P5023A mutation specific prediction model for ventricular arrhythmias in the phospholamban (PLN) p.Arg14del cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T E Verstraelen ◽  
F H M Van Lint ◽  
L P Bosman ◽  
B G S Abeln ◽  
F W Asselbergs ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Prediction Model ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
P Value ◽  
Icd Implantation ◽  
Specific Prediction ◽  
Repolarization Abnormalities

Abstract Background/Introduction The founder mutation p.Arg14del in the gene encoding phospholamban (PLN) is a known cause of arrhythmogenic cardiomyopathy (ACM) with distinct clinical features, such as microvoltages on the ECG and right or left ventricular dysfunction or both. At present, risk stratification for ICD implantation in carriers of this mutation is based on left ventricular ejection fraction and previous ventricular arrhythmia's, which are not specific risk factors for the PLN p.Arg14del cardiomyopathy. Purpose Our goal is to develop a mutation specific prediction model for incident malignant ventricular arrhythmia to guide ICD implantation. Methods Data were collected from p.Arg14del carriers with no history of malignant VA at baseline, identified between September 2009 and June 2018 in three Dutch university hospitals. Genetic analysis of PLN was performed in a clinical setting in index patients with clinical signs of DCM/ACM, or in family members of p.Arg14del carriers. We collected clinical data from the first cardiac evaluation and follow-ups. Malignant VAs were defined as sustained VA, appropriate ICD intervention or (aborted) sudden cardiac death. A prediction model was developed using Cox Proportional Hazard regression. Candidate baseline predictors were pre specified based on literature and clinical expertise. Age, sex, proband status, sudden cardiac death (SCD) in 1st degree relative, repolarization abnormalities, microvoltages, premature ventricular complexes (PVC) burden on 24hrs Holter monitoring, LVEF and non-sustained ventricular arrhythmias (NSVT) were considered. The multivariable model was fitted using stepwise backward selection based on Akaike's Information Criterion. Results We included 440 p.Arg14del carriers with a mean age of 41±18 years and 41% males. During a median follow-up of 4.7 years (IQR 1.7–7.3), 44 incident malignant VA occurred, 20 sustained VAs and 24 appropriate ICD therapies. The multivariable HR's of selected predictors were: 2.2 for minor and 4.5 for major repolarization abnormalities vs no abnormalities (p value respectively 0.06 and 0.01), 2.2 for LVEF <45% (p value 0,1) and 7.7 for >500PVC/24hrs (p value 0.003). Carriers with and without events could be accurately distinguished with this model, with an optimism corrected C-statistic of 0.81. The model calibrated well, with agreement of observed and predicted 5 year malignant VA risk. Risk groups were split into quintiles of predicted risk, figure 1 shows the Kaplan Meier curve of incident malignant VA per risk group. The 5 year risk of incident malignant VA in the lowest 3 quintiles was 0%, 9% in the 4th quintile and 25.2% in the highest quintile. Conclusion We created a PLN p.Arg14del mutation specific prediction model to estimate risk of incident malignant VA. With this model a clear distinction between high risk and low risk patients can be made and can be used to guide ICD implantation for primary prevention.

scholarly journals LEFT VENTRICULAR LEAD IMPLANTATION FOR DETECTION OF VENTRICULAR ARRHYTHMIAS IN PATIENT WITH IMPLANTABLE CARDIOVERTER DEFIBRILLATOR AND LOW R WAVE IN RIGHT VENTRICLE

2021 ◽  
pp. 17
Author(s):  
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricle ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Systolic Function ◽  
Left Ventricular ◽  
Icd Implantation ◽  
Cardioverter Defibrillator ◽  
Ventricular Lead

Dilated cardiomyopathy (DCM) is a disease characterised as left ventricular (LV) or biventricular dilatation with impaired systolic function. Regardless of underlying cause patients with DCM have a propensity to ventricular arrhythmias and sudden cardiac death. Implantable Cardioverter Defibrillator (ICD) implantation for these patients results in significant reduction of sudden cardiac death [1-3]. ICD devices may be limited by right ventricle (RV) sensing dysfunction with low RV sensing amplitude. We present a clinical case of patient with DCM, implanted ICD and low R wave sensing on RV lead.

scholarly journals Development and Validation of a Multivariable Risk Prediction Model for Sudden Cardiac Death after Myocardial Infarction (PROFID Risk Model): Study Rationale, Design and Protocol

2021 ◽  
Author(s):  
Glen Phillp Martin ◽  
Gerhard Hindricks ◽  
Artur Akbarov ◽  
Zoher Kapacee ◽  
Le Mai Parkes ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Prediction Model ◽  
Risk Prediction ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Risk Prediction Model ◽  
Left Ventricular Ejection ◽  
Analytical Approaches

