Circulation Journal           Official Journal of the Japanese Circulation Society                                        ...
490                                                                                                         JCS Joint Work...
JCS Guidelines for Risks and Prevention of Sudden Cardiac Death                                                           ...
492                                                                                                           JCS Joint Wo...
JCS Guidelines for Risks and Prevention of Sudden Cardiac Death                                                           ...
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JCS Guidelines for Risks and Prevention of Sudden Cardiac Death                                                           ...
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JCS Guidelines for Risks and Prevention of Sudden Cardiac Death                                                           ...
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JCS Guidelines for Risks and Prevention of Sudden Cardiac Death                                                           ...
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JCS Guidelines for Risks and Prevention of Sudden Cardiac Death                                                           ...
Guia riesgo y manejo de  muerte subuta
Guia riesgo y manejo de  muerte subuta
Guia riesgo y manejo de  muerte subuta
Guia riesgo y manejo de  muerte subuta
Guia riesgo y manejo de  muerte subuta
Guia riesgo y manejo de  muerte subuta
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Guia riesgo y manejo de muerte subuta

  1. 1. Circulation Journal Official Journal of the Japanese Circulation Society JCS  GUIDELINES http://www. j-circ.or.jp Guidelines for Risks and Prevention of Sudden Cardiac Death (JCS 2010) – Digest Version – JCS Joint Working Group Table of Contents Introduction to the Revised Guidelines················ 489 7. Dilated Cardiomyopathy············································ 495 I  Prediction of Sudden Death 8. Arrhythmogenic Rght Ventricular Cardiomyopathy···· 495 · and Examinations···················································· 490 9. Other Myocardial Disorders······································ 495 · 10. Brugada Syndrome··················································· 495 1. Clinical Findings························································ 490 11. Congenital Long QT Syndrome· ······························· 496 · 2. Electrocardiogram····················································· 490 12. Wolff-Parkinson-White Syndrome····························· 497 3. Heart Rate Variability················································ 490 · 13. Catecholamine-Induced Polymorphic Ventricular   4. Heart Rate Turbulence·············································· 490 Tachycardia······························································· 497 5. Baroreflex Sensitivity················································· 490 14. Other Arrhythmias····················································· 497 6. T-Wave Alternans· ···················································· 490 · 15. Valvular Heart Disease· ············································ 498 · 7. Late Potential···························································· 490 · 8. Cardiac Electrophysiological Study··························· 490 III  Prevention of Sudden Death in Children············ 498 9. Exercise Stress Test················································· 491 · 1. Sudden Infant Death Syndrome································ 498 10. Genetic Tests···························································· 491 2. Arrhythmias······························································· 499 II  Prevention of Sudden Death· ································· 491 3. Commotio Cordis· ····················································· 500 · 4. Congenital Heart Diseases······································· 501 · 1. Arrhythmias······························································· 491 5. Pediatric Hypertrophic Cardiomyopathy· ·················· 501 · 2. Cardiogenic Syncope················································ 492 6. Kawasaki Disease····················································· 502 3. Heart Failure······························································ 492 4. Ischemic Heart Diseases· ········································· 493 · References···································································· 502 5. Hypertrophic Cardiomyopathy··································· 494 6. Other Heart Diseases Associated With Cardiac   Hypertrophy······························································· 494 (Circ J  2012; 76: 489 – 507) Introduction to the Revised GuidelinesThe best way to prevent sudden death is predicting the occur- fatal tachycardia. Severe bradycardia and asystole, which alsorence of sudden death and providing appropriate preventive may cause sudden death, are described in the present guide-measures. Since many cases of sudden death are arrhythmic lines as needed in relation to pathological conditions and dis-death, the present guidelines describe disease conditions and eases known to lead to bradycardia and asystole. The presenttypical clinical findings that may cause arrhythmic death and guidelines is partly revised and reflect the newest findings tohow to prevent sudden deaths in patients with such predicted be included to the guidelines for the non-pharmacological andfindings. Since ventricular tachyarrhythmias (VTA) including pharmacological treatment of arrhythmia of which the Japa-ventricular fibrillation (VF) play an important role in the devel- nese Circulation Society (JCS) are currently revising.opment of arrhythmic death, and the benefits of implantable The present guidelines are written mainly for the use ofcardioverter defibrillator (ICD) in preventing sudden death due cardiologists since pathological conditions and diseases thatto tachycardia have been demonstrated, the present guidelines may cause arrhythmic death must be carefully assessed bymainly discuss the use of ICD in the treatment of potentially expert cardiologists, and since ICD therapy, the most impor- Released online January 12, 2012 Mailing address:  Scientific Committee of the Japanese Circulation Society, 8th Floor CUBE OIKE Bldg., 599 Bano-cho, Karasuma Aneyakoji, Nakagyo-ku, Kyoto 604-8172, Japan.   E-mail: meeting@j-circ.or.jp This English language document is a revised digest version of Guidelines for Risks and Prevention of Sudden Cardiac Death reported at the Japanese Circulation Society Joint Working Groups performed in 2009. (website: http://www.j-circ.or.jp/guideline/pdf/JCS2010aizawa. d.pdf) Joint Working Groups: The Japanese Circulation Society, The Japanese Coronary Association, The Japanese Association for Thoracic Surgery, Japanese Society of Pediatric Cardiology and Cardiac Surgery, Japanese Association of Cardiovascular Intervention and Therapeutics, The Japanese Society for Cardiovascular Surgery, Japanese College of Cardiology, The Japanese Society of Electrocardiology, The Japanese Heart Failure Society, Japanese Heart Rhythm Society ISSN-1346-9843   doi:  0.1253/circj.CJ-88-0022 1 All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: cj@j-circ.or.jp Circulation Journal  Vol.76,  February  2012
