Risk Of Sudden Death In Patients Research Articles

Multi-metric risk score with quantitative late gadolinium enhancement predicts sudden death risk in patients with hypertrophic cardiomyopathy

Multi-metric risk score with quantitative late gadolinium enhancement predicts sudden death risk in patients with hypertrophic cardiomyopathy

Prediction of Sudden Death Risk in Patients with Congenital Heart Diseases.

Risk stratification for sudden death should be discussed with patients with congenital heart disease at each stage of personal and cardiac development. For most patients, risk is low through teenage years and the critical factors to consider are anatomy, ventricular function, and symptoms. By adulthood, these are supplemented by screening for atrial arrhythmias, ventricular arrhythmias, and pulmonary hypertension. Therapies include medication, ablation, and defibrillator placement.

Personalized computational heart models with T1-mapped fibrotic remodeling predict sudden death risk in patients with hypertrophic cardiomyopathy

Personalized computational heart models with T1-mapped fibrotic remodeling predict sudden death risk in patients with hypertrophic cardiomyopathy

Personalized computational heart models with T1-mapped fibrotic remodeling predict sudden death risk in patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is associated with risk of sudden cardiac death (SCD) due to ventricular arrhythmias (VAs) arising from the proliferation of fibrosis in the heart. Current clinical risk stratification criteria inadequately identify at-risk patients in need of primary prevention of VA. Here, we use mechanistic computational modeling of the heart to analyze how HCM-specific remodeling promotes arrhythmogenesis and to develop a personalized strategy to forecast risk of VAs in these patients. We combine contrast-enhanced cardiac magnetic resonance imaging and T1 mapping data to construct digital replicas of HCM patient hearts that represent the patient-specific distribution of focal and diffuse fibrosis and evaluate the substrate propensity to VA. Our analysis indicates that the presence of diffuse fibrosis, which is rarely assessed in these patients, increases arrhythmogenic propensity. In forecasting future VA events in HCM patients, the imaging-based computational heart approach achieved 84.6%, 76.9%, and 80.1% sensitivity, specificity, and accuracy, respectively, and significantly outperformed current clinical risk predictors. This novel VA risk assessment may have the potential to prevent SCD and help deploy primary prevention appropriately in HCM patients.

Repetitive Squat-to-Stand Provocation of Dynamic Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

Left ventricular outflow tract (LVOT) obstruction is a determinant in the management of patients with hypertrophic cardiomyopathy, including candidacy for septal reduction therapy and risk stratification for sudden cardiac death. 1 Elliott P.M. Gimeno J.R. Tomé M.T. Shah J. Ward D. Thaman R. et al. Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy. Eur Heart J. 2006; 27: 1933-1941 Crossref PubMed Scopus (262) Google Scholar ,2 Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al. 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2020; 76: e159-e240 Crossref PubMed Scopus (136) Google Scholar Obstruction is defined as a maximal instantaneous gradient ≥ 30 mm Hg, and severe obstruction is defined as a maximal instantaneous gradient ≥ 50 mm Hg. 2 Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al. 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2020; 76: e159-e240 Crossref PubMed Scopus (136) Google Scholar Several provocative maneuvers have been used to elicit latent obstruction, including the Valsalva maneuver, amyl nitrite inhalation, and exercise on a treadmill or bike. 3 Marwick T.H. Nakatani S. Haluska B. Thomas J.D. Lever H.M. Provocation of latent left ventricular outflow tract gradients with amyl nitrite and exercise in hypertrophic cardiomyopathy. Am J Cardiol. 1995; 75: 805-809 Abstract Full Text PDF PubMed Scopus (74) Google Scholar , 4 Jensen M.K. Havndrup O. Pecini R. Dalsgaard M. Hassager C. Helqvist S. et al. Comparison of Valsalva manoeuvre and exercise in echocardiographic evaluation of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy. Eur J Echocardiogr. 2010; 11: 763-769 Crossref PubMed Scopus (24) Google Scholar , 5 Shah J.S. Esteban M.T.T. Thaman R. Sharma R. Mist B. Pantazis A. et al. Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy. Heart. 2008; 94: 1288-1294 Crossref PubMed Scopus (165) Google Scholar During a recent shortage of amyl nitrite, our practice implemented a repetitive squat-to-stand maneuver as an adjunct to the Valsalva maneuver when assessing for LVOT obstruction. We sought to evaluate the efficacy of this repetitive squat-to-stand maneuver in eliciting LVOT obstruction compared with the Valsalva maneuver in a retrospective study.

