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.

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.

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.

scholarly journals Multimodality Imaging in Hypertrophic Cardiomyopathy for Risk Stratification

2020 ◽  
Vol 13 (2) ◽  
Author(s):  
Albree Tower-Rader ◽  
Christopher M. Kramer ◽  
Stefan Neubauer ◽  
Sherif F. Nagueh ◽  
Milind Y. Desai
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Left Ventricular Outflow Tract ◽  
Multimodality Imaging ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Left Ventricular Outflow ◽  
Sudden Cardiac Death Risk

In hypertrophic cardiomyopathy, multimodality imaging is crucial to confirm diagnosis, assess for presence and mechanism of left ventricular outflow tract obstruction, and risk stratification for sudden cardiac death. This review will focus on the application of imaging to assess established and emerging factors to be considered in sudden cardiac death risk stratification.

scholarly journals Hypertrophic Cardiomyopathy Mimicking Acute Anterior Myocardial Infarction Associated with Sudden Cardiac Death

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Y. Daralammouri ◽  
M. El Garhy ◽  
K. Same ◽  
B. Lauer
Keyword(s):  
Myocardial Infarction ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Valsalva Maneuver ◽  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Anterior Myocardial Infarction ◽  
Left Ventricular Outflow

Hypertrophic cardiomyopathy is the most common genetic disease of the heart. We report a rare case of hypertrophic obstructive cardiomyopathy mimicking an acute anterior myocardial infarction associated with sudden cardiac death. The patient presented with acute ST elevation myocardial infarction and significant elevation of cardiac enzymes. Cardiac catheterization showed some atherosclerotic coronary artery disease, without significant stenosis. Echocardiography showed left ventricular hypertrophy with a left ventricular outflow tract obstruction; the pressure gradient at rest was 20 mmHg and became severe with the Valsalva maneuver (100 mmHg). There was no family history of sudden cardiac death. Six days later, the patient suffered a syncope on his way to magnetic resonance imaging. He was successfully resuscitated by ventricular fibrillation.

Left Ventricular Outflow Tract Obstruction as a Risk Factor for Sudden Cardiac Death in Hypertrophic Cardiomyopathy

2009 ◽  
Vol 104 (5) ◽  
pp. 695-699 ◽  
Author(s):  
Georgios K. Efthimiadis ◽  
Despina G. Parcharidou ◽  
Georgios Giannakoulas ◽  
Efstathios D. Pagourelias ◽  
Panagiotis Charalampidis ◽  
...  
Keyword(s):  
Risk Factor ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Ventricular Outflow Tract Obstruction ◽  
Left Ventricular Outflow

Sudden Cardiac Death: A Modern Pathology Approach to Hypertrophic Cardiomyopathy

2015 ◽  
Vol 139 (3) ◽  
pp. 413-416 ◽  
Author(s):  
Linda Kocovski ◽  
John Fernandes
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Cardiac Myocyte ◽  
Clinical Symptoms ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Exertional Dyspnea ◽  
Left Ventricular Outflow ◽  
Cardiac Myocyte Hypertrophy

Hypertrophic cardiomyopathy is one of the most common causes of sudden cardiac death among young adults and adolescents. Unfortunately, the first manifestation of the condition may be sudden death during exertion, such as sporting activities. Other clinical symptoms include exertional dyspnea, angina, and syncope. Postmortem examination often reveals asymmetrical septal thickening and mural plaque formation in the left ventricular outflow tract. Histologic analysis shows cardiac myocyte hypertrophy, myofiber disarray, and interstitial and replacement fibrosis. Molecular analysis for known genetic abnormalities is essential to genetic counseling of living relatives of decedents to assess and reduce the risk of sudden cardiac death from hypertrophic cardiomyopathy.

The relationship between left ventricular outflow tract gradient and sudden cardiac death in childhood hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Norrish ◽  
T Ding ◽  
E Field ◽  
C O'Mahony ◽  
P.M Elliott ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Inverse Relationship ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Funding Source ◽  
Left Ventricular Outflow ◽  
The Relationship

Abstract Background The most common mode of death in childhood hypertrophic cardiomyopathy (HCM) is sudden cardiac death (SCD). Left ventricular outflow tract obstruction (LVOTO) is an established risk factor for SCD in adults with the disease. In contrast, the prognostic implications of LVOTO in childhood disease is unclear, with recent studies suggesting that it may have an inverse relationship with the risk of SCD. Purpose The aim of this study was to explore the role of LVOTO and the risk of SCD in childhood HCM. Methods A multi-centre, retrospective, longitudinal cohort of 871 children (diagnosed with HCM &lt;16 years of age) was used to explore the relationship between SCD and LVOTO (LVOT gradient ≥30mmHg). Results 189 patients (23%) had LVOTO, which was mild (30–50mmHg), moderate (50–100mmHg) or severe (&gt;100mmHg) in 58 (6.7%), 98 (11.3%) and 33 (3.8%), respectively. The risk of SCD showed an inverse relation to LVOT gradient severity compared to those with no obstruction: mild HR 1.75 (95% CI 0.89–3.44), moderate HR 1.04 (95% 0.55–1.98), and severe HR 0.7 (0.36–1.35) [figure].On univariable analysis [table] LVOTO was associated with heart failure symptoms (NYHA&gt;1) [p &lt;0.001], maximal wall thickness (MWT) [p &lt;0.001], left atrial (LA) diameter [p &lt;0.001], and future myectomy occurring during follow up [p &lt;0.001]. The inverse relationship observed was not altered by the presence or absence of other traditional risk factors. Conclusions LVOTO appears to have a complex relationship with the risk of SCD in childhood HCM, with multiple contributing factors. The pathophysiological mechanisms behind this observation need further exploration, which may be limited by low patient numbers Cummulative incidence of SCD by LVOTO Funding Acknowledgement Type of funding source: Other. Main funding source(s): British Heart Foundation

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 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.

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