Sudden death

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  • ARVC= arrhythmogenic right ventricular cardiomyopathy; LVH = left ventricular hypertrophy
  • AD = autosomal dominant; AR = autosomal recessive; CM = cardiomyopathy; HB = heart block; MD = muscular dystrophy; VA = ventricular arrhythmias; + = low-frequency event; ++ = intermediate-frequency event; +++ = high-frequency event.
  • *These may also accumulate to toxic levels with co-administration of inhibitor drugs like ketoconazole. Data are from Roden DM, Anderson ME. Proarrhythmia. In: Kass RS, Clancey CE, editors. Handbook of Experimental Pharmacology: vol. 171. Basis and Treatment of Cardiac Arrhythmias. Boston: Springer Verlag, 2006:288–304 (1021).
  • *These may also accumulate to toxic levels with co-administration of inhibitor drugs like ketoconazole. Data are from Roden DM, Anderson ME. Proarrhythmia. In: Kass RS, Clancey CE, editors. Handbook of Experimental Pharmacology: vol. 171. Basis and Treatment of Cardiac Arrhythmias. Boston: Springer Verlag, 2006:288–304 (1021).
  • *These may also accumulate to toxic levels with co-administration of inhibitor drugs like ketoconazole. Data are from Roden DM, Anderson ME. Proarrhythmia. In: Kass RS, Clancey CE, editors. Handbook of Experimental Pharmacology: vol. 171. Basis and Treatment of Cardiac Arrhythmias. Boston: Springer Verlag, 2006:288–304 (1021).
  • *These may also accumulate to toxic levels with co-administration of inhibitor drugs like ketoconazole. Data are from Roden DM, Anderson ME. Proarrhythmia. In: Kass RS, Clancey CE, editors. Handbook of Experimental Pharmacology: vol. 171. Basis and Treatment of Cardiac Arrhythmias. Boston: Springer Verlag, 2006:288–304 (1021).
  • Adapted with permission from Roden DM. Drug-induced prolongation of the QT interval. N Engl J Med 2004;350:1013–22. Copyright © 2004 Massachusetts Medical Society. DNA = deoxyribonucleic acid.
  • Sudden death

    1. 1. ACC Ventricular Arrhythmias & Sudden Cardiac Death Slide Deck <ul><li>Based on the ACC/AHA/ESC 2006 </li></ul><ul><li>Guidelines for Management of Patients </li></ul><ul><li>With Ventricular Arrhythmias and the </li></ul><ul><li>Prevention of Sudden Cardiac Death </li></ul><ul><li>February 2007 </li></ul>
    2. 2. Supported by Medtronic, Inc. Medtronic, Inc. was not involved in the development of this slide deck and in no way influenced its contents.
    3. 3. Miguel A. Quinones, MD, FACC Gabriel Gregoratos, MD, FACC, FAHA Cynthia Tracy, MD, FACC, FAHA ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death Developed by:
    4. 4. Applying Classification of Recommendations and Level of Evidence Class III Risk ≥ Benefit No additional studies needed Procedure/Treatment should NOT be performed/administered SINCE IT IS NOT HELPFUL AND MAY BE HARMFUL Class IIb Benefit ≥ Risk Additional studies with broad objectives needed; Additional registry data would be helpful Procedure/Treatment MAY BE CONSIDERED Class IIa Benefit >> Risk Additional studies with focused objectives needed IT IS REASONABLE to perform procedure/administer treatment Class I Benefit >>> Risk Procedure/ Treatment SHOULD be performed/ administered is not recommended is not indicated should not is not useful/effective/beneficial may be harmful may/might be considered may/might be reasonable usefulness/effectiveness is unknown /unclear/uncertain or not well established is reasonable can be useful/effective/ beneficial is probably recommended or indicated should is recommended is indicated is useful/effective/ beneficial
    5. 5. Applying Classification of Recommendations and Level of Evidence Class III • Recommen-dation that procedure or treatment not useful/effective and may be harmful • Sufficient evidence from multiple randomized trials or meta-analyses Class IIb • Recommen-dation’s usefulness/ efficacy less well established • Greater conflicting evidence from multiple randomized trials or meta-analyses Class IIa • Recommen-dation in favor of treatment or procedure being useful/ effective • Some conflicting evidence from multiple randomized trials or meta-analyses Class I • Recommen-dation that procedure or treatment is useful/ effective • Sufficient evidence from multiple randomized trials or meta-analyses Level A Multiple (3-5) population risk strata evaluated General consistency of direction and magnitude of effect
    6. 6. Applying Classification of Recommendations and Level of Evidence Class III • Recommen-dation that procedure or treatment not useful/effective and may be harmful • Limited evidence from single randomized trial or non-randomized studies Class IIb • Recommen-dation’s usefulness/ efficacy less well established • Greater conflicting evidence from single randomized trial or non-randomized studies Class IIa • Recommen-dation in favor of treatment or procedure being useful/effective • Some conflicting evidence from single randomized trial or non-randomized studies Class I • Recommen-dation that procedure or treatment is useful/ effective • Limited evidence from single randomized trial or non-randomized studies Level B Limited (2-3) population risk strata evaluated
    7. 7. Applying Classification of Recommendations and Level of Evidence Class III • Recommend-ation that procedure or treatment not useful/effective and may be harmful • Only expert opinion, case studies, or standard-of-care Class IIb • Recommen-dation’s usefulness/ efficacy less well established • Only diverging expert opinion, case studies, or standard-of-care Class IIa • Recommen-dation in favor of treatment or procedure being useful/effective • Only diverging expert opinion, case studies, or standard-of-care Class I • Recommen-dation that procedure or treatment is useful/ effective • Only expert opinion, case studies, or standard-of-care Level C Very limited (1-2) population risk strata evaluated
    8. 8. Evidence Review The writing committee considered the following in preparing the recommendations: <ul><li>Therapy to be offered (ICD, AA drugs, and miscellaneous) </li></ul><ul><li>Point at which therapy is offered (primary prevention for patients at risk or secondary prevention for those patients who have already experienced an event) </li></ul><ul><li>Purpose of therapy (life preservation or symptom reduction/improved quality of life) </li></ul><ul><li>Etiology of arrhythmia substrate (CHD, cardiomyopathy, or other conditions) </li></ul><ul><li>Functional status of the patient (NYHA functional class) </li></ul><ul><li>Status of left ventricular function (LVEF) </li></ul><ul><li>Specific arrhythmia concerned (e.g., sustained monomorphic VT, polymorphic VT, or ventricular fibrillation) </li></ul>
    9. 9. Epidemiology of VA & SCD Classification of Ventricular Arrhythmia by Clinical Presentation <ul><li>Hemodynamically stable </li></ul><ul><li>♥ Asymptomatic </li></ul><ul><li>♥ Minimal symptoms, e.g., palpitations </li></ul><ul><li>Hemodynamically unstable </li></ul><ul><ul><ul><li>♥ Presyncope </li></ul></ul></ul><ul><ul><ul><li>♥ Syncope </li></ul></ul></ul><ul><ul><ul><li>♥ Sudden cardiac death </li></ul></ul></ul><ul><ul><ul><li>♥ Sudden cardiac arrest </li></ul></ul></ul>
    10. 10. Epidemiology of VA & SCD Classification of Ventricular Arrhythmia by Electrocardiography <ul><li>Nonsustained ventricular tachycardia (VT) </li></ul><ul><ul><ul><li>♥ Monomorphic </li></ul></ul></ul><ul><ul><ul><li>♥ Polymorphic </li></ul></ul></ul><ul><li>Sustained VT </li></ul><ul><ul><ul><li>♥ Monomorphic </li></ul></ul></ul><ul><ul><ul><li>♥ Polymorphic </li></ul></ul></ul><ul><li>Bundle-branch re-entrant tachycardia </li></ul><ul><li>Bidirectional VT </li></ul><ul><li>Torsades de pointes </li></ul><ul><li>Ventricular flutter </li></ul><ul><li>Ventricular fibrillation </li></ul>
    11. 11. Nonsustained Monomorphic VT
    12. 12. Nonsustained LV VT
    13. 13. Sustained Monomorphic VT 72-year-old woman with CHD
    14. 14. Nonsustained Polymorphic VT
    15. 15. Sustained Polymorphic VT Exercise induced in patient with no structural heart disease
    16. 16. Bundle Branch Reentrant VT
    17. 17. Ventricular Flutter Spontaneous conversion to NSR (12-lead ECG)
    18. 18. VF with Defibrillation (12-lead ECG)
    19. 19. Wide QRS Irregular Tachycardia: Atrial Fibrillation with antidromic conduction in patient with accessory pathway – Not VT
    20. 20. Epidemiology of VA & SCD Classification of Ventricular Arrhythmia by Disease Entity <ul><li>Chronic coronary heart disease </li></ul><ul><li>Heart failure </li></ul><ul><li>Congenital heart disease </li></ul><ul><li>Neurological disorders </li></ul><ul><li>Structurally normal hearts </li></ul><ul><li>Sudden infant death syndrome </li></ul><ul><li>Cardiomyopathies </li></ul><ul><ul><ul><li>♥ Dilated cardiomyopathy </li></ul></ul></ul><ul><ul><ul><li>♥ Hypertrophic cardiomyopathy </li></ul></ul></ul><ul><ul><ul><li>♥ Arrhythmogenic right ventricular (RV) </li></ul></ul></ul><ul><ul><ul><li>cardiomyopathy </li></ul></ul></ul>
    21. 21. Reused with permission from Myerburg RJ, Kessler KM, Castellanos A. Circulation 1992;85:12-10. Epidemiology of VA & SCD Incidence of Sudden Cardiac Death
    22. 22. <ul><li>Ventricular fibrillation - 62.4% </li></ul><ul><li>Bradyarrhythmias (including advanced AV block and asystole) - 16.5% </li></ul><ul><li>Torsades de pointes - 12.7% </li></ul><ul><li>Primary VT - 8.3% </li></ul>Mechanisms of Sudden Cardiac Death in 157 Ambulatory Patients Mechanisms and Substrates Bayes de Luna et al. Am Heart J 1989;117:151–9.
