Sudden Cardiac Death Risk Assessment Research Articles

Sudden Cardiac Death Risk Stratification in Heart Failure With Preserved Ejection Fraction.

Heart failure with preserved ejection fraction (HFpEF) poses a significant clinical challenge, with sudden cardiac death (SCD) emerging as one of the leading causes of mortality. Despite advancements in cardiovascular medicine, predicting and preventing SCD in HFpEF remains complex due to multifactorial pathophysiological mechanisms and patient heterogeneity. Unlike heart failure with reduced ejection fraction, where impaired contractility and ventricular remodeling predominate, HFpEF pathophysiology involves heavy burden of comorbidities such as hypertension, obesity, and diabetes. Diverse mechanisms, including diastolic dysfunction, microvascular abnormalities, and inflammation, also contribute to distinct disease and SCD risk profiles. Various parameters such as clinical factors and electrocardiogram features have been proposed in SCD risk assessment. Advanced imaging modalities and biomarkers offer promise in risk prediction, yet comprehensive risk stratification models specific to HFpEF ar0e lacking. This review offers recent evidence on SCD risk factors and discusses current therapeutic strategies aimed at reducing SCD risk in HFpEF.

An ECG-based artificial intelligence model for assessment of sudden cardiac death risk.

Conventional ECG-based algorithms could contribute to sudden cardiac death (SCD) risk stratification but demonstrate moderate predictive capabilities. Deep learning (DL) models use the entire digital signal and could potentially improve predictive power. We aimed to train and validate a 12 lead ECG-based DL algorithm for SCD risk assessment. Out-of-hospital SCD cases were prospectively ascertained in the Portland, Oregon, metro area. A total of 1,827 pre- cardiac arrest 12 lead ECGs from 1,796 SCD cases were retrospectively collected and analyzed to develop an ECG-based DL model. External validation was performed in 714 ECGs from 714 SCD cases from Ventura County, CA. Two separate control group samples were obtained from 1342 ECGs taken from 1325 individuals of which at least 50% had established coronary artery disease. The DL model was compared with a previously validated conventional 6 variable ECG risk model. The DL model achieves an AUROC of 0.889 (95% CI 0.861-0.917) for the detection of SCD cases vs. controls in the internal held-out test dataset, and is successfully validated in external SCD cases with an AUROC of 0.820 (0.794-0.847). The DL model performs significantly better than the conventional ECG model that achieves an AUROC of 0.712 (0.668-0.756) in the internal and 0.743 (0.711-0.775) in the external cohort. An ECG-based DL model distinguishes SCD cases from controls with improved accuracy and performs better than a conventional ECG risk model. Further detailed investigation is warranted to evaluate how the DL model could contribute to improved SCD risk stratification.

Long-term follow-up in patients with hypertrophic cardiomyopathy after defibrillator implantation: analysis of appropriate and inappropriate therapy

Abstract Background Treatment with implantable cardioverter-defibrillators (ICD) is effective for prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Assessment of SCD risk and identification of patients most likely to benefit from primary prevention ICD is challenging. Purpose To investigate the long-term incidence of appropriate and inappropriate ICD therapy in patients with HCM. Methods This was a retrospective cohort study including all patients with HCM and ICDs implanted between 1995 to 2022 in our region (population 2.6 million). Both patients treated with primary and secondary prevention ICD were included. Data was retrieved from medical records. Patients with an ICD implanted for primary prevention were stratified into risk groups according to European Society of Cardiology HCM Risk-SCD score. Results We included 187 patients (65% male) with HCM and ICDs implanted for primary (80%) or secondary (20%) prevention. The median age at ICD implantation was 50 years (IQR: 40 to 61 years). During a mean follow-up of 9 years (IQR: 4 to 12 years), 53 patients (28%) experienced appropriate ICD therapy (antitachycardia pacing and/or shock), while 17 (9%) patients received inappropriate therapy. Patients with an ICD as secondary prevention were almost twice as likely to receive appropriate ICD therapy compared to patients with a primary prevention ICD (42% vs. 25%, p=0.04). The proportion of patients receiving appropriate shocks was 7 times higher in patients with an HCM Risk-SCD score ≥6% compared to patients with a risk score <4% (29% vs. 4%, p<0.001). There was no significant difference in the proportion of appropriate shocks between patients with a HCM Risk-SCD score ≥6% and patients with a risk score of 4%-6% (29% vs. 21%, p=0.49). (Figure 1). Conclusion One in four patients with HCM treated with an ICD experienced appropriate device intervention during 9 years of follow up. Patients with an HCM Risk-SCD score ≥6% had a 7-fold higher risk of appropriate ICD therapy compared to patients with an HCM Risk-SCD score <4%. There was no significant difference in the risk of appropriate shock therapy between patients with a risk score ≥6% and patients with a risk score of 4%-6%. The findings indicate the need for improved selection of patient with HCM for implantation of ICD’s.