Introduction Sudden cardiac death (SCD) is the leading cause of death in patients with myocardial infarction (MI) and can be prevented by the implantable cardioverter defibrillator (ICD). Currently, risk stratification for SCD and decision on ICD implantation are based solely on impaired left ventricular ejection fraction (LVEF). However, this strategy leads to over- and under-treatment of patients because LVEF alone is insufficient for accurate assessment of prognosis. Thus, there is a need for better risk stratification. This is the study protocol for developing and validating a prediction model for risk of SCD in patients with prior MI. Methods and Analysis The EU funded PROFID project will analyse 23 datasets from Europe, Israel and the US (~225,000 observations). The datasets include patients with prior MI or ischemic cardiomyopathy with reduced LVEF<50%, with and without a primary prevention ICD. Our primary outcome is SCD in patients without an ICD, or appropriate ICD therapy in patients carrying an ICD as a SCD surrogate. For analysis, we will stack 18 of the datasets into a single database (datastack), with the remaining analysed remotely for data governance reasons (remote data). We will apply 5 analytical approaches to develop the risk prediction model in the datastack and the remote datasets, all under a competing risk framework: 1) Weibull model, 2) flexible parametric survival model, 3) random forest, 4) likelihood boosting machine, and 5) neural network. These dataset-specific models will be combined into a single model (one per analysis method) using model aggregation methods, which will be externally validated using systematic leave-one-dataset-out cross-validation. Predictive performance will be pooled using random effects meta-analysis to select the model with best performance. Ethics and dissemination Local ethical approval was obtained. The final model will be disseminated through scientific publications and a web-calculator. Statistical code will be published through open-source repositories.

scholarly journals Updating the Risk Stratification for Sudden Cardiac Death in Cardiomyopathies: The Evolving Role of Cardiac Magnetic Resonance Imaging. An Approach for the Electrophysiologist

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 541 ◽  
Author(s):  
Ourania Kariki ◽  
Christos-Konstantinos Antoniou ◽  
Sophie Mavrogeni ◽  
Konstantinos A. Gatzoulis
Keyword(s):  
Cardiac Magnetic Resonance ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Magnetic Resonance ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
High Risk Patients ◽  
Risk Patients

The prevention of sudden cardiac death (SCD) in cardiomyopathies (CM) remains a challenge. The current guidelines still favor the implantation of devices for the primary prevention of SCD only in patients with severely reduced left ventricular ejection fraction (LVEF) and heart failure (HF) symptoms. The implantation of an implantable cardioverter-defibrillator (ICD) is a protective barrier against arrhythmic events in CMs, but the benefit does not outweigh the cost in low risk patients. The identification of high risk patients is the key to an individualized prevention strategy. Cardiac magnetic resonance (CMR) provides reliable and reproducible information about biventricular function and tissue characterization. Furthermore, late gadolinium enhancement (LGE) quantification and pattern of distribution, as well as abnormal T1 mapping and extracellular volume (ECV), representing indices of diffuse fibrosis, can enhance our ability to detect high risk patients. CMR can also complement electro-anatomical mapping (EAM), a technique already applied in the risk evaluation and in the ventricular arrhythmias ablation therapy of CM patients, providing a more accurate assessment of fibrosis and arrhythmic corridors. As a result, CMR provides a new insight into the pathological substrate of CM. CMR may help identify high risk CM patients and, combined with EAM, can provide an integrated evaluation of scar and arrhythmic corridors in the ablative therapy of ventricular arrhythmias.

Idiopathic HFrEF. Is there room left for defibrillators?

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
F Gama ◽  
M.S Carvalho ◽  
G Rodrigues ◽  
F.M Costa ◽  
D Matos ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Sudden Cardiac Death ◽  
Ejection Fraction ◽  
Medical Therapy ◽  
Remote Monitoring ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Optimal Medical Therapy ◽  
Left Ventricular Ejection ◽  
Icd Implantation

Abstract Background and aim Prophylactic implantation of an implantable cardioverter-defibrillator (ICD) is class 1 recommendation for heart failure (HF) patients with reduced ejection fraction (HFrEF) even though its proven advantage is weaker among nonischemic aetiology. In fact, in an era where both optimal medical therapy (OMT) and cardiac resynchronization therapy (CRT) significantly reduce sudden cardiac death (SCD), it is questionable whether ICD still have additional value. The aim of this study was to assess the current benefit of ICDs in preventing sudden cardiac death through resuscitated cardiac arrest (RCA), appropriate therapy for sustained ventricular tachycardia (VT) or fibrillation (VF) in a contemporary population of idiopathic HFrEF patients. Methods Single-centre retrospective study of consecutive symptomatic (NYHA class II to IV) idiopathic HFrEF patients with an ICD (either alone or in association with CRT), and remote monitoring with the corresponding software (MerlinTM, LatitudeTM, CarelinkTM, MicroPortTM or BiotronikTM) to assure appropriate event supervising. Idiopathic aetiology was assumed after excluding other probable causes. Coronary angiogram was required to exclude ischemic aetiology. Only those with prophylactic ICD implantation were included. RCA was defined as collapse with clinical signs of cardiac arrest and VF or VT appropriately terminated by ICD. In order to be sustained, VT episode had to have last at least 30 seconds. Results From 781 remote monitoring controlled patients, a total of 187 consecutive symptomatic idiopathic HFrEF patients with an ICD (125 men, mean age 64±18 years) were enrolled. Patients were on optimal medical therapy (ACEi/ARB: n=168, 90%; BB: n=154, 82%; mineralocorticoid antagonists: n=91, 49%; CRT: n=130, 70%; see Table). After a median follow-up of 99 months (IQR 62.2), RCA occurred in 10.7% (n=20) and 36.9% (n=69) had appropriately terminated VT. Both left ventricular ejection fraction (LVEF) improvement and CRT implantation did not independently reduce the incidence of RCA and VT requiring ICD therapy (OR, 1.02; 95% CI, 0.99–1.05; P=0.146 and OR, 0.85; 95% CI, 0.34–2.13; P=0.728; respectively). All cause mortality was 20 (10.7%). Inappropriate therapy was given as shocks to 41 patients (21.9%) and as antitachycardia pacing (ATP) to 30 (16%), opposing with appropriately given therapy to 43 (23%) and 63 (33.7%) patients, respectively (see Figure), contributing to a net clinical benefit (NCB) of 18.8%, favouring ICD implantation. Conclusion In this contemporaneous real-world population of symptomatic idiopathic HFrEF patients, episodes of impending cardiac death were frequent. Prophylactic ICD implantation seems to have added further benefit reducing SCD on top of optimal medical therapy, LVEF improvement and coexisting CRT. Funding Acknowledgement Type of funding source: None