    23. 23. Clinical Presentations of Patients with VA & SCD <ul><li>Asymptomatic individuals with or without electrocardiographic </li></ul><ul><li>abnormalities </li></ul><ul><li>Persons with symptoms potentially attributable to ventricular </li></ul><ul><li>arrhythmias </li></ul><ul><ul><li>♥ Palpitations </li></ul></ul><ul><ul><li>♥ Dyspnea </li></ul></ul><ul><ul><li>♥ Chest pain </li></ul></ul><ul><ul><li>♥ Syncope and presyncope </li></ul></ul><ul><li>VT that is hemodynamically stable </li></ul><ul><li>VT that is not hemodynamically stable </li></ul><ul><li>Cardiac arrest </li></ul><ul><ul><li>♥ Asystolic (sinus arrest, atrioventricular block) </li></ul></ul><ul><ul><li>♥ VT </li></ul></ul><ul><ul><li>♥ Ventricular fibrillation (VF) </li></ul></ul><ul><ul><li>♥ Pulseless electrical activity </li></ul></ul>
    24. 24. Resting Electrocardiogram Resting 12-lead ECG is indicated in all patients who are evaluated for ventricular arrhythmias. General Evaluation for Documented or Suspected VA
    25. 25. Exercise Testing Exercise testing is recommended in adult patients with ventricular arrhythmias who have an intermediate or greater probability of having CHD by age, gender, and symptoms to provoke ischemic changes or ventricular arrhythmias. Exercise testing, regardless of age, is useful in patients with known or suspected exercise-induced ventricular arrhythmias, including catecholaminergic VT, to provoke the arrhythmia, achieve a diagnosis, and determine the patient’s response to tachycardia. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    26. 26. Exercise Testing Exercise testing can be useful in evaluating response to medical or ablation therapy in patients with known exercise-induced ventricular arrhythmias. Exercise testing may be useful in patients with ventricular arrhythmias and a low probability of CHD by age, gender, and symptoms. Exercise testing may be useful in the investigation of isolated premature ventricular complexes (PVCs) in middle-aged or older patients without other evidence of CHD. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    27. 27. Exercise Testing See Table 1 in the ACC/AHA 2002 Guideline Update for Exercise Testing for contraindications. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III
    28. 28. Ambulatory Electrocardiography Ambulatory ECG is indicated when there is a need to clarify the diagnosis by detecting arrhythmias, QT- interval changes, T-wave alternans (TWA), or ST changes to evaluate risk, or to judge therapy. Event monitors are indicated when symptoms sporadic to establish whether or not they are caused by transient arrhythmias. Implantable recorders are useful in patients sporadic symptoms suspected to be related to arrhythmias such as syncope when a symptom-rhythm correlation cannot be established by conventional diagnostic techniques. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    29. 29. Electrocardiographic Techniques and Measurements It is reasonable to use T-wave alternans to improve the diagnosis and risk stratification of patients with ventricular arrhythmias or who are at risk for developing life-threatening ventricular arrhythmias. ECG techniques such as signal-averaged ECG (SAECG), heart rate variability (HRV), baroreceptor reflex sensitivity, and heart rate turbulence may be useful to improve the diagnosis and risk stratification of patients with ventricular arrhythmias or who are at risk of developing life-threatening ventricular arrhythmias. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III A
    30. 30. Left Ventricular Function and Imaging Echocardiography is recommended in patients with ventricular arrhythmias who are suspected of having structural heart disease. Echocardiography is recommended for the subset of patients at high risk for the development of serious ventricular arrhythmias or SCD, such as those with dilated, hypertrophic, or RV cardiomyopathies, AMI survivors, or relatives of patients with inherited disorders associated with SCD. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    31. 31. Left Ventricular Function and Imaging Exercise testing with an imaging modality―(echocardiography or nuclear perfusion [single-photon emission computed tomography (SPECT)])―is recommended to detect silent ischemia in patients with ventricular arrhythmias who have an intermediate probability of having CHD by age, symptoms, and gender and in whom ECG assessment is less reliable because of digoxin use, LVH, greater than 1-mm ST-segment depression at rest, Wolf-Parkinson-White (WPW) syndrome, or Left Bundle Branch Block (LBBB). Pharmacological stress testing with an imaging modality (echocardiography or myocardial perfusion SPECT) is recommended to detect silent ischemia in patients with ventricular arrhythmias who have an intermediate probability of having CHD by age, symptoms, and gender and are physically unable to perform a symptom-limited exercise test. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    32. 32. Left Ventricular Function and Imaging MRI, cardiac computed tomography (CT), or radionuclide angiography can be useful in patients with ventricular arrhythmias when echocardiography does not provide accurate assessment of left ventricular (LV) and RV function and/or evaluation of structural changes. Coronary angiography can be useful in establishing or excluding the presence of significant obstructive CHD in patients with life-threatening ventricular arrhythmias or in survivors of SCD, who have an intermediate or greater probability of having CHD by age, symptoms, and gender. LV imaging can be useful in patients undergoing biventricular pacing. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    33. 33. Conditions Associated With VA That Can Be Diagnosed With Echocardiography Disease Entity Diagnostic Accuracy Dilated cardiomyopathy Ischemic cardiomyopathy Hypertension with moderate to severe LVH Valvular heart disease Arrhythmogenic right ventricular cardiomyopathy (ARVC) Brugada syndrome High High High High Moderate Poor General Evaluation for Documented or Suspected VA
    34. 34. Electrophysiological Testing in Patients With CHD EP testing is recommended for diagnostic evaluation of patients with remote MI with symptoms suggestive of ventricular tachyarrhythmias, including palpitations, presyncope, and syncope. EP testing is recommended in patients with CHD to guide and assess the efficacy of VT ablation. EP testing is useful in patients with CHD for the diagnostic evaluation of wide-QRS-complex tachycardias of unclear mechanism. EP testing is reasonable for risk stratification in patients with remote MI, NSVT, and LVEF equal to or less than 40%. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    35. 35. Electrophysiological Testing in Patients With Syncope EP testing is recommended in patients with syncope of unknown cause with impaired LV function or structural heart disease. EP testing can be useful in patients with syncope when bradyarrhythmias or tachyarrhythmias are suspected and in whom noninvasive diagnostic studies are not conclusive. General Evaluation for Documented or Suspected VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    36. 36. <ul><li>Antiarrhythmic Drugs </li></ul><ul><li>♥ Beta Blockers: Effectively suppress ventricular ectopic beats & arrhythmias; reduce incidence of SCD </li></ul><ul><li>♥ Amiodarone: No definite survival benefit; some studies have shown reduction in SCD in patients with LV dysfunction especially when given in conjunction with BB. Has complex drug interactions and many adverse side effects (pulmonary, hepatic, thyroid, cutaneous) </li></ul><ul><li>♥ Sotalol: Suppresses ventricular arrhythmias; is more pro-arrhythmic than amiodarone, no survival benefit clearly shown </li></ul><ul><li>♥ Conclusions: Antiarrhythmic drugs (except for BB) should not be used as primary therapy of VA and the prevention of SCD </li></ul>Therapies for VA
    37. 37. Non-antiarrhythmic Drugs ♥ Electrolytes: magnesium and potassium administration can favorably influence the electrical substrate involved in VA; are especially useful in setting of hypomagnesemia and hypokalemia ♥ ACE inhibitors, angiotensin receptor blockers and aldosterone blockers can improve the myocardial substrate through reverse remodeling and thus reduce incidence of SCD ♥ Antithrombotic and antiplatelet agents: may reduce SCD by reducing coronary thrombosis ♥ Statins: have been shown to reduce life-threatening VA in high-risk patients with electrical instability ♥ n-3 Fatty acids: have anti-arrhythmic properties, but conflicting data exist for the prevention of SCD Therapies for VA
    38. 38. Therapies for VA ICDs: Results from Primary and Secondary Prevention Trials 0.6 0.8 1.0 1.2 1.4 MADIT-I AVID 1.6 0.4 CABG-Patch MADIT-II 1996 1997 1997 2002 Aborted cardiac arrest N = 196 N = 1016 N = 900 N = 1232 0.46 0.62 1.07 0.69 Hazard ratio ICD better SCD-HeFT N = 1676 2005 0.77 1.8 LVEF, other features 0.35 or less, NSVT, EP positive 0.30 or less, prior MI 0.35 or less, LVD due to prior MI and NICM 0.35 or less, abnormal SAECG and scheduled for CABG CASH* 2000 N = 191 Aborted cardiac arrest DEFINITE 2004 N = 458 0.65 0.35 or less, NICM and PVCs or NSVT CIDS 2000 N = 659 0.82 Aborted cardiac arrest or syncope DINAMIT 2004 N = 674 1.08 0.35 or less, MI within 6 to 40 days and impaired cardiac autonomic function Trial Name, Pub Year 0.83
    39. 39. <ul><li>Primary Prevention of SCD (1) </li></ul><ul><li>Recommendations in previously published guidelines for prophylactic </li></ul><ul><li>ICD therapy based on LVEF are inconsistent: </li></ul><ul><ul><li>♥ Different LVEFs were chosen for inclusion in trials </li></ul></ul><ul><ul><li>♥ Average EF in such trials was substantially lower than the cutoff value for enrollment </li></ul></ul><ul><ul><li>♥ Subgroup analyses in various trials have not been consistent in their implications </li></ul></ul><ul><ul><li>♥ No trials contained randomized patients with intermediate </li></ul></ul><ul><ul><li>LVEF </li></ul></ul>Therapies for VA
