Evaluating the Association between Anomalous Aortic Origin of the Right Coronary Artery from the Left Sinus with Interarterial Course at Coronary CT Angiography and Sudden Cardiac Death.

Purpose To investigate the association between the anomalous aortic origin of the right coronary artery (R-AAOCA) from the left coronary sinus with interarterial course (IAC) found at coronary CT angiography and sudden cardiac death using a large data set from five university hospitals. Materials and Methods From a total of 89 314 CCTA scans (January 2009 to December 2016) that were retrospectively collected, 316 patients with R-AAOCA from the left sinus with IAC were retrospectively collected. After excluding patients with less than 2 years of follow-up, patients who had already undergone cardiovascular surgery or intervention, and patients with arrhythmia or heart failure before undergoing coronary CT angiography, 224 patients were analyzed. Follow-up was terminated upon the occurrence of major adverse cardiovascular events (MACE). Logistic regression was used to identify clinical and radiologic information as independent predictors of MACE. Results The period prevalence of R-AAOCA from the left sinus with IAC was 0.354%. The mean age was 62.03 years, with a male-to-female ratio of 182:134. During follow-up, 19 of 224 patients (8.5%) experienced MACE, but none had sudden cardiac death. Of these cases, only seven (3.13%) were suspected of being due to R-AAOCA from the left sinus with IAC and all of them had unstable angina. Coronary artery disease was significantly associated with MACE (P < .001), while no significant correlation was observed with radiologic features. Conclusion Sudden cardiac death was not associated with R-AAOCA from the left sinus with IAC found at coronary CT angiography. The occurrence of MACE was low, with coronary artery disease being the sole significant predictor of a patient's prognosis. Keywords: Anomalous Aortic Origin of the Right Coronary Artery, Left Coronary Sinus with Interarterial Course, Coronary CT Angiography, Sudden Cardiac Death Supplemental material is available for this article. © RSNA, 2024.

Sudden cardiac arrest in patients with cancer in the general population: insights from the Paris-SDEC registry.

BACKGROUND: Data on the management of patients with cancer presenting with sudden cardiac arrest (SCA) are scarce. We aimed to assess the characteristics and outcomes of SCA according to cancer history. METHODS: Prospective, population-based registry including every out-of-hospital SCA in adults in Paris and its suburbs, between 2011 and 2019, with a specific focus on patients with cancer. RESULTS: Out of 4069 patients who had SCA admitted alive in hospital, 207 (5.1%) had current or past medical history of cancer. Patients with cancer were older (69.2 vs 59.3 years old, p<0.001), more often women (37.2% vs 28.0%, p=0.006) with more frequent underlying cardiovascular disease (41.1% vs 32.5%, p=0.01). SCA happened more often with a non-shockable rhythm (62.6% vs 43.1%, p<0.001) with no significant difference regarding witness presence and cardiopulmonary resuscitation (CPR) performed. Cardiac causes were less frequent among patients with cancer (mostly acute coronary syndromes, 25.5% vs 46.8%, p<0.001) and had more respiratory causes (pulmonary embolism and hypoxaemia in 34.2% vs 10.8%, p<0.001). Still, no difference regarding in-hospital survival was found after SCA in patients with cancer versus other patients (26.2% vs 29.8%, respectively, p=0.27). Public location, CPR by witness and shockable rhythm were independent predictors of in-hospital survival after SCA in the cancer group. CONCLUSIONS: One in 20 SCA occurs in patients with a history of cancer, yet with fewer cardiac causes than in patients who are cancer-free. Still, in-hospital outcomes remain similar even in patients with known cancer. Cancer history should therefore not compromise the initiation of resuscitation in the context of SCA.

Atrial fibrillation and risk of sudden cardiac arrest in young adults.