  2. 2. 490 JCS Joint Working Grouptant method to prevent such conditions, is performed by car- are also essential for resuscitated patients. We hope that thediologists. Since most arrhythmic deaths occur as a result of present guidelines will help emergency medicine specialistsan out-of-hospital cardiac event, it is needless to say that suc- and practitioners in the front lines who see patients with car-cess or failure of resuscitation is extremely important, and diovascular diseases.appropriate measures to prevent recurrence of cardiac events I  Prediction of Sudden Death and ExaminationsThere are laboratory examinations that are believed to be use-ful in identifying those patients at a high risk for sudden death, 3.  Heart Rate Variabilityespecially arrhythmic death. The majority of these examina-tions assess electrical activity of the heart and the activity of Class IIathe autonomic nervous system. Clinical symptoms of cardiac •  rediction of sudden death in patients after myocardial Pdysfunction and heart failure are also important findings to infarction (MI)8identify patients at a high risk for sudden death. Although Class IIbpatients at a risk for sudden death can be identified (predicted) •  rediction of sudden death in patients with cardiomyopa- Pwith these examinations, only a few of these examinations thy9,10have been demonstrated to be useful in terms of primary pre-vention of sudden death in clinical research. The current use-fulness of clinical findings and laboratory examinations in 4.  Heart Rate Turbulenceterms of predicting sudden death is listed by the class of rec-ommendation, although there is some controversy as to the Class IIbvalidity of methods used in the evaluation of predictive factors •  rediction of sudden death in patients after MI and patients Pfor arrhythmia and sudden death. with heart failure11Classification of RecommendationsClass I: Conditions for which there is general agreement 5.  Baroreflex Sensitivity that a given clinical finding/laboratory examina- tion is useful in predicting sudden death Class IClass IIa: Conditions for which there is some divergence of •  rediction of sudden death in patients with cardiac dys- P opinion, but weight of opinion is in favor of use- function after MI12 fulnessClass IIb: Conditions for which there is some divergence of opinion, but weight of opinion is against useful- 6.  T-Wave Alternans nessClass III: Conditions for which there is general agreement Class IIa that the clinical finding/laboratory examination is •  rediction of cardiac sudden death in patients after MI and P not useful in predicting sudden death in patients who have ischemic cardiomyopathy with car- diac dysfunction13 Class III clinical findings/laboratory examinations are not Class IIbdescribed in this document as a rule. •  rediction of cardiac sudden death in patients with (non- P ischemic) dilated cardiomyopathy (DCM) or heart fail- ure14,15 1.  Clinical FindingsClass I 7.  Late Potential •  decrease in ejection fraction (≤30 to 35%) in patients A with ischemic and non-ischemic heart failure1–3 Class IIb •  rediction of sudden death in patients after MI16 P 2.  Electrocardiogram 8.  Cardiac Electrophysiological StudyClass I •  valuation of ventricular arrhythmia with standard 12- E Class I lead electrocardiogram (ECG) •  atients after MI who have palpitation, near syncope or PClass IIb syncope suspected to be caused by VTA •  valuation of patients with heart failure, cardiac hypertro- E •  valuation of patients following catheter ablation for the E phy (hypertrophic cardiomyopathy [HCM], hypertension, treatment of ventricular tachycardia (VT) and aortic stenosis), and arrhythmogenic right ventricular •  atients with syncope of unknown cause who have cardiac P cardiomyopathy (ARVC) based on QT dispersion (QTD)4–6 dysfunction, organic heart disease, a family history of sud- •  valuation of patients with syncope associated with long E den death and/or abnormal ECG findings QT syndrome (LQTS) or HCM according to the magni- Class IIa tude of transmural dispersion of repolarization (TDR)7 •  atients after MI who have nonsustained ventricular P Circulation Journal  Vol.76,  February  2012
  3. 3. JCS Guidelines for Risks and Prevention of Sudden Cardiac Death 491 tachycardia (NSVT) and a left ventricular ejection fraction rhythmia (LVEF) of ≤40%17 Class IIb •  atients with syncopes that are suspected to be caused by P •  atients with ventricular arrhythmia who are unlikely to P bradycardia or tachyarrhythmia but not confirmed with have coronary diseases non-invasive examinations •  valuation of premature ventricular contraction (PVC) in E patients in middle-age or older 9.  Exercise Stress Test 10.  Genetic TestsClass I •  xercise induced VTA in patient suspected to have coro- E Class IIa nary disease •  ardiac ion channel gene mutations (LQTS mutations, es- C •  atients who have or are suspected to have exercise P pecially LQT1, LQT2 and LQT3 mutations) and site of induced ventricular arrhythmia mutation19,20 •  bnormal blood pressure reactions in patients with HCM18 A Class IIbClass IIa •  bnormalities of the genes of ryanodine receptor (RyR2) A •  valuation of the efficacy of drug treatment or catheter E or calsequestrin (CASQ) 2 in catecholamine-induced poly- ablation in patients with exercise induced ventricular ar- morphic ventricular tachycardia (CPVT)21 II  Prevention of Sudden DeathThis section lists major pathological conditions and diseases Class III measures are not described in this guideline.that are known to precede sudden death, and describes rela- For example, in this guideline, ICD therapy and antiarrhyth-tionship between these conditions and arrhythmic death. This mic drugs are listed as Class I measures, but this does notsection also describes typical signs/symptoms and laboratory mean that ICD therapy and antiarrhythmic drugs are similarlyfindings of the pathological conditions/diseases believed to effective in the prevention of sudden death. This means thatcause sudden death as possible predictive factors and lists there is evidence or consensus that Class I measures decreasemeasures to prevent the patient at a high risk for sudden car- the incidence of arrhythmic death as compared with controldiac death as either primary or secondary prevention of ar- groups. Limitation of exercise is a Class I measure to preventrhythmic death. exercise induced arrhythmia. Measures listed in the same class Types and indications of preventive measures are listed by do not necessarily target the same goal of treatment. Physi-the class of recommendation. cians should be aware of this to select appropriate measures for individual patients.Classification of RecommendationsClass I: Conditions for which there is general agreement that a given measure is indicated 1.  ArrhythmiasClass IIa: Conditions for which there is some divergence of opinion, but weight of opinion is in favor of the 1.  Cardiac Arrest (Resuscitated Cases) use of a given measure Sustained ventricular tachycardia (SVT) and VF are the mostClass IIb: Conditions for which there is some divergence of frequent causes of cardiac arrest. Cardiac arrest recurs fre- opinion, but weight of opinion is against the use quently and the risk of sudden death is extremely high. ICD of a given measure therapy is the most effective secondary preventive measure forClass III: Conditions for which there is general agreement sudden death.22–24 that the measure is not useful Table 1.  Prevention of Sudden Death in Patients With Sustained Ventricular Tachycardia or Ventricular Fibrillation Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  ardiac arrest (VF) C ICD Amiodarone* or sotalol* •  atients with SVT who have syncope during tachycardia, or P ICD Amiodarone* or sotalol* patients with LVEF 40% with a low blood pressure (80 mmHg) •  atients with hemodynamically stable SVT with underlying heart P ICD Amiodarone* or sotalol* disease in whom drugs are not effective or contraindicated •  atients with underlying heart disease and in whom SVT is no P ICD longer induced after catheter ablation Amiodarone* or sotalol* •  atients with SVT associated with underlying heart disease and P ICD + effective drugs a LVEF of ≥40% who are responding to drug treatment *Use to control VT/VF episodes in patients contraindicated for ICD therapy or patients already undergoing ICD therapy. ICD, implantable cardioverter defibrillator; LVEF, left ventricular ejection fraction; SVT, sustained ventricular tachy- cardia; VF, ventricular fibrillation; VT, ventricular tachycardia. Circulation Journal  Vol.76,  February  2012