    40. 40. <ul><li>Primary Prevention of SCD (2) </li></ul><ul><li>Because of these inconsistencies, the recommendations in this </li></ul><ul><li>guideline were constructed to apply to patients with an EF ≤ to a </li></ul><ul><li>range of values </li></ul><ul><li>The next several slides compare the recommendations of </li></ul><ul><li>previously published guidelines with those in this one and the </li></ul><ul><li>reasoning behind the writing committee’s decision </li></ul>Therapies for VA
    41. 41. <ul><li>Primary Prevention of SCD (3) </li></ul><ul><li>LV dysfunction due to MI, LVEF ≤ 30%, NYHA class II, III </li></ul><ul><ul><li>2005 ACC/AHA HF: Class I; LOE B </li></ul></ul><ul><ul><li>2005 ESC HF: Class I; LOE A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: Class IIa; LOE B </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD: Class IIa; LOE B </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class I; LOE A </li></ul></ul><ul><li>Note: The VA/SCD Guideline has combined all trials that enrolled patients with LV dysfunction due to MI into one recommendation </li></ul>Therapies for VA
    42. 42. <ul><li>Primary Prevention of SCD (4) </li></ul><ul><li>LV dysfunction due to MI, LVEF 30-35%, NYHA class II, III </li></ul><ul><ul><li>2005 ACC/AHA HF: Class IIa; LOE B </li></ul></ul><ul><ul><li>2005 ESC HF: Class I; LOE A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: N/A </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD: N/A </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class I; LOE A </li></ul></ul><ul><li>Note: The VA/SCD Guideline has combined all trials that enrolled patients with LV dysfunction due to MI into one recommendation </li></ul>Therapies for VA
    43. 43. <ul><li>Primary Prevention of SCD (5) </li></ul><ul><li>LV dysfunction due to MI, LVEF 30-40%, NSVT, positive EP study </li></ul><ul><ul><li>2005 ACC/AHA HF: N/A </li></ul></ul><ul><ul><li>2005 ESC HF: N/A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: Class I; LOE B </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD: Class IIb; LOE B </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class I; LOE A </li></ul></ul><ul><li>Note: The VA/SCD Guideline has combined all trials that enrolled patients with LV dysfunction due to MI into one recommendation </li></ul>Therapies for VA
    44. 44. <ul><li>Primary Prevention of SCD (6) </li></ul><ul><li>LV dysfunction due to MI, LVEF ≤ 30%, NYHA class I </li></ul><ul><ul><li>2005 ACC/AHA HF: Class IIa; LOE B </li></ul></ul><ul><ul><li>2005 ESC HF: N/A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: N/A </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD: N/A </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class IIa; LOE B </li></ul></ul><ul><li>Note: The VA/SCD Guideline has expanded the range of LVEF ≤ 30-35% for patients with LVD due to MI and NYHA class I into one recommendation </li></ul>Therapies for VA
    45. 45. <ul><li>Primary Prevention of SCD (7) </li></ul><ul><li>LV dysfunction due to MI, LVEF ≤ 31-35%, NYHA class I </li></ul><ul><ul><li>2005 ACC/AHA HF: N/A </li></ul></ul><ul><ul><li>2005 ESC HF: N/A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: N/A </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD: N/A </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class IIa; LOE B </li></ul></ul><ul><li>Note: The VA/SCD Guideline has expanded the range of LVEF ≤ 30-35% for patients with LVD due to MI and NYHA class I into one recommendation </li></ul>Therapies for VA
    46. 46. <ul><li>Primary Prevention of SCD (8) </li></ul><ul><li>Nonischemic cardiomyopathy , LVEF ≤ 30%, NYHA class II, III </li></ul><ul><ul><li>2005 ACC/AHA HF: Class I; LOE B </li></ul></ul><ul><ul><li>2005 ESC HF: Class I; LOE A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: N/A </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD:N/A </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class I; LOE B </li></ul></ul><ul><li>Note: The VA/SCD Guideline has combined all trials of nonischemic cardiomyopathy, NYHA class II, III into one recommendation </li></ul>Therapies for VA
    47. 47. <ul><li>Primary Prevention of SCD (9) </li></ul><ul><li>Nonischemic cardiomyopathy , LVEF 30-35%, NYHA class II, III </li></ul><ul><ul><li>2005 ACC/AHA HF: Class IIa; LOE B </li></ul></ul><ul><ul><li>2005 ESC HF: Class I; LOE A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: N/A </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD:N/A </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class I; LOE B </li></ul></ul><ul><li>Note: The VA/SCD Guideline has combined all trials of nonischemic cardiomyopathy, NYHA class II, III into one recommendation </li></ul>Therapies for VA
    48. 48. <ul><li>Primary Prevention of SCD (10) </li></ul><ul><li>Nonischemic cardiomyopathy , LVEF ≤ 30%, NYHA class I </li></ul><ul><ul><li>2005 ACC/AHA HF: Class IIb; LOE C </li></ul></ul><ul><ul><li>2005 ESC HF: N/A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: N/A </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD:N/A </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class IIb; LOE B </li></ul></ul><ul><li>Note: The VA/SCD Guideline has expanded the range of LVEF to ≤ 30-35% for patients with nonischemic cardiomyopathy and NYHA class I into one recommendation </li></ul>Therapies for VA
    49. 49. <ul><li>Primary Prevention of SCD (11) </li></ul><ul><li>Nonischemic cardiomyopathy , LVEF ≤ 31-35%, NYHA class I </li></ul><ul><ul><li>2005 ACC/AHA HF: N/A </li></ul></ul><ul><ul><li>2005 ESC HF: N/A </li></ul></ul><ul><ul><li>2004 ACC/AHA STEMI: N/A </li></ul></ul><ul><ul><li>2002 ACC/AHA/NASPE PM/ICD: N/A </li></ul></ul><ul><ul><li>2006 ACC/AHA/ESC VA/SCD: Class IIb; LOE B </li></ul></ul><ul><li>Note: The VA/SCD Guideline has expanded the range of LVEF to ≤ 30-35% for patients with nonischemic cardiomyopathy and NYHA class I into one recommendation </li></ul>Therapies for VA
    50. 50. Ablation Ablation is indicated in patients who are otherwise at low risk for SCD and have sustained predominantly monomorphic VT that is drug resistant, who are drug intolerant, or who do not wish long-term drug therapy. Ablation is indicated in patients with bundle-branch reentrant VT. Ablation is indicated as adjunctive therapy in patients with an ICD who are receiving multiple shocks as a result of sustained VT that is not manageable by reprogramming or changing drug therapy or who do not wish long-term drug therapy. Ablation is indicated in patients with WPW syndrome resuscitated from sudden cardiac arrest due to AF and rapid conduction over the accessory pathway causing VF. Therapies for VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    51. 51. Ablation Ablation can be useful therapy in patients who are otherwise at low risk for SCD and have symptomatic nonsustained monomorphic VT that is drug resistant, who are drug intolerant or who do not wish long-term drug therapy. Ablation can be useful therapy in patients who are otherwise at low risk for SCD and have frequent symptomatic predominantly monomorphic PVCs that are drug resistant or who are drug intolerant or who do not wish long-term drug therapy. Ablation can be useful in symptomatic patients with WPW syndrome who have accessory pathways with refractory periods less than 240 ms in duration. Therapies for VA I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    52. 52. Ablation Ablation of Purkinje fiber potentials may be considered in patients with ventricular arrhythmia storm consistently provoked by PVCs of similar morphology. Ablation of asymptomatic PVCs may be considered when the PVCs are very frequent to avoid or treat tachycardia- induced cardiomyopathy. Ablation of asymptomatic relatively infrequent PVCs is not indicated. Therapies for VA
    53. 53. Management of Cardiac Arrest After establishing the presence of definite, suspected, or impending cardiac arrest, the first priority should be activation of a response team capable of identifying the specific mechanism and carrying out prompt intervention. CPR should be implemented immediately after contacting a response team. In an out-of-hospital setting, if an AED is available, it should be applied immediately and shock therapy administered according to the algorithms contained in the documents on CPR developed by the AHA in association with the International Liaison Committee on Resuscitation (ILCOR) and/or the European Resuscitation Council (ERC). Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    54. 54. Management of Cardiac Arrest For victims with ventricular tachyarrhythmic mechanisms of cardiac arrest, when recurrences occur after a maximally defibrillating shock (generally 360 J for monophasic defibrillators), intravenous amiodarone should be the preferred antiarrhythmic drug for attempting a stable rhythm after further defibrillations. For recurrent ventricular tachyarrhythmias or nontachyarrhythmic mechanisms of cardiac arrest, it is recommended to follow the algorithms contained in the documents on CPR developed by the AHA in association with ILCOR and/or the ERC. Reversible causes and factors contributing to cardiac arrest should be managed during advanced life support, including management of hypoxia, electrolyte disturbances, mechanical factors, and volume depletion. Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    55. 55. Management of Cardiac Arrest For response times greater than or equal to 5 min, a brief (less than 90 to 180 s) period of CPR is reasonable prior to attempting defibrillation. A single precordial thump may be considered by health care professional providers when responding to a witnessed cardiac arrest. Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    56. 56. VT Associated With Low Troponin MI Patients presenting with sustained VT in whom low-level elevations in cardiac biomarkers of myocyte injury/ necrosis are documented should be treated similarly to patients who have sustained VT and in whom no biomarker rise is documented. Acute Management of Specific Arrhythmias
