Recommendations for participation in leisure-time physical activity and competitive sports of patients with arrhythmias and potentially arrhythmogenic conditions. Part II: ventricular arrhythmias, channelopathies and implantable defibrillators.

This consensus paper on behalf of the Study Group on Sports Cardiology of the European Society of Cardiology follows a previous one on guidelines for sports participation in competitive and recreational athletes with supraventricular arrhythmias and pacemakers. The question of imminent life-threatening arrhythmias is especially relevant when some form of ventricular rhythm disorder is documented, or when the patient is diagnosed to have inherited a pro-arrhythmogenic disorder. Frequent ventricular premature beats or nonsustained ventricular tachycardia may be a hallmark of underlying pathology and increased risk. Their finding should prompt a thorough cardiac evaluation, including both imaging modalities and electrophysiological techniques. This should allow distinguishing idiopathic rhythm disorders from underlying disease that carries a more ominous prognosis. Recommendations on sports participation in inherited arrhythmogenic conditions and asymptomatic gene carriers are also discussed: congenital and acquired long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, arrhythmogenic right ventricular cardiomyopathy and other familial electrical disease of unknown origin. If an implantable cardioverter defibrillator is indicated, it is no substitute for the guidelines relating to the underlying pathology. Moreover, some particular recommendations for patients/athletes with an implantable cardioverter defibrillator are to be observed.     

Cardiac arrhythmias in the athlete

Cardiac arrhythmias in the athlete are a frequent cause for concern. Some arrhythmias may be benign and asymptomatic, but others may be life threatening and a sign that serious cardiovascular disease is present. Physicians often are consulted with regard to arrhythmias, or symptoms consistent with arrhythmias, in athletes. Sinus bradyarrhythmias are common and even expected in athletes. These bradyarrhythmias are rarely a cause for concern. Heart block is unusual and merits a thorough workup. Atrial fibrillation may be more common in the athlete. Supraventricular tachycardias other than atrial fibrillation generally warrant consideration of radiofrequency ablation for cure of the tachyarrhythmia. Ventricular arrhythmias in the athlete generally occur in the setting of structural heart disease that is genetically determined (hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia, anomalous coronary arteries) or acquired (coronary artery disease, myocarditis, idiopathic dilated cardiomyopathies). In these conditions, the arrhythmia generally is life threatening. Ventricular arrhythmias that occur in the athlete without structural heart disease are not thought to be life threatening. Athletes with structural heart disease and syncope and those with exertional syncope merit a complete evaluation.     

Catheter-based Ablation for Ventricular Arrhythmias

Catheter ablation for patients with recurrent ventricular arrhythmias has emerged as an important and effective treatment option. The approach to ablation, and the risks and likely efficacy are determined by the nature of the severity and type of underlying heart disease. Although implantable defibrillators remain the corner stone for prevention of sudden cardiac death, ablation successfully reduces tachycardia recurrences and storms of ventricular arrhythmias triggering defibrillator shocks in patients with structural heart disease. Our understanding of idiopathic ventricular tachycardia (VT) has grown substantially with several new sites of VT origin recognized in recent years. Ablation is often curative for idiopathic VT. This review discusses common mechanisms and clues to diagnosis of the various VTs, and current advances in ablation options. In particular, endocardial ablation techniques have been complemented by newer approaches such as percutaneous epicardial ablation. In rare cases, transcoronary alcohol ablation can be effective for life-threatening arrhythmia.     

Long-term outcome of pharmacological and nonpharmacological treatment for ventricular arrhythmias

Recent advances of nonpharmacological therapy such as catheter ablation and implantable cardioverter defibrillator and lessons from the Cardiac Arrhythmia Suppression Trial(CAST) have changed the strategy for ventricular arrhythmias. The safety and efficacy of radiofrequency catheter ablation of symptomatic sustained monomorphic ventricular tachycardia without structural heart disease has made ablation the firstline curative therapy. In idiopathic ventricular fibrillation such as Brugada syndrome, an implantable cardioverter defibrillator is the most effective treatment to prevent sudden cardiac death. In patients with asymptomatic ventricular tachyarrhythmias in heart failure, class I antiarrhythmic drugs should be avoided due to proarrhythmic and negative inotropic effects that may be responsible for increased mortality in some trials. In such patients, amiodarone and beta-blocker may reduce sudden cardiac death. For patients with sustained ventricular tachycardia or ventricular fibrillation in heart failure, amiodarone or implantable cardioverter defibrillator should be considered. In comparison with amiodarone, implantable cardioverter defibrillator markedly reduced sudden death in ventricular tachycardia and ventricular fibrillation survivors in Antiarrhythmics Versus Implantable Defibriltors(AVID). Although better patient selection and clarification of mapping criteria improved the successful ablation rate in patients with structural heart disease, candidates of ablation are few. In patients with extensive structural heart disease, multiple ventricular tachycardias are often present. Catheter ablation of a single ventricular tachycardia may be only palliative. Therefore, implantable cardioverter defibrillator is the most effective treatment to prevent sudden cardiac death, with amiodarone and ablation as the adjunctive therapy to prevent frequent ventricular tachycardia. Furthermore, an implantable cardioverter defibrillator improved survival in selected patients with depressed ventricular function after myocardial infarction, who also have nonsustained and inducible sustained ventricular tachycardia in Multicenter Automatic Defibrillator Implantation Trial(MADIT) and Multicenter Unsustained Tachycardia Trial(MUSTT).     