  4. 4. 492 JCS Joint Working Group Table 2.  Prevention of Sudden Death in Patients With Cardiogenic Syncope (Syncope Other Than Those Caused by Non-Cardiac Causes) Findings Class I Class IIa Class IIb •  atients in whom unstable SVT or VF is induced, and drug effi- P ICD cacy cannot be assessed •  atients with underlying heart disease in whom hemodynamically P ICD stable SVT is induced and drug treatment and catheter ablation are ineffective •  atients with underlying heart disease and cardiac dysfunction in P ICD whom hemodynamically unstable SVT or VF is induced and drug efficacy has not been assessed •  atients with dilated or hypertrophic cardiomyopathy in whom P ICD hemodynamically unstable SVT or VF is not induced •  atients with a history of asystole associated with sick sinus P Pacemaker syndrome or atrioventricular block Note) efer to the Guidelines for Non-Pharmacological Therapy of Cardiac Arrhythmias22 reported by the Japanese R Circulation Society for the management of patients with bradycardia. The management of primary arrhythmia is described in a later section. ICD, implantable cardioverter defibrillator; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation.2.  Sustained Ventricular Tachycardia 1.  resence of cardiac disorders that may cause transient PIn Japan, the underlying disease of SVT is old MI in about 30%, asystoleand non-ischemic heart disease in the majority of patients.22,25 2.  VT or VF induced during electrophysiological study SSVT is treated mainly with procaineamide and lidocaine.26,27 (EPS)Amiodarone and nifekalant are recommended for patients 3.  uspected to have unstable SVT in a patient with a his- Swith refractory SVT.27 Patients with a history of high-rate, tory of stable SVThemodynamic deterioration of SVT require measures to pre- 4.  resence of cardiomyopathy without SVT or VF induced Pvent recurrence, and should be considered for the indication during EPSfor ICD therapy first. Since patients with hemodynamically 5.  resence of primary arrhythmic disorder Pstable SVT associated with organic heart disease may often 6.  resence of sinus node dysfunction or atrioventricular Pexperience high-rate episodes or new onset of VT with differ- blockent waveforms, their prognoses are not good and ICD therapy Treatment strategies to prevent arrhythmic deaths mainlyis thus recommended for them.28 Patients following successful consist of ICD therapy. See the sections of Brugada syndromecatheter ablation for the treatment of SVT are often indicated and LQTS for the prevention of arrhythmic death in patientsfor ICD therapy since their long-term prognosis is unclear and with these diseases.recurrent SVT is occasionally observed (Table 1).3. Premature Ventricular Contraction and 3.  Heart Failure Nonsustained Ventricular TachycardiaWhen no heart diseases are present, the prognosis of individu- The mortality of patients with heart failure is higher in patientsals with PVC and NSVT is good, and these arrhythmias do not with more severe disease by New York Heart Associationrepresent risk factors for sudden death. Whether primary pre- (NYHA) classification.36 In recent large-scale clinical studiesvention of sudden death is indicated for patients with PVC or of patients with heart failure, sudden cardiac death developedNSVT depends on the type of underlying diseases, which are in 9 to 22% of the patients.37–41 The percentage of suddendescribed in the following sections. death cases among all-cause death cases is higher in patients with milder heart failure (NYHA Class I or II) than in severer4.  Bradycardia patients (NYHA Class III or IV). The most likely causes ofBradycardia accounts for about 10% of all deaths due to sudden cardiac death among patients with heart failure arearrhythmia.29,30 Bradycardia gradually leading to asystole has SVT and VF.42been observed in patients with arrhythmic death.30 The most About half of patients with out-of-hospital cardiac arrestcommon causes of bradycardia leading to arrhythmic death are (resuscitated) or patients with SVT (in Japan) are consideredsick sinus syndrome and atrioventricular block, which require to have cardiac dysfunction.43–47 Patients with proven VF ortreatment with a pacemaker.31–33 The pacemaker treatment of SVT should undergo secondary preventive treatment mainlythese conditions is described in the Guidelines for Non-Phar- using an ICD.macological Therapy of Cardiac Arrhythmias reported by the In patients after MI, cardiac dysfunction is an independentJCS.22 predictive factor of poor prognosis. ICD therapy has been demonstrated to be useful as a primary preventive measure for sudden death in patients with cardiac dysfunction after MI.1 2.  Cardiogenic Syncope (Table 2) ICD therapy has also been demonstrated to be useful in the primary prevention of sudden death in patients with DCM.2,3Cardiogenic syncope is defined as syncope other than those due Although a number of studies have reported that amioda-to non-cardiac causes such as orthostatic syncope and syncopes rone decreases the incidence of sudden death, some studiesdue to vasovagal reflex and seizures.34,35 Cardiogenic syncope have denied such effect. β-blockers improve the prognosis anddue to arrhythmia may be identified with the following condi- decrease the incidence of sudden death in patients with chronictions: heart failure.41,48 Angiotensin converting enzyme (ACE) in­ Circulation Journal  Vol.76,  February  2012
  5. 5. JCS Guidelines for Risks and Prevention of Sudden Cardiac Death 493 Table 3.  Prevention of Sudden Death in Patients After Myocardial Infarction Clinical findings Class I Class IIa Class IIb Secondary prevention •  atients with a history of SVT, VF or resuscitated cardiac P ICD Amiodarone, sotalol, arrest catheter ablation Primary prevention •  atients with syncope (+), NSVT (+) and LVEF 40% in whom P ICD SVT or VF is induced and drug treatment is ineffective •  atients with syncope (–), NSVT (+) and LVEF 35% in whom P ICD SVT or VF is induced and drug treatment is ineffective or drug efficacy has not been assessed •  atients with cardiac dysfunction (LVEF 35%) P β-blockers, ACE ICD inhibitors, anti-  aldosterone drugs Note) rug treatment is generally believed to improve the prognosis, but no consensus has been achieved. The D significance of drug treatment is not identical to those of ICD and catheter ablation. ACE, angiotensin converting enzyme; ICD, implantable cardioverter defibrillator; LVEF, left ventricular ejection frac- tion; NSVT, nonsustained ventricular tachycardia; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation. Table 4.  Prevention of Sudden Death in Patients With Variant Angina Purpose of treatment/findings Class I Class IIa Class IIb Primary/secondary prevention •  resence of SVT or VF during anginal attack P Calcium channel blockers Nitrates, nicorandil ICD ICD, implantable cardioverter defibrillator; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation.hibitors have also been demonstrated to decrease all-cause ods are sudden death as a result of out-of-hospital cardiacmortality and sudden deaths in this patient population.49 In arrest. Patients at a high risk of sudden death after MI arepatients with severe heart failure, aldosterone antagonists de- identified on the basis of cardiac function and the presence ofcreased the incidence of sudden death.50 Cardiac resynchroni- ventricular arrhythmia. Patients with post-MI VT/VF arezation therapy with defibrillator (CRT-D) is currently per- mainly treated with ICD therapy for secondary prevention offormed for patients with heart failure who are indicated for sudden death.ICD therapy.51 It has been demonstrated that ICD therapy as primary pre- vention of post-MI sudden death improves the prognosis of patients who have cardiac dysfunction (LVEF 40%), NSVT 4.  