Guidelines for Non-Pharmacotherapy of Cardiac Arrhythmias ...€¦ · macotherapy of Cardiac Arrhythmias was published,1 a sub-stantial number of patients underwent catheter ablation

Circulation Journal Vol.77, January 2013

Circulation JournalOfficial Journal of the Japanese Circulation Societyhttp://www.j-circ.or.jp

In 2001 when the first edition of the Guidelines for Non-Phar-macotherapy of Cardiac Arrhythmias was published,1 a sub-stantial number of patients underwent catheter ablation for the treatment of a wide variety of heart diseases including Wolff-Parkinson-White (WPW) syndrome and supraventricular tachy-cardia, and the guidelines clearly described the indications of catheter ablation. However, catheter ablation for the treatment of complex arrhythmias such as atrial fibrillation (AF), atypi-

cal atrial flutter, and ventricular tachycardia associated with structural heart diseases was indicated for only selected patients. Three-dimensional (3D) mapping systems that have been devel-oped since the 1990 s have contributed to the clarification of the mechanisms of these complex arrhythmias and the improve-ment of the outcome of ablation procedures. After the approval of the CARTO system in 2000 and the EnSite system in 2006, catheter ablation therapy became available for many patients

Introduction of the Revised Guidelines

Released online November 17, 2012Mailing 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: [email protected] English language document is a revised digest version of Guidelines for Non-Pharmacotherapy of Cardiac Arrhythmias reported at the

Japanese Circulation Society Joint Working Groups performed in 2010 (website: http://www.j-circ.or.jp/guideline/pdf/JCS2011_okumura_d.pdf).

Joint Working Groups: The Japanese Circulation Society, The Japanese Association for Thoracic Surgery, The Japanese Society for Arti-ficial Organs, The Japanese Society for Cardiovascular Surgery, The Japanese College of Cardiology, The Japanese Society of Electro-cardiology, The Japanese Heart Failure Society, The Japanese Heart Rhythm Society

ISSN-1346-9843 doi: 10.1253/circj.CJ-66-0054All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: [email protected]

Guidelines for Non-Pharmacotherapy of Cardiac Arrhythmias (JCS 2011)

– Digest Version –JCS Joint Working Group

Table of Contents Introduction of the Revised Guidelines ··············· 249I Background and Concept of the Guidelines ··· 250 1. Current Status of Non-Pharmacotherapy of

Cardiac Arrhythmias ················································· 250 2. Contents of the Guidelines ······································· 251 3. Evidence and Class of Recommendations ·············· 252 4. Matters to Be Considered in Determining the

Indications for Non-Pharmacotherapy ······················ 253 5. Requirements for Physicians Providing Non-

Pharmacological Procedures of Arrhythmias ··········· 253 6. Informed Consent ····················································· 254II Clinical Cardiac Electrophysiological Study ··· 255 1. Bradyarrhythmia ······················································· 255 2. Tachyarrhythmia ······················································· 255III Cardiac Pacing ······················································· 256 1. Atrioventricular Block ················································ 256 2. Bifascicular or Trifascicular Block ····························· 257 3. Sick Sinus Syndrome ··············································· 257 4. Bradycardic Atrial Fibrillation ···································· 257 5. Hypersensitive Carotid Sinus Syndrome and

Reflex Syncope ························································ 257 6. Hypertrophic Obstructive Cardiomyopathy ·············· 257 7. Pacing in Children ···················································· 258IV Catheter Ablation ·················································· 258 1. Wolff-Parkinson-White Syndrome and

Atrioventricular Nodal Reentrant Tachycardia ········· 258 2. Atrial Fibrillation ························································ 259

3. Atrial Flutter and Atrial Tachycardia ························· 260 4. Atrioventricular Junction Ablation for

Supraventricular Tachyarrhythmia ··························· 260 5. Premature Ventricular Contraction ··························· 260 6. Ventricular Tachycardia ············································ 261 7. Special Considerations for Children ························· 261V Implantable Cardioverter-Defibrillator Therapy···· 262 1. Implantable Cardioverter-Defibrillator Therapy

for Secondary Prevention ········································· 262 2. Primary Prevention in Patients With Structural

Heart Disease ··························································· 262 3. Syncope of Unknown Etiology ·································· 263 4. Implantable Cardioverter-Defibrillator Therapy in

Specific Cardiac Diseases ········································ 263 5. Implantable Cardioverter-Defibrillators in Children ···· 264VI Cardiac Resynchronization Therapy and

Cardiac Resynchronization Therapy Device That Incorporates Both Pacing and Defibrillation Capabilities ··························· 265

VII Surgery ··································································· 266 1. Atrial Fibrillation ························································ 266 2. Other Supraventricular Tachycardia ························· 266 3. Ventricular Tachycardia ············································ 267References ···································································· 267

(Circ J 2013; 77: 249 – 274)

JCS GUIDELINES


250 JCS Joint Working Group

with arrhythmias not responding to the conventional treatment. Since catheter ablation became a standard procedure in the treatment of arrhythmia, the guidelines were revised in 20062 to expand the indications for catheter ablation for supraven-tricular tachycardia as well as complex arrhythmias including AF. After the revision in 2006, catheter ablation became more common especially in the treatment of paroxysmal AF in which pulmonary vein isolation became the standard catheter abla-tion procedure. In the “Guidelines for Pharmacotherapy of Atrial Fibrillation (JCS 2008)3” and the “Guidelines for Drug Treat-ment of Arrhythmias (JCS 2009)4”, catheter ablation is recom-mended as second-line therapy for drug-resistant AF. In these guidelines, catheter ablation is also recommended as sec-ond-line therapy for other types of arrhythmias not respond to pharmacotherapy and even as first-line therapy for selected patients. The performance of 3D mapping systems was improved as the CARTO XP and CARTO 3, the second and third gen-erations of the CARTO system were approved in 2008 and 2011, respectively, and EnSite NavX, a new feature of EnSite, was approved in 2009. Irrigation tip catheters, that had been unavailable in Japan despite the availability in other countries, were introduced in Japan in 2009. With the advancement of these techniques to support catheter ablation, there is the need for wider and clearer indications of catheter ablation in the treatment of complex arrhythmias especially refractory ones such as AF and ventricular tachycardia.

When the first edition of the guidelines was prepared in 1999 to 2000, the use of implantable cardioverter-defibrillator (ICD) was quite limited in Japan, and almost no evidence was available. In the guidelines, ICD therapy was thus indicated mainly for secondary prevention of sudden cardiac death (SCD) according to the findings of large-scale clinical studies in Europe and the United States and consensus of specialists in Japan, as well as for primary prevention of SCD in patients with a high risk of fatal arrhythmias who have either nonsustained ven-tricular tachycardia or syncope of unknown etiology, show ventricular dysfunction, and have inducible ventricular tachy-cardia/fibrillation in electrophysiological studies (EPS) accord-ing to the results of the MADIT (Multicenter Automatic Defibrillator Implantation Trial)-I in 19965 and the MUSTT (Multicenter Unsustained Tachycardia Trial) in 1999.6 After the publication of the first guidelines in 2001, investigators in Europe and the United States published the results of clinical studies of ICD therapy for primary prevention of SCD such as the MADIT-II in 20027 and the SCD-HeFT (Sudden Cardiac Death in Heart Failure Trial) in 2005.8 The indications of ICD therapy for primary prevention of SCD were expanded accord-ingly. In Japan, indications of ICD therapy for secondary pre-vention of SCD were clearly described, and that for primary prevention of SCD were expanded to patients who have symp-

tomatic heart failure and ventricular dysfunction in the 2006 edition of the guidelines. With the technical advantages and miniaturization of the devices, more patients started to receive ICD therapy, and the guidelines were required to revise to reflect new evidence. In 2007, the Japanese Circulation Soci-ety (JCS) published the “Guidelines for Diagnosis and Man-agement of Patients with Long QT Syndrome and Brugada Syndrome (JCS 2007)9”, which described the indications of ICD therapy for the prevention of fatal arrhythmias in these patient populations. The present guidelines had to be revised to reflect these indications.

In parallel with the advancement of ICD therapy, biven-tricular pacing, which is referred to as cardiac resynchroniza-tion therapy (CRT) was developed and advanced as a treat-ment for patients with chronic heart failure associated with intraventricular conduction disturbance. A lot of clinical stud-ies have demonstrated that CRT is effective in alleviating symptoms and improving exercise capacity and life prognosis especially in patients who have wide QRS complexes of left bundle branch block pattern, chronic heart failure (New York Heart Association [NYHA] Class III or IV symptoms) and ven-tricular dysfunction. In the United States, the Food and Drug Administration (FDA) approved CRT in 2001, and CRT device that incorporates both pacing and defibrillation capabilities (CRT-D) in 2002 according to the results of the COMPANION (Comparison of Medical Therapy, Pacing, and Defibrillation in Heart Failure) study in which CRT-D was demonstrated to improve life prognosis of patients with chronic severe heart failure by preventing death due to heart failure and sudden death.10 In Japan, CRT and CRT-D were included in the National Health Insurance (NHI) price list in 2004 and 2006, respectively. Following the publication of the 2006 edition of the guidelines, investigators reported the benefits of CRT-D in patients with wide QRS complexes, ventricular dysfunction, and mild or moderate heart failure (NYHA Class II or III symp-toms), and the investigators of the RAFT (Resynchronization-Defibrillation for Ambulatory Heart Failure Trial) in 2010 demonstrated that CRT-D was more effective than ICD in improving life prognosis in patients with NYHA Class II symp-toms of heart failure.11 The guidelines should be revised to provide up-to-date indications of CRT and CRT-D, and we also have to consider the criteria for the indication of CRT such as the width of QRS complex, level of cardiac function, severity of heart failure and complication of AF.

With the recent advancement of medical engineering, non-pharmacotherapy of cardiac arrhythmias have improved sig-nificantly, and evidence supporting widespread use of such procedures has been obtained in many large-scale clinical stud-ies. We thus modified the 2006 edition of the guidelines to prepare the 2011 edition.

1. Current Status of Non-Pharmacotherapy of Cardiac Arrhythmias

In Japan, the use of implantable pacemakers for the treatment of bradyarrhythmia started to be covered with the NHI in 1974. It rapidly became common and widespread. The number of patients implanted with pacemakers increased over time in the 1980 s as smaller and more physiological devices with a long battery life became available, and increased to about 42,700 patients in 2002 and about 57,500 patients (new device implan-

tation in about 36,000 patients and device replacement in about 21,000 patients) in 2010. On the other hand, patients with tachyarrhythmia have been treated with drugs since the 1950 s, but the report of the CAST (Cardiac Arrhythmia Suppression Trial)12 in 1989 significantly changed the methodology used to treat tachyarrhythmia, and enhanced the position of non-pharmacotherapy. Various surgical procedures to successfully treat tachyarrhythmia were developed in the 1970 s and 1980 s. Catheter ablation and ICD were developed in the 1980 s and rapidly became common in Europe and the United States in

I Background and Concept of the Guidelines


251JCS Guidelines for Non-Pharmacological Therapy of Arrhythmias

the 1990 s. Catheter ablation has been established as a mini-mally invasive, curative technique to target the sources of arrhythmia. In the United States, about 400,000 people die from SCD each year, and 80 to 90% of them are considered to have ventricular fibrillation/tachycardia,13,14 and ICD therapy is posi-tioned as the most effective preventive measure against sud-den death. The presence of intraventricular conduction distur-bance of left bundle branch block pattern, a finding observed in 20 to 30% of patients with chronic heart failure, was found to be an independent prognostic factor, and CRT (i.e., biven-tricular pacing) was established and rapidly becoming com-mon in Europe and the United States. CRT-D were also devel-oped on the basis of the finding that sudden death accounted for 40 to 50% of the deaths in patients with chronic heart fail-ure, and ventricular fibrillation was considered the most com-mon cause of sudden death in this population.

