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.

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.

Diabetes and arrhythmias

ESC CardioMed ◽  
2018 ◽  
pp. 941-944
Author(s):  
Heikki Huikuri ◽  
Lars Rydén
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Vitamin K ◽  
Cardiac Death ◽  
Vitamin K Antagonists ◽  
Left Ventricular ◽  
Sustained Ventricular Tachycardia ◽  
Patients With Diabetes ◽  
Diagnostic Work

Cardiac arrhythmias are more common in subjects with diabetes mellitus (DM) than in their counterparts without diabetes. Atrial fibrillation (AF) is present in 10–20% of the DM patients, but the association between DM and AF is mostly due to co-morbidities of DM patients increasing the vulnerability to AF. When type 2 DM and AF coexist, there is a substantially higher risk of cardiovascular mortality, stroke, and heart failure, which indicates screening of AF in selected patients with DM. Anticoagulant therapy either with vitamin K antagonists or non-vitamin K antagonist oral anticoagulants is recommended for DM patients with either paroxysmal or permanent AF, if not contraindicated. Palpitations, premature ventricular beats, and non-sustained ventricular tachycardia are common in patients with DM. The diagnostic work-up and treatment of these arrhythmias does not differ between the patients with or without DM. The diagnosis and treatment of sustained ventricular tachycardia, either monomorphic or polymorphic ventricular tachycardia, or resuscitated ventricular fibrillation is also similar between the patients with or without DM. The risk of sudden cardiac death is higher in DM patients with or without a diagnosed structural heart disease. Patients with diabetes and a left ventricular ejection fraction less than 30–35% should be treated with a prophylactic implantable cardioverter defibrillator according to current guidelines. Beta-blocking therapy is recommended for DM patients with left ventricular dysfunction or heart failure to prevent sudden cardiac death due to arrhythmia.

Abstract 10947: Longer and Faster Non-sustained Ventricular Tachycardia in Hypertrophic Cardiomyopathy Patients Predicts for Implantable Cardioverter Defibrillator Shocks and Sudden Cardiac Death

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Weijia Wang ◽  
Zhesi Lian ◽  
Ethan Rowin ◽  
Martin Maron ◽  
Mark Link
Keyword(s):  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Medical Center ◽  
Multivariable Analysis ◽  
Sustained Ventricular Tachycardia ◽  
Cardioverter Defibrillator

Introduction: Non-sustained ventricular tachycardia (NSVT) may be underestimated in patients with hypertrophic cardiomyopathy (HCM). Its impact on the risk of sudden cardiac death (SCD) in HCM is controversial. There is no distinction made in the guidelines as to the length or rate of NSVT as a risk marker for SCD. Hypothesis: NSVT may be nearly universal in HCM patients with high risk of SCD and not found because of the limited time frame of monitoring. NSVT may be associated with appropriate Implantable Cardioverter Defibrillator (ICD) shocks and SCD. Methods: A retrospective study of 181 HCM patients who had an ICD and were followed for at least 6 months from 2000 to 2013 at Tufts Medical Center was performed. The pre-operative evaluations as well as routine ICD follow up notes were reviewed. Results: ICD was implanted in 175 (96.7%) patients as primary prevention and in 6 (3.3%) patients as secondary prevention for SCD. Ninety six (53.0%) patients total had NSVT, including 48 (26.5%) before and 77 (42.5%) after ICD implantation. The agreement for detecting NSVT between Holter monitoring and ICD interrogation was poor (Kappa=0.18, p=0.054). Eighteen (18.75%) patients with NSVT and 6 (7.06%) patients without NSVT had appropriate ICD shocks or SCD (Figure 1). In multivariable analysis, NSVT was independently associated with appropriate ICD shocks and SCD (OR 3.69, 95%CI: 1.31 - 10.43) and remained significant in the 175 patients who had ICD implanted as primary prevention (OR 3.86, 95%CI: 1.13 - 13.18). More rapid NSVT (Cl < 310ms) predicted appropriate ICD shocks and SCD (OR 7.7, 95%CI: 1.6, 36.8), and longer NSVT (> 18beats) also predicted appropriate ICD shocks and SCD (OR=23.7, 95%CI: 2.7, 204.9). Conclusion: The agreement for detecting NSVT between Holter and ICD interrogation is poor. NSVT is significantly associated with appropriate ICD shocks and SCD. Faster and longer NSVT are even more predictive. Extending rhythm monitor time merits consideration in HCM patients.

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.

Sign in / Sign up

Export Citation Format

Share Document