AIMS: Evidence of an association between atrial fibrillation (AF) and sudden cardiac arrest (SCA) in young adults is limited. In this study, we aim to evaluate this association in a general population aged between 20 and 39 years. METHODS AND RESULTS: Young adults who underwent health check-ups between 2009 and 2012 were screened from a nationwide healthcare database in South Korea. A history of AF diagnosis before the health check-ups was identified based on the relevant International Classification of Diseases, 10th edition codes reported in the database. Associations between an established diagnosis of AF and the risk of SCA during follow-up were examined. A total of 6 345 162 young people were analysed with a mean follow-up duration of 9.4 years. The mean age was 30.9 ± 5.0 years, and 5875 (0.09%) individuals were diagnosed with AF. During follow-up, SCA occurred in 5352 (0.08%) individuals, and the crude incidence was 0.56 and 0.09 events per 1000 person-years for participants with and without AF, respectively. Individuals with AF had a 3.0-fold higher risk in a multivariate model adjusted for age, sex, lifestyle, anthropometric data, and medical comorbidities (adjusted hazard ratio 2.96, 95% confidence interval 1.99-4.41, P < 0.001). Both incident and prevalent AFs were associated with an increased risk of SCA, with no significant differences between the two groups. CONCLUSION: Atrial fibrillation was associated with a significantly higher risk of SCA developing in healthy young adults. Whether the rate or rhythm control influences the risk of SCA in young patients with AF remains to be examined.

[Bibliometrics Analysis of Studies on Hypertrophic Cardiomyopathy From 2018 to 2022].

Objective To analyze the research progress and hot topics in hypertrophic cardiomyopathy from 2018 to 2022.Methods The publications in the field of hypertrophic cardiomyopathy from January 1,2018 to December 31,2022 were retrieved from Web of Science core collection database and included for a bibliometric analysis.Results A total of 6355 publications were included,with an average citation frequency of 7 times.The year 2021 witnessed the most publications (1406).The analysis with VOSviewer showed that the research on sudden death related to hypertrophic cardiomyopathy,especially the predictive value of late gadolinium-enhanced cardiac MRI in sudden death,was a hot topic.In addition,gene detection and the new drug mavacamten became hot research topics.The United States was the country with the largest number of publications and the highest citation frequency in this field.Chinese scholars produced the second largest number of publications,which,however,included few high-quality research results.Conclusions Risk stratification and prevention of sudden death is still an important and hot research content in the field of hypertrophic cardiomyopathy.Chinese scholars should carry out multi-center cooperation in the future to improve the research results.

Occurrence of premature battery depletion in a large multicentre registry of subcutaneous cardioverter-defibrillator patients.

AIMS: Subcutaneous implantable cardioverter-defibrillators (S-ICDs) have become established in preventing sudden cardiac death, with some advantages over transvenous defibrillator systems, including a lower incidence of lead failures. Despite technological advancements, S-ICD carriers may suffer from significant complications, such as premature battery depletion (PBD), that led to an advisory for nearly 40 000 patients. This multicentre study evaluated the incidence of PBD in a large set of S-ICD patients. METHODS AND RESULTS: Data from patients implanted with S-ICD models A209 and A219 between October 2012 and July 2023 across nine centres in Europe and the USA were reviewed. Incidence and implications of PBD, defined as clinically observed sudden drop in battery longevity, were analysed and compared to PBD with the definition of battery depletion within 60 months. Prospectively collected clinical data were obtained retrospectively from medical records, device telemetry, and manufacturer reports. This registry is listed on ClinicalTrials.gov (NCT05713708). Of the 1112 S-ICD devices analysed, 547 (49.2%) were equipped with a potentially affected capacitor linked to PBD occurrence, currently under Food and Drug Administration advisory. The median follow-up time for all patients was 46 [inter-quartile range (IQR) 24-63] months. Clinically suspected PBD was observed in 159 (29.1%) of cases, with a median time to generator removal or replacement of 65 (IQR 55-72) months, indicative of significant deviations from expected battery lifespan. Manufacturer confirmation of PBD was made in 91.7% of devices returned for analysis. No cases of PBD were observed in devices that were not under advisory. CONCLUSION: This manufacturer-independent analysis highlights a notable incidence of PBD in patients equipped with S-ICD models under advisory, and the rate of PBD in this study corresponds to the rate currently estimated by the manufacturer. To the best of our knowledge, this provides the largest contemporary peer-reviewed study cohort investigating the actual incidence of PBD in S-ICD patients. These findings emphasize the importance of post-market registries in collaboration between clinicians and the manufacturer to optimize safety and efficacy in S-ICD treatment.