Air pollution and sudden death risk in patients with Parkinson’s disease: Assessing the evidence to date

Air pollution and sudden death risk in patients with Parkinson’s disease: Assessing the evidence to date

Circulating miRNAs and Risk of SuddenDeath in Patients With CoronaryHeartDisease.

This study evaluated whether plasma miRNAs were specifically associated with sudden cardiac and/or arrhythmic death (SCD) in a cohort of patients with coronary heart disease (CHD), most of whom were without primary prevention implantable cardioverter-defibrillators. Novel biomarkers for sudden death risk stratification are needed in patients with CHD to more precisely target preventive therapies, such as implantable cardioverter-defibrillators. miRNAs have been implicated in regulating inflammation and cardiac fibrosis in cells, and plasma miRNAs have been shown to predict cardiovascular death in patients with CHD. We performed a nested case control study within a multicenter cohort of 5,956 patients with CHD followed prospectively for SCD. Plasma levels of 18 candidate miRNAs previously associated with cardiac remodeling were measured in 129 SCD cases and 258 control subjects matched on age, sex, race, and left ventricular ejection fraction. miR-150-5p, miR-29a-3p, and miR-30a-5p were associated with increased SCD risk (odds ratios and 95% confidence intervals: 2.03 [1.12 to 3.67]; p=0.02; 1.93 [1.07 to 3.50]; p=0.02; 0.55 [0.31 to 0.97]; p=0.04, respectively, for third vs. first tertile miRNA level). Unfavorable levels of all 3 miRNAs was associated with a 4.8-fold increased SCD risk (1.59 to 14.51; p=0.006). A bioinformatics-based approach predicted miR-150-5p, miR-29a-3p, and miR-30a-5p to be involved in apoptosis, fibrosis, and inflammation. These findings suggest that plasma miRNAs may regulate pathways important for remodeling and may be useful in identifying patients with CHD at increased risk of SCD.

Enlarged left atrium and sudden death risk in hypertrophic cardiomyopathy patients with or without atrial fibrillation

BackgroundThe relationships among enlarged left atrial dimension (LAD), the presence or absence of atrial fibrillation (AF), and sudden death risk in patients with hypertrophic cardiomyopathy (HCM) remain unclear. The aim of this study was to evaluate the impact of enlarged LAD on sudden death risk in HCM patients with or without documented AF. MethodsThis study included 564 HCM patients (follow-up period: 10.8±7.4 years). LAD was measured from the parasternal long-axis view as the antero-posterior linear diameter at end-systole. Sudden death was defined as the combined endpoint of sudden cardiac death and potentially lethal arrhythmic events, and log-rank tests and Cox proportional hazards models were applied to evaluate the impact of LAD enlargement on the combined endpoint. ResultsThe proportions of patients with sudden death and potentially lethal arrhythmic events were significantly higher among patients with enlarged LAD (≥48mm, N=86) compared with those without enlarged LAD (19.8% vs. 8.2%; p=0.002). However, enlarged LAD was not identified as an independent determinant of sudden death risk in multivariate analysis of all study HCM patients [adjusted hazard ratio (HR): 1.83; 95% confidence interval (CI): 0.95–3.53; p=0.071]. Among patients without documented AF during the follow-up periods, enlarged LAD was an independent determinant of sudden death risk (adjusted HR: 5.23; 95% CI: 2.17–12.58; p<0.001), although there was no significant difference in sudden death risk between patients with and without enlarged LAD in patients with documented AF (adjusted HR: 0.77; 95% CI: 0.31–1.90; p=0.567). ConclusionsThese results suggest that the relationship between LAD and outcome is influenced by the presence or absence of AF in HCM patients. It may thus be necessary to consider the need to prevent sudden death in LAD-enlarged HCM patients without documented AF.

Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden death in the young, although not all patients eligible for sudden death prevention with an implantable cardioverter-defibrillator are identified. Contrast-enhanced cardiovascular magnetic resonance with late gadolinium enhancement (LGE) has emerged as an in vivo marker of myocardial fibrosis, although its role in stratifying sudden death risk in subgroups of HCM patients remains incompletely understood. We assessed the relation between LGE and cardiovascular outcomes in 1293 HCM patients referred for cardiovascular magnetic resonance and followed up for a median of 3.3 years. Sudden cardiac death (SCD) events (including appropriate defibrillator interventions) occurred in 37 patients (3%). A continuous relationship was evident between LGE by percent left ventricular mass and SCD event risk in HCM patients (P=0.001). Extent of LGE was associated with an increased risk of SCD events (adjusted hazard ratio, 1.46/10% increase in LGE; P=0.002), even after adjustment for other relevant disease variables. LGE of ≥15% of LV mass demonstrated a 2-fold increase in SCD event risk in those patients otherwise considered to be at lower risk, with an estimated likelihood for SCD events of 6% at 5 years. Performance of the SCD event risk model was enhanced by LGE (net reclassification index, 12.9%; 95% confidence interval, 0.3-38.3). Absence of LGE was associated with lower risk for SCD events (adjusted hazard ratio, 0.39; P=0.02). Extent of LGE also predicted the development of end-stage HCM with systolic dysfunction (adjusted hazard ratio, 1.80/10% increase in LGE; P<0.03). Extensive LGE measured by quantitative contrast enhanced CMR provides additional information for assessing SCD event risk among HCM patients, particularly patients otherwise judged to be at low risk.

Risk of Sudden Death and Outcome in Patients With Hypertrophic Cardiomyopathy With Benign Presentation and Without Risk Factors

Patients with hypertrophic cardiomyopathy (HC) are reported to have a mortality rate of about 1.0% per year, and those patients without sudden death risk factors and with no or mild symptoms are generally considered to have a benign clinical presentation. However, the risk of sudden death and the outcome in this latter subgroup have not been investigated systematically and remain unresolved. We assessed the risk of sudden death and outcome in 653 consecutive patients with HC without risk factors and with no or mild symptoms. Over a median follow-up of 5.3years, 35 patients (5.4%) died of HC-related causes. Mean age at death was 46 ± 20years in patients who died suddenly and 66 ± 15 and 72 ± 9years, respectively, in patients who died of heart failure or stroke. Event rate was 0.6% per year for sudden death, 0.2% per year for heart failure death, and 0.1% per year for stroke-related death. Sudden death risk was independently and inversely related to age, and risk of heart failure or stroke death was directly related to age (p= 0.020). At 10years after the initial evaluation, sudden death risk was 5.9%, with sudden death rate being the lowest (0.3% per year) in patients with normal left atrial dimension (≤40mm). In conclusion, in patients with HC without conventional risk factors and with no or mild symptoms, the risk of sudden death was not negligible, with an event rate of 0.6% per year. Heart failure and stroke-related death were less common and largely confined to older patients. These results underscore the need for a more accurate assessment of the sudden death risk in patients with HC.

Heart rate: The hummingbird effect

with heart failure, in whom risk of all-cause mortality and sudden death mortality is markedly increased, betaadrenergic antagonists are part of the standard of care, which is defined by randomized prospective clinical trials to pharmacologically extend longevity. A meta-analysis of 23 beta-blocker trials in patients with heart failure demonstrated that the protective effect associated with these drugs was proportional to the heart rate reduction and not related to the dose of the beta-blocker. 7 Ivabradine is a new agent with a direct effect on the funny current (If,) which is an important determinant of the pacemaker potential and periodicity in the sinoatrial node. A randomized prospective trial of ivabradine in patients with heart failure demonstrated a reduction in the primary composite end point of cardiovascular death and heart failure hospital admissions associated with ivabradine treatment. 8 Both reports provide strong evidence of a direct connection between an improvement in cardiovascular outcomes and a pharmacological effect on the resting heart rate. In this issue of HeartRhythm, Teodorescu et al 9 provide more evidence for an important link between resting heart rate and sudden death risk in patients with known coronary artery disease. For their case-control analysis, they identified 378 cases of sudden cardiac death older than 35 years that had prior electrocardiograms (ECGs) available for analysis. Cases were identified from the ongoing Oregon Sudden Unexpected Death Study. The control cases were chosen from the same geographic region and sex and age matched to cases. All the control patients also had coronary artery disease. Resting heart rate was defined by a 12-lead ECG identified from medical records before the sudden death event. The patient characteristics in the 2 groups were remarkable for a number of statistically significant asymmetries. Among cases, diabetes mellitus, chronic obstructive pulmonary disease, chronic renal insufficiency, and severe left ventricular systolic dysfunction (LVSD) were more prevalent. Among heart rate-modulating drugs, only digoxin and beta2-agonists were more commonly used among cases. After adjustment for major comorbidities such as diabetes, pulmonary, and renal disease as well as the effects of heart rate-modulating drugs, the authors found a 26% increase in risk associated with each 10-beats/min increase in resting heart rate. Subsequent adjustment for the impact of LVSD attenuated this relationship to a minor degree. Digoxin use had the strongest predictive value for sudden death, but only 12 controls were on the drug as opposed to 62 controls, of