Implantable cardioverter-defibrillators

Implantable defibrillators have become the dominant therapeutic modality for patients with life-threatening ventricular arrhythmias. Current defibrillators are small (<60 mL) and implanted with techniques similar to standard pacemakers. They provide high-energy shocks for ventricular fibrillation and rapid ventricular tachycardia, antitachycardia pacing for monomorphic ventricular tachycardia, as well as antibradycardia pacing. Newer devices incorporating an atrial lead allow dual-chamber pacing and better discrimination between ventricular and supraventricular tachyarrhythmias. Randomized controlled trials have shown superior survival with implantable defibrillators than with antiarrhythmic drugs in survivors of life-threatening ventricular tachyarrhythmias and in high-risk patients with coronary artery disease. Complications associated with implantable defibrillator therapy include infection, lead failure, and spurious shocks for supraventricular tachyarrhythmias. Most patients adapt well to living with an implantable defibrillator, although driving often has to be restricted. Limited evidence suggests that implantable defibrillator therapy is cost-effective when compared with other widely accepted treatments. The use of implantable defibrillators is likely to continue to expand in the future. Ongoing clinical trials will define further prophylactic indications of the implantable defibrillator and clarify its cost-effectiveness ratio in different clinical settings.     

Do the spatial characteristics of myocardial scar tissue determine the risk of ventricular arrhythmias?

Sudden cardiac death is one of the main causes of mortality in patients with structural heart disease. Although an implantable cardioverter defibrillator significantly reduces the mortality rate, many patients never receive a shock. Identification of high-risk patients would reduce the costs associated with this therapy and prevent the deleterious effect of inappropriate discharges. As scar tissue is the substrate of ventricular arrhythmias in patients with structural heart disease, scar characterization could allow stratification of the risk. The objective of this article is to review the role of scar characteristics in the pathogenesis of ventricular arrhythmias in patients with structural heart disease.     

Arrhythmogene rechtsventrikuläre Kardiomyopathie

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart muscle disease and a major cause of sudden cardiac death and ventricular tachyarrhythmia in young, apparently healthy individuals and athletes. Patients affected by ARVC should be excluded from competitive sports and vigorous training. To provide optimal treatment, early diagnosis and risk stratification are mandatory and genetic counseling of families is recommended. Tailored treatment strategies aim at the prevention of ventricular tachyarrhythmia and sudden death as well as the preclusion of disease progression and symptomatic heart failure. Patients with a low risk of sudden death need either no specific treatment or can be treated with beta blockers or antiarrhythmic drugs, depending on the clinical manifestation of the arrhythmia. Catheter ablation in ARVC constitutes a symptom-oriented and palliative approach for frequently relapsing ventricular tachycardia refractory to antiarrhythmic medication. However, despite good acute results of catheter ablation, there is a high incidence of recurrence during long-term follow-up. In patients with ARVC at high risk of sudden death, implantation of an implantable cardioverter defibrillator (ICD) improves long-term survival by detection and termination of life-threatening ventricular arrhythmias. In the long term, however, the cumulative incidence of mainly lead-related complications of ICD therapy must be considered in the young population with ARVC, particularly when the indications are for primary prevention of sudden death or life-threatening arrhythmias. The proposed algorithm of therapeutic management in ARVC is under constant validation, development and refinement.     

Ventricular Tachycardia/Ventricular Fibrillation Ablation in the Setting of Ischemic Heart Disease

Recurrent ventricular tachycardia (VT) in the setting of coronary artery disease is frequently a life-threatening electrophysiologic emergency. Even in patients with an implantable defibrillator, recurrent VT is frequently accompanied by repeated and disabling shock therapy. Catheter ablative therapy offers the ability to provide immediate control of recurrent VT. Long-term elimination of VT should be anticipated in most patients. This article reviews the strategies, tools, techniques, and expected outcome for catheter ablation of stable and unstable ventricular arrhythmias in the setting ischemic heart disease.     