Ischemic Heart Diseases and SVT induced during EPS,46 and patients with severe car- diac dysfunction (LVEF 30%) (Table 3).1–3Known risk factors for sudden death in patients with coronary Amiodarone is used to prevent arrhythmic death in patientsheart disease include elderly, male sex, a history of syncope after MI,39,40 but some studies have denied the preventive ef-and a family history of sudden cardiac death. However, treat- fects in this patient population.3 β-blockers and spironolactonement to prevent sudden death is not initiated because of the are effective in decreasing sudden death in post-MI patientspresence of such risk factors only. with heart failure. Catheter ablation may be effective in con- trolling monomorphic VT, when VT is no longer induced by1.  Acute Myocardial Infarction programmed electrical stimulation. However, since manyAcute myocardial infarction (AMI) is the most common acute post-MI patients have polymorphic SVT or cannot undergocondition leading to fatal arrhythmia. VF often develops in electrophysiological mapping, patients often have to receivepatients with AMI, especially those in a very early phase of other treatment methods in addition to catheter ablation.the disease. Secondary VF may develop in association with In Europe and the United States, supplementation of poly-cardiogenic shock and pump failure. unsaturated fatty acids is recommended to prevent sudden VF in patients with AMI may be effectively controlled with death.56 Since the number of stenotic lesions, the results ofECG monitoring and electrical defibrillation. Defibrillation by reperfusion therapy during AMI, and the patency of the arteryemergency medical service and treatment with an automated responsible for MI after treatment affect the incidence ofexternal defibrillator (AED) are expected effective for patients arrhythmic accidents, it is important to improve myocardialwith out-of-hospital VF. The Guidelines for Management of ischemia including asymptomatic myocardial ischemia as muchAcute Coronary Syndrome without Persistent ST Segment as possible.Elevation reported by the JCS describe how to treat VT/VFand atrioventricular block to prevent sudden death in patients 3.  Variant Anginawith AMI.52 Patients with variant angina may experience angina attacks leading to fatal ventricular arrhythmia.57,58 Patients with vari-2.  Post-Myocardial Infarction ant angina often die from sudden death, which is believed toThe one-year mortality of patients after MI was as high as be associated with multi-vessel coronary spasm. The progno-10 to 20%, and especially 6-month mortality after onset is sis of patients with variant angina associated with VT is poor.the highest.53–55 Most of the death cases during these peri- Patients with a history of coronary spastic attacks should be Circulation Journal  Vol.76,  February  2012
  6. 6. 494 JCS Joint Working Group Table 5.  Prevention of Sudden Death in Patients With Hypertrophic Cardiomyopathy Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  atients with cardiac arrest, SVT or VF P ICD  Amiodarone Limitation of exercise Primary prevention •  atients with more than one risk factor* P Limitation of exercise ICD, amiodarone •  atients with one risk factor* P Limitation of exercise Amiodarone ICD *Recurrent syncopal attacks, a family history of sudden death, presence of severe left ventricular wall thickening (≥30 mm) or an insufficient increase in blood pressure during exercise (≤20 mmHg). Note) requent (≥5 times/day) or multiple repetitive runs of NSVT (at least 10 beats). F ICD, implantable cardioverter defibrillator; NSVT, nonsustained ventricular tachycardia; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation. Table 6.  Prevention of Sudden Death in Patients With Dilated Cardiomyopathy Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  atients with SVT or VF P ICD  Aldosterone   Amiodarone ACE inhibitors/  antagonists β-blockers Primary prevention •  atients with syncope (+), LVEF ≤40% in whom SVT or P ICD Amiodarone VF is induced, and drug treatment is ineffective •  atients with syncope (–), LVEF ≤40% in whom SVT or P ICD,   Amiodarone VF is induced, and drug treatment is ineffective aldosterone   antagonists •  atients with syncope (+), LVEF ≤40% in whom SVT or P ICD,   VF is induced, and drug efficacy has not been assessed amiodarone •  atients with LVEF ≤36% and NSVT or frequent PVC P ICD (≥10 times/hr)* •  atients with LVEF ≤30% and NSVT P Amiodarone *According to the DEFINITE study. ACE, angiotensin converting enzyme; DEFINITE, DEFibrillators In Non-Ischemic Cardiomyopathy Treatment Evalua- tion; ICD, implantable cardioverter defibrillator; LVEF, left ventricular ejection fraction; NSVT, nonsustained ventricular tachycardia; PVC, premature ventricular contraction; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation.treated with calcium channel blockers to prevent recurrence of (5)  decrease or insufficient increase (≤20 mmHg) in sys- Aattacks. ICD therapy may be considered for patients who still tolic blood pressure during exercise stress test18,59,70have coronary spastic attacks after treatment with sufficient The indication for primary prevention of sudden death shoulddoses of calcium channel blockers and have severe ventricular be determined according to the presence of above risk factorsarrhythmia, but it is unclear whether ICD therapy may improve (Table 5). In the Guidelines for Diagnosis and Treatment ofthe vital prognosis of these patients (Table 4). Patients with Hypertrophic Cardiomyopathy reported by the JCS,71 primary prevention is indicated according to whether VT/VF is induced during EPS. 5.  Hypertrophic CardiomyopathyThe annual mortality of patients with HCM has been reported 6.  Other Heart Diseases Associatedas 1 to 2%.59–63 Although HCM does not necessarily represent With Cardiac Hypertrophya poor prognosis, more than half of death cases among patientswith HCM are sudden death. HCM is an important cause of Left ventricular hypertrophy is observed in patients with hyper-sudden death especially in young patients. Patients resusci- tension, those with aortic stenosis and those with athlete’s heart.tated from cardiac arrest and patients with SVT are at high risk The incidences of cardiovascular accidents such as suddenfor sudden death, and are indicated for ICD therapy as second- death, arrhythmia, heart failure, MI and stroke are higher inary prevention. individuals with left ventricular hypertrophy than those without Risk factors for sudden death in patients with HCM it.72–74 It is unclear that left ventricular hypertrophy per seinclude: increases the risk for sudden death or not, while the incidence (1)  ecurrent syncopal attacks (especially syncopes occur- R of sudden death is considered to be high among patients with ring in children during exercise)61,63,64 left ventricular hypertrophy complicated with arrhythmia,75–78 (2)  first-degree family history of sudden death or multiple A coronary artery disease79,80 or heart failure.81,82 family history of sudden death61,64,65 Secondary prevention of sudden death with ICD therapy is (3)  omplication with NSVT66–68 C indicated for patients with left ventricular hypertrophy associ- (4)  ignificant left ventricular wall thickening (maximum S ated with SVT or VF, but the results of primary prevention thickness ≥30 mm)69 have not been reported. Circulation Journal  Vol.76,  February  2012
