The electromechanical window for arrhythmia-risk assessment.

The electromechanical window (EMW) is calculated by subtracting the repolarization duration from a mechanical reference representing contraction duration in the same heartbeat (eg, aortic valve closure during echocardiography with simultaneous electrocardiography). Here we review the current knowledge on the role of the EMW as an independent parameter for ventricular arrhythmia-risk stratification. We (1) provide a standardized approach to echocardiographic EMW assessment, (2) define relevant cutoff values for both abnormal EMW negativity and positivity, (3) discuss pathophysiological underpinnings of EMW negativity, and (4) outline the potential future role of cardiac electromechanical relations in patients with proarrhythmic conditions.

Assessment of ICD eligibility in non-ischaemic cardiomyopathy patients: a position statement by the Task Force of the Dutch Society of Cardiology.

International guidelines recommend implantation of an implantable cardioverter-defibrillator (ICD) in non-ischaemic cardiomyopathy (NICM) patients with a left ventricular ejection fraction (LVEF) below 35% despite optimal medical therapy and a life expectancy of more than 1 year with good functional status. We propose refinement of these recommendations in patients with NICM, with careful consideration of additional risk parameters for both arrhythmic and non-arrhythmic death. These additional parameters include late gadolinium enhancement on cardiac magnetic resonance imaging and genetic testing for high-risk genetic variants to further assess arrhythmic risk, and age, comorbidities and sex for assessment of non-arrhythmic mortality risk. Moreover, several risk modifiers should be taken into account, such as concomitant arrhythmias that may affect LVEF (atrial fibrillation, premature ventricular beats) and resynchronisation therapy. Even though currently no valid cut-off values have been established, the proposed approach provides a more careful consideration of risks that may result in withholding ICD implantation in patients with low arrhythmic risk and substantial non-arrhythmic mortality risk.

Arrhythmias and ion channelopathies causing sudden cardiac death in Hispanic/Latino and Indigenous populations.

The limited literature and increasing interest in studies on cardiac electrophysiology, explicitly focusing on cardiac ion channelopathies and sudden cardiac death in diverse populations, has prompted a comprehensive examination of existing research. Our review specifically targets Hispanic/Latino and Indigenous populations, which are often underrepresented in healthcare studies. This review encompasses investigations into genetic variants, epidemiology, etiologies, and clinical risk factors associated with arrhythmias in these demographic groups. The review explores the Hispanic paradox, a phenomenon linking healthcare outcomes to socioeconomic factors within Hispanic communities in the United States. Furthermore, it discusses studies exemplifying this observation in the context of arrhythmias and ion channelopathies in Hispanic populations. Current research also sheds light on disparities in overall healthcare quality in Indigenous populations. The available yet limited literature underscores the pressing need for more extensive and comprehensive research on cardiac ion channelopathies in Hispanic/Latino and Indigenous populations. Specifically, additional studies are essential to fully characterize pathogenic genetic variants, identify population-specific risk factors, and address health disparities to enhance the detection, prevention, and management of arrhythmias and sudden cardiac death in these demographic groups.