A Patch Wearable Cardioverter-Defibrillator for Patients at Risk of Sudden Cardiac Arrest.

BACKGROUND: For many patients, sudden cardiac arrest (SCA) risk is elevated temporarily. Wearable cardioverter-defibrillators (WCDs) can monitor and treat SCA during these temporary periods. Traditional WCDs can be uncomfortable, require frequent maintenance, and cannot be used when showering, resulting in poor compliance and avoidable SCA deaths. The Jewel is a novel, water-resistant patch-wearable cardioverter-defibrillator (P-WCD) with a machine learning detection algorithm designed to improve compliance and protection against SCA. OBJECTIVES: This study aims to demonstrate the safety and clinical effectiveness of a novel P-WCD. METHODS: The Jewel IDE Study, a prospective, single-arm study conducted at 30 U.S. sites, enrolled patients at SCA risk due to ventricular tachycardia/ventricular fibrillation who were not candidates for or refused an implantable defibrillator. The primary safety endpoint was <15% patients with clinically significant cutaneous adverse device effects and the primary effectiveness endpoint was <2 inappropriate shocks/100 patient-months. Secondary endpoints were ≥1 successful ventricular tachycardia/ventricular fibrillation conversion and wear time compliance of >14.1 h/d. RESULTS: A total of 305 patients (mean age: 57.9 years; 30.2% female, 27.9% non-White) were enrolled, of which 290 had available device data. The clinically significant cutaneous adverse device effect rate was 2.30% (upper 1-sided 98% CI: 4.80); none were severe. No device-related deaths or serious adverse events were reported. The inappropriate shock rate was 0.36/100 patient-months (upper 1-sided 98% CI: 1.53). Of 11 shocks in 9 patients, 9 shocks were adjudicated to be appropriate. Eight of 9 shocks were successful with a single shock. Median wear time compliance was 23.5 (20.7-23.9) h/d. CONCLUSIONS: The novel P-WCD is a safe and effective WCD with high patient compliance. There were no deaths due to noncompliance and a high number of successful conversions (Jewel IDE study [A Clinical Evaluation of the Jewel P-WCD in Subjects at High Risk for Sudden Cardiac Arrest]; NCT05201495).

Depression and Implantable Cardioverter-Defibrillator Implantation in Black Patients at Risk for Sudden Cardiac Death.

BACKGROUND: Black patients meeting indications for implantable cardioverter-defibrillators (ICDs) have lower rates of implantation compared with White patients. There is little understanding of how mental health impacts the decision-making process among Black patients considering ICDs. Our objective was to assess the association between depressive symptoms and ICD implantation among Black patients with heart failure. METHODS AND RESULTS: This is a secondary analysis of the VIVID (Videos to Address Racial Disparities in ICD Therapy via Innovative Designs) randomized trial, which enrolled self-identified Black individuals with chronic systolic heart failure. Depressive symptoms were assessed by the Patient Health Questionnaire-2 and the Mental Component Summary of the 12-Item Short-Form Health Survey. Decisional conflict was measured by an adapted Decisional Conflict Scale (DCS). ANCOVA was used to assess differences in Decisional Conflict Scale scores. Multivariable logistic regression was used to examine the association between depressive symptoms and ICD implantation. Among 306 participants, 60 (19.6%) reported depressed mood, and 142 (46.4%) reported anhedonia. Participants with the lowest Mental Component Summary of the 12-Item Short-Form Health Survey scores (poorer mental health and higher likelihood of depression) had greater decisional conflict regarding ICD implantation compared with those with the highest Mental Component Summary of the 12-Item Short-Form Health Survey scores (adjusted mean difference in Decisional Conflict Scale score, 3.2 [95% CI, 0.5-5.9]). By 90-day follow-up, 202 (66.0%) participants underwent ICD implantation. There was no association between either the Patient Health Questionnaire-2 score or the Mental Component Summary of the 12-Item Short-Form Health Survey score and ICD implantation. CONCLUSIONS: Depressed mood and anhedonia were prevalent among ambulatory Black patients with chronic systolic heart failure considering ICD implantation. The presence of depressive symptoms did not impact the likelihood of ICD implantation in this population.