scholarly journals CMR-Based Risk Stratification of Sudden Cardiac Death and Use of Implantable Cardioverter–Defibrillator in Non-Ischemic Cardiomyopathy

2021 ◽  
Vol 22 (13) ◽  
pp. 7115
Author(s):  
Laura Keil ◽  
Céleste Chevalier ◽  
Paulus Kirchhof ◽  
Stefan Blankenberg ◽  
Gunnar Lund ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Ischemic Cardiomyopathy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Cardioverter Defibrillator

Non-ischemic cardiomyopathy (NICM) is one of the most important entities for arrhythmias and sudden cardiac death (SCD). Previous studies suggest a lower benefit of implantable cardioverter–defibrillator (ICD) therapy in patients with NICM as compared to ischemic cardiomyopathy (ICM). Nevertheless, current guidelines do not differentiate between the two subgroups in recommending ICD implantation. Hence, risk stratification is required to determine the subgroup of patients with NICM who will likely benefit from ICD therapy. Various predictors have been proposed, among others genetic mutations, left-ventricular ejection fraction (LVEF), left-ventricular end-diastolic volume (LVEDD), and T-wave alternans (TWA). In addition to these parameters, cardiovascular magnetic resonance imaging (CMR) has the potential to further improve risk stratification. CMR allows the comprehensive analysis of cardiac function and myocardial tissue composition. A range of CMR parameters have been associated with SCD. Applicable examples include late gadolinium enhancement (LGE), T1 relaxation times, and myocardial strain. This review evaluates the epidemiological aspects of SCD in NICM, the role of CMR for risk stratification, and resulting indications for ICD implantation.

Beyond ejection fraction: Novel clinical approaches towards sudden cardiac death risk stratification in patients with dilated cardiomyopathy

2021 ◽  
Vol 17 ◽  
Author(s):  
Issa Pour-Ghaz ◽  
Mark Heckle ◽  
Ikechukwu Ifedili ◽  
Sharif Kayali ◽  
Christopher Nance ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Ejection Fraction ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Ventricular Tachyarrhythmia ◽  
Total Mortality ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Icd Therapy

: Implantable cardioverter-defibrillator (ICD) therapy is indicated for patients at risk for sudden cardiac death due to ventricular tachyarrhythmia. The most commonly used risk stratification algorithms use left ventricular ejection fraction (LVEF) to determine which patients qualify for ICD therapy, even though LVEF is a better marker of total mortality than ventricular tachyarrhythmias mortality. This review evaluates imaging tools and novel biomarkers proposed for better risk stratifying arrhythmic substrate, thereby identifying optimal ICD therapy candidates.

New techniques for risk assessment in sudden cardiac death

ESC CardioMed ◽  
2018 ◽  
pp. 2327-2330
Author(s):  
Juan Fernandez-Armenta ◽  
Antonio Berruezo ◽  
Juan Acosta ◽  
Diego Penela
Keyword(s):  
Risk Assessment ◽  
Sudden Cardiac Death ◽  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Ventricular Ejection ◽  
Left Ventricular Ejection ◽  
Individual Risk ◽  
Ventricular Ejection Fraction

Risk stratification for sudden cardiac death (SCD) is one of the main objectives of clinical arrhythmology. Despite increased knowledge of the fundamental basis and predictors of SCD, the estimation of individual risk remains challenging. To date, symptomatic heart failure and reduced left ventricular ejection fraction are the main variables used to identify patients at high risk of SCD who could potentially benefit from preventive therapies. Beyond left ventricular ejection fraction, new diagnostic tools have been proposed to better stratify patients at risk of SCD. Among them, cardiovascular magnetic resonance imaging, which allows direct visualization of the arrhythmogenic substrate, is considered particularly promising. Genetic testing and serum biomarkers may also have a role in SCD risk assessment.

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.

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