In Japan, catheter ablation, ICD therapy, CRT and CRT-D therapy were started to be covered with the NHI in 1994, 1996, 2004 and 2006, respectively (Figure 1). These non-pharma-cological procedures are rapidly improving, and the numbers of patients receiving ICD therapy and CRT/CRT-D therapy have been increasing every year (Figure 2). Although much is still not known for SCD in Japan,15 it is estimated that 60,000 to 80,000 people died from it each year. Although investiga-tors believe that the most common direct cause of SCD is ventricular tachyarrhythmia,16,17 but the distribution of under-lying heart diseases in Japanese patients differ from that in Western patients.18 Coronary artery disease is less common,

and dilated cardiomyopathy, hypertrophic cardiomyopathy and idiopathic ventricular fibrillation are more common in Japanese patients (Figure 3). Physicians should select the most appro-priate option from drugs and various non-pharmacological pro-cedures including catheter ablation, ICD therapy and surgery to treat patients with tachyarrhythmia. It is of great clinical significance to provide updated guidelines for non-pharmaco-therapy of patients with tachyarrhythmia.

2. Contents of the Guidelines

The present revised guidelines propose indications for non-pharmacological procedures for the treatment of cardiac arrhyth-mias according to the latest findings. The present guidelines describe the significance and indications of clinical cardiac EPS, which is essential to determine whether non-pharmaco-logical procedures are indicated and assess the efficacy of such procedures, and list indications of cardiac pacing, catheter abla-tion, ICD therapy, CRT-D therapy and surgery. The guidelines also describe cardiac pacing for patients with hypertrophic obstructive cardiomyopathy and biventricular pacing (i.e., CRT) for patients with heart failure.

It is difficult to provide evidence-based recommendations for non-pharmacotherapy of children with tachyarrhythmia because they are extremely small in number and few clinical studies have been conducted in this patient population. In the present guidelines, many recommendations on the indications

Figure 1. History of pharmacotherapy and non-pharmacotherapy of cardiac arrhythmias. 3D, three-dimensional; CAST, Cardiac Arrhythmia Suppression Trial; CRT, cardiac resynchronization therapy; CRT-D, cardiac resynchronization therapy device that in-corporates both pacing and defibrillation capabilities; ICD, implantable-cardioverter defibrillator; NHI, National Health Insurance; WPW, Wolff-Parkinson-White.



of catheter ablation and ICD therapy in children are graded considering the particularity of these procedures according to the consensus of the members of Joint Working Groups.

3. Evidence and Class of Recommendations

In Japan, non-pharmacological procedures for the treatment of arrhythmias other than cardiac pacing have not been exten-

Figure 2. Changes over time of cases of ICD therapy and CRT in Japan: total cases of new implantation and replacement. CRT, cardiac resynchronization therapy; CRT-D, cardiac resynchronization therapy device that incorporates both pacing and defibril-lation capabilities; CRT-P, cardiac resynchronization therapy device that provides pacing but not defibrillation capability; ICD, implantable-cardioverter defibrillator; NHI, National Health Insurance.

Figure 3. Distribution of underlying heart diseases among patients undergoing ICD therapy in Japan and the United States. ARVC, arrhythmogenic right ventricular cardiomyopathy; DCM, dilated cardiomyopathy; HCM, hypertrophic cardiomyopathy; ICD, im-plantable-cardioverter defibrillator. Modified from Kasanuki H. Indications for ICD. In: Implantable Cardioverter Defibrillator As-sessment Committee of the Japanese Society of Cardiac Pacing and Electrophysiology, editor. Clinical practice of implantable cardioverter defibrillators. p24. Igaku-Shoin Ltd., Tokyo, 1998.18



sively developed and not commonly used. Evidence on the benefits of non-pharmacological procedures for the treatment of arrhythmias is thus limited. In the preparation of the present guidelines, we reviewed the literature providing evidence in Europe and the United States, critically examined the level of evidence, and collected data in Japan to discuss the relevant literature and data at the Joint Working Groups meetings and determine the level of evidence according to the experience and opinions of the members of Joint Working Groups and col-laborators. Although the Joint Working Groups reviewed pre-vious and latest American College of Cardiology/American Heart Association (ACC/AHA) guidelines19–23 as well as guide-lines by the Canadian Diabetes Association24 as references, we did not provide level of evidence in the present guidelines as in the previous editions25–27 since it is difficult to evaluate evi-dence available in Japan. The following Class of Recommen-dations are used in the present guidelines, according to the grading system used in ACC/AHA guidelines as well as the four-rank grading proposed by the Canadian Diabetes Asso-ciation.

1. Class I: There is evidence and/or general agreement that a given procedure or treatment is useful and indicated.

2. Class IIa: Weight of opinion is in favor of usefulness.3. Class IIb: Usefulness is less well established by opinion.4. Class III: General agreement that the procedure or treat-

ment is not useful or may even be harmful and is not indi-cated.

4. Matters to Be Considered in Determining the Indications for Non-Pharmacotherapy

Non-pharmacotherapy of cardiac arrhythmia is performed to 1) prevent SCD and improve life prognosis (reduce mortality); 2) alleviate symptoms related to arrhythmias and improve well-being (reduce morbidity); and 3) improve satisfaction with social life of the patients. The items 2) and 3) are necessary to improve the quality of life (QOL) of the patients. In other words, non-pharmacological procedures should therefore be evaluated and selected from the medical (biological) and social aspects of each patient. The medical aspects include whether the patient has Adams-Strokes attack (symptoms of cerebral ischemia such as syncope and dizziness with black out), palpitations, chest distress, chest pain, symptoms of heart failure, and hemody-namic instability in association with arrhythmias and whether these symptoms may become fatal. Also, the patient should be evaluated for the presence and severity of underlying heart dis-eases, cardiac functions, type of arrhythmias (tachyarrhythmia or bradyarrhythmia), and effects of drugs and exercise, and should undergo clinical EPS, signal-averaged electrocardiog-raphy (ECG), and T wave alternans measurement and others. The social aspects should be evaluated to understand his or her personality and what makes him/her satisfied and what he/she wants in the workplace, community and home. Physicians should consider the patient’s occupation (e.g., airline pilot, car/train driver, or a worker engaged in potentially hazardous activities such as high place work); younger people; especially desire to continue sports, become pregnant, and/or drive; liv-ing place (whether living in a remote place where access to medical services is limited); frequency of recreational travel or business trip (especially overseas business trips facing time differences); and stress during work (whether having a physi-cally and mentally demanding job).

5. Requirements for Physicians Providing Non-Pharmacological

Procedures of Arrhythmias

Non-pharmacological procedures of arrhythmias are rapidly evolving field and require advanced medical techniques. Pro-cedures listed in the present guidelines must be performed by experienced physicians in suitable medical institutions. Physi-cians must meet the following requirements:

1. Physicians must have sufficient knowledge and experience in clinical cardiac EPS.

2. Physicians must have sufficient knowledge and experience in treatment with anti-arrhythmic drugs.

3. Physicians must have sufficient knowledge and medical techniques in non-pharmacological procedures for the treat-ment of arrhythmias such as cardiac pacing, catheter abla-tion, ICD therapy, CRT/CRT-D therapy and surgeries, and must be able to conduct emergency surgery or other appro-priate measures to cope with complications.

1. Implantable-Cardioverter DefibrillatorIn the NHI Price List 2010, the following requirements for medical institutions are set for reimbursement of fee for ICD implantation.

1. Medical institutions shall have notified the prefectural gov-ernment office to provide medical services of cardiology and cardiovascular surgery.

2. Medical institutions shall have performed a minimum of 50 cardiac EPS annually, including a minimum of 5 studies in patients with ventricular tachyarrhythmia.

3. Medical institutions shall have performed a minimum of 30 cases of open heart surgery, coronary bypass surgery or aor-tocoronary bypass surgery annually and a minimum of 10 cases of pacemaker implantation annually.

4. Medical institutions shall have a minimum of two full-time cardiologists and two full-time cardiovascular surgeons, and at least two of them must have completed required training.

2. Catheter AblationIn 1990, the Japanese Society of Cardiac Pacing and Electro-physiology (currently named: the Japanese Heart Rhythm Soci-ety) proposed the following requirements for medical institu-tions performing catheter ablation,28 many of which are still valid.

1. Medical institutions must have physicians with sufficient knowledge and experience in cardiac EPS.

2. Medical institutions must have physicians who have suffi-cient knowledge and experience in treatment with anti-arrhythmic drugs.

3. Medical institutions must have physicians who have suffi-cient knowledge and experience in pacemaker therapy.

4. Medical institutions must ensure that during catheter abla-tion cardiovascular surgeons and medical staffs are ready for emergency surgery whenever required. Catheter ablation, which is referred to as percutaneous cath-

eter-based ablation of the myocardium in the NHI Price List, is listed as a Category 1 procedure required to be performed in institutions that meet the following standards for those per-forming procedures listed in the items 5 and 6 of the General Rules of the Chapter 2, Section 10: Surgeries in of the NHI



Price List in 2010.

1. Medical institutions shall have systems to perform the pro-cedure including those to cope with emergent situations in association with the procedure.

2. Medical institutions shall have physicians required to con-duct the procedure.

3. Medical institutions shall indicate the number of cases per year for the relevant procedure at a place easily visible to patients and visitors.

4. Medical institutions shall ensure that all patients are explained about their surgery using appropriate documents.

3. Cardiac PacingIn the NHI Price List 2010, the following requirement for medical institutions is set for reimbursement of fee for cardiac pacing.

1. Medical institutions shall have a minimum of one physician who has practiced in cardiology or cardiovascular surgery for 5 years or more. Medical institutions categorized into clinics may notify to the relevant governmental office as an institution where the procedure is provided.

Although not mentioned in the above requirement, it is pref-erable that medical institutions also be able to perform cardiac EPS and outpatient services for pacemaker users as well.

4. Cardiac Resynchronization TherapyIn the NHI Price List 2010, the following requirements for medical institutions are set for reimbursement of fee for CRT.

1. Medical institutions shall have notified the prefectural gov-ernment office to provide medical services of cardiology and cardiovascular surgery.

2. Medical institutions shall have performed a minimum of 50 cardiac EPS annually, including a minimum of 5 studies in patients with ventricular tachyarrhythmia.

3. Medical institutions shall have performed a minimum of 50 cases of open heart surgery, aortocoronary bypass surgery or coronary bypass surgery annually and a minimum of 10 cases of pacemaker implantation annually.

4. Medical institutions shall have sufficient experience in treat-ing patients with severe heart failure using mechanical car-diac assist systems including external devices.

5. Medical institutions shall have a minimum of two full-time cardiologists and two full-time cardiovascular surgeons, and at least two of them must have completed required training.

In addition to the above requirements, it is necessary to ensure appropriate human resources, equipment, and operating sys-tems with the aim of performing non-pharmacological proce-dures effectively and safely.

