Joint Position Paper of the Working Group of Pacing and Electrophysiology of the French Society of Cardiology (SFC) and the Société française d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV) on magnetic resonance imaging in patients with cardiac electronic implantable devices.

Magnetic resonance imaging (MRI) has become the reference imaging for the management of a large number of diseases. The number of MR examinations increases every year, simultaneously with the number of patients receiving a cardiac electronic implantable device (CEID). A CEID was considered an absolute contraindication for MRI for years. The progressive replacement of conventional pacemakers and defibrillators by MR-conditional CEIDs and recent data on the safety of MRI in patients with "MR-nonconditional" CEIDs have progressively increased the demand for MRI in patients with a CEID. However, some risks are associated with MRI in CEID carriers, even with "MR-conditional" devices because these devices are not "MR-safe". A specific programing of the device in "MR-mode" and monitoring patients during MRI remain mandatory for all patients with a CEID. A standardized patient workflow based on an institutional protocol should be established in each institution performing such examinations. This joint position paper of the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Société française d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV) describes the effect and risks associated with MRI in CEID carriers. We propose recommendations for patient workflow and monitoring and CEID programming in MR-conditional, "MR-conditional nonguaranteed" and MR-nonconditional devices.

Influence of the time on the prevalence of drug-related resuscitated sudden death during these past 20 years.

UNLABELLED: The use of drugs is the subject of numerous recommendations. The purpose of this study was to evaluate the prevalence of drug-related sudden deaths (SD) and the possible changes during these past 20 years. METHODS: 271 patients, 205 men, 66 women aged from 12 to 88 years (mean 59 ± 15) were admitted after SD resuscitation outside the acute phase of myocardial infarction, 146 before 2000 (group I), 125 between 2000 and 2010 (group II). Complete check-up was performed. RESULTS: Ischemic HD (41%) vs (37%), idiopathic dilated cardiomyopathy (12%) vs (11%), various HD (5%) vs (8%) were as frequent in groups I and II. Valvular HDs were more frequent in group I than II (12%) vs 6% (p<0.01). Abnormalities at ECG (preexcitation syndrome, conduction disturbance, atrial fibrillation or ion channel disorders) were less frequent in group I than II (8%) vs (18%) (p<0.02). Drug-facilitated or related SD's did not change in groups I and II: 54 patients presented a drug-related ventricular fibrillation or asystole, 16% in group I and 24% (NS) in group II. SD was caused by hypokalemia, QT interval increase or conduction disturbance. HD or abnormal ECG was present in 42 patients. Digoxin, diuretics, calcium inhibitors, betablockers, antiarrhythmic drugs alone or in association were mainly implicated. CONCLUSION: Drug-related arrhythmias continue to explain or favour at least 20% of SDs. Despite numerous recommendations on the use of drugs, the prevalence of fatal events that may be attributed to a cardiovascular drug does not decrease between the years before 2000 and after 2000.

Does syncope require rhythmic and non-rhythmic evaluation in patients with previous MI?

BACKGROUND: Multiple factors, in addition to left ventricular ejection fraction (LVEF) influence the risk of mortality in coronary artery disease. The purpose of this study was to evaluate the main causes of syncope after myocardial infarction (MI) and to propose an algorithm of management. METHODS: 356 patients consecutively admitted for syncope and history of MI (>1 month), without ventricular tachycardia (VT), underwent echocardiography, Holter monitoring, head-up tilt test, exercise testing, signal-averaged ECG, electrophysiological study (EPS) and evaluation of coronary status. The mean follow-up was 4±2 years. RESULTS: Monomorphic VT, ventricular flutter or fibrillation (VF) and supraventricular tachyarrhythmia were respectively induced at EPS in 87, 63 and 39 patients; conduction disturbances were noted in 23 patients, and 57 patients had several abnormalities. Among the 144 patients with negative EPS, coronary ischaemia was identified in 37 patients, and hypervagotonia in 27 patients. All studies remain negative in 84 patients (23.6%), more frequently women (p<0.001). Four patients died suddenly during follow-up. A longer QRS duration, a lower LVEF and grade IVa,b of Lown on Holter ECG were associated with the induction of VT. LVEF<40% and VT/VF induction were predictors of cardiac mortality, VT was a predictor of sudden death, and low LVEF and advanced age were predictors of death by heart failure. CONCLUSION: Myocardial ischaemia, hypervagotonia, conduction abnormalities, ventricular or supraventricular tachyarrhythmias were identified in 76% of patients with syncope after MI. Several factors of syncope were found in 57 patients (16%). Non-invasive rhythmological and systematic coronary status assessment should be recommended in patients with syncope following MI.

