scholarly journals Effectiveness of the 2014 European Society of Cardiology guideline on sudden cardiac death in hypertrophic cardiomyopathy: a systematic review and meta-analysis

Heart ◽  
2018 ◽  
pp. heartjnl-2018-313700 ◽  
Author(s):  
Constantinos O’Mahony ◽  
Mohammed Majid Akhtar ◽  
Zacharias Anastasiou ◽  
Oliver P Guttmann ◽  
Pieter A Vriesendorp ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
European Society ◽  
Cardiac Death ◽  
Meta Analysis ◽  
Registration Number ◽  
Risk Patients ◽  
European Society Of Cardiology

ObjectiveIn 2014, the European Society of Cardiology (ESC) recommended the use of a novel risk prediction model (HCM Risk-SCD) to guide use of implantable cardioverter defibrillators (ICD) for the primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). We sought to determine the performance of HCM Risk-SCD by conducting a systematic review and meta-analysis of articles reporting on the prevalence of SCD within 5 years of evaluation in low, intermediate and high-risk patients as defined by the 2014 guidelines (predicted risk <4%, 4%–<6% and ≥6%, respectively).MethodsThe protocol was registered with PROSPERO (registration number: CRD42017064203). MEDLINE and manual searches for papers published from October 2014 to December 2017 were performed. Longitudinal, observational cohorts of unselected adult patients, without history of cardiac arrest were considered. The original HCM Risk-SCD development study was included a priori. Data were pooled using a random effects model.ResultsSix (0.9%) out of 653 independent publications identified by the initial search were included. The calculated 5-year risk of SCD was reported in 7291 individuals (70% low, 15% intermediate; 15% high risk) with 184 (2.5%) SCD endpoints within 5 years of baseline evaluation. Most SCD endpoints (68%) occurred in patients with an estimated 5-year risk of ≥4% who formed 30% of the total study cohort. Using the random effects method, the pooled prevalence of SCD endpoints was 1.01% (95% CI 0.52 to 1.61) in low-risk patients, 2.43% (95% CI 1.23 to 3.92) in intermediate and 8.4% (95% CI 6.68 to 10.25) in high-risk patients.ConclusionsThis meta-analysis demonstrates that HCM Risk-SCD provides accurate risk estimations that can be used to guide ICD therapy in accordance with the 2014 ESC guidelines.Registration numberPROSPERO CRD42017064203;Pre-results.

Predictors of sudden cardiac death and ventricular tachyarrhythmias in patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Rineiska ◽  
E Zakharava ◽  
S Komissarova ◽  
I Haidel
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Myocardial Fibrosis ◽  
Cardiac Death ◽  
T1 Mapping ◽  
Adverse Outcome ◽  
Low Risk ◽  
Ventricular Tachyarrhythmias ◽  
Risk Patients

Abstract Background Determining additional predictors that allow more accurate identification of patients who need primary prevention of sudden cardiac death (SCD) and ventricular tachyarrhythmias (VT) in patients with hypertrophic cardiomyopathy (HCM) is one of the most important tasks. High-risk patients can be more accurately identified using modern non-invasive research methods. Purpose Identification of new risk predictors for SCD and ventricular tachyarrhythmias based on cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) and T1-mapping in patients with hypertrophic cardiomyopathy. Materials and methods Clinical data of 98 HCM subjects (58 males and 40 females, median age 46.5 [35.2; 54.7] treated in our centre in the period between 2013 and 2017 have been studied. All patients underwent CMR with LGE and T1-mapping. Results According to the existing ESC-2014 scale, 45 (46%) of the 98 patients included in the study had a low risk, an intermediate risk was identified in 26 (26%) patients and a high risk of SCD in 27 (28%). During the follow-up period 16 episodes of sudden cardiac death were recorded. Of these, only 8 (50%) patients had a high risk on the ESC-2014 scale. High-risk patients show significantly greater myocardial fibrosis (Me 28.5%; [21.9; 44.1]) compared with patients with intermediate (Me 17.6% [8.0; 22.5]) and low risk of SCD (Me 11.7%; [5.8; 17.6]), p&lt;0.001. A threshold level of fibrosis volume associated with an adverse outcome was determined, which was 15% (based on determining the maximum rank of statistics). For patients with 15% fibrosis volume, the cumulative survival rate on the Kaplan-Mayer curve over the observation period was 96% (95% CI 88.6–100), while for patients with fibrosis volume ≥15% - 72.4% (95% CI 60.6–86.4). The study showed that in 15 (93.7%) patients out of 16 who had an adverse outcome, the volume of myocardial fibrosis was ≥15%. Episodes of nonsustained ventricular tachycardia were detected in 48 patients (48.7%) according to daily ECG monitoring. To identify patients with ventricular tachyarrhythmias by ROC analysis, the threshold value of extracellular myocardial volume was determined using CMR with LGE and T1 mapping, which was 32.5% (sensitivity 74% and specificity 86%). According to multivariate analysis, one of the independent predictors of ventricular tachyarrhythmias was the level of extracellular myocardial volume ≥32.5% (HR 1.2; 95% CI 1.03–1.4). Conclusion Predictors detected by CMR with LGE and T1 mapping can identify patients with HCM with a high risk of sudden cardiac death and ventricular tachyarrhythmias. Funding Acknowledgement Type of funding source: None

Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy

ESC CardioMed ◽  
2018 ◽  
pp. 2316-2319
Author(s):  
Philipp Attanasio ◽  
Wilhelm Haverkamp
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Prediction Model ◽  
Cardiac Death ◽  
Cardioverter Defibrillator ◽  
Life Threatening ◽  
Esc Guidelines ◽  
European Society Of Cardiology

Identification of patients with hypertrophic cardiomyopathy (HCM) who are at high risk of sudden cardiac death (SCD) is essential, as life-threatening arrhythmic events can be effectively treated with implantable cardioverter defibrillator therapy. Various models for risk stratification of patients with HCM have been proposed. The latest clinical risk prediction model was developed in 2013. It is based on the HCM Risk-SCD study that included 3675 patients. Risk stratification using this model is recommended in the 2014 European Society of Cardiology (ESC) Guidelines for management of HCM and in the 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of SCD. This chapter summarizes novelties in the prediction model and the resulting recommendations, and discusses potential limitations of this approach.

scholarly journals A validation study of the European Society of Cardiology guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy

EP Europace ◽  
2019 ◽  
Vol 21 (10) ◽  
pp. 1559-1565 ◽  
Author(s):  
Gabrielle Norrish ◽  
Tao Ding ◽  
Ella Field ◽  
Karen McLeod ◽  
Maria Ilina ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
European Society ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Sustained Ventricular Tachycardia ◽  
Rank Test ◽  
European Society Of Cardiology

Abstract Aims Sudden cardiac death (SCD) is the most common cause of death in children with hypertrophic cardiomyopathy (HCM). The European Society of Cardiology (ESC) recommends consideration of an implantable cardioverter-defibrillator (ICD) if two or more clinical risk factors (RFs) are present, but this approach to risk stratification has not been formally validated. Methods and results Four hundred and eleven paediatric HCM patients were assessed for four clinical RFs in accordance with current ESC recommendations: severe left ventricular hypertrophy, unexplained syncope, non-sustained ventricular tachycardia, and family history of SCD. The primary endpoint was a composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate ICD therapy, or sustained ventricular tachycardia), defined as a major arrhythmic cardiac event (MACE). Over a follow-up period of 2890 patient years (median 5.5 years), MACE occurred in 21 patients (7.5%) with 0 RFs, 19 (16.8%) with 1 RFs, and 3 (18.8%) with 2 or more RFs. Corresponding incidence rates were 1.13 [95% confidence interval (CI) 0.7–1.73], 2.07 (95% CI 1.25–3.23), and 2.52 (95% CI 0.53–7.35) per 100 patient years at risk. Patients with two or more RFs did not have a higher incidence of MACE (log-rank test P = 0.34), with a positive and negative predictive value of 19% and 90%, respectively. The C-statistic was 0.62 (95% CI 0.52–0.72) at 5 years. Conclusions The incidence of MACE is higher for patients with increasing numbers of clinical RFs. However, the current ESC guidelines have a low ability to discriminate between high- and low-risk individuals.

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