In Japan, the Japanese Heart Rhythm Society has hold edu-cation/training seminars on catheter ablation, ICD therapy, CRT/CRT-D therapy, and others to provide basic/standard knowledge and up-to-date information. The Japanese Heart Rhythm Society and the Japanese Society of Electrocardiology are cooperatively developing a system to certify specialists in cardiac arrhythmia who can provide appropriate and up-to-date treatment, and will implement the certification system in 2012. Also, the Japanese Heart Rhythm Society has provided “Car-diac Pacemaker Technician Training Seminars” to foster health-care professionals specialized in cardiac arrhythmia, EPS, pace-maker therapy, ICD/CRT-D therapy, catheter ablation and other

advanced techniques, and issued certificates of Cardiac Device Representatives (CDR) who are working in manufactures of cardiac devices such as pacemaker and ICD to provide device information.

6. Informed Consent

When a patient decides whether he/she will receive advanced medical technique treatment such as non-pharmacotherapy of cardiac arrhythmia, he/she must be fully explained the treat-ment in words which can be understood and express informed consent to treatment of his/her own will. Although informa-tion provided should be selected by individual physicians based on their knowledge and experience and may differ among phy-sicians, the following information should be provided, basi-cally: 1) Information about the disease (e.g., type and severity of arrhythmia, and underlying heart disease); 2) Information about the contents of treatment procedures and its effects (in addition to general information, the results in the medical insti-tution should be provided) such as the purpose and contents of treatment procedures (including the model name and manu-facturer of the pacemaker, ICD or CRT/CRT-D to be used), the clinical efficacy and success rate; the type, severity and incidence of acute phase complications; the type, severity and frequency of long-term complications, and reasons for selec-tion of the treatment procedure; 3) Information about alterna-tive treatment procedures (pharmacotherapy, other non-phar-macological procedures, and procedures also available in other institutions) and their expected effects (e.g., success rates and complications for the procedures mentioned); 4) Information about consequences when the disease is left untreated without undergoing the treatment procedure (e.g., expected outcomes and likelihood); and 5) Information about the position of the treatment procedure for different types of arrhythmias, the pos-sibility of occurring unexpected short- and long-term compli-cations), and expected advancement of antiarrhythmic treat-ment in the future. When the patient seeks second opinions by other physicians and medical institutions, the physician must cooperate with the patient.

However, it is not easy to provide the above information to patients undergoing non-pharmacotherapy of cardiac arrhyth-mia in the clinical setting due to some incompleteness of the current medicine, limited knowledge and experience of the attending physician, or the attending physician’s concern that explanation may lead to confusion. Selection of appropriate and necessary contents to be communicated is an important issue. It is expected that medical institutions will be required to dis-close whether the institution meets the relevant requirements and the physicians have been certified by the relevant academic societies as well as the number of cases and the results (suc-cess rates and incidence of complications) in the future.

Although the “will of the patient” is the most important fac-tor in determining whether non-pharmacotherapy of cardiac arrhythmia should be performed, physicians should not over-estimate the “strong desire” and “unwillingness” of the patient since he/she may have made his/her decision based on biased medical information. Physicians should be committed to be able to clearly explain accurate and up-to-date information to the patients and their family members based on the level of their understanding, and should be aware that informed consent is an only opportunity for the patient to weigh the merits and demerits of the treatment procedure and select a way to expe-rience the “true benefits”.



1. Bradyarrhythmia

Cardiac Electrophysiological Study for Diagnosis of BradyarrhythmiaClass I1. Patients with symptoms such as syncope and dizziness in

whom a causal relation between bradycardia and the symp-toms is unclear.

2. Patients with syncope and dizziness that are suspected to be caused by bradycardia.

Class IIa1. Patients with sinus node dysfunction or atrioventricular (AV)

block who are indicated for pacemaker therapy and require evaluation of sinus node function and AV conduction dis-turbance.

2. Asymptomatic patients with Mobitz type II second degree AV block, third degree AV block, bifascicular block or tri-fascicular block for whom the site of block must be speci-fied and sinus node function must be evaluated.

Class IIb1. Asymptomatic patients with chronic bifascicular block.

Class III1. Patients with asymptomatic sinus bradycardia, first degree

AV block, or Wenckebach second degree AV block.

Cardiac Electrophysiological Study to Evaluate the Efficacy of Pharmacotherapy for BradyarrhythmiaClass INone.

Class IIa1. Patients with sinus node dysfunction in whom the cause of

bradycardia is required to specify from intrinsic causes, autonomic dysfunction or drugs.

2. Patients with bradycardia-tachycardia syndrome in whom drugs to control tachycardia exacerbate bradycardia.

3. Patients with asymptomatic sinus node dysfunction who need treatment with drugs that may exacerbate sinus node dysfunction.

4. Patients with asymptomatic AV block or intraventricular conduction disturbance who need treatment with drugs that may exacerbate conduction disturbance.

Cardiac Electrophysiological Study to Confirm the Efficacy of Cardiac PacingClass I1. Patients with neurally mediated syncope or hypertrophic

obstructive cardiomyopathy in whom temporary pacing is used to confirm the efficacy of cardiac pacing.

Class IIa1. Patients with bradycardic AF in whom temporary pacing is

used to evaluate the efficacy of cardiac pacing and decide whether an implantable pacemaker is indicated or not.

2. Patients with heart failure in whom temporary pacing is used to confirm the efficacy of biventricular pacing (i.e., CRT).

2. Tachyarrhythmia

Cardiac Electrophysiological Study for Diagnosis of TachyarrhythmiaClass I1. Symptomatic patients with narrow QRS tachycardia.2. Patients with wide QRS tachycardia.3. Patients with WPW syndrome who have palpitation attacks

associated with syncope and dizziness.4. Patients with syncope and dizziness that may be caused by

tachycardia.

Class IIa1. Patients with palpitation attacks of which the cause is sus-

pected to be tachyarrhythmia but could not confirmed with ECG or other tests.

2. Asymptomatic patients with narrow QRS tachycardia.

EPS is useful for symptomatic patients with narrow QRS (less than 120 msec) tachycardia29–31 and asymptomatic and symp-tomatic patients with wide QRS tachycardia including ven-tricular tachycardia32,33 since reentry may be a cause. Patients possibly requiring catheter ablation often undergo EPS and catheter ablation at the same time.31,34

Cardiac Electrophysiological Study to Evaluate the Efficacy of Pharmacotherapy for TachyarrhythmiaClass INone.

Class IIa1. Evaluation of drug efficacy and proarrhythmic effects of

drugs in patients with sustained monomorphic ventricular tachycardia.

2. Patients who need long-term follow-up of catheter ablation for ventricular tachycardia.

Class IIb1. Evaluation of the efficacy in patients with AV nodal reen-

trant tachycardia or AV reciprocating tachycardia.2. Evaluation of drug efficacy in patients with sinus nodal

reentrant tachycardia, atrial tachycardia or atrial flutter.3. Patients who need long-term follow-up of catheter ablation

in patients with supraventricular tachyarrhythmia.

The efficacy of drugs is evaluated by their ability in prevent-ing the inducibility of tachycardia during EPS. This style of treatment is referred to as EPS-guided antiarrhythmic therapy. Although it has been reported that EPS-guided or Holter ECG-guided therapy is useful to ensure the efficacy of amiodarone,35 empiric therapy is still prevalent. In the AVID (Antiarrhythmics Versus Implantable Defibrillators) study,36 ICD therapy was superior in improving the prognosis as compared to pharma-cotherapy for secondary prevention of ventricular tachycardia/fibrillation. Since ICD therapy is now quite common, EPS-guided therapy only plays a supplemental role such as reducing the incidence of recurrence.

II Clinical Cardiac Electrophysiological Study



Cardiac Electrophysiological Study to Assess the Risk of PatientsClass I1. Patients resuscitated from cardiac arrest.2. Patients with nonsustained ventricular tachycardia associ-

ated with structural heart disease who have syncopal attacks of unknown etiology and/or left ventricular dysfunction.

3. Asymptomatic patients with WPW syndrome who have a family history of sudden death or engage in dangerous occu-pations.

Class IIa1. Patients with nonsustained ventricular tachycardia or fre-

quent episodes of premature ventricular contractions who have structural heart disease and positive ventricular late potentials on signal-averaged ECG.

2. Patients with Brugada syndrome who have a history of syn-cope and/or a family history of sudden death.

Class IIb1. Patients with nonsustained ventricular tachycardia who have

structural heart disease without ventricular dysfunction.

2. Patients with frequent episodes of premature ventricular con-tractions or with nonsustained ventricular tachycardia who show positive ventricular late potentials on signal-averaged ECG but do not have structural heart disease.

3. Patients with long QT syndrome (LQTS) who have a his-tory or family history of palpitation attacks with syncope and/or dizziness.

The risk of sudden death is assessed by examining whether fatal arrhythmia may be induced or not, but it is not easy to specify high-risk patients. It should be evaluated comprehensively using Holter ECG, exercise stress tests, signal-averaged ECG, and T wave alternans measurement. Risk assessment using EPS should be actively conducted in patients with nonsustained ven-tricular tachycardia who have structural heart disease and left ventricular dysfunction.37–40

EPS are less useful in risk assessment in patients with LQTS. There is controversy regarding whether electrical induction of ventricular fibrillation should be used or not to assess the risk in patients showing Brugada type ECG. It is believed that EPS are less useful in patients with frequent episodes of premature ventricular contractions without structural heart disease.

Since the launch of commercially available implantable pace-makers in the 1960 s, the devices have been improved year by year, and smaller sophisticated devices have been launched. With the rapid downsizing, pacemakers have significantly advanced in terms of function. Currently-available devices can mimic physiological heart rates almost completely using AV sequential pacing and rate responsive pacing. Although per-manent cardiac pacing has been established as the leading treat-ment method for bradyarrhythmia that is far more beneficial than other procedures including pharmacotherapy and is being used to improve the life prognosis and QOL, investigators have pointed out ethical and economic problems caused by overuse. In the light of these problems, physicians have called for more strict guidelines for the use of implantable pacemakers. In the United States, the ACC/AHA Task Force published the first guidelines for implantation of cardiac pacemakers and antiar-rhythmia devices in 1984 which were substantially revised in 1991 and 199819,20 before publishing the latest revision. In Japan, physicians have considered the use of implantable devices according to the AHA/ACC guidelines, but the Japanese Soci-ety of Cardiac Pacing and Electrophysiology (currently named: the Japanese Heart Rhythm Society) published its guidelines for implantation of cardiac pacemakers in 1995.41

When cardiac pacing is considered, it is most important to understand the nature and severity of symptoms and a causal relationship between symptoms and bradyarrhythmia. Symp-toms of bradyarrhythmia include syncope, black out, severe dizziness, and lightheadedness caused by transient cerebral isch-emia, and decreased exercise capacity and symptoms of heart failure caused by long-lasting bradycardia. Physicians should also assess their patients for underlying cardiac diseases that may be exacerbated by bradycardia, and cerebrovascular lesions that may lead to cerebral infarction when bradycardia occurs, especially when they need treatment with drugs that may exac-erbate bradycardia. Abnormal findings of Holter ECG and car-diac EPS are important as well.

Physicians should also consider the age, occupation (e.g., working in places easily exposed to electromagnetic interfer-

ence and high places, car drivers), physical activity, family environment, living environment, personality, and patient’s and his/her family members’ desire.