  7. 7. JCS Guidelines for Risks and Prevention of Sudden Cardiac Death 495 Table 7.  Prevention of Sudden Death in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  atients with cardiac arrest or VF P ICD Primary prevention •  atients in whom SVT is induced, having a family history of P ICD Amiodarone, sotalol sudden death or LP-positive patients with right heart failure ICD, implantable cardioverter defibrillator; LP, late potential on the signal averaged body surface electrocardiogram; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation. Table 8.  Prevention of Sudden Death in Patients With Brugada Syndrome Purpose of treatment Class I Class IIa Class IIb Secondary prevention •  atients in whom cardiac arrest or VF/TdP is confirmed P ICD Primary prevention •  atients who meet at least 2 criteria of (1) a history of syncope (2) a family P ICD history of sudden death and (3) VF is induced during electrophysiological study •  atients who meet one of the above 3 criteria P ICD ICD, implantable cardioverter defibrillator; TdP, torsades de pointes; VF, ventricular fibrillation. tients with extensive abnormal right ventricular wall motion, 7.  Dilated Cardiomyopathy those with VT induced during EPS, and those affecting the right and left ventricles.95,96 Monomorphic VT is commonlyMajor causes of death of patients with DCM are heart failure induced and catheter ablation is often successful, but long-(about 50%) and sudden death (30 to 40%).83,84 Many cases of term outcome of catheter ablation remains unknown. ICDsudden death are caused by VT or VF, but severe bradycardia therapy is the first-choice secondary preventive method. Dur-may lead to sudden death.38 Patients with a history of SVT or ing a 3-year follow-up of patients undergoing ICD therapy,VF are indicated for secondary prevention using an ICD. ICD was activated in about half of the patients.Some patients with monomorphic SVT may be successfully In the primary prevention, the indication for ICD therapytreated with catheter ablation. should be determined according to the presence/absence of A multivariate analysis of the prognosis of patients with SVT induced during EPS with lesions of larger size and a fam-DCM has revealed that a history of SVT or VF and LVEF are ily history of sudden death (Table 7).risk factors for sudden death in this patient population.85 Theincidence of arrhythmic accidents is high among patientswith NSVT and a LVEF of 30%.86 In the analysis of DCM 9.  Other Myocardial Disorderspatients implanted with an ICD, the incidence of arrhythmiais high among those with low LVEF (≤30%). It has been dem- SVT and VF may develop as a complication of cardiac sar-onstrated that ICD therapy is effective as primary prevention coidosis, muscular dystrophy, chronic lung disease, progres-of sudden death in patients with a LVEF of ≤36% associated sive systemic sclerosis or diabetes mellitus, and so on.97 It iswith either NSVT or frequent PVC.2 difficult to predict occurrence of sudden death, SVT or VF in All-cause mortality and incidence of sudden death are high these patients. When the presence of these conditions is con-among patients with DCM complicated with left bundle firmed or strongly suspected, the use of an ICD should bebranch block. Currently, the types and indications of primary considered.prevention of sudden death among patients with DCM aredetermined according to the symptoms, cardiac function, pres-ence/absence of SVT and VF induced during EPS, and so on 10.  Brugada Syndrome(Table 6).22 Brugada syndrome is a disorder characterized by right bundle branch block pattern with ST segment elevation in V1 to V3 8.  Arrhythmogenic Right Ventricular leads on ECG, and may cause sudden death mainly due to VF Cardiomyopathy at night.98,99 Brugada syndrome is considered to be consistent with sudden unexpected nocturnal death syndrome in South-ARVC is a disease with unknown cause that is characterized east Asia and “Pokkuri disease” in Japan. History of VF orwith fatty infiltration in the right ventricle (often extending to syncope represents a significant risk factor for sudden death inthe left ventricle) and VT originated from the right ventri- patient with Brugada syndrome, and ICD therapy is indicatedcle.87–89 Patients with ARVC start to show right heart failure for such patients. It has been reported that quinidine is effec-in their 40 to 50 s. In Europe and the United States, ARVC tive in preventing arrhythmic attacks and activations of theshould be suspected in cases of sudden death or cardiac arrest ICD in patients with Brugada syndrome.100–103in patients under 35 years old.69,90–93 In Japan, ARVC is the The need of primary prevention including ICD therapy isunderlying disease of SVT in about 10% of patients.22,94 usually determined in each institution on the basis of ECG The incidence of arrhythmic accidents is high among pa- findings and presence/absence of VF induced during EPS, and Circulation Journal  Vol.76,  February  2012
  8. 8. 496 JCS Joint Working Group Table 9.  Prevention of Sudden Death in Patients With Congenital Long QT Syndrome Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  atients with VF or cardiac arrest P ICD Primary prevention •  atients meeting ≥2 of the 3 criteria of (1) a history of TdP or syncope, P ICD (2) a family history of sudden death, (3) not responding to β-blockers •  atients responding to β-blockers with a history of TdP or syncope or P β-blockers ICD a family history of sudden death ICD, implantable cardioverter defibrillator; TdP, torsades de pointes; VF, ventricular fibrillation. Table 10.  Indications of Drug Treatment in Patients With Congenital Long QT Syndrome Purpose of treatment/findings Class I Class IIa Class IIb •  atients with a history of syncope (especially those with LQT1 or LQT2 P β-blockers mutation) •  symptomatic patients with QT prolongation, congenital deafness, being A β-blockers neonates/infants, having a family history of sudden death of siblings, and anxiety for sudden death or strong desire for treatment by patients or their family •  symptomatic patients without congenital deafness, or a family history of A Mexiletine β-blockers sudden death of siblings, and who either have LQT3 mutation with a history of syncope or have LQTS not responding to monotherapy with β- blockers Note) xercise and drugs prolonging QT interval (antiarrhythmic drugs, tricyclic antidepressants and antihistamines) E are contraindicated in all patients. LQTS, long QT syndrome. Table 11.  Prevention of Sudden Death in Patients With WPW Syndrome Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  atients with VF, cardiac arrest or syncope P Catheter   ablation Primary prevention •  atients with a R-R interval during atrial fibrillation of ≤250 msec P Catheter   Amiodarone, Class ablation Ia and Ic drugs •  atients with a refractory period of antegrade conduction over the P Catheter   accessory pathway of ≤270 msec, patients with multiple accessory ablation pathways, patients with a family history of sudden death or athletes VF, ventricular fibrillation; WPW, Wolff-Parkinson-White.so on (Table 8).104 thereafter, the incidence of cardiac accidents is higher in female than male patients. Cardiac accidents during childhood are more common in boys than girls with LQT1 mutation, but 11.  Congenital Long QT Syndrome there are no sex differences among patients with LQT2 and LQT3 mutations.109 The incidence of cardiac event (eg, syn-The congenital LQTS is a group of genetic arrhythmic disor- cope, cardiac arrest or sudden death) by the age of 40 yearsders that cause syncope and sudden death due to ventricular was 63%, 46% and 18% in patients with LQT1 (n=112),arrhythmia characterized by QT prolongation and torsades LQT2 (n=72) and LQT3 (n=62) mutations, respectively, andde pointes (TdP), and include Romano-Ward syndrome and the mortality was 4% in patients with LQT1 and LQT2 muta-Jervell and Lange-Nielsen syndrome.105–108 Since cardiac ar- tions and as high as 20% in patients with LQT3 mutation.109rest is the first manifestation of the congenital LQTS in 10% Congenital LQTS patients with a history of cardiac arrest areof patients, it is quite important to predict and prevent cardiac indicated for ICD therapy combined with β-blockers and lim-arrest. To date, 12 different genetic mutations (referred to as itation of exercise (Tables 9,10).LQT1 through LQT12) have been reported to be related to The risk of occurrence of TdP is high among patients withcongenital LQTS. The prevalence is highest for LQT1, which LQT2 mutation and a QTc of 500 msec and male patientsis followed by LQT2 and LQT3. Patients with LQT1, LQT2 with LQT3 mutation.109 Patients with recurrent syncopesand LQT3 account for most of the patients with congenital despite treatment with β-blockers,112 and patients with a familyLQTS.109,110 history of sudden death are at a high risk for cardiac events.113 In male patients, arrhythmic accidents often occur before TdP often develops during exercise, and especially swimming,adolescence, and the incidence of arrhythmic accidents is among patients with LQT1 mutation; in association with men-higher in male than female patients.111 During adolescence and tal stress, sudden auditory stimuli or immediately after child- Circulation Journal  Vol.76,  February  2012