A comprehensive approach to management of ventricular arrhythmias

This review presents five cases that highlight the complexity of taking care of patients with ventricular arrhythmias. Three of the cases discuss management of patients with nonsustained ventricular tachycardia in the setting of structural heart disease: dilated cardiomyopathy, hypertrophic cardiomyopathy, and after myocardial infarction. A fourth case asks whether data from implantable cardioverter defibrillator (ICD) trials can be extrapolated to older patients, and the fifth case discusses management of recurrent ventricular arrhythmias in a patient with an ICD.     

Malignant Sustained Ventricular Tachyarrhythmias in Women:

Implantable Defibrillators in Women. Clinical rhythm, heart disease, ejection fraction, defibrillation threshold, recurrent arrhythmias, and mortality were compared in 268 consecutive recipients (213 men and 55 women) of their first implantable cardioverter defibrillator for life-threatening ventricular tachycardia or fibrillation. Women were younger than men, less likely to have structural heart disease, and more likely to have clinical ventricular fibrillation, a higher ejection fraction, and a lower defibrillation threshold. Complications of defibrillator placement were similar in both sexes. Unadjusted survival tended to be higher in women than in men (97% vs 90%, respectively, at 2 years, P = 0.08), largely due to fewer deaths from noncardiac causes or cardiac causes other than arrhythmia (P = 0.04). Women also tended to be at lower, albeit still substantial, risk for recurrent arrhythmias during follow-up (37% vs 52% in men at 2 years, P = 0.11). After adjustment for baseline differences, overall survival, arrhythmia death-free survival, nouarrhythmia death-free survival, and frequency of recurrent arrhythmias were not found to be gender related. Despite their apparent "lower risk" status on initial presentation, women remained at substantial risk for recurrent arrhythmias. This underscores the need to avoid being unduly biased by the "appearance" of health in managing women with malignant arrhythmias. That survival and other clinical endpoints were all ultimately independent of gender emphasizes the importance of other clinical variables in assessing risk from ventricular tachyarrhythmias.     

Treatment of choice and long-term prognosis for ventricular arrhythmias

Treatment of choice and long-term prognosis of the patients with ventricular arrhythmias are described in terms of prevention of sudden cardiac death and/or recurrence of life-threatening arrhythmias(ventricular tachycardia and ventricular fibrillation). 1) As to the long-term prognosis of ventricular tachyarrhythmias, presence of organic heart disease and degree of cardiac dysfunction are major determining factors. 2) The prognosis of patients with ventricular arrhythmias depends on how sudden cardiac death and life-threatening arrhythmias can be prevented. Among various methods, the electrophysiological test and its guided-therapy for antiarrhythmic drugs are now believed to be the most effective method for the prediction and prevention of the life-threatening events. We propose that the signal averaged electrocardiography is the best screening method as non-invasive approach for the selection of patients undergoing the electrophysiological test. 3) There are still certain limitations as to the prediction of sudden cardiac death and/or prevention of recurrent life-threatening arrhythmias by antiarrhythmic drug treatments in the certain numbers of patients depending on their basal cardiac disease and functional impairment. At present, catheter ablation procedure and implantable cardioverter defibrillator are the choice of the treatment in these cases. 4) In addition to conventional antiarrhythmic drugs, the treatment for the basal cardiac condition is mandatory for the long-term prognosis in the patients with ventricular arrhythmias.     

Circadian variation of malignant ventricular arrhythmias in patients with ischemic and nonischemic heart disease after cardioverter defibrillator implantation. European 7219 Jewel Investigators.

OBJECTIVES: The purpose of this study was to examine the circadian variation of ventricular arrhythmias detected by an implantable cardioverter defibrillator in patients with and without ischemic heart disease. BACKGROUND: Previous studies have shown a circadian variation of ventricular arrhythmias, sudden death and myocardial infarction with a peak occurrence in the morning hours. The circadian pattern, which is similar for both arrhythmic and ischemic events, suggests that ischemia may play a critical role in the genesis of ventricular arrhythmias and sudden death. We hypothesized that, if ischemia plays an important role in the triggering of ventricular arrhythmias, the circadian pattern should be different in patients with ischemic heart disease compared with patients with nonischemic heart disease. METHODS: The circadian variation of ventricular arrhythmias recorded by an implantable cardioverter defibrillator was studied in 310 patients during a mean follow-up of 181 +/- 163 days. Two hundred four patients had a history of ischemic heart disease and 106 patients had nonischemic heart disease. The times of the episodes of ventricular arrhythmias were retrieved from the data log of each device during follow-up, and the circadian pattern was compared between the two groups. RESULTS: During follow-up, 1,061 episodes of ventricular arrhythmias were recorded by the device in the 310 patients. Six hundred eighty-two episodes occurred in the group of patients with ischemic heart disease and 379 occurred in the nonischemic heart disease group. The circadian variation of the episodes showed a typical pattern with a morning and afternoon peak in both groups of patients with ischemic and nonischemic heart disease, but there was no significant difference between the two groups. CONCLUSIONS: The circadian rhythm of ventricular arrhythmias in patients with ischemic heart disease is similar to patients with nonischemic heart disease, suggesting that the trigger mechanisms of the initiation of ventricular tachyarrhythmias may be similar, irrespective of the underlying heart disease.     

