scholarly journals Risk Stratification in Hypertrophic Cardiomyopathy

2015 ◽  
Vol 10 (1) ◽  
pp. 31 ◽  
Author(s):  
Alexandros Klavdios Steriotis ◽  
Sanjay Sharma ◽  
◽  
Keyword(s):  
Risk Factors ◽  
Hypertrophic Cardiomyopathy ◽  
Risk Stratification ◽  
Ventricular Arrhythmias ◽  
Risk Model ◽  
Predictive Accuracy ◽  
Young Patients ◽  
Left Ventricular ◽  
Adverse Event Rate ◽  
Icd Implantation

Hypertrophic cardiomyopathy (HCM) is a hereditary primary myocardial disease that is most commonly due to mutations within genes encoding sarcomeric contractile proteins and is characterised by left ventricular hypertrophy in the absence of a cardiac or systemic cause. Although the overall prognosis is relatively good with an annual mortality rate <1 %, the propensity to potentially fatal ventricular arrhythmias is the most feared complication. The identification of patients at risk of arrhythmogenic sudden cardiac death (SCD) is an essential component in disease management. Aborted SCD and malignant ventricular arrhythmias are the most powerful risk factors for SCD and ICD implantation is recommended in such circumstances. The selection of patients who may benefit from ICD therapy for primary prevention purposes is more challenging. The heterogeneous nature of the disease and the variation in trigger factors provides an adequate explanation for the low predictive accuracy of most conventional risk factors in isolation. A new risk model for risk stratification proposed by the European Society of Cardiology HCM outcome group shows promise but requires validation in different cohorts. The ICD is the only effective therapy in preventing SCD for the disease with a relatively low adverse event rate, but most deaths occur in relatively young patients. However, it is also difficult to ignore the complications with the ICD, therefore, the strive to perfect risk stratification in HCM should continue to ensure that only the most high-risk patients receive an ICD.

Advanced imaging for risk stratification of sudden death in hypertrophic cardiomyopathy

Heart ◽  
2020 ◽  
Vol 106 (11) ◽  
pp. 793-801 ◽  
Author(s):  
Jay Ramchand ◽  
Agostina M Fava ◽  
Michael Chetrit ◽  
Milind Y Desai
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Risk Stratification ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Small Subset ◽  
Imaging Features ◽  
Icd Implantation ◽  
Increased Risk ◽  
Cardiac Condition

Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac condition, which typically manifests as left ventricular hypertrophy. A small subset of patients with HCM have an increased risk of sudden cardiac death (SCD) from ventricular arrhythmias. Risk of SCD can be effectively reduced following implantation of implantable cardiac defibrillators (ICD), although this treatment carries a risk of complications such as inappropriate shocks. With this in mind, we turn to advances in cardiac imaging to guide risk stratification for SCD and to select the appropriate individual who may benefit from ICD implantation. In this review, we have taken the opportunity to briefly summarise the role of imaging in the diagnosis of HCM before focusing on how specific imaging features influence risk of SCD in patients with HCM.

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

2018 ◽  
Author(s):  
Constantinos O’Mahony
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Clinical Care ◽  
Psychological Impact ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Icd Implantation ◽  
European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

ESC CardioMed ◽  
2018 ◽  
pp. 1462-1466
Author(s):  
Constantinos O’Mahony
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Clinical Care ◽  
Psychological Impact ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Icd Implantation ◽  
European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

scholarly journals 1228 New predictors of sudden cardiac death in hypertrophic cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D M Adamczak ◽  
A Rogala ◽  
M Antoniak ◽  
Z Oko-Sarnowska
Keyword(s):  
Risk Factors ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Volume Index ◽  
Lv Function ◽  
Icd Implantation ◽  
Significant Difference