1. Atrioventricular Block

Class I1. Patients with second degree, high-grade or third degree AV

block who exhibit clear symptoms due to bradycardia.2. Patients with high-grade or third degree AV block who have

at least one of the following conditions. 1) Patients in whom AV block is induced by drug(s) which

cannot be discontinued. 2) Patients who have postoperative AV block that cannot

be predicted to improve or not in the future. 3) Patients who underwent catheter ablation of AV junc-

tion. 4) Patients with AV block associated with progressive neu-

romuscular disease. 5) Patients who have substantial bradycardia or long-lasting

ventricular arrest during awaking.

Class IIa1. Asymptomatic patients with persistent third degree AV

block.2. Asymptomatic patients with second degree or high-grade

AV block with one of the following conditions. 1) Patients with a block within or below the bundle of His. 2) Patients with progressive cardiomegaly in association with

bradycardia. 3) Patients in whom exercise or atropine stress test does not

improve or impairs AV conduction.3. Patients who have symptoms possibly caused by bradycardia

and otherwise unexplained first degree AV block within or below the bundle of His.

III Cardiac Pacing



Class IIb1. Patients with first degree AV block associated with heart

failure in whom pacing at an optimal AV interval is expected to improve hemodynamics.

2. Bifascicular or Trifascicular Block

Class I1. Patients with chronic bifascicular or trifascicular block and

a history of Mobitz type II second degree, high-grade or third degree AV block.

2. Patients with chronic bifascicular or trifascicular block in whom AV block is likely to be induced by drug(s) which cannot be discontinued.

3. Patients with chronic bifascicular or trifascicular block and Wenckebach second degree AV block and in whom occur-rence of high-grade AV block is suspected to cause synco-pal attacks.

Class IIa1. Patients with chronic bifascicular or trifascicular block who

have syncopal attacks of unknown etiology.2. Patients with chronic bifascicular or trifascicular block who

have structural heart disease and show conduction delay or block below the bundle of His during EPS.

Class IIb1. Patients with chronic bifascicular or trifascicular block who

show conduction delay or block below the bundle of His during EPS but do not have structural heart disease.

3. Sick Sinus Syndrome

Class I1. Patients with symptoms such as syncope, convulsion, black

out, dizziness, shortness of breath and easy fatigability, or heart failure that have been confirmed due to bradycardia, sinoatrial block, or sinus arrest associated with sinus node dysfunction or blunted heart rate response during exercise, including patients whose symptoms are caused by long-term treatment with drug(s) which cannot be discontinued.

Class IIa1. Patients with the above-mentioned symptoms as well as

bradycardia and/or ventricular arrest, but the relationships between these are unclear.

2. Patients with bradycardia-tachycardia syndrome in whom drugs to control tachycardia exacerbate bradycardia.

Class IIb1. Asymptomatic patients with sinoatrial block or sinus arrest.

4. Bradycardic Atrial Fibrillation

Class I1. Patients with symptoms such as syncope, convulsion, black

out, dizziness, shortness of breath and easy fatigability, or heart failure that have been confirmed due to bradycardia or ventricular arrest, including patients whose symptoms are caused by long-term treatment with drug(s) which cannot be discontinued.

Class IIa1. Patients with the above-mentioned symptoms as well as

bradycardia and/or ventricular arrest, but the relationships between these are unclear.

5. Hypersensitive Carotid Sinus Syndrome and Reflex Syncope

Class I1. Patients with hypersensitive carotid sinus syndrome who

have repeated syncopal attacks due to bradycardia.2. Patients with reflex syncope who show bradycardia on ECG

and have repeated syncopal attacks.

Class IIa1. Patients with reflex syncope with repeated syncopal attacks

who show a cardioinhibitory response to head-up tilt test-ing.

Class III1. Patients with hypersensitive carotid sinus syndrome or reflex

syncope who do not show a cardioinhibitory response to head-up tilt testing.

In many clinical studies of reflex syncope (neurally mediated syncope), the indications for and efficacy of cardiac pacing have been evaluated using head-up tilt testing. Although it has been reported that cardiac pacing can prevent syncope in about 50% of patients with a cardioinhibitory response on head-up tilt testing,42–46 the corresponding percentage in double blind studies was as low as 17%, and the treatment effect of cardiac pacing is unclear.47 Also, patients with hypersensitive carotid sinus syndrome with a marked cardioinhibitory response to head-up tilt testing are expected to improve by cardiac pac-ing.48–50 However, physicians should be aware that physical counterpressure maneuvers are the first-line therapy since reflex syncope is mainly caused by a decrease in blood pressure.51

6. Hypertrophic Obstructive Cardiomyopathy

Class I1. Patients with hypertrophic obstructive cardiomyopathy who

have a significant outflow gradient that causes symptoms negatively affecting QOL and have other reasons justify the use of an implantable pacemaker (including patients with drug-induced bradycardia).

Class IIa1. Patients with hypertrophic obstructive cardiomyopathy who

have a significant outflow gradient that causes symptoms negatively affecting QOL and in whom the severity of symp-toms is related to outflow gradient, pharmacotherapy is inef-fective or cannot be continued due to adverse drug reac-tions, and other options are inappropriate.

Class III1. Patients with hypertrophic obstructive cardiomyopathy with

neither an outflow gradient nor bradycardia indicated for pacemaker implantation.

It has been reported that cardiac pacing in patients with hyper-trophic obstructive cardiomyopathy is effective in alleviating symptoms caused by left ventricular outflow tract obstruction,



and considered that the efficacy correlates with the reduction of outflow gradient. Maron et al. reported that symptomatic improvement perceived after cardiac pacing was most consis-tent with a substantial placebo effect,52 and the efficacy of cardiac pacing is not enough among patients other than elderly patients. On the other hand, in the PIC (Pacing in Cardiomy-opathy) study,53 patients who responded favorably to tempo-rary pacing without compromising the outflow gradient had a pacemaker implanted, and showed benefits of permanent pac-ing. Although patients with hypertrophic obstructive cardio-myopathy have undergone surgeries, and percutaneous trans-luminal septal myocardial ablation (PTSMA) is listed as a treatment method for this disease by the NHI, pacemaker ther-apy is safer than these invasive measures, and is expected to be effective when patient selection and implantation are care-fully performed. However, pacing for patients with hypertro-phic obstructive cardiomyopathy is not covered with the NHI. Patients who may benefit the most are those with a resting gra-dient of 30 mmHg or more, a provoked gradient of 50 mmHg or more54 (reference values).

7. Pacing in Children

The most common indications for permanent pacemaker implan-tation in children are 1) symptomatic sinus bradycardia, 2) bradycardia-tachycardia syndrome, and 3) high-grade or com-plete AV block. Although the general indications for pacemaker implantation in children are similar to those in adults, there are several important considerations in young patients: 1) Patients with prior congenital heart surgery may have symptoms due to sinus bradycardia or loss of AV synchrony at heart rates,55,56 and appropriate heart rates, which differ from those for chil-dren with normal cardiovascular physiology, should be set; 2) the clinical significance of bradycardia is age dependent; 3) transvenous lead implantation may be extremely difficult among infants and young children or children with venous anomaly or congenital heart disease, and epicardial pacing lead implan-tation should be considered; and 4) because there are no ran-domized clinical studies of cardiac pacing in children or patients with congenital heart disease, the Level of Evidence for most recommendations is C.

Additional details that need to be considered in pacemaker implantation in children include risk of paradoxical embolism due to thrombus formation on an endocardial lead system.57 Physicians should also select an appropriate lead implantation technique to reserve the transvenous routes for future use.

Indications for Permanent Pacing in Children and Patients With Congenital Heart DiseaseClass I1. High-grade or complete AV block associated with symp-

tomatic bradycardia, ventricular dysfunction, or low cardiac output.

2. Sinus node dysfunction with correlation of symptoms dur-ing age-inappropriate bradycardia (the definition of brady-cardia varies with the patient’s age and target heart rate).

3. High-grade or complete AV block that persists at least 7 days after cardiac surgery.

4. Congenital complete AV block with a wide QRS escape rhythm, premature ventricular contractions, or ventricular dysfunction.

5. Congenital complete AV block in the infant with a ventricu-lar rate less than 55 bpm or with congenital heart disease and a ventricular rate less than 70 bpm.

Class IIa1. Patients with congenital heart disease and sinus node dys-

function who have recurrent episodes of intra-atrial reen-trant tachycardia (sinus node dysfunction may be intrinsic or secondary to antiarrhythmic treatment).

2. Congenital complete AV block beyond the first year of life with an average heart rate of 50 bpm or less, abrupt pauses in ventricular rate that are 2 or 3 times the basic cycle length, or associated with symptomatic bradycardia.

3. Sinus bradycardia with complex congenital heart disease with a resting heart rate of 40 bpm or less, or abrupt pauses in ventricular rate longer than 3 seconds.

4. Patients with congenital heart disease and impaired hemo-dynamics due to sinus bradycardia or loss of AV syn-chrony.

5. Syncope of unknown etiology in the patients with prior congenital heart surgery complicated by transient AV block and residual bundle branch block.

Class IIb1. Patients with prior congenital heart surgery complicated by

transient complete AV block and residual bifascicular block.2. Congenital complete AV block in asymptomatic children

with a rate appropriate to their age, a no QRS prolongation, and normal cardiac function.

Class III1. Patients with prior congenital heart surgery complicated by

asymptomatic transient AV block, and AV conduction returns to normal.

2. Bifascicular block with or without first degree AV block after surgery for congenital heart disease in the absence of prior complete AV block.

3. Asymptomatic Wenckebach second degree AV block.4. Asymptomatic sinus bradycardia with the longest RR inter-

val less than 3 seconds and a minimum heart rate of 40 bpm or more.

IV Catheter Ablation

1. Wolff-Parkinson-White Syndrome and Atrioventricular Nodal

Reentrant Tachycardia

Wolff-Parkinson-White SyndromeClass I1. Patients with a history of life-threatening AF or other seri-

ous symptoms such as syncope or patients who had tachy-cardia episodes that were mild but significantly deteriorated QOL.

2. Patients who had tachycardia episodes with or without pre-excitation and desire to undergo catheter ablation.

3. Patients who have preexcitation without tachycardia epi-sodes, and are engaged in occupations such as airline pilots and mass transit drivers with a risk of fatal accidents if an



episode occurs.

Class IIa1. Patients who have preexcitation without tachycardia epi-

sodes, and desire to undergo catheter ablation after receiv-ing explanation.

Atrioventricular Nodal Reentrant TachycardiaClass I1. Patients with a history of serious symptoms such as syn-

cope or a history of tachycardia episodes that significantly deteriorated QOL.

2. Patients with tachycardia episodes receiving or not receiv-ing pharmacotherapy who desire to undergo catheter abla-tion.

Class IIa1. Patients with ECG documentation during spontaneous tachy-

cardia episodes in whom tachycardia was not induced but dual AV nodal pathways alone were found during EPS.

2. AV nodal reentrant tachycardia induced by chance during EPS or catheter ablation for other types of tachycardia.

Class IIb1. Patients without ECG documentation during spontaneous

tachycardia episodes in whom tachycardia was not induced but dual AV nodal pathways alone were found during EPS.

Class III1. Patients without a history of tachycardia episodes in whom

dual AV nodal pathways were found but tachycardia was not induced during EPS.

