A SYSTEMATIC REVIEW FOR SUDDEN CARDIAC DEATH IN HYPERTROPHIC CARDIOMYOPATHY PATIENTS WITH MYOCARDIAL FIBROSIS: A CMR LGE STUDY

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<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

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Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers

EP Europace ◽

10.1093/europace/eup431 ◽

2010 ◽

Vol 12 (3) ◽

pp. 313-321 ◽

Cited By ~ 108

Author(s):

Imke Christiaans ◽

Klaartje van Engelen ◽

Irene M. van Langen ◽

Erwin Birnie ◽

Gouke J. Bonsel ◽

...

Keyword(s):

Systematic Review ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Risk Stratification ◽

Cardiac Death ◽

Risk Markers ◽

Clinical Risk

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

Heart ◽

10.1136/heartjnl-2018-313700 ◽

2018 ◽

pp. heartjnl-2018-313700 ◽

Cited By ~ 6

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.

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Serum Matrix Metalloproteinases as Quantitative Biomarkers for Myocardial Fibrosis and Sudden Cardiac Death Risk Stratification in Patients With Hypertrophic Cardiomyopathy

Journal of Cardiac Failure ◽

10.1016/j.cardfail.2016.03.010 ◽

2016 ◽

Vol 22 (10) ◽

pp. 845-850 ◽

Cited By ~ 13

Author(s):

Julia Münch ◽

Maxim Avanesov ◽

Peter Bannas ◽

Dennis Säring ◽

Elisabeth Krämer ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Matrix Metalloproteinases ◽

Risk Stratification ◽

Myocardial Fibrosis ◽

Cardiac Death ◽

Death Risk ◽

Sudden Cardiac Death Risk

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ADDITIONAL VALUE OF QUANTIFICATION OF MYOCARDIAL FIBROSIS BY CONTRAST-ENHANCED MRI TO IDENTIFY SUBJECTS WITH HYPERTROPHIC CARDIOMYOPATHY AND SUDDEN CARDIAC DEATH RISK

Siberian Medical Journal ◽

10.29001/2073-8552-2019-34-1-33-38 ◽

2019 ◽

Vol 34 (1) ◽

pp. 33-38

Author(s):

S. M. Komissarova ◽

E. Yu. Zakharova ◽

T. V. Ilyina ◽

E. A. Vankovich

Keyword(s):

Magnetic Resonance Imaging ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Magnetic Resonance ◽

Myocardial Fibrosis ◽

Cardiac Death ◽

Low Risk ◽

Resonance Imaging ◽

Death Risk ◽

Sudden Cardiac Death Risk

Background. A 5-year sudden cardiac death risk model in line with ESC-2014 (HCM Risk-SCD score) Guidelines assesses the risk of sudden cardiac death in individuals with hypertrophic cardiomyopathy using clinical parameters without taking into account magnetic resonance imaging indices including myocardial fibrosis.Aim. To compare subjects with low, moderate, and high sudden cardiac death risks, identified based on HCM Risk-SCD score, with delayed enhancement magnetic resonance imaging-evidenced fibrosis and to assess the role of fibrosis in identification of patients with sudden cardiac death risk.Material and Methods. A total of 98 patients with hypertrophic cardiomyopathy underwent an integrative examination including cardiac echocardiography, 24-h electrocardiography monitoring, delayed gadolinium-enhanced magnetic resonance imaging, and an assessment of a 5-year sudden cardiac death risk by HCM Risk-SCD score.Results and Discussion. Out of 98 patients, 45 (46%) patients had low risk of sudden cardiac death, 26 (26%) patients had intermediate risk, and 27 (28%) patients had high sudden cardiac death risk by HCM Risk-SCD score. During the follow-up period, (mean 41 months; 25 to 58 months), 16 adverse events were registered: sudden cardiac death occurred in 9 patients while 7 patients had been successfully resuscitated and implanted with implantable cardioverter defibrillator for secondary prevention of sudden cardiac death. The risk assessment using HCM Risk-SCD score showed high risk in 8 out of 16 (50%) patients; 2 (12.5%) patients had moderate risk, while 6 (37.5%) patients had low risk. High-risk patients demonstrated significantly (p<0.001) larger fibrosis (mean 28.5%; quartiles 21.9%; 44.1%) compared to those with moderate risk (mean 17.6%; quartiles 8.0%; 22.5%) and low risk of sudden cardiac death (mean 11.7%; quartiles 5.8%; 17.6%). The volume of fibrosis by delayed contrast-enhanced magnetic resonance imaging was associated with the adverse outcomes rate, which was 15%. A Log rank test in the KaplanMeier survival analysis showed statistically significant differences (p=0,002) in groups with fibrosis <15% and≥15%. The regression analysis showed that myocardial fibrosis was more significant factor associated with sudden cardiac death (OR 12; 95% CI 1.6–91) compared to SCD ESC-2014 score (OR 2.8 95% CI 1.1–7.5).Conclusion. Therefore, based on regression analysis, patients with hypertrophic cardiomyopathy who had myocardial fibrosis volume ≥15% were identified as a group with risk of sudden cardiac death and adverse arrhythmic events.

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