[Complete electrophysiological study remains necessary in symptomatic advanced cardiomyopathy]. / Une étude électrophysiologique complète reste nécessaire dans une cardiopathie évoluée symptomatique.

Multiple mechanisms can explain a tachycardia associated or not with a heart disease. It is important to establish its origin, particularly if advanced heart disease is present, for the treatment. Complete electrophysiological study remains an important means of diagnosis. We report the case of a 39-year old man, who had a dilated cardiomyopathy since the age of 25 years and who complained of tachycardia. These symptoms were shown to be related to atrioventricular nodal re-entrant tachycardia at electrophysiological study and a specific treatment was indicated.

[Non-valvular cardiac devices endocarditis]. / L'endocardite sur matériel de stimulation intracardiaque.

The risk of infective endocarditis on pacemaker or ICD is not negligible and has increased in recent years. Several host-related, procedure-related, or device-related risk factors have been recognized. Owing to its potential severity, the possibility of infective endocarditis should be envisaged in patients with repeated pulmonary infections or documented bacteremia and transesophageal echocardiography should then be used. The most common germs causing pacemaker endocarditis are staphylococci. Treatment requires prolonged antibiotic therapy and retrieval of the pacemaker and leads.

[Significance of permanent atrial fibrillation in idiopathic dilated cardiomyopathy]. / Signification de la fibrillation auriculaire permanente dans la cardiomyopathie dilatée primitive.

UNLABELLED: The significance of atrial fibrillation (AF) in idiopathic dilated cardiomyopathy (IDCM) remains discussed. The purpose of the study was to evaluate the clinical significance of permanent atrial fibrillation in patients with IDCM. METHODS: Systematic noninvasive and invasive studies including Holter monitoring, measurement of left ventricular ejection fraction (LVEF), electrophysiological study and coronary angiography were performed in 323 patients with IDCM; all patients had a left ventricular ejection fraction (LVEF)<40%. The studies were indicated for spontaneous ventricular tachycardia (VT) in 69 patients, syncope in 103 patients and nonsustained VT on Holter monitoring in 151 asymptomatic patients. Sixty-five patients were in permanent AF (group I). Remaining patients were in sinus rhythm at the time of evaluation (group II). Programmed ventricular stimulation using up to 3 extrastimuli in control state and if necessary after isoproterenol was systematic. Patients were followed 3+/-2 years. RESULTS: Mean age was significantly older in group I (61+/-8 years) than in group II (52+/-12) (P<0.01). Syncope (31 vs 36%), spontaneous sustained VT (18 vs 23%); mean LVEF (28+/-9% vs 29+/-9%), VT induction (25 vs 35%) were similar in both groups. During the follow-up, there were no statistical differences between groups I and II concerning each event: sudden death occurred in 13 patients, 1.5% of group I patients and 5% of group II patients (NS); a death related to heart failure occurred 22 patients, 5% of group I patients and 7% of group II patients (NS); heart transplantation was performed in 13 patients, 8% of group I patients and 3% of group II patients (NS). CONCLUSIONS: An older age is the only significant clinical factor associated with the presence of a permanent atrial fibrillation in idiopathic dilated cardiomyopathy. The presence of permanent AF does not increase the induction of a sustained ventricular tachycardia and does not affect the general prognosis of IDCM.

[Prevalence of sudden cardiac death during sports activities]. / Prévalence de la mort subite au cours du sport.

Sudden death during sport is a rare and unexpected event. It essentially affects young males, and a cardiomyopathy that had not been diagnosed during medical examinations is present in the majority of cases. In young subjects, there is generally hypertrophic cardiomyopathy or arhythmogenic right ventricular dysplasia. This is revealed during sporting activity, and sudden death is often the first symptom of the disease. Competitive sport increases the relative risk of sudden death to 2.5 compared to the risk in a non-sporting subject. The prevalence of sudden death during competitive sport is poorly understood. From the rare studies available, it could be estimated at 2.3/100,000 athletes per year. In Europe, it essentially occurs during football matches. However, the prevalence of sudden death during so-called 'recreational' sports is not precisely known. It could be much higher because these activities involve a larger number of people, and take place without supervision and usually without a medical examination beforehand. The participants are older, and coronary pathology is usually implicated.

