arrest due to VT/VF or experienced hemodynamically unstable VT B or stable sustained VT B not due to reversible causes, if the expected meaningful survival is > 1 year. B Show 2 more Patients with nonischemic cardiomyopathy (management of recurrent VA): Consider initiating amiodarone or sotalol in patients with non-ischemic cardiomyopathy and an ICD experiencing spontaneous ventricular arrhythmia or recurrent appropriate shocks despite optimal device programming and treatment with a -blocker. C Consider performing catheter ablation for reducing recurrent VT and ICD shocks in patients with non-ischemic cardiomyopathy and recurrent sustained monomorphic VT failed or intolerant to antiarrhythmic medications. C Patients with arrhythmogenic right ventricular cardiomyopathy, evaluation: As per ESC 2022 guidelines, obtain cardiac MRI in patients with suspected arrhythmogenic RV cardiomyopathy. B Show 3 more As per HRS/ACC/AHA 2018 guidelines, consider obtaining clinical screening for arrhythmogenic RV cardiomyopathy along with providing genetic counseling and obtaining genetic testing in selected first-degree relatives of patients with arrhythmogenic RV cardiomyopathy, if the proband has a disease-causing mutation. B Show 4 more Patients with arrhythmogenic right ventricular cardiomyopathy, primary prevention of SCD: As per ESC 2022 guidelines, discourage participation in high-intensity exercise in patients with confirmed arrhythmogenic RV cardiomyopathy. B Show 5 more As per HRS/ACC/AHA 2018 guidelines, advise avoiding intensive exercise in patients with a clinical diagnosis of arrhythmogenic RV cardiomyopathy. B https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 12/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway Show 2 more Patients with arrhythmogenic right ventricular cardiomyopathy, secondary prevention of SCD: As per ESC 2022 guidelines, initiate -blockers in patients with arrhythmogenic RV cardiomyopathy and non-sustained or sustained ventricular arrhythmia. B Show 5 more As per HRS/ACC/AHA 2018 guidelines, perform ICD placement in patients with arrhythmogenic RV cardiomyopathy and an additional marker of increased risk of sudden cardiac death (resuscitated sudden cardiac arrest, sustained VT, significant ventricular dysfunction with RV ejection fraction or LVEF 35%), if the expected meaningful survival id > 1 year. B Show 2 more Patients with hypertrophic cardiomyopathy, evaluation: As per ESC 2022 guidelines, obtain cardiac MRI with late gadolinium enhancement for diagnostic workup in patients with hypertrophic cardiomyopathy. B Show 3 more As per HRS/ACC/AHA 2018 guidelines, obtain sudden cardiac death risk stratification at the time of initial evaluation and periodically thereafter in patients with hypertrophic cardiomyopathy. B Show 4 more Patients with hypertrophic cardiomyopathy, primary prevention of SCD: As per ESC 2022 guidelines, consider advising participation in high-intensity exercise in asymptomatic adult patients with hypertrophic cardiomyopathy without risk markers. C Show 5 more As per HRS/ACC/AHA 2018 guidelines, do not perform ICD placement in patients with an identified hypertrophic cardiomyopathy genotype in the absence of sudden cardiac death risk factors. D Patients with hypertrophic cardiomyopathy, secondary prevention of SCD: As per ESC 2022 guidelines, perform ICD placement in patients with hypertrophic cardiomyopathy with hemodynamically not-tolerated VT or VF. B Show 3 more As per HRS/ACC/AHA 2018 guidelines, perform ICD placement in patients with hypertrophic cardiomyopathy survived a sudden cardiac arrest due to VT or VF, or having spontaneous sustained VT causing syncope or hemodynamic compromise, if the expected meaningful survival is > 1 year. B Show 4 more Patients with left ventricular non-compaction cardiomyopathy: consider performing ICD placement for primary prevention of sudden cardiac death in patients with LV non-compaction cardiomyopathy phenotype based on cardiac MRI or echocardiography. C Patients with neuromuscular disorders (evaluation): obtain an annual follow-up with at least a 12-lead ECG in patients with muscular dystrophies, even in the concealed phase of the disease. B Show 4 more https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 13/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway Patients with neuromuscular disorders, management: As per ESC 2022 guidelines, treat patients with neuromuscular disorders having VA or ventricular dysfunction similarly to patients with ventricular arrhythmia without neuromuscular disorders. B Show 8 more As per HRS/ACC/AHA 2018 guidelines, perform ICD placement for primary and secondary prevention in patients with neuromuscular disorders with the same indications as for patients with non-ischemic cardiomyopathy, if the expected meaningful survival is > 1 year. B Show 3 more Patients with myocarditis: As per ESC 2022 guidelines, refer patients with confirmed or clinically suspected acute myocarditis presenting with life-threatening VA to a specialized center. B Show 6 more As per AHA 2018 guidelines: Refer patients with life-threatening VT or VF associated with confirmed or clinically suspected myocarditis to centers with mechanical hemodynamic support and advanced arrhythmia management. B Consider performing ICD placement and/or initiating antiarrhythmic medications in patients with giant cell myocarditis with VF or hemodynamically unstable VT treated according to guideline-directed medical therapy, if the expected meaningful survival is > 1 year. C Patients with cardiac amyloidosis: consider performing ICD placement in patients with light- chain amyloidosis or transthyretin-associated cardiac amyloidosis and hemodynamically not- tolerated VT. C Patients