2. Atrial Fibrillation

Class I1. Patients with drug-resistant symptomatic paroxysmal AF

with neither severe left atrial enlargement, severe left ven-tricular dysfunction, nor severe pulmonary disease who are to undergo catheter ablation in medical institutions perform-ing a minimum of 50 catheter ablation procedures for AF annually.

Class IIa1. Symptomatic drug-resistant paroxysmal or persistent AF.2. Patients engaged in occupations such as airline pilots and

mass transit drivers in which the presence of AF affect their operations.

3. Patients in whom pharmacotherapy is effective but desire to undergo catheter ablation for AF.

Class IIb1. Symptomatic drug-resistant paroxysmal or persistent AF

with severe left atrial enlargement and/or severe left ven-tricular dysfunction.

2. Paroxysmal or persistent AF without symptoms or signifi-cant deterioration of QOL.

Class III1. Patients suspected to have left atrial thrombus.2. Patients contraindicated for anticoagulation therapy.

There are two approaches, rhythm control and rate control, to the treatment of AF. In the AFFIRM (Atrial Fibrillation Fol-

low-up Investigation of Rhythm Management),58 RACE (Rate Control versus Electrical Cardioversion for Persistent Atrial Fibrillation)59 and STAF (Strategies of Treatment of Atrial Fibrillation) studies60 mainly in patients with persistent AF, no differences were found between the two approaches in terms of life prognosis. In the J-RHYTHM (Japanese Rhythm Man-agement Trial for Atrial Fibrillation) study,61 a clinical study conducted in Japan mainly in patients with paroxysmal AF, there were no significant differences between the two approaches in terms of mortality, incidence of cerebral infarction, and hos-pitalization rate. However, the prognosis was better in patients maintaining sinus rhythm in a subanalysis of the AFFIRM study, and the results suggest that any beneficial antiarrhyth-mic effects of antiarrhythmic drugs are offset by their adverse effects.62 On the other hand, many studies have reported that pulmonary vein isolation ablation is better than pharmacother-apy of arrhythmia in terms of maintaining sinus rhythm.63–70 Since many patients with paroxysmal AF have AF triggers originating in the pulmonary veins, bilateral pulmonary vein isolation, an ablation technique to electrically isolate the bilat-eral pulmonary veins from the left atrium, may prevent the recurrence of AF.71 Currently, extensive pulmonary vein isola-tion, a procedure to create an ablation circle around the supe-rior and inferior pulmonary veins on both sides,72 and pulmo-nary vein isolation to ablate around the orifice of each of the four pulmonary veins73 are used. Patients with recurrent AF after the procedure need repeated ablation procedures. The success rate ranges from 45 to 94%, and recent studies have reported that successful treatment was achieved in more than 80%.68,74–87 Sinus rhythm can be maintained in 59 to 93% of patients without drugs. Reported complications include left atrial-esophageal fistula, atrial tachycardia originating from the left atrium, pulmonary vein stenosis, arterial embolism, cardiac tamponade, vagus nerve disorders (gastrointestinal motility dis-order), and phrenic nerve dysfunction.88,89 In an analysis of the prevalence and causes of fatal outcome in catheter ablation of AF in 45,115 procedures performed from 1995 to 2006, the mortality was 0.98 per 1,000 patients (0.098%).90 In the pres-ent guidelines, catheter ablation for patients symptomatic drug-resistant paroxysmal AF is listed as a Class I indication pro-vided that “this procedure is performed in medical institutions performing a minimum of 50 catheter ablation procedures annu-ally”, since advanced technique, experience and equipment are necessary to ensure the safe and consistent implementation of this procedure. Evidence regarding the usefulness of cath-eter ablation has been obtained in medical institutions with a large number of procedures, and constant implementation is believed to be related to the efficacy and safety of catheter ablation for AF.

Catheter ablation of persistent AF is effective in 20 to 61% of patients undergoing pulmonary vein ablation only, and 9 to 85% of patients receiving complex fractionated atrial electro-gram (CFAE) ablation.66,79,91–93 Pulmonary vein ablation alone is not sufficiently effective, and pulmonary vein ablation in combination with CFAE ablation or linear ablation is expected to be effective and require no pharmacotherapy in 47 to 95%, and to achieve maintaining sinus rhythm in about more than 70% of patients.81,85,94–99

A long-term study has reported that 79.5% of patients with paroxysmal AF and normal left ventricular function maintained sinus rhythm about 5 years after extensive pulmonary vein isola-tion (some patients underwent repeated procedures), and clin-ical improvement was observed in 92.5%.100 In another study in patients with paroxysmal (51%) and nonparoxysmal (49%) AF undergoing extensive pulmonary vein isolation (some



underwent additional linear ablation), 87% of patients main-tained sinus rhythm 2 years after ablation (72% achieved AF elimination without antiarrhythmic drugs, and 15% needed antiarrhythmic drugs), and 13% had recurrent AF. QOL scores started to improve significantly at month 3, and continued for 2 years.101

In a retrospective analysis in patients after successful cath-eter ablation of AF, the incidences of cerebral embolism and massive bleeding during the follow-up period of about 2 years were significantly lower in patients who discontinued antico-agulation therapy 3 to 6 months after ablation than patients who continued anticoagulation therapy.102 Patients with a high risk of thromboembolism should continue anticoagulation ther-apy after catheter ablation.103

3. Atrial Flutter and Atrial Tachycardia

Atrial Flutter (Typical and Atypical Atrial Flutter)Class I1. Patients with atrial flutter who have tachycardia, syncope,

symptoms of heart failure, and deterioration of QOL.2. Patients with typical atrial flutter that developed during phar-

macotherapy of AF.3. Patients with typical atrial flutter that confirmed before or

developed during catheter ablation for AF.

Class IIa1. Patients in whom typical atrial flutter was induced by chance

during catheter ablation for other types of tachycardia.2. Patients with drug-resistant, atypical atrial flutter.3. Patients engaged in occupations such as airline pilots and

mass transit drivers in which the presence of atrial flutter affects their operations.

Class IIb1. Patients in whom atypical atrial flutter was induced by

chance during catheter ablation for other types of tachycar-dia.

Atrial flutter is a type of supraventricular tachycardia charac-terized by a regular flutter wave morphology with an atrial rate of about 300 bpm (240 to 440 bpm). Atrial flutter is classified into type I and type II: the atrial rate is relatively slow (240 to 340 bpm) in type I and faster (350 to 450 bpm) in type II. Many cases of type I atrial flutter, referred to as “typical atrial flut-ter”, are macro-reentrant right atrial tachycardia that involves circulation of the reentrant impulse around the tricuspid annu-lus, and the ECG displays a sawtooth waveform in the inferior leads. Typical atrial flutter is further divided into two sub-types, known as counterclockwise atrial flutter and clockwise atrial flutter based on the flutter wave polarity in the inferior leads (negative and positive, respectively). Atypical atrial flut-ter (i.e., type II atrial flutter) is caused by a macro-reentrant circuit rotating around an area other than the tricuspid annulus. Its clinical aspects are similar to AF but the mechanisms differ among subtypes.

The severity of symptoms caused by atrial flutter depends on the rate of AV conduction. Although some patients remain asymptomatic, others may develop 1:1 AV conduction and syncope. Typical atrial flutter can be successfully treated with catheter ablation by creating a line of conduction block at the isthmus between the tricuspid annulus and the inferior vena cava.104,105

Since atypical atrial flutter other than right atrial flutter due

to lower loop reentry106 is right atrial isthmus-independent, tar-gets of catheter ablation differ among patients. Atypical atrial flutter may be caused by intra-atrial reentry in some cases and abnormal automaticity in others. 3D mapping systems are use-ful to localize the reentry circuit and the origin of tachycardia, so as to examine thoroughly the pattern of conduction in the fluttering right or left atrium.

Atrial TachycardiaClass I1. Symptomatic patients with drug-resistant recurrent atrial

tachycardia with a focal origin.2. Patients with incessant atrial tachycardia.

Class IIa1. Symptomatic patients with atrial tachycardia with a focal

origin that responds to pharmacotherapy.2. Patients with asymptomatic atrial tachycardia and ventricu-

lar dysfunction.

Atrial tachycardia may arise from a focal origin, as in cases of abnormal automaticity (i.e., ectopic automaticity, and triggered activity) and micro-reentry, and from macro-reentry circuits in others. It is difficult to clearly differentiate the latter cases from isthmus-independent atrial flutter.107–110 Atrial tachycar-dia arising from a focal origin includes sinus node reentry tachy-cardia, AV nodal reentrant tachycardia, and other types of reen-try tachycardia. Abnormal automaticity is commonly observed in the pulmonary vein, superior vena cava, coronary veins, atrial appendage, crista terminalis, and others.111 Catheter ablation is recommended even for asymptomatic patients since long-lasting atrial tachycardia may induce left ventricular dysfunc-tion by causing tachycardia-induced cardiomyopathy.

4. Atrioventricular Junction Ablation for Supraventricular Tachyarrhythmia

Class I1. Patients with supraventricular tachyarrhythmia who have

serious symptoms or severe ventricular dysfunction due to tachycardia, in whom pharmacotherapy is ineffective or can-not be continued due to adverse drug reactions and for whom conventional catheter ablation for supraventricular arrhyth-mia was not successful or cannot be performed.

Class IIa1. Patients with supraventricular tachyarrhythmia and substan-

tial deterioration of QOL in whom pharmacotherapy is inef-fective or difficult to perform and for whom conventional catheter ablation for supraventricular arrhythmia was not successful or cannot be performed.

Class III1. Patients in whom AV conduction should be maintained con-

sidering the risk and benefit of the procedure.

5. Premature Ventricular Contraction

Class I1. Patients in whom premature ventricular contraction trigger

polymorphic ventricular tachycardia or ventricular fibrilla-tion and in whom pharmacotherapy is ineffective or cannot be continued due to adverse drug reactions.



2. Patients with frequent premature ventricular contractions who have a substantial deterioration of QOL or heart fail-ure in whom pharmacotherapy is ineffective or cannot be continued due to adverse drug reactions.

3. Patients in whom CRT is not effective due to ineffective biventricular pacing caused by frequent premature ventricu-lar contractions and pharmacotherapy is ineffective or can-not be continued due to adverse drug reactions.

Class IIa1. Patients with frequent premature ventricular contractions

originating from the ventricular outflow tract who have ven-tricular dysfunction or structural heart disease.

2. Patients with frequent premature ventricular contractions originating from the ventricular outflow tract who desire to undergo catheter ablation even though pharmacotherapy is effective or has not been initiated.

Since premature ventricular contraction may trigger polymor-phic ventricular tachycardia or ventricular fibrillation,112,113 cath-eter ablation for premature ventricular contraction may prevent occurrence of polymorphic ventricular tachycardia or ventricu-lar fibrillation.114 Since frequent premature ventricular contrac-tions may also deteriorate cardiac function,115 catheter ablation may improve ventricular dysfunction.116,117

Frequent premature ventricular contractions may cause inef-fective biventricular pacing in patients receiving CRT. Treat-ment of premature ventricular contraction may improve ven-tricular dysfunction by improving the efficacy of biventricular pacing.118

Catheter ablation may be considered for patients with fre-quent premature ventricular contractions originating from the ventricular outflow tract and patients with ventricular dysfunc-tion.119

6. Ventricular Tachycardia

Class I1. Patients with monomorphic ventricular tachycardia associ-

ated with ventricular dysfunction or heart failure in whom pharmacotherapy is ineffective or cannot be continued due to adverse drug reactions.