Supraventricular tachyarrhythmia as a cause of sudden cardiac arrest.

INTRODUCTION: Supraventricular tachyarrhythmias (SVTA) are an accepted cause of cardiac arrest in patients with Wolff-Parkinson-White syndrome (WPW) and hypertrophic cardiomyopathy but their participation in other conditions is less well understood. The purpose of the study was to examine the role of SVTA in sudden cardiac arrest (SCA) by comprehensive evaluation of patients successfully resuscitated from SCA. METHODS: A total of 169 survivors of SCA in the absence of acute myocardial infarction underwent systematic evaluation that included echocardiography, Holter monitoring, coronary angiography and electrophysiological study (EPS) with additional testing in selected cases using provocative drug testing with isoproterenol, ajmaline or ergonovine. RESULTS: SVTA was found as the only possible cause or as the cause facilitating SCA in 29 patients: (1) 3 had a WPW syndrome related to accessory pathway with short refractory period; (2) for 12 patients, SVTA was the cause of cardiovascular collapse; heart disease (HD) was present in 11 cases, but disappeared in two of four with dilated cardiomyopathy after the restoration of sinus rhythm; (3) in 14 patients, SVTA degenerated either in a VF or ventricular tachycardia (VT); HD was present in 12 cases, but disappeared in one; two had no HD and recurrent similar arrhythmia was documented by cardiac defibrillator in one of them. SVTA induced coronary ischemia was the main cause of SCA. CONCLUSION: Rapid SVTA was a cause of SCA, either by cardiovascular collapse or by the degeneration in VT or VF. The complication generally occurred in patients with advanced HD or with rapid SVTA-induced cardiomyopathy and rarely in patients without HD. The incidence of SVTA as the only cause or the facilitating cause of SCAs is probably underestimated, because it is difficult to prove.

[Implantable cardioverter defibrillators in pediatric patients: results in a series of 33 cases]. / Le défibrillateur automatique implantable chez l'enfant et l'adolescent: indications, complications et résultats à propos d'une étude multicentrique française de 33 cas.

UNLABELLED: Implantable defibrillator is the recognized treatment of sudden cardiac death. Miniaturization of the devices allows implantation in children. METHODS: This multicentric retrospective study analyzed data of 33 children aged 18 years and less who were implanted from 1990 to 2005. RESULTS: Age of patients are 10 to 18 years, 20 patients were implanted after a resuscitation of sudden death, 10 after a syncope, 2 after a ventricular tachycardia and 1 in a prophylactic way. Tachycardias on primary electrical disease are most frequent (46%). Hypertrophic cardiomyopathy accounts for 22%, DAVD for 14%, congenital cardiopathies for 12%. Seventeen patients received appropriate shocks and 14 patients had inappropriate shocks. There were two unexplained deaths. Five leads fractures and two device infections were noted. CONCLUSION: Implantable defibrillator is an effective treatment for children high-risk of sudden death. Occurrence of inappropriate shocks due to sinusal tachycardia, infections and leads fractures are frequent.

[Prevalence of supraventricular tachycardia and tachyarrhythmias in resuscitated cardiac arrest]. / Incidence des tachycardies et tachyarythmies supraventriculaires dans les morts subites récupérées.

Supraventricular arrhythmias are considered to be benign when the ventricular rate is slowed and treated by anticoagulants. The aim of this study was to determine the possible influence of these arrhythmias in resuscitated cardiac arrest. Between 1980 and 2002, 151 patients were admitted after a cardiac arrest. Supraventricular arrhythrmias were identified as a possible cause of the cardiac arrest in 21 patients. They underwent echocardiography, exercise stress test, Holter ECG monitoring , coronary angiography and electrophysiological investigation. After these investigations, three patients had a malignant form of the Wolff-Parkinson-White syndrome, two were asymptomatic and, in the third patient, ventricular fibrillation was induced by treatment with diltiazem. In 8 patients, a rapid supraventricular arrhythmia was considered to be the cause of cardiac arrest by cardiogenic shock; 2 patients had hypertrophic cardiomyopathy, 5 had severe dilated cardiomyopathy which regressed in one patient. In ten patients, cardiac arrest due to ventricular tachycardia or fibrillation was provoked by a rapid (> 220 beats/min) supraventricular arrhythmia; two patients had no apparent underlying cardiac pathology. In the others, myocardial ischaemia or acute cardiac failure were considered to be the cause of the cardiac arrest. The authors conclude that rapid supraventricular arrhythmias may cause cardiac arrest either by cardiogenic shock or degenerescence to ventricular tachycardia or fibrillation. Usually, this event occurs in patients with severe cardiac disease but it may occur in subjects without cardiac disease or by an arrhythmia-induced cardiomyopathy.