Arrhythmogenic Right Ventricular Cardiomyopathy Post-Mortem Assessment: A Systematic Review.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder characterized by the progressive fibro-fatty replacement of the right ventricular myocardium, leading to myocardial atrophy. Although the structural changes usually affect the right ventricle, the pathology may also manifest with either isolated left ventricular myocardium or biventricular involvement. As ARVC shows an autosomal dominant pattern of inheritance with variable penetrance, the clinical presentation of the disease is highly heterogeneous, with different degrees of severity and patterns of myocardial involvement even in patients of the same familiar group with the same gene mutation: the pathology spectrum ranges from the absence of symptoms to sudden cardiac death (SCD) sustained by ventricular arrhythmias, which may, in some cases, be the first manifestation of an otherwise silent pathology. An evidence-based systematic review of the literature was conducted to evaluate the state of the art of the diagnostic techniques for the correct post-mortem identification of ARVC. The research was performed using the electronic databases PubMed and Scopus. A methodological approach to reach a correct post-mortem diagnosis of ARVC was described, analyzing the main post-mortem peculiar macroscopic, microscopic and radiological alterations. In addition, the importance of performing post-mortem genetic tests has been underlined, which may lead to the correct identification and characterization of the disease, especially in those ARVC forms where anatomopathological investigation does not show evident morphostructural damage. Furthermore, the usefulness of genetic testing is not exclusively limited to the correct diagnosis of the pathology, but is essential for promoting targeted screening programs to the deceased's family members. Nowadays, the post-mortem diagnosis of ARVC performed by forensic pathologist remains very challenging: therefore, the identification of a clear methodological approach may lead to both a reduction in under-diagnoses and to the improvement of knowledge on the disease.

Severe mental illness: cardiovascular risk assessment and management.

Patients with severe mental illness (SMI) including schizophrenia and bipolar disorder die on average 15-20 years earlier than the general population often due to sudden death that, in most cases, is caused by cardiovascular disease. This state-of-the-art review aims to address the complex association between SMI and cardiovascular risk, explore disparities in cardiovascular care pathways, describe how to adequately predict cardiovascular outcomes, and propose targeted interventions to improve cardiovascular health in patients with SMI. These patients have an adverse cardiovascular risk factor profile due to an interplay between biological factors such as chronic inflammation, patient factors such as excessive smoking, and healthcare system factors such as stigma and discrimination. Several disparities in cardiovascular care pathways have been demonstrated in patients with SMI, resulting in a 47% lower likelihood of undergoing invasive coronary procedures and substantially lower rates of prescribed standard secondary prevention medications compared with the general population. Although early cardiovascular risk prediction is important, conventional risk prediction models do not accurately predict long-term cardiovascular outcomes as cardiovascular disease and mortality are only partly driven by traditional risk factors in this patient group. As such, SMI-specific risk prediction models and clinical tools such as the electrocardiogram and echocardiogram are necessary when assessing and managing cardiovascular risk associated with SMI. In conclusion, there is a necessity for differentiated cardiovascular care in patients with SMI. By addressing factors involved in the excess cardiovascular risk, reconsidering risk stratification approaches, and implementing multidisciplinary care models, clinicians can take steps towards improving cardiovascular health and long-term outcomes in patients with SMI.

Regional disparities and risk factors of mortality among patients at high risk of sudden cardiac death in emerging countries: a nonrandomized controlled trial.

BACKGROUND: Comprehensive data on patients at high risk of sudden cardiac death (SCD) in emerging countries are lacking. The aim was to deepen our understanding of the SCD phenotype and identify risk factors for death among patients at high risk of SCD in emerging countries. METHODS: Patients who met the class I indication for implantable cardioverter-defibrillator (ICD) implantation according to guideline recommendations in 17 countries and regions underrepresented in previous trials were enrolled. Countries were stratified by the WHO regional classification. Patients were or were not implanted with an ICD at their discretion. The outcomes were all-cause mortality and SCD. RESULTS: We enrolled 4222 patients, and 3889 patients were included in the analysis. The mean follow-up period was 21.6 ± 10.2 months. There were 433 (11.1%) instances of all-cause mortality and 117 (3.0%) cases of SCD. All-cause mortality was highest in primary prevention (PP) patients from Southeast Asia and secondary prevention (SP) patients from the Middle East and Africa. The SCD rates among PP and SP patients were both highest in South Asia. Multivariate Cox regression modelling demonstrated that in addition to the independent predictors identified in previous studies, both geographic region and ICD use were associated with all-cause mortality in patients with high SCD risk. Primary prophylactic ICD implantation was associated with a 36% (HR = 0.64, 95% CI 0.531-0.802, p < 0.0001) lower all-cause mortality risk and an 80% (HR = 0.20, 95% CI = 0.116-0.343, p < 0.0001) lower SCD risk. CONCLUSIONS: There was significant heterogeneity among patients with high SCD risk in emerging countries. The influences of geographic regions on patient characteristics and outcomes were significant. Improvement in increasing ICD utilization and uptake of guideline-directed medical therapy in emerging countries is urgent. TRIAL REGISTRATION: ClinicalTrials.gov, NCT02099721.

