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 treatment of fever with antipyretic drugs. B Show 9 more https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 16/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway As per HRS/ACC/AHA 2018 guidelines, offer observation without treatment in asymptomatic patients with only inducible type 1 Brugada ECG pattern. B Show 4 more Patients with early repolarization pattern, evaluation: As per ESC 2022 guidelines, diagnose early repolarization pattern as J-point elevation of 1 mm in 2 adjacent inferior and/or lateral ECG leads. B Show 5 more As per HRS/ACC/AHA 2018 guidelines: Do not obtain genetic testing in patients with an early repolarization pattern on ECG. D Do not obtain electrophysiologic testing for risk stratification for ventricular arrhythmia in patients with early repolarization syndromes. D Patients with early repolarization pattern, management: As per ESC 2022 guidelines, perform ICD placement in patients with a diagnosis of early repolarization syndrome survived a cardiac arrest. B Show 7 more As per HRS/ACC/AHA 2018 guidelines: Offer observation without treatment in asymptomatic patients with an early repolarization pattern on ECG. B Perform ICD placement in patients with an early repolarization pattern on ECG and cardiac arrest or sustained ventricular arrhythmia, if the expected meaningful survival is > 1 year. B Patients with catecholaminergic polymorphic VT, evaluation: As per ESC 2022 guidelines, diagnose catecholaminergic polymorphic VT in the presence of a structurally normal heart, normal ECG, and exercise- or emotion-induced bidirectional or polymorphic VT. B Show 4 more As per HRS/ACC/AHA 2018 guidelines, consider providing genetic counseling and obtaining genetic testing in patients with catecholaminergic polymorphic VT and clinical VT or exertional syncope. C Show 2 more Patients with catecholaminergic polymorphic VT, management: As per ESC 2022 guidelines, discourage participation in competitive sports, strenuous exercise, and exposure to stressful environments in all patients with catecholaminergic polymorphic VT. B Show 6 more As per HRS/ACC/AHA 2018 guidelines, initiate -blockers in patients with catecholaminergic polymorphic VT. B Show 2 more 7. Quality improvement Public access defibrillation: https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 17/19 6/29/23, 3:42 AM Ventricular arrhythmias Pathway Ensure public access defibrillation is available at sites where cardiac arrest is more likely to occur. B Promote community training in basic life support to increase bystander CPR rate and automated external defibrillator use. B Clinical findings Symptoms Past medical history Chest discomfort Congenital heart disease Chest pain Congenital long QT syndrome Dizziness Congenital short QT syndrome Dyspnea Coronary artery disease Fatigue HF Lightheadedness VHD Orthopnea Cardiac exam Palpitations Pre-syncope Cardiac arrest Syncope Vital signs Hypotension Tachycardia ECG findings Brugada ECG pattern Early repolarization Premature ventricular complexes Prolonged QT interval Prolonged QTc interval Torsade de pointes VF VT Studies 2022 PAUSE-SCD In patients with cardiomyopathy and monomorphic VT with an indication for ICD implantation, ablation therapy was superior to conventional therapy with respect to the composite outcome of VT recurrence, cardiovascular hospitalization or death. Roderick Tung et al. Circulation. 2022 Jun 21. 2022 PARTITA https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 18/19 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. Show 11 more References 1. Sana M Al-Khatib, William G Stevenson, Michael J Ackerman et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138 13):e272-e391. Open 2. Katja Zeppenfeld, Jacob Tfelt-Hansen, Marta de Riva et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Aug 26;ehac262. Open 3. W Timothy Garvey, Jeffrey I Mechanick, Elise M Brett et al. AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND AMERICAN COLLEGE OF ENDOCRINOLOGY COMPREHENSIVE CLINICAL PRACTICE GUIDELINES FOR MEDICAL CARE OF PATIENTS WITH OBESITY. Endocr Pract. 2016 Jul;22 Suppl 3 1 203. Open 4. Silvia G Priori, Carina Blomstr m-Lundqvist, Andrea Mazzanti 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 Nov 1;36 41 2793 2867. Open 5. William J Groh, Deepak Bhakta, Gordon F Tomaselli et al. 2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Heart Rhythm. 2022 Oct;19 10):e61-e120. Open 6. Michael J Ackerman, Silvia G Priori, Stephan Willems et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society HRS and the European Heart Rhythm Association EHRA . Heart Rhythm. 2011 Aug;8 8 1308 39. Open 7. Silvia G Priori, Arthur A Wilde, Minoru Horie et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013 Dec;10 12 1932 63. Open https://web.pathway.md/diseases/rec2ihfst1ClYcQ5y 19/19