Sudden cardiac arrest occurring in temporal proximity to consumption of energy drinks.

BACKGROUND: Energy drinks potentially can trigger life-threatening cardiac arrhythmias. It has been postulated that the highly stimulating and unregulated ingredients alter heart rate, blood pressure, cardiac contractility, and cardiac repolarization in a potentially proarrhythmic manner. OBJECTIVE: The purpose of this study was to describe our experience regarding sudden cardiac arrest (SCA) occurring in proximity to energy drink consumption in patients with underlying genetic heart diseases. METHODS: The electronic medical records of all SCA survivors with proven arrhythmias referred to the Mayo Clinic Windland Smith Rice Genetic Heart Rhythm Clinic for evaluation were reviewed to identify those who consumed an energy drink before their event. Patient demographics, clinical characteristics, documented energy drink consumption, and temporal relationship of energy drink consumption to SCA were obtained. RESULTS: Among 144 SCA survivors, 7 (5%; 6 female; mean age at SCA 29 ± 8 years) experienced an unexplained SCA associated temporally with energy drink consumption. Of these individuals, 2 had long QT syndrome and 2 had catecholaminergic polymorphic ventricular tachycardia; the remaining 3 were diagnosed with idiopathic ventricular fibrillation. Three patients (43%) consumed energy drinks regularly. Six patients (86%) required a rescue shock, and 1 (14%) was resuscitated manually. All SCA survivors have quit consuming energy drinks and have been event-free since. CONCLUSION: Overall, 5% of SCA survivors experienced SCA in proximity to consuming an energy drink. Although larger cohort studies are needed to elucidate the incidence/prevalence and quantify its precise risk, it seems prudent to sound an early warning on this potential risk.

Occurrence of premature battery depletion in a large multicentre registry of subcutaneous cardioverter-defibrillator patients.

AIMS: Subcutaneous implantable cardioverter-defibrillators (S-ICDs) have become established in preventing sudden cardiac death, with some advantages over transvenous defibrillator systems, including a lower incidence of lead failures. Despite technological advancements, S-ICD carriers may suffer from significant complications, such as premature battery depletion (PBD), that led to an advisory for nearly 40 000 patients. This multicentre study evaluated the incidence of PBD in a large set of S-ICD patients. METHODS AND RESULTS: Data from patients implanted with S-ICD models A209 and A219 between October 2012 and July 2023 across nine centres in Europe and the USA were reviewed. Incidence and implications of PBD, defined as clinically observed sudden drop in battery longevity, were analysed and compared to PBD with the definition of battery depletion within 60 months. Prospectively collected clinical data were obtained retrospectively from medical records, device telemetry, and manufacturer reports. This registry is listed on ClinicalTrials.gov (NCT05713708). Of the 1112 S-ICD devices analysed, 547 (49.2%) were equipped with a potentially affected capacitor linked to PBD occurrence, currently under Food and Drug Administration advisory. The median follow-up time for all patients was 46 [inter-quartile range (IQR) 24-63] months. Clinically suspected PBD was observed in 159 (29.1%) of cases, with a median time to generator removal or replacement of 65 (IQR 55-72) months, indicative of significant deviations from expected battery lifespan. Manufacturer confirmation of PBD was made in 91.7% of devices returned for analysis. No cases of PBD were observed in devices that were not under advisory. CONCLUSION: This manufacturer-independent analysis highlights a notable incidence of PBD in patients equipped with S-ICD models under advisory, and the rate of PBD in this study corresponds to the rate currently estimated by the manufacturer. To the best of our knowledge, this provides the largest contemporary peer-reviewed study cohort investigating the actual incidence of PBD in S-ICD patients. These findings emphasize the importance of post-market registries in collaboration between clinicians and the manufacturer to optimize safety and efficacy in S-ICD treatment.