Wolf-Parkinson-White Syndrome: Diagnosis, Risk Assessment, and Therapy-An Update.

Wolf-Parkinson-White (WPW) syndrome is a disorder characterized by the presence of at least one accessory pathway (AP) that can predispose people to atrial/ventricular tachyarrhythmias and even sudden cardiac death. It is the second most common cause of paroxysmal supraventricular tachycardia in most parts of the world, affecting about 0.1-0.3% of the general population. Most patients with WPW syndrome have normal anatomy, but it may be associated with concomitant congenital heart disease or systemic diseases. Although many individuals are asymptomatic, during supraventricular arrhythmia episodes, they may experience severe symptoms, including syncope or even sudden cardiac death (mainly due to pre-excited atrial fibrillation over rapidly conducting AP). In addition to arrhythmia-related symptoms, for some specific locations of the APs with overt anterograde conduction, there might be a reduction in exercise capacity mediated by a reduction in LV systolic performance due to anomalous LV depolarization. Although it is typically diagnosed through electrocardiography (ECG), additional tests are necessary for risk assessment. Management of WPW syndrome may be quite challenging and can vary from only acknowledging the presence of the accessory pathway to pharmacological treatment or radiofrequency ablation. Early diagnosis, risk assessment, and appropriate treatment are critical steps in the management of WPW syndrome, aiming to improve the quality of life and reduce the risk of life-threatening arrhythmias.

Histological assessment of cardiac amyloidosis in patients undergoing transcatheter aortic valve replacement.

AIMS: Studies have reported a strongly varying co-prevalence of aortic stenosis (AS) and cardiac amyloidosis (CA). We sought to histologically determine the co-prevalence of AS and CA in patients undergoing transcatheter aortic valve replacement (TAVR). Consequently, we aimed to derive an algorithm to identify cases in which to suspect the co-prevalence of AS and CA. METHODS AND RESULTS: In this prospective, monocentric study, endomyocardial biopsies of 162 patients undergoing TAVR between January 2017 and March 2021 at the University Medical Centre Göttingen were analysed by one pathologist blinded to clinical data using haematoxylin-eosin staining, Elastica van Gieson staining, and Congo red staining of endomyocardial biopsies. CA was identified in only eight patients (4.9%). CA patients had significantly higher N-terminal pro-brain natriuretic peptide (NT-proBNP) levels (4356.20 vs. 1938.00 ng/L, P = 0.034), a lower voltage-to-mass ratio (0.73 vs. 1.46 × 10-2 mVm2/g, P = 0.022), and lower transaortic gradients (Pmean 17.5 vs. 38.0 mmHg, P = 0.004) than AS patients. Concomitant CA was associated with a higher prevalence of post-procedural acute kidney injury (50.0% vs. 13.1%, P = 0.018) and sudden cardiac death [SCD; P (log-rank test) = 0.017]. Following propensity score matching, 184 proteins were analysed to identify serum biomarkers of concomitant CA. CA patients expressed lower levels of chymotrypsin (P = 0.018) and carboxypeptidase 1 (P = 0.027). We propose an algorithm using commonly documented parameters-stroke volume index, ejection fraction, NT-proBNP levels, posterior wall thickness, and QRS voltage-to-mass ratio-to screen for CA in AS patients, reaching a sensitivity of 66.6% with a specificity of 98.1%. CONCLUSIONS: The co-prevalence of AS and CA was lower than expected, at 4.9%. Despite excellent 1 year mortality, AS + CA patients died significantly more often from SCD. We propose a multimodal algorithm to facilitate more effective screening for CA containing parameters commonly documented during clinical routine. Proteomic biomarkers may yield additional information in the future.