Abstract BACKGROUND Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium. HCM is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmias. However, in most patients, HCM has a benign course. That is why it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation. The HCM SCD-Risk Calculator is a useful tool for estimating the risk of SCD. The parameters included in the model at evaluation are: age, maximum left ventricular (LV) wall thickness, left atrial (LA) dimension, maximum gradient in left ventricular outflow tract, family history of SCD, non-sustained ventricular tachycardia (nsVT) and unexplained syncope. Nevertheless, there is potential to improve and optimize the effectiveness of this tool in clinical practice. Therefore, the following new risk factors are proposed: LV global longitudinal strain (GLS), LV average strain (ASI) and LA volume index (LAVI). GLS and ASI are sensitive and noninvasive methods of assessing LV function. LAVI more accurately characterizes the size of the left atrium in comparison to the LA dimension. METHODS 252 HCM patients (aged 20-88 years, of which 49,6% were men) treated in our Department from 2005 to 2018, were examined. The follow-up period was 0-13 years (average: 3.8 years). SCD was defined as sudden cardiac arrest (SCA) or an appropriate ICD intervention. All patients underwent an echocardiographic examination. The medical and family histories were collected and ICD examinations were performed. RESULTS 76 patients underwent an ICD implantation during the follow-up period. 20 patients have reached an SCD end-point. 1 patient died due to SCA and 19 had an appropriate ICD intervention. There were statistically significant differences of GLS and ASI values between SCD and non-SCD groups; p = 0.026389 and p = 0.006208, respectively. The average GLS in the SCD group was -12.4% ± 3.4%, and -15.1% ± 3.5% in the non-SCD group. The average ASI values were -9.9% ± 3.8% and -12.4% ± 3.5%, respectively. There was a statistically significant difference between LAVI values in SCD and non-SCD groups; p = 0.005343. The median LAVI value in the SCD group was 45.7 ml/m2 and 37.6 ml/m2 in the non-SCD group. The ROC curves showed the following cut-off points for GLS, ASI and LAVI: -13.8%, -13.7% and 41 ml/m2, respectively. Cox’s proportional hazards model for the parameters used in the Calculator was at the borderline of significance; p = 0.04385. The model with new variables (GLS and LAVI instead of LA dimension) was significant; p = 0.00094. The important factors were LAVI; p = 0.000075 and nsVT; p = 0.012267. CONCLUSIONS The proposed new SCD risk factors were statistically significant in the study population and should be taken into account when considering ICD implantation.

scholarly journals Noninvasive risk factors for the prediction of inducibility on programmed ventricular stimulation in post-MI patients with ejection fraction over 40% at SCD risk, insights from the PRESERVE EF study

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
K Trachanas ◽  
P Arsenos ◽  
I Xenogiannis ◽  
K Tsimos ◽  
K Triantafyllou ◽  
...  
Keyword(s):  
Risk Factors ◽  
Risk Stratification ◽  
Risk Score ◽  
Systolic Function ◽  
High Sensitivity ◽  
Left Ventricular ◽  
Final Decision ◽  
Icd Implantation ◽  
Programmed Ventricular Stimulation ◽  
Ventricular Stimulation

Abstract Funding Acknowledgements Type of funding sources: None. Background Sudden cardiac arrest (SCA) in post myocardial infarction (post-MI) patients with a relatively preserved left ventricular systolic function (LVEF≥40%) has an annual incidence of 1%, in the absence of adequate risk stratification methods and guideline recommendations for primary prevention. In the PRESERVE-EF study we used a two-step SCA risk stratification approach to detect patients at risk for major arrhythmic events. Seven noninvasive risk factors (NIRFs) were extracted from ambulatory electrocardiography (AECG). Patients with at least one NIRF present were referred for invasive programmed ventricular stimulation (PVS). Inducible patients received an ICD. Purpose To assess the performance of NIRFs extracted from 24hr AECG, based on the PRESERVE EF criteria, in predicting inducibility. Methods The PRESERVE EF study enrolled 575 patients. Two hundred and four of them had at least one NIRF and an indication for PVS, but 52 of them declined. Finally, 41 out of 152 patients who underwent PVS were inducible. For the present analysis data from these 152 patients (mean age 60 ± 10years, LVEF 49 ± 6%, 89% males) were analyzed. Chi-square test, univariate logistic regression and areas under ROC curves were calculated for the PVS inducibility endpoint. Results Age, male gender and LVEF for the PVS inducible patients group (n = 41) and the noninducible patients group (n = 111) were, respectively: 61 ± 9years vs 59 ± 10years (p = 0.310), 98% vs 86% (p = 0.048), 45 ± 4% vs 51 ± 7% (p &lt; 0.001). Among NIRFs examined, LVEF ≤ 50%, nsVT≥1/24hour and presence of LPs on SAECG presented high and significant Odds Ratios (ORs) for a positive PVS study end point. A simple risk score based on cutoff points of LVEF ≤ 50%, NSVTepisode≥1/24hour and presence of LPs missed only 1 out of the 41 inducible patients and yielded: OR 14.146 (p = 0.01) with a high sensitivity 98% but low specificity 26% for a positive PVS (AUC = 0.65). Conclusion Cut off points of LVEF ≤ 50%, nsVTepisode≥1/24hour and presence of LPs were important predictors of inducibility. A simple risk score based on these predictors achieves high sensitivity but low specificity.  The final decision for an ICD implantation should be based on a positive PVS, which is irreplaceable in risk stratification.