Diagnostic cost-effectiveness of a two-stage cardiovascular evaluation program in young-adolescent athletes. Role of echocardiography.

BACKGROUND: Sports medical examinations are a tool to detect potentially life-threatening situations during physical activity, but their usefulness and protocols in young athletes remain controversial. The aim was to describe the diagnostic performance of a 2-step ECG and echocardiogram screening model in a large sample of healthy young-adolescent athletes (aged 11-16 years), evaluating its cost-effectiveness for cardiovascular disease (CVD) detection. METHODS: Cross-sectional study of 2617 individuals (81 % male, mean age 15 years), over 20 federated sports (La Rioja-Spain). A cardiological examination included family and personal history, physical examination, ECG, and transthoracic echocardiogram. The previous ECG, conducted at 11-13 years old, was reviewed in 1589 individuals (60 %). ECGs were interpreted according to 2017 international criteria. The prevalence of structural cardiac disease (CD) was investigated, and the diagnostic performance of both tests and the cost of screening was evaluated. RESULTS: CVD was diagnosed in 63 athletes (2.4 %) and 16 (0.6 %) with pathology related to sudden cardiac death (SCD). In 3 cases (0.11 %), cessation of sporting activity was indicated, and in 2 cases (0.07 %) treatment for congenital CD was indicated. ECG alterations were infrequent (2.5 %). Eighty percent of the structural CD diagnosed by echocardiogram had a normal ECG. The cost per CVD detected was €3,080, and for CVD associated with SCD, it was €12,323. CONCLUSIONS: Our study shows the diagnostic cost-effectiveness of a two-step cardiac screening protocol, including ECG, and highlights the role of echocardiography in young adolescent athletes, which could be implemented at a low and reasonable cost.

New York Heart Association functional class and implantable cardioverter-defibrillator in non-ischaemic heart failure with reduced ejection fraction: Extended follow-up of the DANISH trial.

AIMS: Current guidelines recommend implantable cardioverter-defibrillator (ICD) therapy in patients with heart failure, a left ventricular ejection fraction of ≤35%, and New York Heart Association (NYHA) class II-III. However, the evidence regarding the benefit of primary prevention ICD is less consistent in patients with NYHA class III. We investigated the long-term effects of primary prevention ICD implantation according to NYHA class in an extended follow-up study of the DANISH trial. METHODS AND RESULTS: The DANISH trial randomized 1116 patients with non-ischaemic heart failure with reduced ejection fraction (HFrEF) to ICD implantation or usual care. Outcomes were analysed according to NYHA class at baseline (NYHA class II and III/IV). The primary outcome was all-cause mortality. Of the 1116 patients randomized in the DANISH trial, 597 (53.5%) were in NYHA class II at baseline, 505 (45.3%) in NYHA class III, and 14 (1.3%) in NYHA class IV. During a median follow-up of 9.5 years, NYHA class III/IV, compared with NYHA class II, were associated with a greater long-term rate of all-cause mortality (hazard ratio [HR] 1.52, 95% confidence interval [CI] 1.20-1.93) and cardiovascular death (HR 1.95 [1.47-2.60]). ICD implantation, compared with usual care, did not reduce the long-term rate of all-cause mortality (all participants: HR 0.89 [95% CI 0.74-1.08]; NYHA class II: HR 0.85 [0.64-1.13]; NYHA class III/IV: HR 0.89 [0.69-1.14]; pinteraction = 0.78) or cardiovascular death (all participants: HR 0.87 [95% CI 0.70-1.09]; NYHA class II: HR 0.78 [0.54-1.12]; NYHA class III/IV: HR 0.89 [0.67-1.19]; pinteraction = 0.58), irrespective of NYHA class. Similarly, NYHA class did not modify the beneficial effects of ICD implantation on sudden cardiovascular death (all participants: HR 0.60 [95% CI 0.40-0.92]; NYHA class II: HR 0.73 [0.40-1.36]; NYHA class III/IV: HR 0.52 [0.29-0.94]; pinteraction = 0.39). CONCLUSIONS: In patients with non-ischaemic HFrEF, ICD implantation, compared with usual care, did not reduce the overall mortality rate, but it did reduce sudden cardiovascular death, regardless of baseline NYHA class. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov NCT00542945.