Appropriateness of implantable cardioverter-defibrillator device implants in the United States.

BACKGROUND: The appropriate use criteria (AUCs) are a diverse group of indications aimed to better evaluate the benefits of implantable cardioverter-defibrillator (ICD) and cardiac resynchronization therapy. OBJECTIVE: The purpose of this study was to quantify the proportion of ICD and cardiac resynchronization therapy with defibrillator (CRT-D) implants as appropriate, may be appropriate (MA), or rarely appropriate (RA) on the basis of the AUC guidelines. METHODS: This is a multicenter retrospective study of patients within the National Cardiovascular Data Registry undergoing ICD implantation between April 2018 and March 2019 at >1500 US hospitals. The appropriateness of ICD implants was adjudicated using the AUC. RESULTS: Of 309,318 ICDs, 241,438 were primary prevention implants (78.1%) and 67,880 secondary prevention implants (21.9%); 243,532 (79%) were mappable to the AUC. For primary prevention, 185,431 ICDs (96.4%) were appropriate, 5660 (2.9%) MA, and 1205 (0.6%) RA. For secondary prevention, 47,498 ICDs (92.7%) were appropriate, 2581 (5%) MA, and 1157 (2.3%) RA. A significant number of RA devices were implanted in patients with New York Heart Association class IV heart failure who were ineligible for advanced therapies (53.9%) and those with myocardial infarction within 40 days (18.1%). The appropriateness of the pacing lead was more variable, with 48,470 dual-chamber ICD implants (62%) being classified as appropriate, 29,209 (37.4%) MA, and 448 (0.6%) RA. Among CRT-D implants, 63,848 (82.2%) were appropriate, 9900 (12.7%) MA, and 3940 (5.1%) RA for left ventricular pacing. A total of 99,754 implants were deemed appropriate but excluded from Centers for Medicare & Medicaid Services National Coverage Determination. More than 92% of hospitals had an RA implant rate of <4%. CONCLUSION: In this large national registry, 95% of mappable ICD and CRT-D implants were considered appropriate, with <2% of RA implants. Nearly 100,000 appropriate implants are excluded by Centers for Medicare & Medicaid Services National Coverage Determination.

Assessment of Sudden Cardiac Death Risk in Pediatric Primary Electrical Disorders: A Comprehensive Overview.

Sudden cardiac death (SCD) in children is a devastating event, often linked to primary electrical diseases (PED) of the heart. PEDs, often referred to as channelopathies, are a group of genetic disorders that disrupt the normal ion channel function in cardiac cells, leading to arrhythmias and sudden cardiac death. This paper investigates the unique challenges of risk assessment and stratification for channelopathy-related SCD in pediatric patients-Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, idiopathic ventricular fibrillation, long QT syndrome, Anderson-Tawil syndrome, short QT syndrome, and early repolarization syndrome. We explore the intricate interplay of genetic, clinical, and electrophysiological factors that contribute to the complex nature of these conditions. Recognizing the significance of early identification and tailored management, this paper underscores the need for a comprehensive risk stratification approach specifically designed for pediatric populations. By integrating genetic testing, family history, and advanced electrophysiological evaluation, clinicians can enhance their ability to identify children at the highest risk for SCD, ultimately paving the way for more effective preventive strategies and improved outcomes in this vulnerable patient group.

Cardiological Functional Assessment of National Olympic Team of Kazakhstan.