with cardiac sarcoidosis (primary prevention of SCD): perform ICD placement in patients with cardiac sarcoidosis having a LVEF 35%. B Show 4 more Patients with cardiac sarcoidosis, secondary prevention of SCD: As per ESC 2022 guidelines, perform ICD placement in patients with cardiac sarcoidosis having documented sustained VT or aborted cardiac arrest. B Show 2 more As per HRS/ACC/AHA 2018 guidelines, perform ICD placement in patients with cardiac sarcoidosis sustained VT, survived sudden cardiac arrest, or having LVEF of 35%, if the expected meaningful survival is > 1 year. B Show 4 more Patients with Chagas cardiomyopathy: consider initiating amiodarone to reduce the arrhythmia burden in patients with Chagas cardiomyopathy presenting with symptomatic premature ventricular complexes or VT. C Show 2 more Patients with valvular heart disease: Perform programmed electrical stimulation with standby catheter ablation in patients with aortic valve disease and sustained monomorphic VT to identify and ablate bundle branch re- entrant VT, especially if it occurs following a valve intervention. B https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 14/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway Follow dilated cardiomyopathy/hypokinetic non-dilated cardiomyopathy recommendations for ICD placement for primary prevention in patients with VHD and persistent LV dysfunction after surgical correction (if possible). B Patients with congenital heart disease, evaluation: As per ESC 2022 guidelines, consider obtaining electrophysiologic evaluation including programmed electrical stimulation in patients after repair of tetralogy of Fallot with arrhythmia symptoms and non-sustained VT, C or without arrhythmia symptoms but with a combination of other risk factors. C As per HRS/ACC/AHA 2018 guidelines, consider obtaining electrophysiologic testing to evaluate the risk of sustained VT in patients with adult congenital heart disease presenting with syncope or other ventricular arrhythmia symptoms and not meeting indications for a primary prevention ICD. C Show 2 more Patients with congenital heart disease, management: As per ESC 2022 guidelines, perform ICD placement in adult patients with congenital heart disease with biventricular physiology and a left systemic ventricle presenting with symptomatic HF (NYHA II/III) and ejection fraction 35% despite 3 months of optimal medical treatment. B Show 11 more As per HRS/ACC/AHA 2018 guidelines, perform catheter or surgical intervention as feasible for the treatment of hemodynamic abnormalities before consideration of ablation or an ICD in patients with adult congenital heart disease and complex or sustained ventricular arrhythmia in the presence of important residual hemodynamic lesions. B Show 8 more Patients with long QT syndrome, evaluation: As per ESC 2022 guidelines, diagnose long QT syndrome with either QTc 480 ms in repeated 12-lead ECGs with or without symptoms or long QT syndrome diagnostic score > 3. B Show 5 more As per HRS/ACC/AHA 2018 guidelines, provide genetic counseling and obtain genetic testing in patients with clinically diagnosed long QT syndrome. B Show 3 more Patients with long QT syndrome, management: As per ESC 2022 guidelines, consider calculating the arrhythmic risk before initiation of therapy based on the genotype and duration of QTc interval in patients with long QT syndrome. C Show 9 more As per HRS/ACC/AHA 2018 guidelines, initiate -blockers in patients with long QT syndrome with a resting QTc > 470 ms. B Show 5 more Patients with Andersen-Tawil syndrome (evaluation): Suspect Andersen-Tawil syndrome in patients without structural heart disease presenting with at least 2 of the following: https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 15/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway prominent U waves with or without prolongation of the QT interval bidirectional and/or polymorphic premature ventricular complexes/VT dysmorphic features periodic paralysis KCNJ2 pathogenic loss of function mutation. B Obtain genetic testing in patients with suspected Andersen-Tawil syndrome. B Patients with Andersen-Tawil syndrome (management): perform ICD placement in patients with Andersen-Tawil syndrome after aborted cardiac arrest or not-tolerated sustained VT. B Show 3 more Patients with short QT syndrome, evaluation: As per ESC 2022 guidelines, suspect short QT syndrome in the presence of a QTc 320 ms. B Show 5 more As per HRS/ACC/AHA 2018 guidelines, provide genetic counseling and obtain mutation- specific genetic testing in first-degree relatives of patients having a causative mutation for short QT syndrome. B Show 2 more Patients with short QT syndrome, management: As per ESC 2022 guidelines, perform ICD placement in patients with a diagnosis of short QT syndrome: survived an aborted cardiac arrest documented spontaneous sustained VT. B Show 4 more As per HRS/ACC/AHA 2018 guidelines, offer observation without treatment in asymptomatic patients with a short QTc interval. B Show 4 more Patients with Brugada syndrome, evaluation: As per ESC 2022 guidelines, diagnose Brugada syndrome in patients with spontaneous type 1 Brugada ECG pattern and no other heart disease. B Show 5 more As per HRS/ACC/AHA 2018 guidelines, consider obtaining a pharmacological challenge with sodium channel blockers for diagnosis in patients with suspected Brugada syndrome in the absence of a spontaneous type 1 Brugada ECG pattern. C Show 3 more Patients with Brugada syndrome, management: As per ESC 2022 guidelines, offer the following in all patients with Brugada syndrome: avoidance of drugs likely to induce ST-segment elevation in right precordial leads avoidance of cocaine, cannabis, and excessive alcohol intake