Genetic Risk Stratification in Arrhythmogenic Left Ventricular Cardiomyopathy.

Arrhythmogenic left ventricular cardiomyopathy is characterized by early malignant ventricular arrhythmia associated with varying degrees and times of onset of left ventricular dysfunction. Variants in numerous genes have been associated with this phenotype. Here, the authors review the literature on recent cohort studies of patients with variants in desmoplakin, lamin A/C, filamin-C, phospholamban, RBM20, TMEM43, and selected channelopathy genes also associated with structural disease. Unlike traditional sudden cardiac death risk assessment in nonischemic cardiomyopathy, left ventricular systolic function is an insensitive predictor of risk in patients with these genetic diagnoses.

3D substrate complexity analysis using cardiac MRI predicts ICD therapy in post-infarct ventricular tachycardia

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): EACVI, Netherlands Heart Institute. Background Implantable cardiac defibrillator (ICD) implantation can protect against sudden cardiac death (SCD) after a myocardial infarction. However, relatively few patients with an ICD experience a life-threatening arrhythmic event. Imaging studies have proposed metrics based on 2D analysis of late gadolinium enhancement (LGE) characteristics to predict post-infarct malignant arrhythmias and improve SCD risk assessment. However, given the intrinsic 3D nature of the electrical pathways through the infarcted regions, 3D reconstructions of the scar substrate from LGE imaging may be required to fully characterize the pro-arrhythmic nature of the scar substrate. Aim To evaluate the accuracy of LGE based 3D metrics such as conduction corridors (regions of borderzone (BZ) surrounded by scar core) and 3D interface surfaces (boundaries between scar and myocardium) towards predicting ICD therapy. Methods ADAS LV and custom-made software was used to generate 3D patient-specific ventricular models in a prospective cohort of post-infarct patients (n=40) having undergone LGE imaging pre-ICD implantation. The extent of variation in scar-characteristics was evaluated in ADAS by quantifying the BZ, scar core, the number and weight of conduction corridors i.e. BZ surrounded by scar core. Custom-written scripts were used to calculate metrics describing the 3D topology of the scar substrate, specifically the interface area between myocardium and total enhancement (BZ+core), and the interface between BZ and core. These metrics were compared with ICD therapy during follow-up. Results Total corridors were comparable between both groups (6.53 ± 7.9 vs. 4.6 ± 4, p = .38). Corridor weight demonstrated a trend towards higher mass in the event group (2.7 ± 2.1g vs. 1.6 ± 1.4g, p = .06). Patients with an event (n=17) had higher myocardium-total enhancement interface (103.8±35.1cm2 vs. 77.4±33.7cm2, p = .021) and BZ-core interface (76±27.5cm2 vs. 55.2±27.6cm2, p = .024). Cox-regression demonstrated a significant independent association of myocardium-total enhancement interface with an event (HR 2.79; 1.44–5.44, p < .01). Kaplan-Meier analysis (Figure 1) showed a significantly higher event rate in patients with an interface area between myocardium-total enhancement of more than 72cm2 and BZ-core more than 42.3cm2. Conclusion These results demonstrate that patients with appropriate device therapy had larger myocardium-total enhancement and BZ-core surface interface areas. Conceptually, the BZ-core interface could be considered to be related to the reentrant circuit path-length whilst the myocardium-total enhancement interface reflects the surfaces most-likely to initiate unidirectional block, both of which can be consider pro-arrhythmic substrates. These findings emphasize the importance of visualizing and thereby characterizing substrate as a 3D entity instead of the currently applied 2D approach to facilitate early identification of high-risk patients.