2. Patients with frequent ICD discharges and in whom phar-macotherapy is ineffective or cannot be continued due to adverse drug reactions.

3. Patients in whom CRT is not effective due to ineffective biventricular pacing caused by monomorphic ventricular tachycardia and pharmacotherapy is ineffective or cannot be continued due to adverse drug reactions.

4. Patients with symptomatic idiopathic ventricular tachycar-dia and deterioration of QOL who desire to undergo cath-eter ablation even though pharmacotherapy is effective or has not been initiated.

Class IIa1. Patients with asymptomatic idiopathic ventricular tachycar-

dia originating from the ventricular outflow tract and an extremely rapid heart rate.

2. Patients with idiopathic ventricular tachycardia originating from the ventricular outflow tract who desire to undergo catheter ablation even though pharmacotherapy is effective or has not been initiated.

Catheter ablation techniques for the treatment of idiopathic ven-tricular tachycardia have achieved stable results for years.120–127 However, catheter ablation of ventricular tachycardia associ-ated with structural heart disease such as myocardial infarction and cardiomyopathy is often not successful, and arrhythmias often recur.128–134 When patients receiving ICD therapy have deterioration of QOL due to episodes of tachycardia and fre-quent ICD discharges, catheter ablation may be an effective treatment option to decrease the frequency of episodes of ven-tricular tachycardia.135,136

7. Special Considerations for Children

The use of catheter ablation for children should be carefully considered on the basis of the indications for adult patients with arrhythmias as well as specific features in children.25,137 In children, catheter ablation is conducted for the treatment of severe conditions such as arrhythmias associated with con-genital heart diseases138,139 and incessant tachycardia compli-cated by tachycardia-induced ventricular dysfunction.140 It is desirable that these patients be treated with physicians who have experience and knowledge in the treatment of congenital heart disease and catheter ablation for children.

Class I1. Children with WPW syndrome or ventricular tachycardia

who have a history of near-miss sudden death or syncope.2. Children with supraventricular tachycardia or ventricular

tachycardia who have ventricular dysfunction due to sus-tained tachycardia.

3. Children with hemodynamically significant drug-resistant ventricular tachycardia.

Class IIa1. Children with drug-resistant recurrent or symptomatic supra-

ventricular tachycardia.2. Children with tachycardia associated with congenital heart

diseases (especially in children in whom catheterization would be difficult after surgery).

3. Children with incessant supraventricular tachycardia.4. Children with intra-atrial reentrant tachycardia.5. Children with palpitation in whom supraventricular tachy-

cardia is induced during cardiac EPS.6. Children with WPW syndrome who or whose family mem-

ber desire to undergo catheter ablation after careful consid-eration of the natural history and complications of the dis-ease.

Class IIb1. Children with supraventricular tachycardia responding to

pharmacotherapy.2. Catheter ablation of AV junction and pacemaker implanta-

tion in children with recurrent or drug-resistant intra-atrial reentrant tachycardia in whom prior catheter ablation was failed (consider referral to a medical institution with a large number of cases).

3. Children with ventricular tachycardia for which pharmaco-therapy is effective but abnormal hemodynamics remain.

4. Children with nonsustained ventricular tachycardia or non-sustained supraventricular tachycardia.



The indications for ICD therapy are classified into primary prevention and secondary prevention. Secondary prevention refers to the prevention of SCD in those patients who have survived a previous cardiopulmonary arrest, or ECG-docu-mented sustained ventricular tachycardia, or ventricular fibril-lation. Primary prevention refers to the prevention of SCD in patients with nonsustained ventricular tachycardia, patients who have syncope but ECG findings suggesting arrhythmia have not been obtained, or patients with ventricular dysfunction and a high risk of sudden death or arrhythmic death.

1. Implantable Cardioverter-Defibrillator Therapy for Secondary Prevention

Class I1. Patients with clinically documented ventricular fibrillation.2. Patients with structural heart disease and sustained ven-

tricular tachycardia and who meet one or more of the fol-lowing conditions:

1) Patients with syncope in association with ventricular tachycardia.

2) Patients with a blood pressure of 80 mmHg or less, symp-toms of cerebral ischemia or chest pain in association with tachycardia.

3) Patients with polymorphic ventricular tachycardia. 4) Patients with hemodynamically stable monomorphic ven-

tricular tachycardia in whom pharmacotherapy is inef-fective or cannot be continued due to adverse drug reac-tions, or cannot be assessed for drug efficacy, or catheter ablation is ineffective or impossible.

Class IIa1. Patients with structural heart disease in whom sustained ven-

tricular tachycardia becomes no longer inducible after cath-eter ablation.

2. Patients with sustained ventricular tachycardia associated with structural heart disease for whom effective pharmaco-therapy was established by detailed follow-up and drug effi-cacy evaluation.

Class IIb1. Patients who are more likely to develop ventricular tachy-

cardia or ventricular fibrillation triggered by an acute revers-ible disorder (e.g., acute ischemia, electrolyte abnormality, and drugs) and are at high risk for exposure to the disorder again despite sufficient treatment.

Class III1. Patients with ventricular fibrillation or ventricular tachy-

cardia caused by conditions that can be successfully treated with catheter or surgical ablation (e.g., AF/atrial flutter with rapid ventricular rate associated with WPW syndrome, and idiopathic sustained ventricular tachycardia).

2. Patients whose life expectancy is less than 12 months.3. Patients who cannot express consent or cooperate with treat-

ment due to mental disorder or other reasons.4. Patients with ventricular tachycardia/fibrillation with a known

acute reversible disorder (e.g., acute ischemia, electrolyte abnormality, and drugs) in whom ventricular tachycardia/fibrillation is expected to be prevented by eliminating the cause.

5. Patients with frequent ventricular tachycardia/fibrillation that cannot be controlled with antiarrhythmic drugs and/or cath-eter ablation.

6. Patient with severe drug-resistant congestive heart failure and NYHA Class IV symptoms who are not indicated for heart transplantation, CRT, or left ventricular assist device (LVAD).

Many large-scale studies have been conducted to investigate the effects of ICD therapy and pharmacotherapy in patients with sustained ventricular tachycardia and ventricular fibrilla-tion associated with structural heart diseases, and the results demonstrated that ICD therapy was more effective than phar-macotherapy in improving prognosis regardless of the type of structural heart diseases.36,141,142

1. Sustained Ventricular Tachycardia and Ventricular Fibrillation Associated With Coronary Artery Disease

In large-scale studies mainly conducted in Europe and the United States, 70 to 80% of patients had coronary artery diseases, and results indicated that ICD therapy show a favorable effect in the secondary prevention of fatal arrhythmias in patients with coronary artery diseases.36,141,142 The mean left ventricular ejec-tion fraction (LVEF) among patients enrolled in these studies ranged 32 to 45%. It is expected that ICD therapy are espe-cially effective among patients with a LVEF of 35% or less.

ICD therapy may not always be indicated for patients with acute coronary syndrome (ACS),143 since sustained ventricular tachycardia and ventricular fibrillation developing in the acute phase of ACS (the first 48 hours after onset) often do not recur after ischemia is treated and the arrhythmic substrate is stabi-lized.

2. Sustained Ventricular Tachycardia and Ventricular Fibrillation Associated With Nonischemic Dilated Cardiomyopathy

Prospective clinical studies conducted until now have demon-strated that ICD therapy is superior to antiarrhythmic drugs in improving the life prognosis in patients with sustained ven-tricular tachycardia and ventricular fibrillation associated with nonischemic dilated cardiomyopathy as in in the case of patients with coronary artery diseases.36,141,142

2. Primary Prevention in Patients With Structural Heart Disease

Class I1. Patients with chronic heart failure due to coronary artery

disease or dilated cardiomyopathy who have NYHA Class II or III symptoms of heart failure, a LVEF of 35% or less and nonsustained ventricular tachycardia.

2. Patients with NYHA Class I symptoms of heart failure who have left ventricular dysfunction (LVEF of 35% or less) associated with coronary artery disease or dilated cardio-myopathy and nonsustained ventricular tachycardia in whom sustained ventricular tachycardia or ventricular fibrillation induced during EPS.

Class IIa1. Patients with chronic heart failure associated with coronary

artery disease or dilated cardiomyopathy who have NYHA

V Implantable Cardioverter-Defibrillator Therapy



Class II or III symptoms of heart failure despite appropriate pharmacotherapy and a LVEF of 35% or less.

Class III1. Patients with idiopathic nonsustained ventricular tachycar-

dia not associated with structural heart diseases.

Large-scale prospective clinical studies have been conducted mainly in Europe and the United States,5–8,144,145 to assess the effects of ICD therapy for primary prevention of SCD in patients at a high risk of sudden death (i.e., patients with low LVEF or NYHA Class II or III symptoms of heart failure).

1. Primary Prevention of SCD in Patients With Coronary Artery Disease

The results of clinical studies conducted mainly in Europe and the United States support widespread use of ICD therapy for patients with coronary artery disease and low LVEF. On the other hand, cohort studies to evaluate the prognosis of patients with coronary artery disease in Japan have reported that patients have relatively favorable life prognosis and the incidence of sudden death is low.146,147 Accordingly, extended ICD indica-tions supported in Europe and the United States may not be so cost-effective in Japan.

Also, it has been demonstrated that the efficacy of ICD ther-apy is higher in patients with a longer period from the onset of myocardial infarction. ICD implantation as a primary preven-tion method should be performed in patients who survived their first myocardial infarction for at least one month.

2. Primary Prevention of Sudden Cardiac Death in Patients With Nonischemic Dilated Cardiomyopathy

The results of clinical studies conducted in Europe and the United States indicated that although ICD therapy was effec-tive in terms of the decrease in mortality in patients with nonisch-emic dilated cardiomyopathy associated with heart failure, the decrease was not statistically significant8. A meta-analysis of 5 studies on the effects of ICD therapy in this patient popula-tion indicated that relative mortality decreased by 31% with ICD therapy.148

In a cohort study of the prognosis of patients with chronic heart failure in Japan, SCD occurred in 15% of patients with heart failure (LVEF less than 30%).149 It is thus considered that the prognosis and incidence of SCD in patients with nonisch-emic dilated cardiomyopathy in Japan are similar to those in Europe and the United States.

3. Syncope of Unknown Etiology

Class I1. Patients with chronic heart failure associated with coronary

artery disease or dilated cardiomyopathy who have NYHA Class II or III symptoms of heart failure and an LVEF of 35% or less despite appropriate pharmacotherapy.

Class IIa1. Patients with moderate ventricular dysfunction (LVEF 36

to 50% and NYHA Class I symptoms) associated with coro-nary artery disease or dilated cardiomyopathy in whom ven-tricular tachycardia/fibrillation is induced during EPS.

Class III1. Patients without ventricular dysfunction in whom the pres-

ence of causes of fatal arrhythmias such as hypertrophic

cardiomyopathy, Brugada syndrome (including drug-induced Brugada syndrome), preexcitation syndrome and short QT syndrome is excluded, and ventricular tachycardia/fibrilla-tion is not induced during EPS.

Since ventricular tachycardia/fibrillation are major and signifi-cant causes of syncope, ICD therapy is indicated for patients with syncope suspected to be due to ventricular tachycardia/fibrillation.