scholarly journals Sex-specific differences in risk stratification of ventricular arrhythmias among implantable cardioverter defibrillator patients: do women differ from men?

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A.C.J Van Der Lingen ◽  
D.A.M.J Theuns ◽  
M.A.J Becker ◽  
A.C Van Rossum ◽  
V.P Van Halm ◽  
...  
Keyword(s):  
Risk Factors ◽  
Risk Stratification ◽  
Secondary Prevention ◽  
Ventricular Arrhythmias ◽  
Study Cohort ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Prevention Therapy ◽  
Male Patients

Abstract Background Implantable cardioverter defibrillator (ICD) guidelines and risk stratification models of sudden cardiac death (SCD) are applied without differentiation between men and women, based on the assumption that the incidence of ventricular arrhythmias and risk factors of SCD are similar in both sexes. Sex-specific risk factors of SCD may influence studies evaluating the benefit of ICD therapy, for both men and women. Purpose Aim of the study is to assess sex-specific differences in occurrence and predictors of appropriate device therapy (ADT) for ventricular arrhythmias. Methods A multicenter retrospective cohort of 2300 consecutive patients was evaluated, including patients referred for ICD implantation between the years 2009–2018 (age 62±13 years, LVEF 32±12%, 53% ischemic cardiomyopathy [CMP], 28% resynchronization therapy, 65% primary prevention). Exclusion criteria were: (1) patients with hypertrophic CMP, arrhythmogenic right ventricular CMP, systemic infiltrative cardiac disease or channelopathy; (2) lost to follow-up immediately after ICD implantation. Primary endpoint was ADT, defined as anti-tachycardia pacing or shock for ventricular tachyarrhythmia. Secondary endpoints were mortality and inappropriate ICD therapy. Univariable and multivariable Cox regression analyses, stratified by sex, were performed to assess predictors of ADT. Results The cohort primarily consisted of men (75%). After a mean follow-up of 4.8±3.0 years, men experienced more ADT compared to women (25% versus 16%, HR 1.71, p&lt;0.001) and men displayed a higher mortality compared to women (25% versus 19%, HR 1.37, p&lt;0.01). No difference in inappropriate ICD therapy was observed (9% versus 10%, HR 1.01, p=0.94). In the total study cohort, male sex (HR 1.55, p&lt;0.001), higher age (HR 1.15 per 10 years, p&lt;0.0019), left bundle branch block (LBBB, HR 0.74, p=0.01) and secondary prevention indication (HR 1.89, p&lt;0.001) were independently associated with ADT. In male patients, independent predictors of ADT were comparable with the total study cohort: higher age (HR 1.20 per 10 years, p&lt;0.001), LBBB (HR 0.72, p=0.01) and secondary prevention therapy (HR 1.80, p&lt;0.001). In contrast, age (p=0.54) or LBBB (p=0.29) were not associated with ADT in women. In women, only paroxysmal atrial fibrillation (HR 1.76, p=0.03) and secondary prevention therapy (HR 1.78, p&lt;0.01) were independently associated with ADT. Conclusion This study showed that men were at higher risk of ADT compared to women and that risk factors associated with SCD differ between both sexes. The results strongly suggests that SCD risk stratification models are primarily driven by male patients and sex-specific risk models of SCD are needed to identify those women at high risk of SCD. Figure 1 Funding Acknowledgement Type of funding source: None

P887Atrial fibrillation is associated with sudden cardiac death risk factors in patients with hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I A Marcuschamer ◽  
O Zusman ◽  
S Schwartzenberg ◽  
M Vaturi ◽  
Y Shapira ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Nearest Neighbor ◽  
Nyha Class ◽  
Left Ventricular ◽  
Similar Distribution ◽  
Icd Implantation