  9. 9. JCS Guidelines for Risks and Prevention of Sudden Cardiac Death 497 Table 12.  Prevention of Sudden Death in Patients With Catecholamine-Induced Polymorphic Ventricular Tachycardia Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  atients with VF or cardiac arrest P ICD + β-blockers + Calcium channel blockers + Flecainide Primary prevention •  yncope S ICD, left stellectomy •  ale patients with RyR2 mutation or patients M β-blockers ICD with CASQ2 mutation + Calcium channel blockers + Flecainide •  hildren with a family history of sudden death, C β-blockers NSVT or syncope Note) xercise should be limited in all patients. E CASQ2, calsequestrin 2 gene; ICD, implantable cardioverter defibrillator; NSVT, nonsustained ventricular tachycar- dia; RyR2, ryanodine receptor; VF, ventricular fibrillation.birth among those with LQT2 mutation; and during sleep is no sex difference in incidence. In patients with CPVT, exer-among those with LQT3 mutation.110,114 In patients with LQT2 cise and intravenous isoproterenol induce a gradual increasemutation, the risk of occurrence of TdP is high among patients in PVC, which further lead to polymorphic or bidirectionalwith mutations in the pore region of the human-ether-related VT, very fast polymorphic VT (350 to 400/min), and finally togene (HERG gene).20,115 Primary prevention consists of limita- VF. Recently, genetic mutations of RyR221,128–136 and CASQ2tion of exercise and treatment with β-blockers. Mexiletine is genes137,138 have been found in patients with CPVT.effective for patients with LQT3 mutation, and pacing is used Secondary prevention is indicated for patients in whom VFfor patients with bradycardia. or polymorphic VT was observed. The use of an ICD as pri- mary prevention is indicated for patients with a family history of sudden death or a history of syncope. Pharmacological pre- 12.  Wolff-Parkinson-White Syndrome ventive treatment mainly consists of β-blockers. When treat- ment with β-blockers is insufficient, calcium channel blockersIt has been reported that symptomatic Wolff-Parkinson-White such as verapamil may be effective in some cases.139,140 In(WPW) syndrome is observed in 1 to 2/1,000 individuals, the addition to these drugs, strict limitation of exercise is impor-incidence of sudden death is 0.02 to 0.15%/year, and the inci- tant. Male patients with RyR2 mutation and patients withdence of VF is about 3 to 4 fold of the incidence of sudden CASQ2 gene mutation need early implantation of an ICDdeath.116 Even in patients with asymptomatic WPW syndrome, (Table 12).132VF may develop in rare cases during the first episode of atrialfibrillation. WPW syndrome may usually be completely treatedwith catheter ablation,22,117 and the risk of sudden death will 14.  Other Arrhythmiasdisappear after treatment (Table 11). The incidence of VF tends to be higher in young male 1.  Non-Brugada Type Idiopathic Ventricularpatients.118,119 The risk of VF is high among patients with a Fibrillationhistory of atrial fibrillation or reciprocating tachycardia. It has Some cases of idiopathic VF do not represent ECG findingsbeen reported that about half of patients experience VF during characteristic to Brugada syndrome.141,142 It is important to dif-the first episode of atrial fibrillation.118 It is believed that VF ferentiate non-Brugada type idiopathic VF from Brugada syn-develops in 20 to 40% of patients with multiple Kent bun- drome of which characteristic ECG findings may vary and bedles.119 The presence of Ebstein’s anomaly is considered a risk normalized over time. Cases of polymorphic VT initiated infactor for sudden death in patients with WPW syndrome. the Purkinje’s fiber have been recently reported, and are suc- On the other hand, the risk of sudden death is considered cessfully treated with catheter ablation.143 There is a conditionlow in patients with intermittent WPW syndrome and patients called variant Brugada syndrome characterized by idiopathicin whom delta waves disappear after intravenous administra- VF characterized with Brugada syndrome-like ST segmenttion of ajmaline or procainamide.120,121 Patients at a high risk elevation in leads II, III and aVF.of transition from atrial fibrillation to VF may be identified onthe basis of the refractory period of antegrade conduction over 2.  Polymorphic Ventricular Tachycardia Triggered bythe accessory pathway or the shortest R-R interval during Short-Coupled Ventricular Premature Contractionatrial fibrillation induced during EPS.118,122,123 This condition develops as very short-coupled PVC (≤250 msec in many cases), which leads polymorphic VT to VF.144 13.  Catecholamine-Induced Polymorphic 3. Polymorphic Ventricular Tachycardia Resulting Ventricular Tachycardia From Ventricular Parasystole There are cases of polymorphic VT originating from a ven-The first episodes of CPVT often manifest during childhood tricular parasystolic rhythm.145 If it is transient, polymorphicafter infancy as exercise induced polymorphic VT or VF.124–127 VT terminates without causing further changes, while if itCPVT is not associated with organic heart diseases, and there persists, it may lead to VF. Circulation Journal  Vol.76,  February  2012
  10. 10. 498 JCS Joint Working Group Table 13.  Prevention of Sudden Death in Patients With Aortic Stenosis Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  VT or VF S ICD Surgical repair of stenotic valves Primary prevention •  atients with ventricular arrhythmia in whom SVT P Surgical repair of stenotic valves Amiodarone or VF is induced •  atients with severe valvular stenosis P Surgical repair of stenotic valves ICD, implantable cardioverter defibrillator; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation. Table 14.  Prevention of Sudden Death in Patients With Mitral Valve Prolapse Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  VT, VF or cardiac arrest S ICD ICD, implantable cardioverter defibrillator; SVT, sustained ventricular tachycardia; VF, ventricular fibrillation.4.  Ventricular Tachycardia/Fibrillation Associated With 2.  Mitral Valve Prolapse Short QT Intervals Mitral valve prolapse had attracted interest as a cause of suddenA few cases of VT/VF associated with subnormal short QT death since it was the single and only abnormal finding onintervals on ECG have been reported.146 autopsy in patients died from sudden death of unknown The use of an ICD is indicated for secondary prevention of cause.154,155 However, it was then found that sudden death issudden death in these patients, and catheter ablation may cure extremely rare among patients with mitral valve prolapse with-this condition in some cases. It is difficult to predict high risk out mitral regurgitation.156,157 The incidence of sudden death inpatients indicated for primary prevention. patients with mitral regurgitation (1.8%/year) is estimated 50 to 100-fold higher than those without mitral regurgitation. The risk for sudden death among patients with mitral valve prolapsed 15.  