Association of implantable defibrillator therapy risk with body mass index in systolic heart failure

ObjectivesTo determine whether risk for implantable cardioverter-defibrillator (ICD) therapy varies by body mass index (BMI) in systolic heart failure (HF). BackgroundIt is unknown whether obesity increases sudden death risk in patients with systolic HF. MethodsSecondary analysis of patients with HF, left ventricular ejection fraction ≤0.40 and ICD (N = 464) was performed using Cox regression modeling to assess risk for first delivered ICD therapy, with patients grouped by BMI (kg/m2): normal (18.5 to <25), overweight (25 to <30), and obese (≥30). ResultsOverweight patients, compared with patients with normal BMI, had greater adjusted risk for first ICD therapy (HR 1.66; 95% CI 1.02–2.71; P = 0.04), whereas obese BMI was not associated with risk for first ICD therapy. ConclusionsThere was an inverted U-shaped relationship between BMI and risk for first ICD therapy among systolic HF patients, with highest risk in overweight BMI.

Sudden Death Risk in Syncope: The Role of The Implantable Cardioverter Defibrillator

Syncope is generally benign but when it is due to an underlying cardiovascular condition, the prognosis can be guarded. Patients with syncope may be at risk of dying suddenly from a ventricular arrhythmia especially if the collapse is caused by a poorly-tolerated, self-terminating, ventricular tachycardia (VT). If a similar VT recurs, and persists, it could initiate cardiac arrest, leading to sudden cardiac death. However, distinguishing which patient with syncope may benefit most from implantable cardioverter defibrillator (ICD) therapy, which can stop life-threatening and poorly tolerated VT, thereby preventing sudden cardiac death, remains an ongoing challenge. Careful assessment of the patient's underlying cardiovascular conditions, scrupulous attention to historical detail to assess potential causes for syncope, and risk stratification based upon clinical characteristics and short and long-term risks can help. This review focuses on the sudden death risk in patients with syncope and explores the role of the ICD to treat ventricular arrhythmias, prevent symptoms, and prevent death.

“Please implant a defibrillator in my patient”: It's déjà vu all over again

“Please implant a defibrillator in my patient”: It's déjà vu all over again

417 Sudden death risk in patients with tetralogy of Fallot and 22q11.2 deletion syndrome

achieve sufficiently high heparin blood concentration after the initial heparin bolus in 52% of cases and required an additional heparin bolus prior to bypass initiation. Most patients (361, 58%) received additional heparin during the surgery, despite normal activated clotting time ( 400s). Patients in the postHMS era required significantly lower amount of allogeneic red blood cell (87ml/kg vs. 118 ml/kg, P 0.001) and platelets (8ml/kg vs. 14ml/kg, P 0.001). Use of recombinant factor VIIa in post-operative period was less likely in the post-HMS era (1.1% vs. 3.5%, P 0.03). Intubation time (9 vs. 27 hours, P 0.001), intensive care unit stay (2 vs. 3 days, P 0.009) and hospital stay (6.3 vs. 8.0 days, P 0.02) were all shorter in the post-HMS period. CONCLUSION: Failure of the HMS system to achieve sufficient blood heparin concentration after the initial dose shows that further refinements in the pediatric protocol are necessary. Even considering this, the use of the HMS system for all pediatric cardiac surgeries was associated with system-wide improvement in post-surgical outcomes. Although the effect cannot be entirely ascribed to the use of the HMS because of additional concurrent changes in surgical and post-operative protocols, the reduction of allogeneic blood transfusions and bleeding-related complications suggest that the HMS system was a contributor to the improved surgical outcomes observed between the 2 periods.