Abstract Background Atrial Fibrillation (AF) is prevalent in a fourth of patients with Hypertrophic Cardiomyopathy (HCM), but its clinical impact in these patients remains ill-defined. Aim To compare clinical characteristics in HCM patients with vs without AF and assess indirectly potential sudden cardiac death (SCD) risk. Methods Retrospective study in a single tertiary referral HCM center. Patients with HCM and AF were compared with matched controlled HCM patients without AF. NYHA class was assessed by a single physician. Propensity score matching was performed with a ratio of 2:1 by nearest neighbor with adjustment for age, sex, and left ventricular tract obstruction (LVOTO). Ordinal regression was used with NYHA as outcome. Results Among 298 patients with HCM, 68 patients (22.8%) had AF. After propensity matching, 66 patients with AF and 112 without AF had similar distribution of age (67.1 vs. 65.1 years), gender (57.6% vs. 61% males) and Basal Surface Area (1.88 vs. 1.87 m2) respectively. The prevalence of LVOTO (57.6% vs. 58.5%) and apical hypertrophy (19.7% vs 19.5%) was similar in the two groups. Cardiac risk factors including Hypertension (60.6% vs. 60.2%) and Diabetes Mellitus (15.2 vs. 20.3%) were similar in both groups. AF patients were diagnosed with HCM at a younger age than patients without HCM (48.5 vs. 55 years; p=0.01). HCM patients with AF had significantly lower LVOT gradients compared with patients without AF (28.1 mmHg vs 47.4 mmHg, p=0.005), had a higher prevalence of non-sustained ventricular tachycardia (39.4% vs. 9.4%; p<0.01), and ventricular tachycardia (9.1% vs 1.7%; p<0.04) and were more likely to have undergone implantation of an internal cardioverter defibrillator (ICD) (23.1% vs. 8.5%; p=0.001), respectively. Dyspnea was the most prevalent symptom in both groups (51.1% and 46.6% in AF and non-AF respectively). NYHA Class was similar in both groups: 1.88±0.69 in patients with AF vs. 1.73±0.74 in patients without AF (p=0.17). NYHA class did not differ in 26 patients with chronic persistent AF vs. 42 patients with paroxysmal AF (being in sinus rhythm at evaluation). Conclusion AF does not seem to impact functional level class in patients with HCM, but carries a higher burden of sudden cardiac death prognostic factors, incurring a higher rate of ICD implantation. Acknowledgement/Funding None

scholarly journals Prognostic significance of cardiac magnetic resonance-based markers in patients with hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Zsofia Dohy ◽  
Liliana Szabo ◽  
Attila Toth ◽  
Csilla Czimbalmos ◽  
Rebeka Horvath ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Myocardial Fibrosis ◽  
Ventricular Arrhythmias ◽  
Prognostic Significance ◽  
Strain Analysis ◽  
Left Ventricular ◽  
Icd Therapy ◽  
Lv Mass

AbstractThe prognosis of patients with hypertrophic cardiomyopathy (HCM) varies greatly. Cardiac magnetic resonance (CMR) is the gold standard method for assessing left ventricular (LV) mass and volumes. Myocardial fibrosis can be noninvasively detected using CMR. Moreover, feature-tracking (FT) strain analysis provides information about LV deformation. We aimed to investigate the prognostic significance of standard CMR parameters, myocardial fibrosis, and LV strain parameters in HCM patients. We investigated 187 HCM patients who underwent CMR with late gadolinium enhancement and were followed up. LV mass (LVM) was evaluated with the exclusion and inclusion of the trabeculae and papillary muscles (TPM). Global LV strain parameters and mechanical dispersion (MD) were calculated. Myocardial fibrosis was quantified. The combined endpoint of our study was all-cause mortality, heart transplantation, malignant ventricular arrhythmias and appropriate implantable cardioverter defibrillator (ICD) therapy. The arrhythmia endpoint was malignant ventricular arrhythmias and appropriate ICD therapy. The LVM index (LVMi) was an independent CMR predictor of the combined endpoint independent of the quantification method (p < 0.01). The univariate predictors of the combined endpoint were LVMi, global longitudinal (GLS) and radial strain and longitudinal MD (MDL). The univariate predictors of arrhythmia events included LVMi and myocardial fibrosis. More pronounced LV hypertrophy was associated with impaired GLS and increased MDL. More extensive myocardial fibrosis correlated with impaired GLS (p < 0.001). LVMi was an independent CMR predictor of major events, and myocardial fibrosis predicted arrhythmia events in HCM patients. FT strain analysis provided additional information for risk stratification in HCM patients.