    57. 57. Sustained Monomorphic VT Wide-QRS tachycardia should be presumed to be VT if the diagnosis is unclear. Direct current cardioversion with appropriate sedation is recommended at any point in the treatment cascade in patients with suspected sustained monomorphic VT with hemodynamic compromise. Acute Management of Specific Arrhythmias
    58. 58. Sustained Monomorphic VT Intravenous procainamide (or ajmaline in some European countries) is reasonable for initial treatment of patients with stable sustained monomorphic VT. Intravenous amiodarone is reasonable in patients with sustained monomorphic VT that is hemodynamically unstable, refractory to conversion with countershock, or recurrent despite procainamide or other agents. Transvenous catheter pace termination can be useful to treat patients with sustained monomorphic VT that is refractory to cardioversion or is frequently recurrent despite antiarrhythmic medication. Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    59. 59. Sustained Monomorphic VT Intravenous lidocaine might be reasonable for the initial treatment of patients with stable sustained monomorphic VT specifically associated with acute myocardial ischemia or infarction. Calcium channel blockers such as verapamil and diltiazem should not be used in patients to terminate wide-QRS- complex tachycardia of unknown origin, especially in patients with a history of myocardial dysfunction. Acute Management of Specific Arrhythmias
    60. 60. Repetitive Monomorphic VT Intravenous amiodarone, beta blockers, and intravenous procainamide (or sotalol or ajmaline in Europe) can be useful for treating repetitive monomorphic VT in the context of coronary disease and idiopathic VT. Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    61. 61. Polymorphic VT Direct-current cardioversion with appropriate sedation as necessary is recommended for patients with sustained polymorphic VT with hemodynamic compromise and is reasonable at any point in the treatment cascade. Intravenous beta blockers are useful for patients with recurrent polymorphic VT, especially if ischemia is suspected or cannot be excluded. Intravenous loading with amiodarone is useful for patients with recurrent polymorphic VT in the absence of abnormal repolarization related to congenital or acquired LQTS. Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    62. 62. Polymorphic VT Urgent angiography with a view to revascularization should be considered for patients with polymorphic VT when myocardial ischemia cannot be excluded. Intravenous lidocaine may be reasonable for treatment of polymorphic VT specifically associated with acute myocardial ischemia or infarction. Acute Management of Specific Arrhythmias
    63. 63. Torsades de Pointes Withdrawal of any offending drugs and correction of electrolyte abnormalities are recommended in patients presenting with torsades de pointes. Acute and long-term pacing is recommended for patients presenting with torsades de pointes due to heart block and symptomatic bradycardia. Acute Management of Specific Arrhythmias
    64. 64. Torsades de Pointes Management with intravenous magnesium sulfate is reasonable for patients who present with LQTS and few episodes of torsades de pointes. Magnesium is not likely to be effective in patients with a normal QT interval. Acute and long-term pacing is reasonable for patients who present with recurrent pause-dependent torsades de pointes. Beta blockade combined with pacing is reasonable acute therapy for patients who present with torsades de pointes and sinus bradycardia. Isoproterenol is reasonable as temporary treatment in acute patients who present with recurrent pause-dependent torsades de pointes who do not have congenital LQTS. Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    65. 65. Torsades de Pointes Potassium repletion to 4.5 to 5 mM/L may be considered for patients who present with torsades de pointes. Intravenous lidocaine or oral mexiletine may be considered in patients who present with LQT3 and torsades de pointes. Acute Management of Specific Arrhythmias
    66. 66. Incessant VT Revascularization and beta blockade followed by intravenous antiarrythmic drugs such as procainamide or amiodarone are recommended for patients with recurrent or incessant polymorphic VT due to acute MI. Intravenous amiodarone or procainamide followed by VT ablation can be effective in the management of patients with frequently recurring or incessant monomorphic VT. Acute Management of Specific Arrhythmias I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    67. 67. Incessant VT Intravenous amiodarone and intravenous beta blockers separately or together may be reasonable in patients with VT storm. Overdrive pacing or general anesthesia may be considered for patients with frequently recurring or incessant VT. Spinal cord modulation may be considered for some patients with frequently recurring or incessant VT. Acute Management of Specific Arrhythmias
    68. 68. LV Dysfunction Due to Prior MI Aggressive attempts should be made to treat HF that may be present in some patients with LV dysfunction due to prior MI and VTs. Aggressive attempts should be made to treat myocardial ischemia that may be present in some patients with VTs. Coronary revascularization is indicated to reduce the risk of SCD in patients with VF when direct, clear evidence of acute MI is documented to immediately precede the onset of VF. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    69. 69. LV Dysfunction Due to Prior MI If coronary revascularization cannot be carried out and there is evidence of prior MI and significant LV dysfunction, the primary therapy of patients resuscitated from VF should be the ICD in patients who are receiving chronic optimal medical therapy and those who have reasonable expectation of survival with a good functional status for more than 1 year. ICD therapy is recommended for primary prevention to reduce total mortality by a reduction in SCD in patients with LV dysfunction due to prior MI who are at least 40 days post-MI, have an LVEF less than or equal to 30% to 40%, are NYHA functional class II or III, are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III
    70. 70. LV Dysfunction Due to Prior MI The ICD is effective therapy to reduce mortality by a reduction in SCD in patients with LV dysfunction due to prior MI who present with hemodynamically unstable sustained VT, are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD Related to Specific Pathology
    71. 71. LV Dysfunction Due to Prior MI Implantation of an ICD is reasonable in patients with LV dysfunction due to prior MI who are at least 40 days post-MI, have an LVEF of less than or equal to 30% to 35%, are NYHA functional class I on chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. Amiodarone, often in combination with beta blockers, can be useful for patients with LV dysfunction due to prior MI and symptoms due to VT unresponsive to beta-adrenergic– blocking agents. Sotalol is reasonable therapy to reduce symptoms resulting from VT for patients with LV dysfunction due to prior MI unresponsive to beta-blocking agents. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    72. 72. LV Dysfunction Due to Prior MI Adjunctive therapies to the ICD, including catheter ablation or surgical resection, and pharmacological therapy with agents such as amiodarone or sotalol are reasonable to improve symptoms due to frequent episodes of sustained VT or VF in patients with LV dysfunction due to prior MI. Amiodarone is reasonable therapy to reduce symptoms due to recurrent hemodynamically stable VT for patients with LV dysfunction due to prior MI who cannot or refuse to have an ICD implanted. Implantation is reasonable for treatment of recurrent VT in patients post-MI with normal or near normal ventricular function who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    73. 73. LV Dysfunction Due to Prior MI Curative catheter ablation or amiodarone may be considered in lieu of ICD therapy to improve symptoms in patients with LV dysfunction due to prior MI and recurrent hemodynamically stable VT whose LVEF is greater than 40%. Amiodarone may be reasonable therapy for patients with LV dysfunction due to prior MI with an ICD indication, as defined above, in patients who cannot or refuse to have an ICD implanted. VA & SCD Related to Specific Pathology
    74. 74. LV Dysfunction Due to Prior MI Prophylactic antiarrhythmic drug therapy is not indicated to reduce mortality in patients with asymptomatic nonsustained ventricular arrhythmias. Class IC antiarrhythmic drugs in patients with a past history of MI should not be used. A VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III
    75. 75. Valvular Heart Disease Patients with valvular heart disease and ventricular arrhythmias should be evaluated and treated following current recommendations for each disorder. The effectiveness of mitral valve repair or replacement to reduce the risk of SCD in patients with mitral valve prolapse, severe mitral regurgitation, and serious ventricular arrhythmias is not well established. VA & SCD Related to Specific Pathology