3D substrate complexity analysis using cardiac MRI predicts ICD therapy in post-infarct ventricular tachycardia

Abstract Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): EACVI Research Grant Academy Van Leersum grant of the Academy Medical Sciences Fund (Royal Netherlands Academy of Arts & Sciences). Background Implantable cardiac defibrillator (ICD) implantation can protect against sudden cardiac death (SCD) after a myocardial infarction. However, relatively few patients with an ICD experience a life-threatening arrhythmic event. Imaging studies have proposed metrics based on 2D analysis of late gadolinium enhancement (LGE) characteristics to predict post-infarct malignant arrhythmias and improve SCD risk assessment. However, given the intrinsic 3D nature of the electrical pathways through the infarcted regions, 3D reconstructions of the scar substrate from LGE imaging may be required to fully characterize the pro-arrhythmic nature of the scar substrate. Aim To evaluate the accuracy of LGE based 3D metrics such as conduction corridors (regions of borderzone (BZ) surrounded by scar core) and 3D interface surfaces (boundaries between scar and myocardium) towards predicting ICD therapy. Methods ADAS LV and custom-made software was used to generate 3D patient-specific ventricular models in a prospective cohort of post-infarct patients (n=40) having undergone LGE imaging pre-ICD implantation. The extent of variation in scar-characteristics was evaluated in ADAS by quantifying the BZ, scar core, the number and weight of conduction corridors i.e. BZ surrounded by scar core. Custom-written scripts were used to calculate metrics describing the 3D topology of the scar substrate, specifically the interface area between myocardium and total enhancement (BZ+core), and the interface between BZ and core. These metrics were compared with ICD therapy during follow-up. Results Total corridors were comparable between both groups (6.53 ± 7.9 vs. 4.6 ± 4, p = .38). Corridor weight demonstrated a trend towards higher mass in the event group (2.7 ± 2.1g vs. 1.6 ± 1.4g, p = .06). Patients with an event (n=17) had higher myocardium-total enhancement interface (103.8±35.1cm2 vs. 77.4±33.7cm2, p=.021) and BZ-core interface (76±27.5cm2 vs. 55.2±27.6cm2, p=.024). Cox-regression demonstrated a significant independent association of myocardium-total enhancement interface with an event (HR 2.79; 1.44-5.44, p < .01). Kaplan-Meier analysis showed a significantly higher event rate in patients with an interface area between myocardium-total enhancement of more than 72cm2 (Figure 1A) and BZ-core more than 42.3cm2 (Figure 1B). Conclusion These results demonstrate that patients with appropriate device therapy had larger myocardium-total enhancement and BZ-core surface interface areas. Conceptually, the BZ-core interface could be considered to be related to the reentrant circuit path-length whilst the myocardium-total enhancement interface reflects the surfaces most-likely to initiate unidirectional block, both of which can be consider pro-arrhythmic substrates. These findings emphasize the importance of visualizing and thereby characterizing substrate as a 3D entity instead of the currently applied 2D approach to facilitate early identification of high-risk patients.

Personalized Management of Sudden Death Risk in Primary Cardiomyopathies: From Clinical Evaluation and Multimodality Imaging to Ablation and Cardioverter-Defibrillator Implant.

Sudden cardiac death represents the leading cause of death worldwide; although the majority of sudden deaths occur in an elderly population with coronary artery disease, some occur in young and otherwise healthy individuals, as is the case of cardiomyopathies. The aim of the present review is to provide a stepwise hierarchical approach for the global sudden death risk estimation in primary cardiomyopathies. Each individual risk factor is analyzed for its contribution to the overall risk of sudden death for each specific cardiomyopathy as well as across all primary myocardial diseases. This stepwise hierarchical and personalized approach starts from the clinical evaluation, subsequently passes through the role of electrocardiographic monitoring and multimodality imaging, and finally concludes with genetic evaluation and electro-anatomical mapping. In fact, the sudden cardiac death risk assessment in cardiomyopathies depends on a multiparametric approach. Moreover, current indications for ventricular arrhythmia ablation and defibrillator implantation are discussed.

Diagnosing Hypertrophic Cardiomyopathy in Athletes

Sudden cardiac death (SCD) in a young asymptomatic individual is a devastating, unpredictable event, with a widespread impact on the public health system. Hypertrophic cardiomyopathy (HCM) is the most common form of genetic heart disease, and considered one of the leading causes of SCD affecting young and frequently asymptomatic patients. A diagnosis of HCM is challenging particularly in young athletes due to overlapping clinical phenotypesbetween pathological left ventricular hypertrophy (LVH) and exercise-induced physiological LVH. Severalinvestigational tools have been used to differentiate these two distinct entities. This review article focuses upon the diagnosis of HCM in young athletes, SCD risk assessment, and current recommendations for exercise in athletic individuals with HCM.