Athletes carry an increased risk of cardiovascular (CV) conditions. Due to the relatively high loads and intensity of the training process, athletes' CV systems undergo various adaptations, which can combine in the future and provoke unexpected outcomes. Most CV screening protocols have several successive steps. The aim of our study was to perform a cardiological functional assessment of the National Olympic Team of Kazakhstan via several noninvasive protocols to close the gaps between the approaches and collect solid data for the prevention of sudden cardiac death (SCD) incidence among Kazakhstani athletes. METHODS: The methods used in this study were 12-lead resting electrocardiography (ECG), echocardiography, cardiointervalography, cardiopulmonary exercise testing (CPET), and HyperQ stress testing. RESULTS: One case was detected via 12-lead resting ECG. Another case of the slowdown of the heart rate (HR) recovery was detected via cardiointervalography with no clinical signs and normal ECG. The HyperQ stress testing of the women's basketball team detected a positive result in four leads in one athlete. CONCLUSION: Our results demonstrate that the CV systems of athletes require the implementation of several diagnostic methods in rest and stress conditions for more precise evaluation, with each of the methods fulfilling the whole picture for the prevention of such tragic events as sudden cardiac death and sudden cardiac arrest.

Assessment of late gadolinium enhancement in hypertrophic cardiomyopathy improves risk stratification based on current guidelines.

BACKGROUND AND AIMS: Identifying patients with hypertrophic cardiomyopathy (HCM) who are candidates for implantable cardioverter defibrillator (ICD) implantation in primary prevention for sudden cardiac death (SCD) is crucial. The aim of this study was to externally validate the 2022 European Society of Cardiology (ESC) model and other guideline-based ICD class of recommendation (ICD-COR) models and explore the utility of late gadolinium enhancement (LGE) in further risk stratification. METHODS: Seven hundred and seventy-four consecutive patients who underwent cardiac magnetic resonance imaging were retrospectively enrolled. RESULTS: Forty-six (5.9%) patients reached the SCD-related endpoint during 7.4 ± 2.5 years of follow-up. Patients suffering from SCD had higher ESC Risk-SCD score (4.3 ± 2.4% vs. 2.8 ± 2.1%, P < .001) and LGE extent (13.7 ± 9.4% vs. 4.9 ± 6.6%, P < .001). Compared with the 2014 ESC model, the 2022 ESC model showed increased area under the curve (.76 vs. .63), sensitivity (76.1% vs. 43.5%), positive predictive value (16.8% vs. 13.6%), and negative predictive value (98.1% vs. 95.9%). The C-statistics for SCD prediction of 2011 American College of Cardiology (ACC)/American Heart Association (AHA), 2014 ESC, 2020 AHA/ACC, and 2022 ESC models were .68, .64, .76 and .78, respectively. Furthermore, in patients without extensive LGE, LGE ≥5% was responsible for seven-fold SCD risk after multivariable adjustment. Whether in ICD-COR II or ICD-COR III, patients with LGE ≥5% and <15% showed significantly worse prognosis than those with LGE <5% (all P < .001). CONCLUSIONS: The 2022 ESC model performed better than the 2014 ESC model with especially improved sensitivity. LGE enabled further risk stratification based on current guidelines.

Incidence, risk assessment and prevention of sudden cardiac death in cardiomyopathies.

Cardiomyopathies are a significant contributor to cardiovascular morbidity and mortality, mainly due to the development of heart failure and increased risk of sudden cardiac death (SCD). Despite improvement in survival with contemporary treatment, SCD remains an important cause of mortality in cardiomyopathies. It occurs at a rate ranging between 0.15% and 0.7% per year (depending on the cardiomyopathy), which significantly surpasses SCD incidence in the age- and sex-matched general population. The risk of SCD is affected by multiple factors including the aetiology, genetic basis, age, sex, physical exertion, the extent of myocardial disease severity, conduction system abnormalities, and electrical instability, as measured by various metrics. Over the past decades, the knowledge on the mechanisms and risk factors for SCD has substantially improved, allowing for a better-informed risk stratification. However, unresolved issues still challenge the guidance of SCD prevention in patients with cardiomyopathies. In this review, we aim to provide an in-depth discussion of the contemporary concepts pertinent to understanding the burden, risk assessment and prevention of SCD in cardiomyopathies (dilated, non-dilated left ventricular, hypertrophic, arrhythmogenic right ventricular, and restrictive). The review first focuses on SCD incidence in cardiomyopathies and then summarizes established and emerging risk factors for life-threatening arrhythmias/SCD. Finally, it discusses validated approaches to the risk assessment and evidence-based measures for SCD prevention in cardiomyopathies, pointing to the gaps in evidence and areas of uncertainties that merit future clarification.