Even patients with mild or moderate ventricular dysfunction (LVEF 36 to 50%) due to coronary artery disease or nonisch-emic dilated cardiomyopathy and NYHA Class I symptoms should be considered at a high risk of sudden death when ven-tricular tachycardia/fibrillation is induced during EPS, and may be indicated for ICD therapy.150–153

4. Implantable Cardioverter-Defibrillator Therapy in Specific Cardiac Diseases

1. Hypertrophic CardiomyopathyClass I1. Patients with a history of sustained ventricular tachycardia,

ventricular fibrillation or cardiopulmonary arrest.

Class IIa1. Patients who have nonsustained ventricular tachycardia; a

family history of sudden death; syncope; a left ventricular wall thickness of 30 mm or more; or abnormal blood pres-sure response during exercise.

Hypertrophic cardiomyopathy is an important myocardial dis-ease that causes sudden death especially in young patients under 40 years of age, and is often inheritable.154,155 Since patients with a history of sustained ventricular tachycardia or ventricu-lar fibrillation have a recurrence rate of about 10% per year, widespread use of ICD therapy is recommended.156 It has been reported that major risk factors for consideration of primary prevention with ICD therapy include nonsustained ventricular tachycardia; a family history of sudden death; syncope; a left ventricular wall thickness of 30 mm or more; and abnormal blood pressure response during exercise.

2. Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Class I1. Patients with a history of cardiac arrest, ventricular fibril-

lation, or hemodynamically unstable sustained ventricular tachycardia.

Class IIa1. Patients diagnosed with arrhythmogenic right ventricular

cardiomyopathy/dysplasia who have syncope of unknown etiology.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia is a major underlying heart disease that may cause SCD in young patients.157–161 Factors predicting appropriate ICD discharge include a history of cardiac arrest and hemodynamically unsta-ble sustained ventricular tachycardia.162 Regarding the use of the ICD for primary prevention of SCD, a history of syncope is believed to predict appropriate ICD discharge.163



3. Brugada SyndromeClass I1. Patients resuscitated from cardiac arrest.2. Patients with documentation of spontaneously terminating

ventricular fibrillation or polymorphic ventricular tachycar-dia.

Class IIa1. Patients who have Brugada type (coved type) ECG (Includ-

ing findings during pharmacological stress testing and ECG findings documented in a first intercostal space.) and meet at least two of the following three conditions:

1) History of syncope. 2) Family history of sudden death. 3) Ventricular fibrillation induced during EPS.

Class IIb1. Patients with Brugada type (coved type) ECG (Including

findings during pharmacological stress testing and ECG findings documented in a first intercostal space.) and meet only one of the above three conditions.

It has been reported that the 3-year incidence of arrhythmic events in patients with symptomatic Brugada syndrome is about 30%,164 while the incidence of sudden death is low in asymp-tomatic patients.165–168 However, ICD therapy is considered even for patients in whom ventricular fibrillation has not been documented, when they have typical ECG findings of Brugada syndrome; a history of syncopal attacks; polymorphic ven-tricular tachycardia induced during programmed stimulation; or a family history of sudden death.166,169,170

4. Congenital Long QT SyndromeClass I1. Patients with a history of ventricular fibrillation or cardiac

arrest.

Class IIa1. Patients with a history of torsade de pointes or syncope

who do not respond to β-blockers.2. Patients with a family history of sudden death who do not

respond to β-blockers.

Class IIb1. Patients with a history of torsade de pointes or syncope who

respond to β-blockers.2. Patients with a family history of sudden death who respond

to β-blockers.

Note: The efficacy of β-blockers should be evaluated accord-ing to symptoms and severity of QT prolongation during stress testing. β-blockers are considered ineffective in patients diag-nosed with LQTS type 3 (LQT3).

ICD therapy for the treatment of LQTS should be considered in 1) patients with recurrent syncope not responding to β-block-ers or other drugs, or patients resuscitated from sudden death; 2) patients whose first manifestation was resuscitated from cardiac arrest; 3) patients with a family history of SCD; and 4) patients with none-adherence to pharmacotherapy or patients meet the criteria for intolerance.171 However, the ICD is not a curative therapy, and may cause mental disorder due to fre-quent ICD discharges after implantation. The indication for ICD implantation in children especially should be evaluated carefully.172

Left Cervicothoracic Sympathetic Ganglionectomy for Congenital Long QT SyndromeClass INone.

Class IIb1. Patients with frequent ICD discharges after implantation

despite of treatment with β-blockers.2. Patients with syncope associated with torsade de pointes

despite of treatment with β-blockers.

Although few patients have undergone left cervicothoracic sympathetic ganglionectomy in Japan, this procedure has been used to treat drug-resistant patients in Europe and the United States, and favorable results have been reported in the treat-ment of drug-resistant congenital LQTS.173–175

5. Implantable Cardioverter-Defibrillators in Children

Few children have undergone ICD implantation176 since the prevalence of fatal arrhythmia associated with structural heart disease such as ischemic heart disease is low among children, and the rate of resuscitation is lower in children than adults,177 among other reasons. Since the most common causes of SCD in children include cardiomyopathy, congenital heart diseases and arrhythmias,178 and SCD due to arrhythmia is especially common,159,179 children receiving ICD implantation after resus-citation is expected to increase in the future. In fact, successful resuscitation of children has increased in Japan, since auto-mated external defibrillator (AED) become widely available in Japan; public education was provided to emphasize the impor-tance of basic life support (BLS); and the use of AED by emergency medical technicians and bystanders was allowed in 2003 and July 2007, respectively. In the schools, it is highly likely that children can survive without sequelae when teach-ers and nursing teachers try to resuscitate them using BLS and AED. The results of multicenter clinical studies of ICD ther-apy in children and patients with congenital heart disease have been published.180 The following recommendations were pre-pared according to the guidelines for the diagnosis and treat-ment of arrhythmia in children proposed by the Japanese Soci-ety of Pediatric Cardiology and Cardiac Surgery181 and the ACC/AHA/Heart Rhythm Society (HRS) 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities.54

1. Indications for ICD Implantation in Children and Patients With Congenital Heart Diseases

ICD implantation in children is quite rare in Japan, and in most cases ICD implantation is conducted for secondary prevention.

Class I1. Patients resuscitated from cardiac arrest after evaluation to

define the cause of the event and to exclude any reversible causes.

2. Patients with symptomatic sustained ventricular tachycar-dia associated with congenital heart disease (catheter abla-tion or surgical repair may offer possible alternatives in carefully selected patients).

Class IIa1. Patients with congenital heart disease with recurrent syncope

of unknown etiology in the presence of either ventricular dysfunction or inducible ventricular arrhythmias during EPS.



Class III1. Patients whose life expectancy is less than 12 months.2. Patients with frequent episodes of ventricular tachycardia/

fibrillation.3. Patients with severe mental disorders which may exacerbate

after ICD implantation or in whom follow-up after ICD implantation is expected to be difficult.

4. Patients with drug-resistant severe heart failure and NYHA Class IV symptoms who are not indicated for heart trans-plantation, CRT, or LVAD.

5. Patients with syncope of unknown etiology in the absence of both underlying heart disease and inducible ventricular tachycardia during EPS.

6. Patients with ventricular fibrillation/tachycardia who are amenable to catheter or surgical ablation (e.g., WPW syn-drome or idiopathic sustained ventricular tachycardia).

7. Patients with tachycardia due to reversible causes (e.g., elec-trolyte abnormality, drugs) in whom recurrent ventricular tachycardia/fibrillation may be prevented by eliminating the cause.

2. Hypertrophic CardiomyopathyA major cause of death among young patients with hypertro-phic cardiomyopathy is sudden death.182–195 The risk of sudden death is high in patients with nonsustained ventricular tachy-cardia;192 those with ventricular tachycardia;193 those with a left ventricular wall thickness of 30 mm or more;192 those with increased interventricular septal thickness or left ventricular posterior wall thickness;194 those with increased QTc disper-sion (20 ms or more) or myocardial bridge in the anterior descending branch of left coronary artery confirmed by coro-

nary angiography;193 those with heart failure;177,194 and young children under 3 years of age with heart failure.187 Indications for ICD therapy in children with hypertrophic cardiomyopathy are similar to those in adult patients.

3. Long QT SyndromeAmong arrhythmias considered as the cause of sudden death of children in the schools, LQTS is the most common cause.195–204 Among patients with congenital LQTS diagnosed during the neonatal and infantile periods, the incidence of clinically sig-nificant symptoms is highest among those with LQTS type 2 (LQT2) and LQT3.205 Patients who developed symptoms related to drug-resistant LQTS receive pacemaker therapy or ICD implantation as secondary prevention.206–210 For detailed infor-mation regarding ICD therapy in children with LQTS, readers should refer to the “Guidelines for Diagnosis and Manage-ment of Patients with Long QT Syndrome and Brugada Syn-drome (JCS 2007; 2005–2006 Joint Working Groups Report9”.

4. Adult Patients With Congenital Heart DiseaseTetralogy of Fallot and complete transposition of the great arteries are the most common underlying diseases211–216 asso-ciated with postoperative sudden death in adult patients with congenital heart diseases.217,218 Among patients with tetralogy of Fallot, sudden death has been related to the use of transan-nular patch;213 moderate or severe pulmonary regurgitation;214, 217 a history of ventricular tachycardia;217 a QRS interval more than equal to 180 msec217 or more than 120 msec;216 a LVEF less than 40%;217 complete AV block lasting for 3 days or more after surgery;215 and longer duration after the surgery,216,217 among other factors.

Patients with heart failure may often develop intraventricular conduction disturbance, AV dyssynchrony, intraventricular dyssynchrony, and interventricular dyssynchrony. CRT with biventricular pacing can reduce these dyssynchrony conditions. CRT may prevent the progression of heart failure and improve the prognosis in patients with moderate or severe chronic heart failure who have cardiac contractile dysfunction and cardiac dyssynchrony.219 However, CRT may not be effective in all patients with heart failure. Important factors predicting the efficacy of CRT include low LVEF and wide QRS complex on the ECG. Many clinical studies have been conducted in patients with a QRS interval of 120 to 150 msec. In the present guidelines, a wide QRS complex is defined as a QRS interval of 120 msec or more. CRT is highly effective in patients with a wide QRS complex of 150 msec or more (see below). Fur-ther studies are required to consider the indications of CRT in patients with mild heart failure. Although diagnostic imaging techniques such as echocardiography are expected to detect left ventricular contraction dyssynchrony, no standard criteria have been developed to detect the presence of dyssynchrony and evaluate responses to and effectiveness of CRT.

On the other hand, it is believed that CRT may decrease the total mortality of patients with heart failure but does not affect the incidence of SCD.220 It has been reported that CRT with ICD backup may further decrease total mortality by prevent-ing SCD.221 CRT-D is recommended for patients who are indicated for both CRT and ICD (Class I or IIb).

Cardiac Resynchronization Therapy (CRT-P [CRT Device That Provides Pacing But Not Defibrillation Capability])Class I1. Patients with NYHA Class III or ambulatory Class IV symp-

toms of chronic heart failure despite optimal pharmacother-apy, a LVEF of 35% or less, a QRS interval of 120 msec or more, and sinus rhythm.

Class IIa1. Patients with NYHA Class III or ambulatory Class IV symp-

toms of chronic heart failure despite optimal pharmaco-therapy, a LVEF of 35% or less, a QRS interval of 120 msec or more, and AF.