Ventricular arrhythmias in congestive heart failure

Despite advances in the treatment of congestive heart failure (CHF), the mortality rate continues to be high. A large number of the deaths are sudden, presumably due to ventricular arrhythmias. Complex ventricular arrhythmias are recorded in as many as 80% of patients with CHF, with nonsustained ventricular tachycardia occurring in 40%. The latter appears to be an independent predictor of mortality. Chronic structural abnormalities responsible for CHF may be the basis for the capability of a ventricle to support life-threatening arrhythmias, which are triggered by premature ventricular contractions. The pathogenesis of arrhythmias is multifactorial. Electrolyte abnormalities, ischemia, catecholamines, inotropic and antiarrhythmic drugs may worsen arrhythmias and increase susceptibility of a ventricle to sustained arrhythmias. Beta-adrenergic blockers and angiotensin-converting enzyme inhibitors have a beneficial effect. The role of various drugs in the pathogenesis and treatment of ventricular arrhythmias is discussed. The efficacy of antiarrhythmic therapy targeted to asymptomatic nonsustained ventricular tachycardia, in order to prevent sudden death, is controversial. Pharmacotherapy guided by electrophysiologic testing is the treatment of choice for patients who have manifest sustained ventricular tachycardia, but patients resuscitated from ventricular fibrillation may require automatic implantable cardioverter defibrillator.     

Development of device therapy for ventricular arrhythmias

The past 25 years have seen the implantable cardioverter defibrillator emerge as the treatment of choice for ventricular arrhythmias with reduction in size but increased therapeutic options. Understanding the complex mechanisms of ventricular arrhythmias and defibrillation in normal and diseased hearts has been the focus of many research teams including that of John Uther at the Westmead Hospital Department of Cardiology. Marked improvements in capacitor and battery technologies, arrhythmia discrimination, pacing algorithms, shock waveforms and monitoring capabilities enable wider use and patient acceptance. Emergence of cardiac resynchronisation therapy and the implantable defibrillator for treatment of chronic heart failure is not only giving quality of life and extended survival for heart failure patients but has also cast new light on the evolution of heart failure.     

Should an abnormal serum potassium concentration be considered a correctable cause of cardiac arrest?

According to American Heart Association/American College of Cardiology Practice Guidelines, electrolyte abnormalities, including abnormal serum potassium concentrations, are considered a correctable cause of a life-threatening ventricular arrhythmia. Ventricular defibrillator therapy in this situation is a class III indication, and thought to be ineffective and perhaps harmful, although there are minimal data to support this recommendation. The steady-state serum potassium concentration frequently changes during a cardiac arrest. Additionally, the vast majority of cardiac arrest patients have structural heart disease and are commonly treated with a variety of medications that can alter the serum potassium concentration. In the Antiarrhythmics Versus Implantable Defibrillators (AVID) trial, patients with a correctable cause of an electrolyte imbalance were excluded from study participation but were followed in the AVID registry. Similar outcomes were observed among patients in the AVID registry and the main trial. Spironolactone therapy in patients with congestive heart failure decreases all-cause mortality and sudden and nonsudden cardiac death. In a preliminary study of 169 patients with an episode of a sustained ventricular arrhythmia treated with an implantable defibrillator, freedom from appropriate defibrillator therapy was 18% after five years. The probability of appropriate defibrillator therapy was independent of the initial serum potassium concentration. For these reasons, our current clinical practice is to use an implantable defibrillator to treat an initial episode of sustained ventricular tachycardia or ventricular fibrillation that occurs in a patient with structural heart disease and an abnormal serum potassium concentration.     

Implantable cardioverter-defibrillators for secondary prevention: is it worth it in the elderly?