Cost-effectiveness and diagnostic accuracy of focused cardiac ultrasound in the pre-participation screening of athletes: the SPORT-FoCUS study.

AIMS: The role of pre-participation screening (PPS) modalities in preventing sudden cardiac death (SCD) in athletes is debated due to a high false-positive rate. Focused cardiac ultrasound (FoCUS) has shown higher sensitivity and specificity, but its cost-effectiveness remains uncertain. This study aimed to determine the diagnostic performance and cost-effectiveness of FoCUS use in PPS. METHODS AND RESULTS: A total of 2111 athletes (77.4% male, mean age 24.9 ± 15.2years) underwent standardized family and medical history collection, physical examination, resting electrocardiography (ECG), FoCUS (10 min/5 views protocol), comprehensive echocardiography and exercise stress test. We prospectively evaluated three PPS incremental models: Model A, standardized medical history and physical examination Model B, Model A plus resting and stress ECG and Model C, Model B plus FoCUS (10 min/5 views protocol). We determined their incremental diagnostic accuracy and cost-effectiveness ratio. A total of 30 athletes were diagnosed with a cardiac condition associated with SCD: 3 were identified by Model A, 14 by Model B, and 13 athletes by Model C. The introduction of FoCUS markedly increased the sensitivity of PPS, compared with Model A and Model B (sensitivity 94% vs. 19% vs. 58% specificity 93% vs. 93% vs. 92%). The total screening costs were as follows: Model A 35.64 euros, Model B 87.68 euros, and Model C 120.89 euros. Considering the sole conditions at risk of SCD, the incremental cost-effectiveness ratio was 135.62 euros for Model B and 114.31 for Model C. CONCLUSIONS: The implementation of FoCUS into the PPS allows to identify a significantly greater number of athletes at risk of SCD and markedly lowers the false negative rate. Furthermore, the incorporation of FoCUS into the screening process has shown to be cost-effective.

A significant electrocardiography false-positive rate makes pre-participation screening (PPS) for sudden cardiac death (SCD) in athletes controversial. Focused cardiac ultrasound (FoCUS) may increase sensitivity and specificity however, its cost-effectiveness is unknown. This study evaluates the diagnostic performance and cost-effectiveness of FoCUS in PPS. Incorporating a simplified echocardiographic exam called FoCUS into PPS resulted in higher diagnostic reliability, with a lower rate of false negatives and a higher number of athletes at risk for SCD identified.The integration of FoCUS into the screening process resulted to be cost-effective in our athletes' cohort.

Utilization and Outcomes of Primary Prevention Implantable Cardioverter-Defibrillators in Patients With Hypertrophic Cardiomyopathy.