[Prevalence of Brugada syndrome among 35,309 inhabitants of Lorraine screened at a preventive medicine centre]. / Prevalence du syndrome de Brugada dans une population de médecine préventive en Lorraine a propos de 35,309 cas.

UNLABELLED: Brugada syndrome is a recently identified cause of sudden death. Its primary prevention remains controversial, and epidemiology poorly defined. PATIENT POPULATION AND METHODS: Electrocardiograms (ECG) of 35,309 individuals (mean age = 37.2 years, 47% men) recorded over a 1-year period were reviewed and classified as (1) typical, (2) suspicious, and (3) negative. Subjects whose ECG was suspicious were offered a provocative test with flecainide, 2 mg/kg, i.v., and individuals whose ECG was typical were advised to undergo programmed ventricular stimulation (PVS). RESULTS: In 14 men and 6 women between the ages of 24 and 77 years (mean =47.5), ECGs were typical (n=6) or suspicious (n=14). Among 6 subjects with typical ECGs, 3 underwent PVS, which was positive in 1, who received an implantable cardioverter defibrillator (ICD). Among 14 subjects whose ECGs were suspicious, 5 declined further investigations and 5 developed typical ECG characteristics of Brugada syndrome after flecainide administration. PVS was negative in 4 subjects who consented to the procedure. Overall, among 35,309 individuals screened, 11 had ECG findings consistent with Brugada syndrome and, over a follow-up of 30 months, all had remained free of adverse cardiac event. CONCLUSIONS: we estimated a prevalence of Brugada syndrome of 0.3% in Lorraine. A single patient received an ICD for inducible ventricular tachyarrhythmia during PVS, representing a potential 30 per million asymptomatic adult rate of ICD implantation for this indication.

[New systems of mapping and navigation in electrophysiology]. / Nouveaux systèmes de cartographie et de navigation en électrophysiologie.

The indications of radiofrequency ablation of arrhythmias have considerably increased since the introduction of the technique in the early 1990s. Interventional rhythmologists now treat arrhythmias which are more and more complex by their mechanism. This requires accurate representation of the ablation catheter position and the integration of spatial and temporal data to identify the arrhythmogenic substrate. The systems of mapping and navigation developed over the last ten years are important tools for interventional rhythmologists. They are very useful for the identification of complex arrhythmogenic substrates which require "individualised" ablations in specific cases. The aim of this article is to review different systems of mapping, and/or navigation currently on the market and their principal characteristics without entering into the details of their use in interventional electrophysiology.

[Does syncope change the results of programmed ventricular stimulation in patients with previous myocardial infarction?]. / La syncope change-t-elle les résultats de la stimulation ventriculaire programmée chez les patients ayant un antécédent d'infarctus du myocarde?

UNLABELLED: The induction of a ventricular tachycardia (VT) after myocardial infarction (MI) is associated with a high risk of VT and sudden death (SD) in asymptomatic patients; the purpose of the study was to know if syncope modifies the results of programmed ventricular stimulation (PVS) and the clinical consequences. METHODS: PVS using two and three extra stimuli delivered in two sites of right ventricle was performed in 1057 patients without spontaneous VT or resuscitated SD at least 1 month after an acute MI; 836 patients (group I) were asymptomatic and were studied for a low ejection fraction or nonsustained VT on Holter monitoring or late potentials; 228 patients (group II) were studied for unexplained syncope. The patients were followed up to 5 years of heart transplantation. RESULTS: Sustained monomorphic VT (< 280 b/min) was induced in 238 group I patients (28%) and 62 group II patients (29%); ventricular flutter (VT > 270 b/min) or ventricular fibrillation (VF) was induced in 245 group I patients (29%) and 42 group II patients (18%) (P < 0.05); PVS was negative in 353 group I patients (42%) and 124 (55%) group II patients (NS). The patients differ by their prognosis; cardiac mortality was 13% in group I patients and 34% in group II patients with inducible VT < 280 b/min (P < 0.01), 4% in group I patients and 13% in group II patients with inducible VF (P < 0.05), 5% in group I patients and 7% in group II patients with negative study (NS). In conclusion, syncope did not change the results of programmed ventricular stimulation after myocardial infarction. However, syncope increased significantly cardiac mortality of patients with inducible ventricular tachycardia, flutter or fibrillation.