Aging is associated with structural alterations in the heart that predispose the elderly to life-threatening ventricular arrhythmias. The majority of sudden cardiac deaths occur in people aged 65 and older. As the proportion of elderly in our population continues to grow, a greater number of elderly patients with malignant ventricular arrhythmias will require appropriate medical management. Clinical outcome trials have demonstrated that implantable cardioverter-defibrillators (ICDs) improve overall survival compared with pharmacologic therapy when used for the secondary prevention of cardiac arrest. Despite proven efficacy, physicians may be reluctant to implant a defibrillator in an older patient. This review summarizes the data pertaining to the use of defibrillators for secondary prevention in the elderly. ICD use for secondary prevention reduces all-cause mortality and appears to be economically advantageous in an older patient population. Currently, there is no convincing data to suggest that ICD therapy should be withheld from a patient based on age alone.     

How to manage athletes with syncope

Athletes with syncope warrant a full evaluation, as it is possible that athletics may unmask heart disease, either structural or electrical, and these athletes may be at risk for sudden cardiac death. The work-up and treatment of syncope in the athlete offers some unique challenges: to diagnose potentially life-threatening arrhythmias, to avoid pharmacological therapy, and to maximize ability to play. This article addresses the topic of syncope and possible solutions for those athletes in whom it is diagnosed.     

Near-fatal incident on routine induction of ventricular fibrillation at the replacement of an implantable cardioverter defibrillator

The case of a 54-year-old patient with severe coronary artery disease and storms of ventricular tachyarrhythmias is presented. On the replacement of an implantable cardioverter defibrillator due to battery depletion, he suffered life-threatening complications associated with arrhythmia induction. Performing a routine induction of ventricular arrhythmias on the occasion of pulse generator replacement may be reconsidered in selected patients.     

Treatment of athletes with cardiac disease or arrhythmias

Opinion statement Ventricular arrhythmias and sudden cardiac death in the athlete are uncommon but extremely visible because of the high profile of amateur and professional athletes. In athletes under the age of 30 years, the incidence of sudden death is low and in most cases occurs in individuals with inherited heart disease. In the older athlete, sudden death is more common and is generally due to arrhythmias in the context of coronary artery disease. Many athletes with aborted sudden death, arrhythmia-related syncope, or high-risk genetic disorders benefit from therapy with implantable cardioverterdefibrillators (ICDs). Although ICD therapy can effectively abort sudden death, implantation of an ICD generally prohibits an individual from all competitive athletics except low-intensity sports. Recommendations for participation in competitive athletics generally follow the recently published 36th Bethesda Conference Eligibility Recommendations for Competitive Athletes with Cardiovascular Abnormalities.     

Implantable defibrillator event rates in patients with unexplained syncope and inducible sustained ventricular tachyarrhythmias: a comparison with patients known to have sustained ventricular tachycardia

OBJECTIVES

To assess the clinical significance of inducible ventricular tachyarrhythmias among patients with unexplained syncope.

BACKGROUND

Induction of sustained ventricular arrhythmias at electrophysiology study in patients with unexplained syncope and structural heart disease is usually assigned diagnostic significance. However, the true frequency of subsequent spontaneous ventricular tachyarrhythmias in the absence of antiarrhythmic medications is unknown.

METHODS

In a retrospective case-control study, the incidence of implantable cardiac defibrillator (ICD) therapies for sustained ventricular arrhythmias among patients with unexplained syncope or near syncope (syncope group, n = 22) was compared with that of a control group of patients (n = 32) with clinically documented sustained ventricular tachycardia (VT). Sustained ventricular arrhythmias were inducible in both groups and neither group received antiarrhythmic medications. All ICDs had stored electrograms or RR intervals. Clinical variables were similar between groups except that congestive cardiac failure was more common in the syncope group.

RESULTS

Kaplan-Meier analysis of the time to first appropriate ICD therapy for syncope and control groups produced overlapping curves (p = 0.9), with 57 ± 11% and 50 ± 9%, respectively, receiving ICD therapy by one year. In both groups, the induced arrhythmia was significantly faster than spontaneous arrhythmias, but the cycle lengths of induced and spontaneous arrhythmias were positively correlated (R = 0.6, p < 0.0001). During follow-up, three cardiac transplantations and seven deaths occurred in the syncope group, and two transplantations and five deaths occurred in the control group (36-month survival without transplant 52 ± 11% and 83 ± 7%, respectively, p = 0.03).

CONCLUSIONS

In patients with unexplained syncope, structural heart disease and inducible sustained ventricular arrhythmias, spontaneous sustained ventricular arrhythmias occur commonly and at a similar rate to patients with documented sustained VT. Thus, electrophysiologic testing in unexplained syncope can identify those at risk of potentially life-threatening tachyarrhythmias, and aggressive treatment of these patients is warranted.