Rare Case of Pediatric Hypertrophic Obstructive Cardiomyopathy (HOCM) in A 6-year-old Boy: How to Recognize, Assess, and Manage the Risk?

Highlights: It discusses pediatric HCM which is usually caused by autosomal dominant traits caused by mutation in cardiac sarcomere protein genes. It explains how to recognize the cardiac risk Abstract: Hypertrophic Obstructive Cardiomyopathy (HOCM) is a disease characterized by increased left ventricular (LV) wall thickness and accompanied by obstructive physiology measured by increased LV outflow tract gradient. It is mainly inherited in autosomal dominant traits caused by a mutation in cardiac sarcomere protein genes. In pediatrics, HOCM is rare and comprises a different diagnosis and management approach compared to adults. The risk of sudden cardiac death (SCD) is also higher in the pediatric population. Case Summary. This report is about a case of HOCM found incidentally in a 6-year-old boy and a discussion based on the latest literature review. The patient first came for evaluation for cardiac murmur and abnormality in ECG and chest x-ray. Diagnosis of HOCM was made through echocardiography assessment. Discussion. ICD implantation for primary prevention of SCD was considered based on individualized 5-year SCD risk assessment which is around 7%. Optimal pharmacological therapy with beta-blocker, careful planning of ICD implantation with balanced benefit and risk, and septal reduction surgery when indicated should extend the life expectancy and quality of life of pediatric HOCM. It is both essential and interesting to recognize pediatric HOCM diagnostic findings and to pursue further research about therapies of this rare disease.

Sudden cardiac death multiparametric classification system for Chagas heart disease's patients based on clinical data and 24-hours ECG monitoring.

About 6.5 million people are infected with Chagas disease (CD) globally, and WHO estimates that $ > million people worldwide suffer from ChHD. Sudden cardiac death (SCD) represents one of the leading causes of death worldwide and affects approximately 65% of ChHD patients at a rate of 24 per 1000 patient-years, much greater than the SCD rate in the general population. Its occurrence in the specific context of ChHD needs to be better exploited. This paper provides the first evidence supporting the use of machine learning (ML) methods within non-invasive tests: patients' clinical data and cardiac restitution metrics (CRM) features extracted from ECG-Holter recordings as an adjunct in the SCD risk assessment in ChHD. The feature selection (FS) flows evaluated 5 different groups of attributes formed from patients' clinical and physiological data to identify relevant attributes among 57 features reported by 315 patients at HUCFF-UFRJ. The FS flow with FS techniques (variance, ANOVA, and recursive feature elimination) and Naive Bayes (NB) model achieved the best classification performance with 90.63% recall (sensitivity) and 80.55% AUC. The initial feature set is reduced to a subset of 13 features (4 Classification; 1 Treatment; 1 CRM; and 7 Heart Tests). The proposed method represents an intelligent diagnostic support system that predicts the high risk of SCD in ChHD patients and highlights the clinical and CRM data that most strongly impact the final outcome.

Sudden cardiac risk assessment in hypertrophic cardiomyopathy: role of the imaging

The current knowledge about imaging in the sudden cardiac death (SCD) risk assessment in hypertrophic cardiomyopathy (HCMP) is discussed. The overall risk of SCD in patients with HCMP is not high, but it remain the main cause of death in young patients. Implantation of cardioverter-defibrillator, surgical treatment of HCMP allow to prevent SCD and other negative cardiovascular events (heart failure and stroke) in patients with HCMP. Thus identification of predictors of negative cardiovascular events is essential in HCMP. Imaging methods (echocardiography and magnetic resonance imaging) enable to assess risk factors: left ventricular hypertrophy, left ventricular outflow tract obstruction, left atrial dimension, apical aneurysm, midventricular obstruction and extent of the late gadolinium enhancement. Role of the risk factors in SCD risk assessment in HCMP is discussed in the article.

Updated risk assessments for sudden cardiac death in hypertrophic cardiomyopathy patients with implantable cardioverter-defibrillator.