Valvular Heart Disease cannot be predicted with the presence/absence of arrhythmia.158 Also, QTD and the presence/absence of ventricular arrhythmia1.  Aortic Stenosis induced during EPS are not considered helpful in predictingAortic stenosis is the most common valvular heart disease caus- sudden death. Although the presence/absence of mitral regurgi-ing sudden death. It has been reported that sudden death occurs tation significantly affects the incidence of sudden death, thein 15 to 20% of adult patients with aortic stenosis (mean age: significance of echocardiography in risk assessment has not60 years) and 44 of 70 patients died from sudden death during been investigated in detail. Patients with SVT or VF are indi-survey.147,148 The most common causes of sudden death in pa- cated for secondary prevention using an ICD (Table 14).tients with aortic stenosis are considered VF and SVT. Patientswith SVT and VF (after cardiopulmonary resuscitation) should 3.  Patients After Prosthetic Valve Replacementundergo secondary prevention mainly with ICD therapy. It has been reported that the incidence of sudden death during Although aortic pressure gradient is an effective measure of the late phase after valve replacement with the St. Jude Medi-severity of valvular stenosis, it is not useful in predicting sudden cal prosthesis ranged 0.5 to 2.4%,159,160 while the incidence ofdeath. Arrhythmia findings of Holter ECG correlate with the sudden death after replacement with bioprosthetic valves wasinterventricular septal wall thickness, left ventricular mass, and as low as 0.2 to 1%.161 It is believed that sudden death inthe decrement of LVEF.149–152 Patients with a QTD of ≥70 msec patients using mechanical valves is often related to heart fail-are at high risks of developing syncopes and cardiac arrest.5,153 ure, MI or fatal arrhythmia.161 Secondary prevention is essen- Patients with hemodynamically significant valvular stenosis tial for patients with VT/VF, but prediction and primary pre-are indicated for surgery (Table 13). vention of sudden death among patients after valve replacement have not been established yet. III  Prevention of Sudden Death in ChildrenThe most common causes of sudden cardiac death in childrenin Japan are cardiomyopathy, congenital heart diseases and 1.  Sudden Infant Death Syndromearrhythmias.162,163 HCM is the most common cardiomyopathyleading to sudden death in children, and arrhythmias leading Several factors and causes have been pointed out for suddento sudden death include VF, complete atrioventricular block, death in infants. Although there is an opinion that suddenLQTS and WPW syndrome. Major pathological conditions death with known causes should not be included in suddenand diseases are described in the following subsections. infant death syndrome (SIDS), this section includes sudden death with known causes to ensure appropriate management. Circulation Journal  Vol.76,  February  2012
  11. 11. JCS Guidelines for Risks and Prevention of Sudden Cardiac Death 499 Table 15.  Prevention of Sudden Infant Death Syndrome in Infants With Long QT Syndrome Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention • nfants resuscitated from SIDS with a QTc of ≥440 msec I ICD β-blockers Primary prevention • nfants whose elder siblings died from SIDS with a QTc I ICD of ≥440 msec β-blockers • nfants without a family history of SIDS with a QTc of I β-blockers ≥440 msec ICD, implantable cardioverter defibrillator; SIDS, sudden infant death syndrome. Table 16.  Prevention of Sudden Infant Death Syndrome in Infants With Impulse Conduction Disorders Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  esuscitated infants R Pacemaker Primary prevention • nfants with familial impulse conduction disorders I Pacemaker Table 17.  Prevention of Sudden Infant Death Syndrome in Infants With Abnormal Fatty Acid Metabolism Purpose of treatment/findings Class I Class IIa Class IIb Primary prevention Common preventive methods: Frequent high carbo low fat meals Limit long-chain fatty acids and take middle-chain fatty acids •  arnitine transporter deficiency C •  PT-II deficiency C •  A translocase deficiency C •  CAD deficiency L +L-carnitine •  LCAD deficiency V +L-carnitine •  CHAD/trifunctional protein deficiency L +L-carnitine CA translocase, carnitine-acylcarnitine translocase; CPT-II, carnitine palmitoyltransferase type II; LCAD, long-chain acyl-coenzyme A dehydrogenase; LCHAD, long-chain 3-hydroxyacyl-CoA dehydrogenase; VLCAD, very-long-chain acyl-CoA dehydrogenase.1.  Long QT Syndrome tion, atrioventricular block, left bundle branch block and otherIn an investigation of a relationship between SIDS during types of arrhythmia responsible for SIDS.170 Appropriate dietaryneonatal period and the QT prolongation, infants with a QTc therapy and drug treatment are recommended for children with≥440 msec were at a high risk of sudden death (Table 15).164 abnormal fatty acid metabolism (Table 17).2.  SCN5A Gene MutationSCN5A gene mutation was reported in about 2% of infants 2.  Arrhythmiasdied from SIDS.165–167 Since it is difficult to predict suddendeath, patients with SCN5A gene mutation are indicated for 1.  WPW Syndromesecondary prevention mainly using an ICD. However, it is Although there are electrophysiological findings suggesting adifficult to implant an ICD to children with small body size. high risk of sudden death (VF), it has been pointed out sudden death in children cannot be predictable with EPS.119,123,171 Cura-3.  Impulse Conduction Disorders tive treatment using catheter ablation is indicated for patientsFasciculoventricular tracts were significantly frequently with WPW syndrome to prevent sudden death (see Table 11).observed during autopsy of infants died from SIDS.168,169 It isdifficult to predict such findings. Children with a history of 2.  Ventricular Tachycardia and Catecholamine-Inducedcardiac arrest due to atrioventricular block are indicated for Polymorphic Ventricular Tachycardiapacemaker. Primary prevention is recommended for children Sudden death due to VT is observed even in children under 5with familial impulse conduction disorders (Table 16). years.172 ICD therapy is indicated for children with VT/CPVT, but implantation is not feasible due to small body size and lead4.  Abnormal Fatty Acid Metabolism troubles associated with bodily movement and development. ItIt has been reported that disorders of mitochondrial fatty acid has been reported that catheter ablation is effective. The treat-β-oxidation cause accumulation of long-chain acylcarnitine, ment are similar to those recommended for adult patients.124,132,139which may lead to VT, atrial tachycardia, sinus node dysfunc- See the section of CPVT in adults. Circulation Journal  Vol.76,  February  2012