    76. 76. Congenital Heart Disease ICD implantation is indicated in patients with congenital heart disease who are survivors of cardiac arrest after evaluation to define the cause of the event and exclude any reversible causes. ICD implantation is indicated in patients who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. Patients with congenital heart disease and spontaneous sustained VT should undergo invasive hemodynamic and EP evaluation. Recommended therapy includes catheter ablation or surgical resection to eliminate VT. If that is not successful, ICD implantation is recommended. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    77. 77. Congenital Heart Disease Invasive hemodynamic and EP evaluation is reasonable in patients with congenital heart disease and unexplained syncope and impaired ventricular function. In the absence of a defined and reversible cause, ICD implantation is reasonable in patients who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. EP testing may be considered for patients with congenital heart disease and ventricular couplets or NSVT to determine the risk of a sustained ventricular arrhythmia. Prophylactic antiarrhythmic therapy is not indicated for asymptomatic patients with congenital heart disease and isolated PVCs. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    78. 78. Myocarditis, Rheumatic Disease, and Endocarditis Temporary pacemaker insertion is indicated in patients with symptomatic bradycardia and/or heart block during the acute phase of myocarditis. Acute aortic regurgitation associated with VT should be treated surgically unless otherwise contraindicated. Acute endocarditis complicated by aortic or annular abscess and AV block should be treated surgically unless otherwise contraindicated. VA & SCD Related to Specific Pathology
    79. 79. Myocarditis, Rheumatic Disease, and Endocarditis ICD implantation can be beneficial in patients with life- threatening ventricular arrhythmias who are not in the acute phase of myocarditis, as indicated in the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. Antiarrhythmic therapy can be useful in patients with symptomatic NSVT or sustained VT during the acute phase of myocarditis. ICD implantation is not indicated during the acute phase of myocarditis. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    80. 80. Infiltrative Cardiomyopathies In addition to managing the underlying infiltrative cardiomyopathy, life-threatening arrhythmias should be treated in the same manner that such arrhythmias are treated in patients with other cardiomyopathies, including the use of ICD and pacemakers in patients who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD Related to Specific Pathology
    81. 81. Endocrine Disorders and Diabetes The management of ventricular arrhythmias secondary to endocrine disorders should address the electrolyte (potassium, magnesium, and calcium) imbalance and the treatment of the underlying endocrinopathy. Persistent life-threatening ventricular arrhythmias that develop in patients with endocrine disorders should be treated in the same manner that such arrhythmias are treated in patients with other diseases, including use of ICD and pacemaker implantation as required in those who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. Patients with diabetes with ventricular arrhythmias should generally be treated in the same manner as patients without diabetes. VA & SCD Related to Specific Pathology
    82. 82. End-Stage Renal Failure The acute management of ventricular arrhythmias in end- stage renal failure should immediately address hemodynamic status and electrolyte (potassium, magnesium, and calcium) imbalance. Life-threatening ventricular arrhythmias, especially in patients awaiting renal transplantation, should be treated conventionally, including the use of ICD and pacemaker as required, in patients who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD Related to Specific Pathology
    83. 83. Obesity, Dieting, and Anorexia Life-threatening ventricular arrhythmias in patients with obesity, anorexia, or when dieting should be treated in the same manner that such arrhythmias are treated in patients with other diseases, including ICD and pacemaker implantation as required. Patients receiving ICD implantation should be receiving chronic optimal medical therapy and have reasonable expectation of survival with a good functional status for more than 1 year. Programmed weight reduction in obesity and carefully controlled re-feeding in anorexia can effectively reduce the risk of ventricular arrhythmias and SCD. Prolonged, unbalanced, very low calorie, semistarvation diets are not recommended; they may be harmful and provoke life-threatening ventricular arrhythmias. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    84. 84. Pericardial Diseases Ventricular arrhythmias that develop in patients with pericardial disease should be treated in the same manner that such arrhythmias are treated in patients with other diseases including ICD and pacemaker implantation as required. Patients receiving ICD implantation should be receiving chronic optimal medical therapy and have reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD Related to Specific Pathology
    85. 85. Pulmonary Arterial Hypertension Prophylactic antiarrhythmic therapy generally is not indicated for primary prevention of SCD in patients with pulmonary arterial hypertension (PAH) or other pulmonary conditions. VA & SCD Related to Specific Pathology
    86. 86. Transient Arrhythmias of Reversible Cause Myocardial revascularization should be performed, when appropriate, to reduce the risk of SCD in patients experiencing cardiac arrest due to VF or polymorphic VT in the setting of acute ischemia or MI. Unless electrolyte abnormalities are proved to be the cause, survivors of cardiac arrest due to VF or polymorphic VT in whom electrolyte abnormalities are discovered in general should be evaluated and treated in a manner similar to that of cardiac arrest without electrolyte abnormalities. VA & SCD Related to Specific Pathology
    87. 87. Transient Arrhythmias of Reversible Cause Patients who experience sustained monomorphic VT in the presence of antiarrhythmic drugs or electrolyte abnormalities should be evaluated and treated in a manner similar to that of patients with VT without electrolyte abnormalities or antiarrhythmic drugs present. Antiarrhythmic drugs or electrolyte abnormalities should not be assumed to be the sole cause of sustained monomorphic VT. Patients who experience polymorphic VT in association with prolonged QT interval due to antiarrhythmic medications or other drugs should be advised to avoid exposure to all agents associated with QT prolongation. A list of such drugs can be found on the Web sites www.qtdrugs.org and www.torsades.org. VA & SCD Related to Specific Pathology I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    88. 88. Dilated Cardiomyopathy (Nonischemic) EP testing is useful to diagnose bundle-branch reentrant tachycardia and to guide ablation in patients with nonischemic DCM. EP testing is useful for diagnostic evaluation in patients with nonischemic DCM with sustained palpitations, wide-QRS-complex tachycardia, presyncope, or syncope. VA Associated With Cardiomyopathies
    89. 89. Dilated Cardiomyopathy (Nonischemic) An ICD should be implanted in patients with nonischemic DCM and significant LV dysfunction who have sustained VT or VF, are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. ICD therapy is recommended for primary prevention to reduce total mortality by a reduction in SCD in patients with nonischemic DCM who have an LVEF less than or equal to 30% to 35%, are NYHA functional class II or III, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. VA Associated With Cardiomyopathies I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    90. 90. Dilated Cardiomyopathy (Nonischemic) ICD implantation can be beneficial for patients with unexplained syncope, significant LV dysfunction, and nonischemic DCM who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. ICD implantation can be effective for termination of sustained VT in patients with normal or near normal ventricular function and nonischemic DCM who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. VA Associated With Cardiomyopathies I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    91. 91. Dilated Cardiomyopathy (Nonischemic) Amiodarone may be considered for sustained VT or VF in patients with nonischemic DCM. Placement of an ICD might be considered in patients who have nonischemic DCM, LVEF of less than or equal to 30% to 35%, who are NYHA functional class I receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. VA Associated With Cardiomyopathies
    92. 92. Hypertrophic Cardiomyopathy (HCM) ICD therapy should be used for treatment in patients with HCM who have sustained VT and/or VF and who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. ICD implantation can be effective for primary prophylaxis against SCD in patients with HCM who have 1 or more major risk factor for SCD and who are receiving chronic optimal medical therapy and in patients who have reasonable expectation of survival with a good functional status for more than 1 year. Amiodarone therapy can be effective for treatment in patients with HCM with a history of sustained VT and/or VF when an ICD is not feasible. VA Associated With Cardiomyopathies I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    93. 93. Hypertrophic Cardiomyopathy EP testing may be considered for risk assessment for SCD in patients with HCM. Amiodarone may be considered for primary prophylaxis against SCD in patients with HCM who have 1 or more major risk factor for SCD if ICD implantation is not feasible. VA Associated With Cardiomyopathies