Hypertrophic cardiomyopathy (HCM) is a genetic disease associated with a risk of malignant ventricular tachyarrhythmias and sudden cardiac death (SCD). Assessment of the SCD risk is crucial for its clinical management, and there has been considerable interest in developing risk stratification strategies. An implantable cardioverter-defibrillator (ICD) is a life-saving treatment for patients with HCM who are at a high-risk of ventricular tachyarrhythmias and SCD. However, a substantial number of ICD recipients experience adverse effects arising from inappropriate device therapy and implant-related complications. This has led to numerous investigations of the risk of SCD and the indications for ICD implantation. American guidelines were recently updated to include new risk markers, including left ventricular systolic dysfunction, apical aneurysm, and extensive late gadolinium enhancement, while European guidelines recommend individualized estimated 5-year SCD risk assessment models. Studies evaluating other risk factors for SCD in patients with HCM have also been published. Drawing on recent guidelines and publications on clinical risk factors, we focus this review on updated risk assessments for SCD with ICD therapy in patients with HCM.

Cardiac magnetic resonance imaging predictors of ventricular arrhythmia in mid-cavity obstructive hypertrophic cardiomyopathy

Abstract Background/Introduction Left ventricular (LV) mid-cavity obstruction (LVMCO) in hypertrophic cardiomyopathy (HCM) is an uncommon phenotypic feature predisposed to the formation of myocardial fibrosis and apical aneurysms (LVAA). These features may be independently proarrhythmic, and LVAA is considered a class 2a indication for implantable cardioverter defibrillator (ICD) in current US, but not European guidelines for the primary prevention of sudden cardiac death (SCD). Cardiac magnetic resonance (CMR) imaging is the preferred modality for detecting these and other phenotypic features critical to SCD risk assessment. Purpose To assess the ability of CMR imaging parameters to predict occurrence of non-sustained ventricular tachycardia (NSVT) in HCM patients with Doppler-derived evidence of LVMCO. Methods Multi-modality imaging records were retrospectively assessed to identify HCM patients with Doppler-LVMCO and CMR scans. CMR images were assessed by an investigator blinded to clinical status. Late gadolinium enhancement (LGE) was quantified using the full-width, half-maximum technique. CMR imaging parameters were assessed for predictive ability using Cox proportional hazards during univariate and multivariate analyses, accounting for time to event (NSVT or censorship of follow-up). Results The study cohort included 58 patients (57±11 years, 74% male) with a median follow-up of 6.2 (IQR 4.3) years. Mean mid-cavity gradient was 33±23 mmHg. NSVT was detected in 27/58 (47%) patients, was 4 beats or longer in 23/27 (85%) and was monomorphic in 21/27 (77%). On univariate analysis, predictors of NSVT during follow-up include LV mass index (HR 1.02, 95% CI 1.00–1.04, p=0.03), LGE in grams (HR 1.04, 95% CI 1.01–1.06, p=0.005), and LVAA (HR 2.57, 95% CI 1.14–5.79, p=0.023). After multivariate adjustment (Table 2), none were significantly associated. Conclusions In LVMCO, magnitude of LV hypertrophy, extent of LGE and the presence of an apical aneurysm may not be independent predictors of ventricular arrhythmias. SCD algorithms based on qualitative assessments of these features may overestimate risk. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): National Institute of Health Research (NIHR)

Pediatric Hypertrophic Cardiomyopathy: Exploring the Genotype-Phenotype Association.

Pediatric hypertrophic cardiomyopathy (HCM) is the most common form of cardiomyopathy in children and a leading cause of sudden cardiac death. Yet, the association between genotype variation, phenotype expression, and adverse events in pediatric HCM has not been fully elucidated. Although the literature on this topic is evolving in adult HCM, the evidence in children is lacking. Solidifying our understanding of this relationship could improve risk stratification as well as improve our comprehension of the underlying pathophysiological characteristics of pediatric HCM. In this state‐of‐the‐art review, we examine the current literature on genetic variations in HCM and their association with outcomes in children, discuss the current approaches to identifying cardiovascular phenotypes in pediatric HCM, and explore possible avenues that could improve sudden cardiac death risk assessment.