  12. 12. 500 JCS Joint Working Group Table 18.  Prevention of Sudden Death in Children With Long QT Syndrome Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  atients with syncope not responding P ICD to drug treatment +β-blockers Mexiletine*1 Mg*2 •  ymptomatic patients S Pacemaker +β-blockers Mexiletine*1 Mg*2 Primary prevention •  symptomatic patients A Drug treatment same as above Appropriate guid- ance on exercise *1LQT2 or LQT3 mutation with severe atrioventricular block is often observed in neonates and infants with long QT syndrome. The good efficacy of mexiletine in this patient population has been described in many reports. *2Use for torsades de pointes. ICD, implantable cardioverter defibrillator; Mg, magnesium. Table 19.  Prevention of Sudden Death Related to Commotio Cordis Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  esuscitated patients R Prohibit from exercise which may cause chest wall impact Use chest protectors Use softer balls Primary prevention Use chest protectors Use softer balls Table 20.  Prevention of Sudden Cardiac Death in Children With Hypertrophic Cardiomyopathy Purpose of treatment/findings Class I Class IIa Class IIb Secondary prevention •  hildren resuscitated from near-miss sudden death C ICD with a history of syncope or symptomatic VT or a Drug treatment† first-degree family history of sudden death Primary prevention •  ymptomatic children or high-risk children* S ICD*, β-blockers, calcium channel block- ers‡, disopyramide (or cibenzoline) *See the section of adult HCM. †Digitalis, ACE inhibitors and nitrates are contraindicated for patients without heart failure (special care should be taken for infants) or dilated phase HCM. ‡Calcium channel blockers are not recom- mended for patients with severe left ventricular outflow obstruction. ACE, angiotensin converting enzyme; HCM, hypertrophic cardiomyopathy; ICD, implantable cardioverter defibrillator; VT, ventricular tachycardia.3.  Long QT Syndrome Holter ECG). Since it is well known that children with LQT1LQTS is one cause of sudden cardiac death due to arrhythmia mutation often experience drowning or near-drowning acci-in children. ECG check-ups in schools are common in Japan, dents during swimming, swimming is commonly prohibitedand the prevalence of asymptomatic QT prolongation is esti- or limited in these children. It is important to provide appro-mated as 1 in 1,200 children.173 Symptoms are believed to priate guidance on exercise (Table 18). See the section ofdevelop in 1 in 10 children with asymptomatic LQTS. SIDS for the relationship between LQTS and SIDS. Onset of LQTS during neonatal period and infancy is prev- The Research Committee on Management of Children withalent among patients with LQT2 or LQT3 mutations, and LQTS of Japanese Society of Pediatric Cardiology and Car-mexiletine has been reported to be effective in the treatment diac Surgery is conducting a prospective study of childrenof LQTS.174,175 Poor compliance with drug treatment is a risk with asymptomatic LQTS, and is expected to propose guide-factor of arrhythmic accidents.175 lines for primary prevention for such children.182 Patients who still have arrhythmias during treatment withantiarrhythmic drugs such as β-blockers and mexiletine, apacemaker or an ICD is implanted.176–181 There are no reports 3.  Commotio Cordisof large-scale studies in children with LQTS. Treatment strategies for LQTS are determined according to Commotio cordis is a noticeable condition defined as suddenthe results of genetic tests, family histories of sudden death death triggered by relatively mild mechanical impact to theand syncope and ECG findings (eg, exercise stress ECG and chest observed among athletes.183,184 The cause of death is Circulation Journal  Vol.76,  February  2012
  13. 13. JCS Guidelines for Risks and Prevention of Sudden Cardiac Death 501 Table 21.  Classification of Severity of Cardiovascular Lesions in Kawasaki Disease Severity classification Findings (echocardiography and selective coronary angiography) I  coronary dilatation No Patients with no coronary dilatation including those in the acute phase II  Transient coronary dilatation Patients with slight and transient coronary dilatation which typically subsides during the acute phase within 30 days after onset III  Regression Patients who exhibit coronary aneurysms on day 30 after onset, but disappear- ance in the b]ilateral coronary artery systems within one year after onset, and do not meet the criteria for Group V IV  Residual coronary aneurysms Patients with unilateral or bilateral coronary aneurysms detected by coronary angiography in the second year or later and do not meet the criteria for Group V V  Coronary stenotic lesions Patients with coronary stenotic lesions detected by coronary angiography (a)  o ischemic findings: patients without ischemic signs/symptoms detectable N by laboratory tests or other examinations (b)  ith ischemic findings: patients with ischemic signs/symptoms detectable W by laboratory tests or other examinations Note) ther clinical symptoms of findings: When patients have moderate or severe valvular heart disease, heart failure, O severe arrhythmia, or other cardiac disease, such conditions should be described in addition to the severity of Kawasaki disease. Adapted from Guidelines for Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease (JCS 2008).199 Table 22.  Indications of Treatment by Classification of Severity of Coronary Artery Lesions in Kawasaki Disease Treatment Class I Class II Class III Antiplatelet drugs*1 IV, V III I, II Anticoagulant drugs*2 IV, V III I, II Coronary vasodilators*3 V IV I, II, III Drugs for heart failure*4 V IV I, II, III Coronary intervention*5 V(b) V(a) I, II, III, IV Coronary bypass surgery V(b) V(a) I, II, III, IV *1Aspirin, dipyridamole, and ticlopidine, *2Warfarin, heparin, *3Such as calcium cannel blockers and nitrates, *4ACE inhibitors, angiotensin receptor blockers and β-blockers, *5Intracoronary thrombolysis (urokinase, tissue plasminogen activators), coronary balloon angioplasty, stenting, and coronary angioplasty using rotablators. ACE, angiotensin converting enzyme. Adapted from Guidelines for Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease (JCS 2008).199believed to be arrhythmias triggered by blunt trauma to thechest wall. Commotio cordis often develops in players of 5.  Pediatric Hypertrophic Cardiomyopathybaseball, soft ball and ice hockey, but has also been reportedduring football, soccer, rugby, lacrosse, boxing and karate as HCM is the most important cause of sudden death in chil-well as when someone deliver a knee kick to the chest or hit dren.91,190–194 Children with HCM with a history of resuscitatedthe chest with a hand or fist.184,185 from near-miss sudden death, syncope or symptomatic VT or Prompt cardiopulmonary resuscitation should be initiated. a first-degree family history of sudden death are indicated forChest protecters are used to prevent commotion cordis, but the ICD therapy according to the criteria in each institution orbenefit is limited since it has been reported that 28% of indi- American College of Cardiology/American Heart Associa-viduals died from commotio cordis wore chest protecters.185 tion/North American Society of Pacing and ElectrophysiologyFurther safety measures such as using softer balls should be (ACC/AHA/NASPE) recommendations.117considered (Table 19). It is unclear that whether septal myectomy, pacemaker implantation and percutaneous transluminal septal myocardial ablation (PTSMA) used for adult patients with HCM are effec- 4.  Congenital Heart Diseases tive secondary preventive methods for children or not.190,195 Drug treatment is recommended as both primary and second-Sudden death after surgery for congenital heart diseases has ary prevention. β-blockers, calcium channel blockers, diso-been observed.186,187 The incidence of sudden death after sur- pyramide (or cibenzoline) are used (Table 20).71,190 It has beengery is relatively high in patients with tetralogy of Fallot and reported that highdose β-blocker treatment (5 to 23 mg/kg/day)patients with complete transposition of great arteries.187–189 is highly effective,160 but some studies have denied suchSecondary prevention is indicated for patients who experi- effects.71enced syncope, symptomatic VT or resuscitated from near- Pediatric HCM is characterized by the higher prevalence ofmiss sudden death. Since criteria for ICD therapy in children heart failure and poorer prognosis among children in whomwith congenital heart diseases are not available, guidelines for HCM developed during infancy than those with later onset.196,197adult patients should be referred.22 Physicians should refer to the School Activity Management Table published by the Japanese Society of School Health for level of exercise limitation and lifestyle guidance.71 Circulation Journal  Vol.76,  February  2012

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