    94. 94. Risk Factors for Sudden Cardiac Death in Hypertrophic Cardiomyopathy Major Risk Factors Possible in Individual Patients Cardiac arrest (VF) Spontaneous sustained VT Family history of premature sudden death Unexplained syncope LV thickness greater than or equal to 30 min Abnormal exercise BP Nonsustained spontaneous VT AF Myocardial ischemia LV outflow obstruction High-risk mutation Intense (competitive) physical exertion Modified with permission from Maron BJ et al. J Am Coll Cardiol 2003;42:1687-713. VA Associated With Cardiomyopathies
    95. 95. Arrhythmogenic Right Ventricular Cardiomyopathy ICD implantation is recommended for the prevention of SCD in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. ICD implantation can be effective for the prevention of SCD in patients with ARVC with extensive disease, including those with LV involvement, 1 or more affected family member with SCD, or undiagnosed syncope when VT or VF has not been excluded as the cause of syncope, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. VA Associated With Cardiomyopathies I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    96. 96. Arrhythmogenic Right Ventricular Cardiomyopathy Amiodarone or sotalol can be effective for treatment of sustained VT or VF in patients with ARVC when ICD implantation is not feasible. Ablation can be useful as adjunctive therapy in management of patients with ARVC with recurrent VT, despite optimal antiarrhythmic drug therapy. EP testing might be useful for risk assessment of SCD in patients with ARVC. VA Associated With Cardiomyopathies I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    97. 97. Arrhythmogenic RV Cardiomyopathy (RV conduction delay, inverted T-waves V1-V5)
    98. 98. Arrhythmogenic RV Cardiomyopathy 12-lead ECG showing Epsilon wave
    99. 99. Sustained Monomorphic VT 49-year-old man with ARVC
    100. 100. Neuromuscular Disorders Patients with neuromuscular disorders who have ventricular arrhythmias should generally be treated in the same manner as patients without neuromuscular disorders. Permanent pacemaker insertion may be considered for neuromuscular diseases such as myotonic muscular dystrophy, Kearns-Sayre syndrome, Erb dystrophy, and peroneal muscular atrophy with any degree of AV block (including first-degree AV block) with or without symptoms, because there may be unpredictable progression of AV conduction disease. VA Associated With Cardiomyopathies
    101. 101. VA Associated With Cardiomyopathies Frequency of Events in Neuromuscular Disorders Associated With Heart Disease Note: + to +++ represents a comparison between the various medical conditions and the relative frequency of an event. + ++ +++ -- Kearns-Sayre syndrome +++ + -- AR Friedich’s ataxia Other conditions ++ +++ +++ X-linked, AD, AR Emery-Dreifus MD and associated disorders + +++ +++ AD Myotonic MD +++ + + AR Limb-girdle 2C-2F ++ +++ +++ AD Limb-girdle 1B +++ + -- X-linked X-linked dilated CM +++ + + X-linked Becker +++ + + X-linked Duchenne Muscular dystrophies CM VA HB Inheritance
    102. 102. ICD therapy is recommended for secondary prevention of SCD in patients who survived VF or hemodynamically unstable VT, or VT with syncope and who have an LVEF less than or equal to 40%, who are receiving chronic optimal medical therapy, and who have a reasonable expectation of survival with a good functional status for more than 1 year. ICD therapy is recommended for primary prevention to reduce total mortality by a reduction in SCD in patients with LV dysfunction due to prior MI who are at least 40 days post- MI, have an LVEF less than or equal to 30% to 40%, are NYHA functional class II or III receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. Heart Failure
    103. 103. ICD therapy is recommended for primary prevention to reduce total mortality by a reduction in SCD in patients with nonischemic heart disease who have an LVEF less than or equal to 30% to 35%, are NYHA functional class II or III, are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 year. Amiodarone, sotalol, and/or other beta blockers are recommended pharmacological adjuncts to ICD therapy to suppress symptomatic ventricular tachyarrhythmias (both sustained and non-sustained) in otherwise optimally treated patients with HF. Amiodarone is indicated for the suppression of acute hemodynamically compromising ventricular or supraventricular tachyarrhythmias when cardioversion and/or correction of reversible causes have failed to terminate the arrhythmia or prevent its early recurrence. Heart Failure I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    104. 104. ICD therapy combined with biventricular pacing can be effective for primary prevention to reduce total mortality by a reduction in SCD in patients with NYHA functional class III or IV, are receiving optimal medical therapy, in sinus rhythm with a QRS complex of at least 120 ms, and who have reasonable expectation of survival with a good functional status for more than 1 year. ICD therapy is reasonable for primary prevention to reduce total mortality by a reduction in SCD in patients with LV dysfunction due to prior MI who are at least 40 days post-MI, have an LVEF of less than or equal to 30% to 35%, are NYHA functional class I, are receiving chronic optimal medical therapy, and have reasonable expectation of survival with a good functional status for more than 1 year. Heart Failure I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    105. 105. ICD therapy is reasonable in patients who have recurrent stable VT, a normal or near normal LVEF, and optimally treated HF and who have a reasonable expectation of survival with a good functional status for more than 1 year. Biventricular pacing in the absence of ICD therapy is reasonable for the prevention of SCD in patients with NYHA functional class III or IV HF, an LVEF less than or equal to 35%, and a QRS complex equal to or wider than 160 ms (or at least 120 ms in the presence of other evidence of ventricular dyssynchrony) who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. Heart Failure I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    106. 106. Amiodarone, sotalol, and/or beta blockers may be considered as pharmacological alternatives to ICD therapy to suppress symptomatic ventricular tachyarrhythmias (both sustained and nonsustained) in optimally treated patients with HF for whom ICD therapy is not feasible. ICD therapy may be considered for primary prevention to reduce total mortality by a reduction in SCD in patients with nonischemic heart disease who have an LVEF of less than or equal to 30% to 35%, are NYHA functional class I receiving chronic optimal medical therapy, and who have a reasonable expectation of survival with a good functional status for more than 1 year. Heart Failure
    107. 107. Long QT Syndrome Lifestyle modification is recommended for patients with an LQTS diagnosis (clinical and/or molecular). Beta blockers are recommended for patients with an LQTS clinical diagnosis (i.e., in the presence of prolonged QT interval). Implantation of an ICD along with use of beta blockers is recommended for LQTS patients with previous cardiac arrest and who have reasonable expectation of survival with a good functional status for more than 1 year. Genetic Arrhythmia Syndromes I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    108. 108. Long QT Syndrome Beta blockers can be effective to reduce SCD in patients with a molecular LQTS analysis and normal QT interval. Implantation of an ICD with continued use of beta blockers can be effective to reduce SCD in LQTS patients experiencing syncope and/or VT while receiving beta blockers and who have reasonable expectation of survival with a good functional status for more than 1 year. Left cardiac sympathetic neural denervation may be considered for LQTS patients with syncope, torsades de pointes, or cardiac arrest while receiving beta blockers. Implantation of an ICD with the use of beta blockers may be considered for prophylaxis of SCD for patients in categories possibly associated with higher risk of cardiac arrest such as LQT2 and LQT3 and who have reasonable expectation of survival with a good functional status for more than 1 year. Genetic Arrhythmia Syndromes I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    109. 109. Long QT Syndrome in a 16-year-old girl QT=520 ms; Atrial Tachycardia with 2:1 AV conduction
    110. 110. Torsades de Pointes Spontaneous conversion to NSR (continuous lead II monitor strip)
    111. 111. Genetic Arrhythmia Syndromes Long QT Syndrome Subtypes I Kr beta subunit 21p22.1-22-2 KCNE1 JLN2 I Ks alpha subunit 11p15.5 KCNQ1 JLN1 I Ca alpha subunit 12p13.3 CACNA1C LQT8 I K1 17p23.1-24.2 KCNJ2 LQT7 I Kr beta subunit 21p22.1-22-2 KCNE2 LQT6 I Ks beta subunit 21p22.1-22-2 KCNE1 LQT5 Targeting protein 4q25-2 ANK2 LQT4 I Na alpha subunit 3p21-23 SCN5A LQT3 I Kr alpha subunit 7q35-35 KCNH2 LQT2 I Ks alpha subunit 11p15.5 KCNQ1 LQT1 Function Chromosome Gene Variant
    112. 112. Genetic Arrhythmia Syndromes Genetic Variants of Short QT Syndrome References: Brugada et al. Circulation 2004;109:30–5; Bellocq et al. Circulation 2004;109:2394–7; Priori et al; Circ Res 2005;96:800–7. Bellocq et al. I Ks potassium channel alpha subunit (KvLQT1) KCNQ1 Autosomal dominant 11p15.5 SQTS2 Priori et al. I K1 potassium channel (Kir 2.1) KCNJ2 Autosomal dominant 17q23.1-q24.2 SQTS3 Brugada et al. I Kr potassium channel alpha subunit (HERG) KCNH2 Autosomal dominant 7q3p2135-q36 SQTS1 Reference Protein Gene Symbol Inheritance Chromosomal Locus Locus Name