Arrhythmogenic right ventricular cardiomyopathy: Unveiling clinical presentations, CMR insights and prognosis in a single-center retrospective study.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition with a prevalence ranging from 1 in 2000 to 1 in 5000 individuals. ARVC is a significant contributor to sudden cardiac death, particularly in young individuals and athletes, and remains challenging to diagnose definitively. We conducted a single-center retrospective study to evaluate the presentations, electrocardiogram findings, and imaging characteristics of ARVC patients evaluated at our center between 2021 and 2023. Notably, our study is the second investigation of ARVC conducted in Pakistan. We report divergent symptom prevalence as compared to the current literature and have incorporated the Task Force Criteria. Despite limited access to cardiac magnetic resonance (CMR) facilities worldwide, our findings underscore the critical role ofCMR in ARVC diagnosis. Our cohort had a mortality rate of 17 % highlighting the importance of early detection and the need for improved diagnostic facilities for ARVC in the region.

Magnetic Resonance Imaging Characterization and Clinical Outcomes of Dilated and Arrhythmogenic Left Ventricular Cardiomyopathies.

BACKGROUND: Nondilated left ventricular cardiomyopathy (NDLVC) has been recently differentiated from dilated cardiomyopathy (DCM). A comprehensive characterization of these 2 entities using cardiac magnetic resonance (CMR) and genetic testing has never been performed. OBJECTIVES: This study sought to provide a thorough characterization and assess clinical outcomes in a large multicenter cohort of patients with DCM and NDLVC. METHODS: A total of 462 patients with DCM (227) or NDLVC (235) with CMR data from 4 different referral centers were retrospectively analyzed. The study endpoint was a composite of sudden cardiac death or major ventricular arrhythmias. RESULTS: In comparison to DCM, NDLVC had a higher prevalence of pathogenic or likely pathogenic variants of arrhythmogenic genes (40% vs 23%; P < 0.001), higher left ventricular (LV) systolic function (LV ejection fraction: 51% ± 12% vs 36% ± 15%; P < 0.001) and higher prevalence of free-wall late gadolinium enhancement (LGE) (27% vs 14%; P < 0.001). Conversely, DCM showed higher prevalence of pathogenic or likely pathogenic variants of nonarrhythmogenic genes (23% vs 12%; P = 0.002) and septal LGE (45% vs 32%; P = 0.004). Over a median follow-up of 81 months (Q1-Q3: 40-132 months), the study outcome occurred in 98 (21%) patients. LGE with septal location (HR: 1.929; 95% CI: 1.033-3.601; P = 0.039) was independently associated with the risk of sudden cardiac death or major ventricular arrhythmias together with LV dilatation, older age, advanced NYHA functional class, frequent ventricular ectopic activity, and nonsustained ventricular tachycardia. CONCLUSIONS: In a multicenter cohort of patients with DCM and NDLVC, septal LGE together with LV dilatation, age, advanced disease, and frequent and repetitive ventricular arrhythmias were powerful predictors of major arrhythmic events.

Candidacy and long-term outcomes of subcutaneous implantable cardioverter-defibrillators in current practice in patients with hypertrophic cardiomyopathy.