[The electrocardiogram in ventricular tachycardias]. / Electrocardiogramme des tachycardies ventriculaires.

The electrocardiogram, as much as the clinical examination, is a basic tool for the cardiologist. Technological advances have led to a certain lack of interest in learning to read the electrocardiogram, for which close analysis can allow precise diagnosis, notably in the field of cardiac rhythm disorders. This article concerns the electrocardiogram in ventricular tachycardias with two themes: differential diagnosis of wide QRS complex tachycardias and recognition of the site of origin of a ventricular tachycardia. "Fine" analysis of the electrocardiogram is not an intellectual "game". Actually, careful analysis of the 12 lead ECG allows exact and rapid diagnosis in a large majority of cases, distinguishing a ventricular tachycardia from a supraventricular tachycardia with conduction defect; the appropriate management can be selected without delay. At the same time, close reading of the electrocardiogram also allows the site of origin of a ventricular tachycardia to be recognised. Combining this information with elements of the patient's record can allow the arrhythmia to be related to a known pathology or to prompt a targeted aetiological investigation.

Adult presentation of MCAD deficiency revealed by coma and severe arrythmias.

We report the case of a 33-year-old man who presented with headaches and vomiting. Soon after admission he became drowsy and agitated, developed ventricular tachycardia and his neurological state worsened (Glasgow coma score 6). Blood analysis showed respiratory alkalosis, hyperlactacidemia (8 mmol/l), hyperammonemia (390 micro mol/l) and hypoglycaemia (2.4 mmol/l). Subsequently, he developed supraventricular tachycardia, ventricular tachycardia and ultimately ventricular fibrillation resulting in cardiac arrest, which was successfully treated. A CT scan of the head revealed cerebral oedema. Whilst in the intensive care unit, he developed renal failure and rhabdomyolysis. The metabolic abnormalities seen at the time of admission normalised within 48 h with IV glucose infusion. Biological investigations, including urinary organic acids and plasma acylcarnitines, showed results compatible with MCAD deficiency. Mutation analysis revealed the patient was homozygous for the classical mutation A985G. This is one of only a few reports of severe cardiac arrhythmia in an adult due to MCAD deficiency. This condition is probably under-diagnosed in adult patients with acute neurological and/or cardiac presentations.

[Cardiac stimulation in the infant and young child]. / Stimulation cardiaque chez le nourrisson et le jeune enfant. Avantages et inconvénients respectifs de la voie endocavitaire et de la voie épicardique.

This study evaluates the problems and the evolution of cardiac stimulation in infants (aged < 3.5 years) by comparing the endocavity and epicardial routes in a retrospective series of 37 patients. Thirty seven patients aged 1.2 +/- 0.9 years treated with epicardial (n = 19) or endocavity (n = 18) stimulation were followed for 10.9 +/- 6.4 years (0.75-24). The 2 patient groups did not differ in age or weight. Four patients were lost to follow up, and 1 died. The functional duration of the first stimulator was not significantly different if the initial approach was epicardial or endocavity. The endocavity probes were introduced by venous denudation in 15 cases and by subclavian puncture in 3 cases. Fourteen of the 19 children fitted by the epicardial route went on to endocavity stimulation, of which 10 were at the first replacement. None of the 18 patients fitted by the endocavity route went on to epicardial stimulation. Out of 11 endocavity probe replacements and 9 atrialisations, the homolateral venous approach was always possible except in 2 cases. In conclusion, the results for the epicardial and endocavity routes are comparable. For technical reasons (calibre of the veins, size of the stimulator) it would appear reasonable if the endocavity route was used, making do initially with a mono chamber stimulation. The advances in the epicardial electrodes abolishes the major handicap (threshold elevation) of this approach which can be advocated when double chamber stimulation seems preferable.