Sudden cardiac death risk stratification in hypertrophic cardiomyopathy: discrepancies persist among guidelines

Abstract Background Differing criteria have been provided on sudden cardiac death (SCD) risk stratification in hypertrophic cardiomyopathy (HCM) by current guidelines. Recently, AHA/ACC proposed an algorithm with novel clinical markers. It remains to be established the impact of this new approach. Purpose Evaluate the impact of the 2020 AHA/ACC guideline on the 2014 ESC and 2011 ACCF/AHA criteria regarding SCD risk assessment and primary prevention implantable cardioverter-defibrillator (ICD) in HCM. Methods The database of a HCM center non-referred cohort was accessed for SCD risk profile between March 2007 and March 2020. The agreement for primary prevention ICD recommendations among guidelines was assessed with the Cohen's and Fleiss' Kappa coefficient, P<0.05. SCD or appropriate ICD shock were defined as the primary end-point. Results A total of 100 patients, age 60±13 years, 55 (55%) females, were followed by 5±3 years. The maximal left ventricular (LV) wall thickness was 18±4 mm, 38 (38%) patients showed a family history of SCD, 22 (22%) syncope, 6 (6%) ejection fraction ≤50%, 2 (2%) LV apical aneurysm, 1 (1%) massive LV hypertrophy, 26 (26%) non-sustained ventricular tachycardia, and 23 (23%) extensive late gadolinium enhancement. An ICD was placed in 17 (17%) patients. According to the 2020 AHA/ACC guideline, 57 (57%) patients met class IIa recommendation, 27 (27%) class IIb, and 16 (16%) class III. The 2014 ESC model classified 14 (14%) in class IIa, 18 (18%) in class IIb, and 68 (68%) in class III. The 2011 ACCF/AHA considered 66 (66%) in class IIa, 6 (6%) in class IIb, and 28 (28%) in class III. The Cohen's Kappa was 0.200 (95% CI 0.292–0.107), P=0.0005, between the 2020 AHA/ACC and the 2014 ESC, and 0.520 (95% CI 0.651–0.388), P=0.0005, between the North American approaches. The Fleiss' Kappa was 0.219 (95% CI 0.303–0.135) P=0.0005 among the three guidelines, whereas it reached 0.221 (95% CI 0.334–0.108) for class IIa, 0.244 (95% CI 0.357–0.131) for class IIb and 0.202 (95% CI 0.315–0.089) for class III, P=0.0005. Figure 1 shows the patient's reclassification with the new guideline. The primary end-points occurred in 7 (7%) patients in a median follow-up of 6 (17–0.4) years. All of them were classified as IIa with the 2011 ACCF/AHA guideline, but only 4 (4%) met this class under the 2020 AHA/ACC, and none in the 2014 ESC model. Conclusion A low agreement was found among guidelines, especially between the 2020 AHA/ACC and the 2014 ESC criteria. The North American systems differed moderately, but the new approach has reduced the cases in class IIa and III recommendation for primary prevention ICD. In contrast, the recent 2020 guideline has increased the number of patients in class IIa in relation to the European model, but both strategies have not protected the totality of patients with SCD or appropriate shock. Funding Acknowledgement Type of funding sources: Public hospital(s). Main funding source(s): Hospital de Clínicas de Porto Alegre Figure 1

The DAPA Trial in the Context of Previous Prophylactic ICD Landmark Trials.

In patients with ischaemic cardiomyopathy and severely reduced left ventricular ejection fraction (LVEF), an arrhythmogenic milieu is created by a complex interplay between myocardial scarring (assessed by cardiac MRI) and multiple other factors (ventricular ectopy, ischaemia and autonomic imbalance), favouring the occurrence of arrhythmic sudden cardiac death (SCD). Currently, a dynamic and robust model of dichotomised SCD risk assessment after primary percutaneous coronary intervention (PCI) is lacking, underlining the urgent need for further refinement of the widely accepted and guidelines-based criteria (ischaemic cardiomyopathy, LVEF ≤35%) for primary prevention. This review addresses the potential additional value of the recently published Defibrillator After Primary Angioplasty (DAPA) trial results. The DAPA trial conveys important messages and provides novel perspectives regarding left ventricular function post-primary PCI as an (early) risk marker for SCD and the impact of prophylactic ICD implantation on survival in this cohort. In the context of other previous primary prevention trials, DAPA was the first trial including only ST-elevation MI patients all treated with acute PCI.

Predicting risk of sudden cardiac death in patients with cardiac sarcoidosis using multimodality imaging and personalized heart modeling in a multivariable classifier.