Background There is uncertainty about the appropriate use of primary prevention implantable cardioverter-defibrillators (ICDs) among older patients with hypertrophic cardiomyopathy. Methods and Results Patients with hypertrophic cardiomyopathy who received a primary prevention ICD between 2010 and 2016 were identified using the National Cardiovascular Data Registry ICD Registry. Trends in ICD utilization and patient characteristics were assessed over time. Using linked Centers for Medicare and Medicaid Service claims data, Cox proportional hazard models assessed factors associated with mortality and postdischarge hospitalization for cardiac arrest/ventricular arrhythmia. Of 5571 patients with hypertrophic cardiomyopathy, 1511 (27.1%) were ≥65 years old. ICD utilization increased over time in all age groups. There were no changes in the prevalence of risk factors for sudden cardiac death over time. The variables most strongly associated with postdischarge mortality were older age (adjusted hazard ratio (aHR) 1.80 [95% CI, 1.47-2.21]), New York Heart Association class (III/IV versus I/II aHR 2.17 [95% CI, 1.57-2.98]), and left ventricular ejection fraction (left ventricular ejection fraction ≤35% versus >50% aHR 2.34 [95% CI, 1.58-3.48]; left ventricular ejection fraction 36%-50% versus >50% aHR 2.98 [95% CI, 2.02-4.40]), while history of nonsustained ventricular tachycardia (aHR 2.38 [95% CI, 1.62-3.51]) and New York Heart Association class (III/IV versus I/II aHR 1.84 [95% CI, 1.22-2.78]) were strongly associated with hospitalization for ventricular arrhythmia/cardiac arrest. Conclusions Primary prevention ICD utilization in patients with hypertrophic cardiomyopathy increased over time, including among those ≥65 years old. Among older patients, the strongest risk factors for hospitalization for ventricular arrhythmia/cardiac arrest following ICD implantation were history of nonsustained ventricular tachycardia and New York Heart Association class.

Sudden Death Risk Assessment in Hypertrophic Cardiomyopathy Across the Lifespan: Reconciling the American and European Approaches.

Hypertrophic cardiomyopathy (HCM) is the most prevalent inherited cardiac disease. Since the modern description of HCM more than seven decades ago, great focus has been placed on preventing its most catastrophic complication: sudden cardiac death (SCD). Implantable cardioverter-defibrillators (ICD) have been recognized to provide effective prophylactic therapy. Over the years, two leading societies, the European Society of Cardiology (ESC) and the American Heart Association/American College of Cardiology (AHA/ACC), have proposed risk stratification models to assess SCD in adults. European guidelines rely on a risk calculator, the HCM Risk-SCD, while American guidelines propose a stand-alone risk factor approach. Recently, risk prediction models were also developed in the pediatric population. This article reviews the latest recommendations on the risk stratification of SCD in HCM and summarises current indications for ICD use.

Manual vs. automatic assessment of the QT-interval and corrected QT.

AIMS: Sudden cardiac death (SCD) is challenging to predict. Electrocardiogram (ECG)-derived heart rate-corrected QT-interval (QTc) is used for SCD-risk assessment. QTc is preferably determined manually, but vendor-provided automatic results from ECG recorders are convenient. Agreement between manual and automatic assessments is unclear for populations with aberrant QTc. We aimed to systematically assess pairwise agreement of automatic and manual QT-intervals and QTc. METHODS AND RESULTS: A multi-centre cohort enriching aberrant QTc comprised ECGs of healthy controls and long-QT syndrome (LQTS) patients. Manual QT-intervals and QTc were determined by the tangent and threshold methods and compared to automatically generated, vendor-provided values. We assessed agreement globally by intra-class correlation coefficients and pairwise by Bland-Altman analyses and 95% limits of agreement (LoA). Further, manual results were compared to a novel automatic QT-interval algorithm. ECGs of 1263 participants (720 LQTS patients; 543 controls) were available [median age 34 (inter-quartile range 35) years, 55% women]. Comparing cohort means, automatic and manual QT-intervals and QTc were similar. However, pairwise Bland-Altman-based agreement was highly discrepant. For QT-interval, LoAs spanned 95 (tangent) and 92 ms (threshold), respectively. For QTc, the spread was 108 and 105 ms, respectively. LQTS patients exhibited more pronounced differences. For automatic QTc results from 440-540 ms (tangent) and 430-530 ms (threshold), misassessment risk was highest. Novel automatic QT-interval algorithms may narrow this range. CONCLUSION: Pairwise vendor-provided automatic and manual QT-interval and QTc results can be highly discrepant. Novel automatic algorithms may improve agreement. Within the above ranges, automatic QT-interval and QTc results require manual confirmation, particularly if T-wave morphology is challenging.