BACKGROUND: In patients with Hypertrophic Cardiomyopathy (HCM) S-ICD is usually the preferred option as pacing is generally not indicated. However, limited data are available on its current practice adoption and long-term follow-up. METHODS: Consecutive HCM patients with S-ICD implanted between 2013 and 2021 in 3 international centers were enrolled in this observational study. Baseline, procedural and follow-up data were regularly collected. Efficacy and safety were compared with a cohort of HCM patients implanted with a tv-ICD. RESULTS: Seventy patients (64% males) were implanted with S-ICD at 41 ± 15 years, whereas 168 patients with tv-ICD at 49 ± 16 years. For S-ICD patients, mean ESC SCD risk score was 4,5 ± 1.9%: 25 (40%) at low-risk, 17 (27%) at intermediate and 20 (33%) at high-risk. Patients were followed-up for 5.1 ± 2.3 years. Two patients (0.6 per 100-person-years, vs 0.4 per 100 person-years with tv-ICD, p = 0.45) received an appropriate shock on VF, 17 (24%) were diagnosed with de-novo AF. Inappropriate shocks occurred in 4 patients (1.2 per 100-person-years, vs 0.9 per 100 person-years with tv-ICD, p = 0.74), all before Smart-Pass algorithm implementation. Four patients experienced device-related adverse events (1.2 per 100-person-years, vs 1 per 100 person-years with tv-ICD, p = 0.35%). CONCLUSIONS: S-ICDs were often implanted in patients with an overall low-intermediate ESC SCD risk, reflecting both the inclusion of additional risk markers and a lower decision threshold. S-ICDs in HCM patients followed for over 5 years showed to be effective in conversion of VF and safe. Greater scrutiny may be required to avoid overtreatment in patients with milder risk profiles.

Evaluation of new predictive scores for sudden cardiac death in childhood hypertrophic cardiomyopathy in a French cohort.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is rare in children, and sudden cardiac death (SCD) is difficult to predict. Two prognostic scores - HCM Risk-Kids and Precision Medicine for Cardiomyopathy (PRIMaCY) - were developed to assess the risk of SCD in the next 5 years in children with HCM. AIMS: To test the ability of these scores to predict SCD in children with HCM. Also, to identify factors associated with a severe cardiac rhythmic event (SCRE) (ventricular fibrillation, sustained ventricular tachycardia, heart transplant for rhythmic reasons or SCD). METHODS: Retrospective, multicentre, observational study at 10 medical centres in the Nord-Pas-de-Calais region, France. RESULTS: This study included 72 paediatric patients with HCM during 2009-2019 who were followed for a median (interquartile range [IQR]) of 8.5 (5.0-16.2) years. Eleven patients (15.3%) presented with SCRE. HCM Risk-Kids was high, with a median (IQR) score of 6.2% (2.1-12.8%; significant threshold≥6.0%) and the PRIMaCY median (IQR) score was 7.1% (2.6-15.0%; significant threshold≥8.3%). The positive predictive value was only 27.1% (95% confidence interval [CI] 21.5-32.5%) for HCM Risk-Kids (with a threshold of≥6.0%) and 33.2% (95% CI 27.1-38.9%) for the PRIMaCY score (with a threshold of≥8.3%). The negative predictive values were 95.4% (95% CI 92.3-97.7%) and 93.0% (95% CI 89.8-96.2%), respectively. Three of 28 patients with an implantable cardioverter defibrillator (ICD) experienced complications (including inappropriate shocks). CONCLUSION: HCM Risk-Kids and the PRIMaCY score have low positive predictive values to predict SCD in paediatric patients. If used alone, they could increase the rate of ICD implantation and thus ICD complications. Therefore, the scores should be used in combination with other data (genetic and magnetic resonance imaging results).

[Sudden death in adults : Data from 305 consecutive autopsy cases in Algeria]. / Mort subite de l'adulte : données de 305 cas d'autopsies consécutives en Algérie.

BACKGROUND-AIMS: Sudden death in a young adult who showed no prodrome or complaint during his lifetime is a tragedy. The death often remains unexplained by doctors and is often the subject of a judicial investigation following which an autopsy is ordered. Our study joins several studies around the world, where the results have linked sudden death in adults to a cardiac origin. METHODS: Through a series of 305 autopsies carried out in the forensic medicine department of the Frantz Fanon hospital in the city of Bejaia in Algeria over a period of two years, 57 cases corresponded to unexplained sudden deaths, i.e. an incidence of 3 cases per 100,000 inhabitants per year. RESULTS: Sudden death was of cardiac origin in 50.8% of cases (N=28). Two epidemiologic profiles emerge in our study: the first is that of a man aged between 50 and 60 years of age, with several deleterious lifestyle habits (in particular smoking) with a cardiovascular history, previously followed by a cardiologist, who died suddenly out-of-hospital, from ischemic heart disease. The second is that of a young adult under 40 years of age, of average build, with no particular medical history, having not previously consulted a cardiologist, who died suddenly of hypertrophic cardiomyopathy. CONCLUSIONS: In many instances, we observed major anatomical lesion, which had not motivated any prior medical consultation either with a general practitioner or with a cardiologist.