Angina Pectoris Due to Severe Muscular Bridge in Hypertrophic Cardiomyopathy

1998 ◽  
Vol 6 (2) ◽  
pp. 132-134
Author(s):  
M Şah Topcuoĝlu ◽  
Ayhan Usal ◽  
Cem Kayhan ◽  
Aladdin Pekedis ◽  
Acar Tokcan ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Calcium Antagonist ◽  
Hypertrophic Cardiomyopathy ◽  
Angina Pectoris ◽  
Left Ventricular Outflow Tract ◽  
Young Patients ◽  
Ventricular Outflow Tract ◽  
Myocardial Scintigraphy ◽  
Left Ventricular ◽  
Left Ventricular Outflow

We report the case of a 39-year-old male with hypertrophic cardiomyopathy who complained of angina pectoris. The patient was treated with a beta blocker and a calcium antagonist without effect. Myocardial scintigraphy revealed anterior ischemia. Cardiac catheterization and ventriculography revealed severe systolic narrowing of the left anterior descending coronary artery and no significant pressure gradient across the left ventricular outflow tract. Myotomy was performed on a muscular bridge over the left anterior descending coronary artery and the patient's angina was relieved. In young patients with hypertrophic cardiomyopathy who develop angina refractory to medical therapy, a coexisting muscular bridge should be sought.

scholarly journals Baseline clinical features in a large-scale registration survey of patient with hypertrophic cardiomyopathy throughout Japan: J-HCM registry study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Kubo ◽  
H Takano ◽  
M Takayama ◽  
Y.L Doi ◽  
Y Minami ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Clinical Features ◽  
Clinical Characteristics ◽  
Large Scale ◽  
Risk Group ◽  
Nyha Class ◽  
Left Ventricular ◽  
Registry Study ◽  
Icd Implantation ◽  
Successful Recovery

Abstract Background Hypertrophic cardiomyopathy (HCM) is a most prevalent primary myocardial disorder with heterogeneous clinical features. However, there have been few studies on clinical features of HCM as a prospective cohort. In 2015, we established a large-scale registration survey of patients with HCM throughout Japan, named J-HCM registry study. Purpose The aim of this study was to clarify the clinical features of Japanese patients with HCM. Methods J-HCM registry study is a prospective, multicenter investigation, consisting of 24 hospitals. This time, we present the baseline clinical characteristics in this survey. Results Total 1484 patients were registered. The ages at registration and at diagnosis were 65±15 and 56±17 years, respectively, and 806 patients (54%) were men. Majority of the patients (95%) was NYHA class I or II. With regard to subtypes of HCM, there were 526 patients (36%) in the HCM with left ventricular (LV) outflow tract obstruction, 126 patients (8%) in the mid-ventricular obstruction, 57 patients (4%) in the end-stage phase characterized by LV ejection fraction &lt;50%, and 197 patients (14%) in apical HCM. At registration, 80 patients (6%) had prior successful recovery from sustained ventricular tachycardia or ventricular fibrillation, 162 patients (11%) suffered from heart failure hospitalization, and 64 patients (4%) had history of embolic event. Regarding invasive treatment, 160 patients (10%) had prior septal reduction therapy and 162 patients (11%) had ICD implantation. According to the 2014 European Society of Cardiology Guidelines on sudden cardiac death (SCD) prevention, the study patients were divided into 3 categories by the HCM Risk-SCD calculator: patients distribution, 4% in the high risk group (≥6% calculated HCM Risk-SCD at 5 years), 7% in the intermediate risk group (4% to &lt;6%), 69% in the low risk group (&lt;4%), and 16% in the patients with extreme characteristics (Figure 1). Conclusions In this multicenter registration survey of patients with HCM, the baseline clinical characteristics were almost similar to several retrospective large-scale cohorts in Western countries except older age and less symptomatic state. This study will provide important knowledge regarding management of HCM. Figure 1 Funding Acknowledgement Type of funding source: None

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