    113. 113. Brugada Syndrome An ICD is indicated for Brugada syndrome patients with previous cardiac arrest receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. An ICD is reasonable for Brugada syndrome patients with spontaneous ST-segment elevation in V 1 , V 2 , or V 3 who have had syncope with or without mutations demonstrated in the SCN5A gene and who have reasonable expectation of survival with a good functional status for more than 1 year. Clinical monitoring for the development of a spontaneous ST-segment elevation pattern is reasonable for the management of patients with ST-segment elevation induced only with provocative pharmacological challenge with or without symptoms. Genetic Arrhythmia Syndromes I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    114. 114. Brugada Syndrome An ICD is reasonable for Brugada syndrome patients with documented VT that has not resulted in cardiac arrest and who have reasonable expectation of survival with a good functional status for more than 1 year. Isoproterenol can be useful to treat an electrical storm in the Brugada syndrome. EP testing may be considered for risk stratification in asymptomatic Brugada syndrome patients with spontaneous ST elevation with or without a mutation in the SCN5A gene. Quinidine might be reasonable for the treatment of electrical storm in patients with Brugada syndrome. Genetic Arrhythmia Syndromes I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    115. 115. Brugada Syndrome (Typical ST-T abnormality V1-V2)
    116. 116. Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Beta blockers are indicated for patients who are clinically diagnosed with CPVT on the basis of the presence of spontaneous or documented stress-induced ventricular arrhythmias. Implantation of an ICD with use of beta blockers is indicated for patients with CPVT who are survivors of cardiac arrest and who have reasonable expectation of survival with a good functional status for more than 1 year. Genetic Arrhythmia Syndromes
    117. 117. Catecholaminergic Polymorphic Ventricular Tachycardia Beta blockers can be effective in patients without clinical manifestations when the diagnosis of CPVT is established during childhood based on genetic analysis. Implantation of an ICD with the use of beta blockers can be effective for affected patients with CPVT with syncope and/or documented sustained VT while receiving beta blockers and who have reasonable expectation of survival with a good functional status for more than 1 year. Beta blockers may be considered for patients with CPVT who were genetically diagnosed in adulthood and never manifested clinical symptoms of tachyarrhythmias. Genetic Arrhythmia Syndromes I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    118. 118. Idiopathic Ventricular Tachycardia Catheter ablation is useful in patients with structurally normal hearts with symptomatic, drug-refractory VT arising from the RV or LV or in those who are drug intolerant or who do not desire long-term drug therapy. EP testing is reasonable for diagnostic evaluation in patients with structurally normal hearts with palpitations or suspected outflow tract VT. Arrhythmias in Structurally Normal Hearts I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    119. 119. Idiopathic Ventricular Tachycardia Drug therapy with beta blockers and/or calcium channel blockers (and/or IC agents in right ventricular outflow tract VT) can be useful in patients with structurally normal hearts with symptomatic VT arising from the RV. ICD implantation can be effective therapy for the termination of sustained VT in patients with normal or near normal ventricular function and no structural heart disease who are receiving chronic optimal medical therapy and who have reasonable expectation of survival for more than 1 year. Arrhythmias in Structurally Normal Hearts I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    120. 120. Electrolyte Disturbances Potassium (and magnesium) salts are useful in treating ventricular arrhythmias secondary to hypokalemia (or hypomagnesmia) resulting from diuretic use in patients with structurally normal hearts. It is reasonable to maintain serum potassium levels above 4.0 mM/L in any patient with documented life-threatening ventricular arrhythmias and a structurally normal heart. It is reasonable to maintain serum potassium levels above 4.0 mM/L in patients with acute MI. Magnesium salts can be beneficial in the management of VT secondary to digoxin toxicity in patients with structurally normal hearts. Arrhythmias in Structurally Normal Hearts I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    121. 121. Alcohol Complete abstinence from alcohol is recommended in cases where there is a suspected correlation between alcohol intake and ventricular arrhythmias. Persistent life-threatening ventricular arrhythmias despite abstinence from alcohol should be treated in the same manner that such arrhythmias are treated in patients with other diseases, including an ICD, as required, in patients receiving chronic optimal medical therapy and who have reasonable expectation of survival for more than 1 year. Arrhythmias in Structurally Normal Hearts
    122. 122. Smoking and Lipids Smoking should be strongly discouraged in all patients with suspected or documented ventricular arrhythmias and/or aborted SCD. Statin therapy is beneficial in patients with CHD to reduce the risk of vascular events, possibly ventricular arrhythmias, and SCD. n-3 polyunsaturated fatty acid supplementation may be considered for patients with ventricular arrhythmias and underlying CHD. Arrhythmias in Structurally Normal Hearts I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    123. 123. Athletes Preparticipation history and physical examination, including family history of premature or SCD and specific evidence of cardiovascular diseases such as cardiomyopathies and ion channel abnormalities, is recommended in athletes. Athletes presenting with rhythm disorders, structural heart disease, or other signs or symptoms suspicious for cardiovascular disorders should be evaluated as any other patient but with recognition of the potential uniqueness of their activity. Athletes presenting with syncope should be carefully evaluated to uncover underlying cardiovascular disease or rhythm disorder. VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    124. 124. Athletes Athletes with serious symptoms should cease competition while cardiovascular abnormalities are being fully evaluated. Twelve-lead ECG and possibly echocardiography may be considered as preparticipation screening for heart disorders in athletes. VA & SCD Related to Specific Populations
    125. 125. Gender and Pregnancy Pregnant women developing hemodynamically unstable VT or VF should be electrically cardioverted or defibrillated. In pregnant women with the LQTS who have had symptoms, it is beneficial to continue beta-blocker medications throughout pregnancy and afterward, unless there are definite contraindications. VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III
    126. 126. Elderly Patients Elderly patients with ventricular arrhythmias should generally be treated in the same manner as younger individuals. The dosing and titration schedule of antiarrhythmic drugs prescribed to elderly patients should be adjusted to the altered pharmacokinetics of such patients. Elderly patients with projected life expectancy less than 1 year due to major comorbidities should not receive ICD therapy. VA & SCD Related to Specific Populations
    127. 127. Pediatric Patients An ICD should be implanted in pediatric survivors of a cardiac arrest when a thorough search for a correctable cause is negative and the patients are receiving optimal medical therapy and have reasonable expectation of survival with a good functional status for more than 1 year. Hemodynamic and EP evaluation should be performed in the young patient with symptomatic, sustained VT. ICD therapy in conjunction with pharmacological therapy is indicated for high-risk pediatric patients with a genetic basis (ion channel defects or cardiomyopathy) for either SCD or sustained ventricular arrhythmias. The decision to implant an ICD in a child must consider the risk of SCD associated with the disease, the potential equivalent benefit of medical therapy, as well as risk of device malfunction, infection, or lead failure and that there is reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD Related to Specific Populations
    128. 128. Pediatric Patients ICD therapy is reasonable for pediatric patients with spontaneous sustained ventricular arrhythmias associated with impaired (LVEF of 35% or less) ventricular function who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. Ablation can be useful in pediatric patients with symptomatic outflow tract or septal VT that is drug resistant, when the patient is drug intolerant or wishes not to take drugs. VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    129. 129. Pediatric Patients Pharmacological treatment of isolated PVCs in pediatric patients is not recommended. Digoxin or verapamil should not be used for treatment of sustained tachycardia in infants when VT has not been excluded as a potential diagnosis. Ablation is not indicated in young patients with asymptomatic NSVT and normal ventricular function. VA & SCD Related to Specific Populations
    130. 130. Patients with ICDs Patients with implanted ICDs should receive regular follow- up and analysis of the device status. Implanted ICDs should be programmed to obtain optimal sensitivity and specificity. Measures should be undertaken to minimize the risk of inappropriate ICD therapies. Patients with implanted ICDs who present with incessant VT should be hospitalized for management. VA & SCD Related to Specific Populations