International Criteria for Reporting Study Quality for Sudden Cardiac Arrest/Death Tool.

BACKGROUND: Studies reporting on the incidence of sudden cardiac arrest and/or death (SCA/D) in athletes commonly lack methodological and reporting rigor, which has implications for screening and preventative policy in sport. To date, there are no tools designed for assessing study quality in studies investigating the incidence of SCA/D in athletes. METHODS AND RESULTS: The International Criteria for Reporting Study Quality for Sudden Cardiac Arrest/Death tool (IQ-SCA/D) was developed following a Delphi process. Sixteen international experts in sports cardiology were identified and invited. Experts voted on each domain with subsequent moderated discussion for successive rounds until consensus was reached for a final tool. Interobserver agreement between a novice, intermediate, and expert observer was then assessed from the scoring of 22 relevant studies using weighted and unweighted κ analyses. The final IQ-SCA/D tool comprises 8 domains with a summated score of a possible 22. Studies are categorized as low, intermediate, and high quality with summated IQ-SCA/D scores of ≤11, 12 to 16, and ≥17, respectively. Interrater agreement was "substantial" between all 3 observers for summated IQ-SCA/D scores and study categorization. CONCLUSIONS: The IQ-SCA/D is an expert consensus tool for assessing the study quality of research reporting the incidence of SCA/D in athletes. This tool may be used to assist researchers, reviewers, journal editors, and readers in contextualizing the methodological quality of different studies with varying athlete SCA/D incidence estimates. Importantly, the IQ-SCA/D also provides an expert-informed framework to support and guide appropriate design and reporting practices in future SCA/D incidence trials.

Relationship Between Genotype Status and Clinical Outcome in Hypertrophic Cardiomyopathy.

BACKGROUND: The genetic basis of hypertrophic cardiomyopathy (HCM) is complex, and the relationship between genotype status and clinical outcome is incompletely resolved. METHODS AND RESULTS: We assessed a large international HCM cohort to define in contemporary terms natural history and clinical consequences of genotype. Consecutive patients (n=1468) with established HCM diagnosis underwent genetic testing. Patients with pathogenic (or likely pathogenic) variants were considered genotype positive (G+; n=312; 21%); those without definite disease-causing mutations (n=651; 44%) or variants of uncertain significance (n=505; 35%) were considered genotype negative (G-). Patients were followed up for a median of 7.8 years (interquartile range, 3.5-13.4 years); HCM end points were examined by cumulative event incidence. Over follow-up, 135 (9%) patients died, 33 from a variety of HCM-related causes. After adjusting for age, all-cause and HCM-related mortality did not differ between G- versus G+ patients (hazard ratio [HR], 0.78 [95% CI, 0.46-1.31]; P=0.37; HR, 0.93 [95% CI, 0.38-2.30]; P=0.87, respectively). Adverse event rates, including heart failure progression to class III/IV, heart transplant, or heart failure death, did not differ (G- versus G+) when adjusted for age (HR, 1.20 [95% CI, 0.63-2.26]; P=0.58), nor was genotype independently associated with sudden death event risk (HR, 1.39 [95% CI, 0.88-2.21]; P=0.16). In multivariable analysis, age was the only independent predictor of all-cause and HCM-related mortality, heart failure progression, and sudden death events. CONCLUSIONS: In this large consecutive cohort of patients with HCM, genotype (G+ or G-) was not a predictor of clinical course, including all-cause and HCM-related mortality and risk for heart failure progression or sudden death. G+ status should not be used to dictate clinical management or predict outcome in HCM.