[Isolated congenital complete atrio-ventricular block]. / Blocs auriculo-ventriculaires complets congénitaux isolés. Suivi à long terme d'une série consécutive de 54 patients.

The objective of this work was to study the long term evolution of a retrospective series of 54 patients affected with congenital isolated complete atrio-ventricular block (CAVB) and to analyse the value of the different methods used for surveillance. Our series included 54 patients affected with isolated CAVB, without associated cardiopathy, diagnosed at an average age of 5.3 +/- 5.5 years, of which 9 were in utero. The average duration of follow up was 14.5 +/- 9.6 years. During the evolution, a cardiac stimulator was placed in 41 patients (76%) at a relatively late average age of 13.3 +/- 9 years, significantly lower for CAVB diagnosed before the age of 1 year (9.6 +/- 7.4 years) than for those diagnosed after the age of 1 year (16.2 +/- 9.2 years) (p < 0.02). The approach was endocavitary in 39 cases and epicardial in 2 cases. The only 2 deaths in our series (4%) concerned 2 patients of 18 and 26 years already fitted with a stimulator. Three patients progressed to severe dilated cardiomyopathy despite implantation of a stimulator. The decisive arguments for implantation of a PM were clinical (11 patients), Holter ECG (25 patients), stress test (17 patients), electrophysiological investigation (5 patients), echocardiography (3 patients) and surgical intervention (2 patients). In conclusion, our study confirms the good prognosis of isolated congenital complete atrio-ventricular block, but underlines the possible progression in rare cases in spite of stimulation towards dilated cardiomyopathy for which the aetiology remains uncertain. Three quarters of the patients required a stimulator at a somewhat late age.

[Interferences in the everyday life of the patient with a cardiac pacemaker or an implantable defibrillator]. / Interférences de la vie quotidienne chez le patient avec stimulateur cardiaque ou défibrillateur implantable.

The electromagnetic sources of interferences being able to deteriorate the operation of cardiac pacemakers or implantable defibrillators are numerous. This potential risk has been known since the release, 40 years ago, of pacemakers incorporating a detection circuit. Many papers, reviewed in this article, have been published about these conflicts. In daily practice, the risk of dangerous interference is weak, but it seems obvious that the implantable defibrillators are much more sensitive to the external environment than the cardiac pacemakers. With some precautions to eliminate manifest risk situation, it is possible to carry out a strictly normal life. Diagnostic memories increasingly sophisticated included in the new prostheses make possible the identification of asymptomatic conflicts, and the specification of the mechanism of a real problem. Provided information may also be useful to find solutions (adjustment, precautions) to decrease, even to remove the risks.

[Characteristics and causes of death in 283 patients with implanted defibrillators]. / Caractéristiques et causes de décès chez 283 patients implantés d'un défibrillateur automatique.

UNLABELLED: The implantable automatic defibrillator has proved its superiority over pharmacological treatments for preventing mortality by serious ventricular arrhythmia. We studied the cause of death in a population of 283 consecutive patients implanted between February 1988 and December 2000 (age at implantation: 58 +/- 14.7 years; extremes: 15-78 years, 45 females, ejection fraction: 0.39 +/- 0.15) and followed up over a median of 25 months (extremes = 1 day-163 months). RESULTS: At the end of follow up, 55 patients had died (average age: 62.7 +/- 12.6 years, extremes: 15-79 years, 7 females). All except 2 had a cardiopathy: ischaemic cardiopathy (n = 38, 36 IDDM), dilated cardiomyopathy (n = 14), arrhythmogenic dysplasia of the right ventricle (n = 1). The median interval between implantation and death was 35 months (extremes = 1 day-137 months). The causes of death were the following: cardiac insufficiency (n = 24), refractory arrhythmias (n = 13), other cardiac causes (n = 8), extra-cardiac pathologies (n = 10). The deceased patients had presented an average of 86.6 +/- 23.4 ventricular arrhythmias (extremes = 0-1309) but 18 of them (33%) did not present any during follow up. CONCLUSIONS: Cardiac insufficiency is the prime cause of death in refractory arrhythmias; on patient in 4 dies from ventricular arrhythmia, despite the defibrillator and one deceased patient in 3 had no arrhythmia during follow up.