Limitations of Ejection Fraction for Prediction of Sudden Death Risk in Patients With Coronary Artery Disease: Lessons From the MUSTT Study

Limitations of Ejection Fraction for Prediction of Sudden Death Risk in Patients With Coronary Artery Disease: Lessons From the MUSTT Study

Limitations of Ejection Fraction for Prediction of Sudden Death Risk in Patients With Coronary Artery Disease: Lessons From the MUSTT Study

We determined the contribution of multiple variables to predict arrhythmic death and total mortality risk in patients with coronary disease and left ventricular dysfunction. We then constructed an algorithm to predict risk of mortality and sudden death. Many factors in addition to ejection fraction (EF) influence the prognosis of patients with coronary disease. However, there are few tools to use this information to guide clinical decisions. We evaluated the relationship between 25 variables and total mortality and arrhythmic death in 674 patients enrolled in the MUSTT (Multicenter Unsustained Tachycardia Trial) study that did not receive antiarrhythmic therapy. We then constructed risk-stratification algorithms to weight the prognostic impact of each variable on arrhythmic death and total mortality risk. The variables having the greatest prognostic impact in multivariable analysis were functional class, history of heart failure, nonsustained ventricular tachycardia not related to bypass surgery, EF, age, left ventricular conduction abnormalities, inducible sustained ventricular tachycardia, enrollment as an inpatient, and atrial fibrillation. The model demonstrates that patients whose only risk factor is EF < or =30% have a predicted 2-year arrhythmic death risk <5%. Multiple variables influence arrhythmic death and total mortality risk. Patients with EF < or =30% but no other risk factor have low predicted mortality risk. Patients with EF >30% and other risk factors may have higher mortality and a higher risk of sudden death than some patients with EF < or =30%. Thus, risk of sudden death in patients with coronary disease depends on multiple variables in addition to EF.

Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy

Left ventricular outflow tract obstruction (LVOTO) is associated with reduced survival in patients with hypertrophic cardiomyopathy (HCM). The influence of LVOTO on survival from SD in relation to other recognized clinical risk markers is unknown. A total of 917 patients with HCM (554 males, 43+/-15 years) were studied; 288 (31.4%) had LVOTO at rest (> or =30 mmHg). During follow-up [median 61 (30;99) months], 54 (5.9%) patients died suddenly (SD), survived ventricular fibrillation, or had an appropriate ICD discharge; 25 (2.7%) died from heart failure or were transplanted; 17 (1.8%) died from other cardiovascular causes. Five-year survival from all-cause death or cardiac transplantation was lower in patients with LVOTO [86.5% (95% CI: 81.7-91.2) vs. 90.1% (95% CI: 87.3-92.8), P=0.006], with a trend towards higher all-cause death and transplantation with increasing LVOTO [(RR per 20 mmHg=1.24 (95% CI: 1.08-1.42), P=0.003)]. In patients with obstruction, there was a significant relation between 5-year survival from all-cause death and functional limitation (NYHA class I: 91.0%; NYHA class II: 83.3%; NYHA class III/IV: 82.6%, P=0.002). LVOTO was associated with reduced survival from SD and ICD discharge (SD/ICD) [91.4% (95% CI: 87.4-95.3) vs. 95.7% (95% CI: 93.8-97.6), P=0.0004]. Magnitude of LVOTO was related to a higher occurrence of SD/ICD [RR per 20 mmHg=1.36 (95% CI: 1.12-1.65), P=0.001]. There was no relation between survival from SD/ICD, LVOTO, and NYHA class. The annual rate of SD/ICD in patients with LVOTO and no risk factors was 0.37% (95%CI: 0.05-1.35). There was a trend towards lower survival from SD/ICD, with increasing numbers of risk factors in patients with and without LVOTO (P=0.002 and P=0.002, respectively). Multivariable analysis demonstrated that LVOTO was an independent predictor of SD/ICD, with a 2.4-fold (P=0.003) increase in the risk of SD/ICD. LVOTO is associated with an increased risk of SD/ICD that is related to the severity of obstruction and the presence of other recognized risk factors for SD. The low sudden death mortality in asymptomatic patients with LVOTO and no other SD risk markers suggests that aggressive interventions to reduce LVOTO are unwarranted in this group. Further studies are required to determine the most appropriate treatment strategies (ICD or gradient reduction) in patients with additional risk factors.