    131. 131. Patients with ICDs Catheter ablation can be useful for patients with implanted ICDs who experience incessant or frequently recurring VT. In patients experiencing inappropriate ICD therapy, EP evaluation can be useful for diagnostic and therapeutic purposes. VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    132. 132. Digitalis Toxicity An antidigitalis antibody is recommended for patients who present with sustained ventricular arrhythmias, advanced AV block, and/or asystole that are considered due to digitalis toxicity. Patients taking digitalis who present with mild cardiac toxicity (e.g., isolated ectopic beats only) can be managed effectively with recognition, continuous monitoring of cardiac rhythm, withdrawal of digitalis, restoration of normal electrolyte levels (including serum potassium greater than 4 mM/L), and oxygenation. Magnesium or pacing is reasonable for patients who take digitalis and present with severe toxicity (sustained ventricular arrhythmias, advanced AV block, and/or asystole). VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    133. 133. Digitalis Toxicity Dialysis for the management of hyperkalemia may be considered for patients who take digitalis and present with severe toxicity (sustained ventricular arrhythmias; advanced AV block, and/or asystole). Management by lidocaine or phenytoin is not recommended for patients taking digitalis and who present with severe toxicity (sustained ventricular arrhythmias, advanced AV block, and/or asystole). VA & SCD Related to Specific Populations
    134. 134. Drug Interactions Causing Arrhythmias Increased Concentration of Arrhythmogenic Drug (1 of 2) *These may also accumulate to toxic levels with co-administration of inhibitor drugs like ketoconazole. Data from Roden DM. Proarrhythmia. In: Kass RS, ed. Handbook of Experimental Pharmacology: vol. 171. Boston: Springer Verlag, 2006:288–304. Erythromycin* Clarithromycin Some calcium channel blockers* Some HIV protese inhibitors (especially ritanovir) Terfenadine, astemizole Itraconazole Cisapride Increased drug levels Ketoconazole Quinidine Digoxin toxicity Cyclosporine Itraconazole Erythromycin Increased digoxin bioavailability, reduced biliary and renal excretion due to P-glycoprotein inhibition Amiodarone Quinidine Verapamil Digoxin Eliminating gut flora that metabolize digoxin Some antibiotics Digoxin Effect Interacting Drug Drug
    135. 135. Increased Concentration of Arrhythmogenic Drug (2 of 2) Drug Interactions Causing Arrhythmias Data from Roden DM. Proarrhythmia. In: Kass RS, ed. Handbook of Experimental Pharmacology: vol. 171. Boston: Springer Verlag, 2006:288–304. Increased plasma dofetilde concentration Verapamil Cimetidine Trimethoprim Ketoconazole Megestrol Dofetilide Increased adverse effects Some tricyclic antidepressants Flecainide Increased beta blockade Increased beta blockade Quinidine (even ultra-low dose) Fluoxetine Beta blockers propafenone Effect Interacting Drug Drug
    136. 136. Decreased Concentration of Antiarrhythmic Drug Drug Interactions Causing Arrhythmias Data from Roden DM. Proarrhythmia. In: Kass RS, ed. Handbook of Experimental Pharmacology: vol. 171. Boston: Springer Verlag, 2006:288–304. Induced drug metabolism Rifampin, barbiturates Quinidine, mexiletine Increased P-glycoprotein activity Rifampin Decreased digoxin effect due to decreased absorbtion Antacids Digoxin Effect Interacting Drug Drug
    137. 137. Synergistic Pharmalogical Activity Causing Arrhythmias Drug Interactions Causing Arrhythmias Increased and persistent vasodilation; risk of myocardial ischemia Nitrates Sildenafil Amiodarone, beta blockers, digoxin, dilitiazem, verapamil Clonidine Beta blockers, clonidine, digoxin, dilitiazem, verapamil Amiodarone Amiodarone, beta blockers, clonidine, digoxin, verapamil Diltiazem Amiodarone, beta blockers, clonidine, digoxin, dilitiazem Verapamil Amiodarone, beta blockers, clonidine, dilitiazem, verapamil Digoxin Bradycardia when used in combination Amiodarone, clonidine, digoxin, dilitiazem, verapamil Beta blockers Increased T de P risk due to diuretic-induced hypokalemia Diuretics QT-prolonging antiarrhythmics Effect Interacting Drug Drug
    138. 138. Examples of Drugs Causing Torsades de Pointes * (e.g., hospitalization for monitoring recommended during drug initiation in some circumstances) Adapted with permission from Roden DM. N Engl J Med 2004;350:1013-22. VA & SCD Related to Specific Populations <ul><li>Ajmaline </li></ul><ul><li>Sotalol </li></ul><ul><li>Quinidine </li></ul><ul><li>Procainamide </li></ul><ul><li>Ibutilide </li></ul><ul><li>Dofetilide </li></ul><ul><li>Disopyramide </li></ul>Frequent (greater than 1%)* <ul><li>Opioid dependence agents: methadone </li></ul><ul><li>Antipsychotics: chlorpromazine, haloperidol, mesoridazine, thioridazine, pimozide </li></ul><ul><li>Antiemetics: domperidone, droperidol </li></ul><ul><li>Anti-infectives: clarithromycin, erythromycin, halofantrine; pentamidine, sparfloxacin </li></ul><ul><li>Cisapride </li></ul><ul><li>Bepridil </li></ul><ul><li>Arsenic trioxide </li></ul><ul><li>Amiodarone </li></ul>Less Frequent
    139. 139. Adapted with permission from Roden DM. N Engl J Med 2004;350:1013-22. VA & SCD Related to Specific Populations Risk Factors for Drug-Induced Torsades de Pointes <ul><li>Combination of QT-prolonging drug with its metabolic inhibitor </li></ul><ul><li>Rapid rate of intravenous drug administration </li></ul><ul><li>Concomitant use of 2 or more drugs that prolong the QT interval </li></ul><ul><li>High drug concentrations (exception: quinidine) , often due to drug interactions </li></ul><ul><li>Severe hypomagnesemia </li></ul><ul><li>Digitalis therapy </li></ul><ul><li>Certain DNA polymorphisms </li></ul><ul><li>Congestive heart failure </li></ul><ul><li>Congenital long QT syndrome </li></ul><ul><li>Recent conversion from atrial fibrillation </li></ul><ul><li>Left ventricular hypertrophy </li></ul><ul><li>Bradycardia </li></ul><ul><li>Ventricular arrhythmia </li></ul><ul><li>Hypokalemia </li></ul><ul><li>Baseline QT prolongation </li></ul><ul><li>Female gender </li></ul>
    140. 140. Drug-Induced Long QT Syndrome In patients with drug-induced LQTS, removal of the offending agent is indicated. Management with intravenous magnesium sulfate is reasonable for patients who take QT-prolonging drugs and present with few episodes of torsades de pointes in which the QT remains long. Atrial or ventricular pacing or isoproterenol is reasonable for patients taking QT-prolonging drugs who present with recurrent torsades de pointes. Potassium ion repletion to 4.5 to 5 mM/L may be reasonable for patients who take QT-prolonging drugs and present with few episodes of torsades de pointes in whom the QT remains long. VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III B
    141. 141. Sodium Channel Blocker-Related Toxicity In patients with sodium channel blocker–related toxicity, removal of the offending agent is indicated. Stopping the drug, reprogramming the pacemaker or repositioning leads can be useful in patients taking sodium channel blockers who present with elevated defibrillation thresholds or pacing requirement. In patients taking sodium channel blockers who present with atrial flutter with 1:1 AV conduction, withdrawal of the offending agent is reasonable. If the drug needs to be continued, additional A-V nodal blockade with diltiazem, verapamil, or beta blocker or atrial flutter ablation can be effective. Administration of a beta blocker and a sodium bolus may be considered for patients taking sodium channel blockers if the tachycardia becomes more frequent or more difficult to cardiovert. VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIa IIb IIb IIb III III III C
    142. 142. *Always includes recognition, continuous monitoring of cardiac rhythm, withdrawal of offending agents, restoration of normal electrolytes (including potassium to greater than 4 mEq/L0, and oxygenation. The order shown is not meant to represent the preferred sequence when more than one treatment is listed. VA & SCD Related to Specific Populations Syndromes of Drug-Induced Arrhythmia and Their Management Ventricular pacing Isoproterenol Recurrent torsades de pointes IV magnesium sulfate (MgSO 4 ) Replete potassium (K+) to 4.5 to 5 mEq/L Torsades de pointes; few episodes, QT remains long QT-prolonging drugs Anti-digitalis antibody Pacing Dialysis for hyperkalemia Severe toxicity; sustained ventricular arrhythmias; advanced AV block; asystole Mild cardiac toxicity (isolated arrhythmias only) Digitalis Management* Clinical Setting Drugs
    143. 143. *Always includes recognition, continuous monitoring of cardiac rhythm, withdrawal of offending agents, restoration of normal electrolytes (including potassium to greater than 4 mEq/L0, and oxygenation. The order shown is not meant to represent the preferred sequence when more than one treatment is listed. VA & SCD Related to Specific Populations Syndromes of Drug-Induced Arrhythmia and Their Management Stop drug; treat arrhythmia Brugada syndrome Beta blocker; sodium Ventricular tachycardia (more frequent; difficult to cardiovert) Diltiazem, verapamil, beta blocker (IV) Atrial flutter with 1:1 AV conduction Stop drug; reposition leads Elevated defibrillation or pacing requirement Sodium channel blockers Management* Clinical Setting Drugs
    144. 144. Other Drug-Induced Toxicity High intermittent doses and cumulative doses exceeding the recommended levels should be avoided in patients receiving anthracyclines such as doxorubicin. All patients receiving 5-fluorouracil therapy should receive close supervision and immediate discontinuation of the infusion if symptoms or signs of myocardial ischemia occur. Further treatment with 5-fluorouracil must be avoided in these individuals. Patients with known cardiac disease should have a full cardiac assessment including echocardiography, which should be undertaken prior to use of anthracyclines such as doxorubicin, and regular long-term follow-up should be considered. VA & SCD Related to Specific Populations I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III I I I IIa IIa IIa IIb IIb IIb III III III IIa IIa IIb IIa IIb III IIb III B III

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