Benefits, harms, and costs of newborn genetic screening for hypertrophic cardiomyopathy: Estimates from the PreEMPT model.

PURPOSE: Population newborn genetic screening for hypertrophic cardiomyopathy (HCM) is feasible, however its benefits, harms, and cost-effectiveness are uncertain. METHODS: We developed a microsimulation model to simulate a US birth cohort of 3.7 million newborns. Those identified with pathogenic/likely pathogenic variants associated with increased risk of HCM underwent surveillance and recommended treatment, whereas in usual care, individuals with family histories of HCM underwent surveillance. RESULTS: In a cohort of 3.7 million newborns, newborn genetic screening would reduce HCM-related deaths through age 20 years by 44 (95% uncertainty interval [UI] = 10-103) however increase the numbers of children undergoing surveillance by 8127 (95% UI = 6308-9664). Compared with usual care, newborn genetic screening costs $267,000 per life year saved (95% UI, $106,000 to $919,000 per life year saved). CONCLUSION: Newborn genetic screening for HCM could prevent deaths but at a high cost and would require many healthy children to undergo surveillance. This study shows how modeling can provide insights into the tradeoffs between benefits and costs that will need to be considered as newborn genetic screening is more widely adopted.

Accuracy of mobile 6-lead electrocardiogram device for assessment of QT interval: a prospective validation study.

INTRODUCTION: Ambulatory assessment of the heart rate-corrected QT interval (QTc) can be of diagnostic value, for example in patients on QTc-prolonging medication. Repeating sequential 12-lead electrocardiograms (ECGs) to monitor the QTc is cumbersome, but mobile ECG (mECG) devices can potentially solve this problem. As the accuracy of single-lead mECG devices is reportedly variable, a multilead mECG device may be more accurate. METHODS: This prospective dual-centre study included outpatients visiting our cardiology clinics for any indication. Participants underwent an mECG recording using a smartphone-enabled 6­lead mECG device immediately before or immediately after a conventional 12-lead ECG recording. Multiple QTc values in both recordings were manually measured in leads I and II using the tangent method and subsequently compared. RESULTS: In total, 234 subjects were included (mean ± standard deviation (SD) age: 57 ± 17 years; 58% males), of whom 133 (57%) had cardiac disease. QTc measurement in any lead was impossible due to artefacts in 16 mECGs (7%) and no 12-lead ECGs. Mean (± SD) QTc in lead II on the mECG and 12-lead ECG was 401 ± 30 and 406 ± 31 ms, respectively. Mean (± SD) absolute difference in QTc values between both modalities was 12 ± 9 ms (r = 0.856; p < 0.001). In 55% of the subjects, the absolute difference between QTc values was < 10 ms. CONCLUSION: A 6-lead mECG allows for QTc assessment with good accuracy and can be used safely in ambulatory QTc monitoring. This may improve patient satisfaction and reduce healthcare costs.

[The role of cardiac magnetic resonance imaging in defining the prognosis of patients with acute ST-segment elevation myocardial infarction. Part 2. Assessment of the disease prognosis].

Currently the incidence of congestive heart failure after ST-segment elevation myocardial infarction (STEMI) tends to increase. Reperfusion therapy is still the only effective method to reduce an infarct size. Therefore, there is a high unmet need of novel cardioprotective treatments that would improve outcomes in such patients. Recent advances in cardiovascular magnetic resonance (CMR) methods enabled the identification of certain new infarct characteristics associated with the development of heart failure and sudden cardiac death. These characteristics can help identify new groups of high risk patients and used as a targets for novel cardioprotective treatments. This part of the review summarizes novel CMR-based characteristics of myocardial infarction and their role in the prognostic stratification of STEMI patients.