Arrhythmogenic right ventricular cardiomyopathy: current diagnostic and management strategies.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease of unknown etiology characterized by the peculiar right ventricular (RV) involvement. Distinctive pathologic features are myocardial atrophy and fibro-fatty replacement of the RV free wall, and clinical presentation is usually related to ventricular tachycardias with a left bundle branch block pattern or ventricular fibrillation leading to cardiac arrest, mostly in young people and athletes. Later in the disease evolution, progression and extension of RV muscle disease and left ventricular involvement may result in right or biventricular heart failure. The diagnosis of ARVC may be difficult because of several problems with specificity of ECG abnormalities, different potential etiologies of ventricular arrhythmias with a left bundle branch morphology, assessment of RV structure and function, and interpretation of endomyocardial biopsy findings. Therefore, standardized diagnostic criteria have been proposed by the Study Group on ARVC of the European Society of Cardiology. According to these guidelines, the diagnosis of ARVC is based on the presence of major and minor criteria encompassing electrocardiographic, arrhythmic, morphofunctional, histopathologic, and genetic factors. Since the assessment of sudden death risk in patients with ARVC is still not well established, there are no precise guidelines to determine which patients need to be treated and what is the best management approach. The therapeutic options include beta-blockers, antiarrhythmic drugs, catheter ablation, and implantable cardioverter defibrillator (ICD). The ICD is the most effective safeguard against arrhythmic sudden death. However, its precise role in changing the natural history of ARVC by preventing sudden and nonsudden death needs to be evaluated by a prospective study of a large series of patients. In patients in whom ARVC has progressed to severe RV or biventricular systolic dysfunction with risk of thromboembolic complications, treatment consists of current therapy for heart failure including anticoagulant therapy. In cases of refractory congestive heart failure, patients may become candidates for heart transplantation.

Clinical diagnosis and management strategies in arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease, often familial, that is characterized by fibro-fatty replacement of the right ventricular (RV) myocardium. The most common clinical manifestations of ARVC consists of ventricular arrhythmias of RV origin, which may lead to sudden death mostly in young people and athletes, electrocardiograph depolarization/repolarization changes mostly localized to right precordial leads, and global and/or regional dysfunction and structural alterations of the RV. The diagnosis of ARVC may be difficult due to several problems with the specificity of the electrocardiograph abnormalities, the different potential etiologies of ventricular arrhythmias with a left bundle branch morphology, the assessment of the RV structure and function, and the interpretation of endomyocardial biopsy findings. Therefore, standardized diagnostic criteria have been proposed by the Study Group on ARVC of the European Society of Cardiology. According to these guidelines, the diagnosis of ARVC is based on the presence of major and minor criteria encompassing electrocardiograph, arrhythmic, morphofunctional, histopathologic, and genetic factors. Because the assessment of sudden death risk in patients with ARVC is still not well established, there are no precise guidelines to determine which are the patients who need to be treated and which is the best management approach. The therapeutic options include beta blockers, antiarrhythmic drugs, catheter ablation, and implantable cardioverter defibrillator. The implantable defibrillator is the most effective safe-guard against arrhythmic sudden death. However, its precise role in changing natural history of ARVC by preventing sudden and nonsudden death needs to be evaluated by a prospective study of a large series of patients. In patients in whom ARVC has progressed to severe RV or biventricular systolic dysfunction with risk of thromboembolic complications, treatment consists of current therapy for heart failure including anticoagulant therapy. In case of refractory congestive heart failure, the patients may become candidates for heart transplantation.