2. Patients with NYHA Class III or ambulatory Class IV symp-toms of chronic heart failure despite optimal pharmaco-therapy, a LVEF of 35% or less, and who have received or are planned to receive permanent pacemaker implantation, and have depended on or are expected to require frequent ventricular pacing.

Class IIb1. Patients with NYHA Class II symptoms of chronic heart

failure despite optimal pharmacotherapy, a LVEF of 35% or less, and who are planned to receive permanent pace-maker implantation and are expected to require frequent ventricular pacing.

VI Cardiac Resynchronization Therapy and Cardiac Resynchronization Therapy Device That Incorporates Both Pacing and Defibrillation Capabilities



Class III1. Asymptomatic patients with low LVEF who are not indi-

cated for pacemaker therapy.2. Patients whose physical function is limited by chronic con-

ditions other than heart failure, or patients whose life expec-tancy is less than 12 months.

Cardiac Resynchronization Therapy Device That Incorporates Both Pacing and Defibrillation CapabilitiesClass I1. Patients with NYHA Class III or ambulatory Class IV symp-

toms of chronic heart failure despite optimal pharmaco-therapy, a LVEF of 35% or less, a QRS interval of 120 msec or more, and sinus rhythm, and who are indicated for ICD therapy.

Class IIa1. Patients with NYHA Class III or ambulatory Class IV symp-

toms of chronic heart failure despite optimal pharmaco-therapy, a LVEF of 35% or less, a QRS interval of 120 msec or more, and AF, and who are indicated for ICD therapy.

2. Patients with NYHA Class II symptoms of chronic heart

failure despite optimal pharmacotherapy, a LVEF of 30% or less, a QRS interval of 150 msec or more, and sinus rhythm, and who are indicated for ICD therapy.

3. Patients with chronic heart failure NYHA Class III or ambu-latory Class IV symptoms with optimal pharmacotherapy, a LVEF of 35% or less, and who have received or are planned to receive ICD therapy, and have depended on or are expected to require frequent ventricular pacing.

Class IIb1. Patients with NYHA Class II symptoms of chronic heart

failure despite optimal pharmacotherapy, a LVEF of 35% or less, and who are planned to receive ICD therapy and are expected to require frequent ventricular pacing.

Class III1. Asymptomatic patients with low LVEF who are not indi-

cated for ICD therapy.2. Patients whose physical function is limited by chronic con-

ditions other than heart failure, or patients whose life expec-tancy is less than 12 months.

Surgeries for cardiac arrhythmias were developed as curative techniques, and have significantly contributed to the under-standing of anatomy and electrophysiology of arrhythmias as well as the advancement of non-pharmacological procedures such as catheter ablation. The first surgical procedure for arrhythmias, ventricular aneurysmectomy developed for patients with ventricular tachycardia, was reported in 1959.222 Electro-physiological procedures such as surgical interruption of the accessory pathway was then established as a surgical proce-dure for patients with WPW syndrome,223,224 and Cox et al. developed the Maze procedure for patients with AF.225,226 As catheter ablation techniques for WPW syndrome become more sophisticated and widespread, the Maze procedure and surgi-cal treatment of ventricular tachycardia become the most com-mon surgical procedures for arrhythmias. The results of long-term follow-up of patients after arrhythmia surgeries should be examined and the development of more curative and mini-mally invasive surgical procedures are awaited.

1. Atrial Fibrillation

Indications for Surgical Treatment of Atrial FibrillationClass I1. Patients with AF associated with mitral valve disease who

undergo cardiac surgery for the treatment of mitral valve disease.

Class IIa1. Patients with AF who undergo cardiac surgery for the treat-

ment of structural heart disease other than mitral valve disease.

2. Patients with AF who are complicated by left atrial throm-bus resistant to thrombolytic and/or anticoagulation ther-apy, or have a history of embolism caused by left atrial thrombus despite appropriate anticoagulation therapy.

3. Patients with AF in whom catheter ablation failed or AF recurred.

Class IIb1. Patients with lone AF who have severe symptoms such as

palpitation and a significant deterioration of QOL, and who cannot receive pharmacotherapy due to intolerance or adverse drug reactions.

2. Patients with drug-resistant paroxysmal AF who have repeat-edly received emergency care such as defibrillation.

The Maze procedure was developed as a curative treatment, and has improved to simplify the techniques, to ensure less invasive procedures and physiological recovery of atrial exci-tation such as by changing atrial incision lines, using cryoabla-tion and radiofrequency energy alternative to surgical inci-sions, and simplifying the incision lines.227–232

In patients with AF associated with mitral valve diseases, the incidence of postoperative cerebral infarction decreases when they receive the Maze procedure concomitant to mitral valve surgery.233 The Maze procedure terminates AF by elec-trical isolation of the pulmonary veins in which rapid repeti-tive excitation is occurring, and by preventing reentry by atrial incision lines. Basically, AF may be terminated after pulmo-nary vein isolation only in some patients,228 while sinus rhythm may not be restored after the Maze procedure in other patients. Further studies such as electrophysiological evaluation through intraoperative mapping should be conducted in the future.234 Although surgical treatment of AF has become straightfor-ward and less invasive using current ablation devices,231 fur-ther advancement of the devices and development of reliable methods to verify successful conduction block are necessary.235

2. Other Supraventricular Tachycardia

Indications for Surgical Treatment of Other Supraventricular TachycardiaClass I1. Patients with typical atrial flutter or intra-atrial reentrant

tachycardia who undergo cardiac surgery for the treatment

VII Surgery



of structural heart disease.2. Patients with supraventricular tachycardia that developed

after the Fontan-type operation (total cavopulmonary con-nection, TCPC) with extracardiac conduit or other tech-niques and in whom catheter ablation is impossible or dif-ficult due to their unique hemodynamics.

3. Patients with drug-resistant supraventricular tachycardia associated with WPW syndrome or other conditions with serious symptoms or a significant deterioration of QOL, and in whom catheter ablation was unsuccessful or tachy-cardia recurred.

Class IIa1. Patients with supraventricular tachycardia that developed

after surgery of congenital heart disease who have hemo-dynamic instability during tachycardia, and in whom cath-eter ablation was unsuccessful or tachycardia recurred.

Surgical treatment of arrhythmias is indicated in patients who have typical atrial flutter or intra-atrial reentrant tachycardia who need surgical treatment of structural heart disease and patients who have isthmus-dependent atrial flutter or incisional atrial reentry that developed in the long-term period after the operation requiring complex atrial incisions such as the Fon-tan-type, Mustard and Senning operations during infancy or early childhood.236–243 Surgical treatment of arrhythmias is also indicated in patients in whom catheter ablation is impossible or was unsuccessful or tachycardia recurred after catheter abla-tion.244–246

3. Ventricular Tachycardia

Indications for Surgical Treatment of Ventricular TachycardiaClass I1. Patients with sustained monomorphic ventricular tachycar-

dia due to underlying heart disease in whom pharmaco-therapy, catheter ablation and ICD therapy are ineffective or cannot be used, and ventricular tachycardia can be induced repeatedly.

2. Patients with idiopathic sustained ventricular tachycardia, severe symptoms and a significant deterioration of QOL, in whom pharmacotherapy is ineffective or cannot be used, in whom catheter ablation was unsuccessful or tachycardia recurred, and in whom repeated catheter ablation is not expected to be successful.

3. Patients with frequent episodes of ventricular tachycardia or frequent ICD discharges that cannot be suppressed by pharmacotherapy and/or catheter ablation.

Class IIa1. Patients with sustained monomorphic ventricular tachycar-

dia associated with myocardial infarction and who have heart failure or thromboembolism due to ventricular aneu-rysm or left ventricular akinesis.

Surgical treatment of ventricular tachycardia is indicated for patients with ventricular tachycardia associated with underly-ing heart disease in whom conventional antiarrhythmic treat-ment is ineffective or cannot be used.247–253 Patients with sus-tained monomorphic ventricular tachycardia should undergo surgery directed by preoperative or intraoperative mapping.252,253 Patients with sustained monomorphic ventricular tachycardia associated with myocardial infarction should undergo surgical

treatment of ventricular tachycardia concomitant to the surgi-cal procedures such as ventricular aneurysmectomy and left ventricular reconstruction for the treatment of heart failure and/or thromboembolism due to ventricular aneurysm or left ventricular akinesis.222 Surgery is indicated for patients with frequent episodes of ventricular tachycardia and frequent ICD discharges, such as those with electrical storm, in whom other procedures would be ineffective.

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AppendixChair: • Ken Okumura, Department of Cardiology, Respiratory Medicine and

Nephrology, Hirosaki University Graduate School of Medicine

Members: • Yoshifusa Aizawa, Department of Research and Development, Tachikawa

Medical Center • Kazutaka Aonuma, Cardiovascular Division, Institute of Clinical Med-

icine, University of Tsukuba Graduate School of Comprehensive Human Sciences

• Nobuhisa Hagiwara, Department of Cardiology, Tokyo Women’s Med-ical University

• Fumitaka Isobe, Department of Cardiac Surgery, Aichi Medical Uni-versity School of Medicine

• Takao Katoh, Nippon Medical School • Kazuo Matsumoto, Department of Cardiology, Saitama Medical Uni-

versity International Medical Center • Yuji Murakawa, Fourth Department of Internal Medicine, Mizonokuchi

Hospital, Teikyo University School of Medicine • Takashi Nitta, Department of Cardiovascular Surgery, Nippon Medical

School • Satoshi Ohnishi, Department of Cardiology, NTT East Kanto Medical

Center • Akihiko Shimizu, Faculty of Health Sciences, Yamaguchi University

School of Medicine

Collaborators: • Kenzo Hirao, Heart Rhythm Center, Tokyo Medical and Dental Uni-

versity • Toshiyuki Ishikawa, Department of Medical Science and Cardiorenal

Medicine, Yokohama City University Graduate School of Medicine • Toru Iwa, Department of Cardiology, Aichi Medical University School

of Medicine • Takashi Kurita, Division of Cardiology, Department of Medicine, Fac-

ulty of Medicine, Kinki University • Kengo Kusano, Department of Cardiovascular Medicine, Okayama

University Graduate School of Medicine, Dentistry and Pharmaceu-tical Sciences

• Yoshihide Nakamura, Department of Pediatrics, Kinki University School of Medicine

• Yuji Nakazato, Department of Cardiology, Juntendo University Urayasu Hospital

• Shinichi Niwano, Department of Cardio-andiology, Kitasato University School of Medicine

• Shingo Sasaki, Department of Advance Management of Cardiac Arrhyth-mias, Hirosaki University Graduate School of Medicine

• Tsuyoshi Shiga, Department of Cardiology, Tokyo Women’s Medical University

• Morio Shoda, Department of Cardiology, Tokyo Women’s Medical University

• Naokata Sumitomo, Department of Pediateics and Child Health, Nihon University School of Medicine

• Jun Umemura, Department of Cardiology, Sakakibara Heart Institute

Independent Assessment Committee: • Hiroshi Kasanuki, Integrated Bioscience and Biomedical Engineering,

Graduate School of Advanced Science and Engineering, Waseda University

• Takeshi Kimura, Department of Cardiovascular of Medicine, Graduate School of Medicine Kyoto University

• Itsuo Kodama, Nagoya University • Tohru Ohe, Department of Cardiology, Sakakibara Heart Institute of

Okayama • Shigeo Tanaka, Sayama Chuo Hospital

(The affiliations of the members are as of July 2012)