Cardiac sarcoidosis (CS), an inflammatory disease characterized by formation of granulomas in the heart, is associated with high risk of sudden cardiac death (SCD) from ventricular arrhythmias. Current "one-size-fits-all" guidelines for SCD risk assessment in CS result in insufficient appropriate primary prevention. Here, we present a two-step precision risk prediction technology for patients with CS. First, a patient's arrhythmogenic propensity arising from heterogeneous CS-induced ventricular remodeling is assessed using a novel personalized magnetic-resonance imaging and positron-emission tomography fusion mechanistic model. The resulting simulations of arrhythmogenesis are fed, together with a set of imaging and clinical biomarkers, into a supervised classifier. In a retrospective study of 45 patients, the technology achieved testing results of 60% sensitivity [95% confidence interval (CI): 57-63%], 72% specificity [95% CI: 70-74%], and 0.754 area under the receiver operating characteristic curve [95% CI: 0.710-0.797]. It outperformed clinical metrics, highlighting its potential to transform CS risk stratification.

Gaps in Evidence for Risk Stratification for Sudden Cardiac Death in Hypertrophic Cardiomyopathy.

Gaps in Evidence for Risk Stratification for Sudden Cardiac Death in Hypertrophic Cardiomyopathy.

Long-term monitoring of arrhythmias in cardiac sarcoidosis

Abstract Introduction Screening for cardiac sarcoidosis (CS) is recommended since it can manifest with ventricular arrhythmias (VA), atrioventricular conduction block (AVB) and sudden cardiac death (SCD). However, risk stratification for SCD is challenging, in particular in patients without overt cardiac symptoms. Purpose This study reports the practice-based risk stratification for SCD and the incidence of arrhythmias and mortality in CS patients by long-term monitoring of arrhythmias. Methods A retrospective, single center cohort study was performed in 537 patients with sarcoidosis screened for cardiac involvement with cardiac MRI and fluorodeoxyglucose PET in an hospital, a Dutch tertiary referral center. CS was diagnosed in 115 of 537 patients (21%), complete follow up was available in 108 patients (94%). After risk assessment for SCD (figure 1) an ICD was implanted in 16 high-risk patients. Within the92 low-risk patients, 80 had an internal loop recorder (ILR) implanted and 12 patients received no device. Chart review was performed to assess the occurrence of VA, AVB, death, ICD therapy and device related complications. Results During a mean follow-up of 31±15 months, 9 out of 80 ILR patients (11.3%) received an ICD of whom 7 (8.8%) based on recorded arrhythmias (VA in 5 and AVB in 2 patients). Five out of the total 25 ICD patients (20%) experienced sustained VA successfully treated with anti-tachycardia pacing in 2 (8%) and terminated spontaneously in all other patients. Two ICD patients experienced a mild pocket infection, treated with antibiotics. Two deaths occurred in the low-risk patients: 1 non-cardiac death and 1 SCD due to asystole. Conclusion The practice-based risk stratification supported an ICD implantation in up to 5% of sarcoidosis patients screened for CS. Sustained VA occurred in 20% of ICD patients.Early detection of important arrhythmias with an ILR can optimize risk assessment for SCD in CS. Practise-based risk stratification Funding Acknowledgement Type of funding source: None

Late gadolinium enhancement location assessed by magnetic resonance and arrhythmogenic risk in hypertrophic cardiomyopathy

Introduction and ObjectivesLate gadolinium enhancement (LGE) extent has emerged as a predictor of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM), however little is known about the arrhythmogenic relevance of its specific location in the left ventricle. Our aim was to analyze the influence of LGE location on the occurrence of ventricular arrhythmias (VA) and SCD in patients with HCM. MethodsWe performed a retrospective analysis of clinical and Holter records of HCM patients who underwent cardiac magnetic resonance at our center. LGE extent and distribution were assessed using the American Heart Association 17-segment model. VA was defined as non-sustained or sustained ventricular tachycardia, ventricular fibrillation or SCD. ResultsSixty-one patients (age 57.0±16.7 years) were included and VA occurred in 24.6% (n=15). Patients with VA showed greater LGE extent than those without (7.40±5.3 vs. 3.52±3.0 segments, p=0.007). Analyzing the distribution of LGE, a set of arrhythmogenic segments (apex/basal inferior/basal anterolateral/mid inferoseptal) was found. The extent of LGE involvement in these segments was also greater in patients with VA (2.07±1.03 vs. 0.65±0.71 segments, p<0.001; area under the curve 0.861 for VA) and this difference remained significant after adjustment for potentially confounding variables. ConclusionsThe extent of LGE involvement of a set of segments with an apparent relation to cardiac areas of increased mechanical stress was significantly and independently associated with the occurrence of VA, suggesting that not only the extent but also the location of LGE is important for the assessment of SCD risk in HCM patients.