Background Cardiac sarcoidosis is associated with malignant arrhythmias, and athletes with implantable cardioverter-defibrillators (ICDs) have historically been restricted from competition. Case Summary A 34-year old professional basketball player experienced cardiac arrest due to monomorphic ventricular tachycardia during competition, however cardiopulmonary resuscitation with automated external defibrillator use led to immediate cardioversion. Work-up revealed findings of probable cardiac sarcoidosis according to current criteria. He was treated with steroids, and an ICD was placed with no further arrhythmia. With shared decision-making, he returned to competition. Discussion The evaluation of cardiac arrest requires coronary, genetic, and myocardial interrogation, with myocardial processes best evaluated using advanced imaging and, selectively, endomyocardial biopsy. Recent data suggest the safety of ICD use in athletes. Take-Home Messages The diagnosis of cardiac sarcoidosis relies largely on clinical findings and noninvasive imaging and exists on a spectrum of definite, probable, possible, or unlikely. Return to play after ICD implantation is an important consideration in competitive athletes with sudden cardiac arrest and requires shared decision-making.
Importance Current risk prediction guidelines for hypertrophic cardiomyopathy predict only sudden cardiac death and are imperfect, leading to avoidable deaths and unnecessary implantable cardioverter defibrillators. Objective To combine prospectively collected clinical history, imaging, genetic, and biomarker data to improve risk prediction of adverse events in hypertrophic cardiomyopathy. Design, Setting, and Participants A total of 2750 patients with hypertrophic cardiomyopathy were prospectively enrolled in the registry-based study from 44 sites in North America and Europe with expertise in hypertrophic cardiomyopathy and cardiac magnetic resonance (CMR) imaging. Participants were enrolled from April 1, 2014, to April 7, 2017. Exposures Patients underwent a health history questionnaire, blood sampling for biomarkers and genotyping, and contrast-enhanced CMR. Patients were followed up yearly by telephone and through records review regarding event documentation. Main Outcomes and Measures The predefined composite adjudicated primary end point was time to first event for hypertrophic cardiomyopathy-related deaths; nonfatal sustained ventricular arrhythmias (VAs) requiring cardioversion or defibrillation; and left ventricular (LV) assist device implant or heart transplant. A secondary end point was a composite of sudden cardiac death and nonfatal VA events. The elastic-net method identified the most important predictors. Cox proportional hazards regression assessed associations with time to the first end point. Results Of the 2750 prospectively enrolled patients, 2698 (98%) had analyzable data after 9 were excluded because they had hypertrophic cardiomyopathy phenocopies and 43 withdrew. Of these remaining patients, 1919 (71%) were male, mean age was 50 years (SD, 11 years), and 423 (16%) were from underrepresented racial and minority groups. The mean follow-up was 6.9 years (SD, 2.1 years). The primary event model in 104 patients included LV scar as a percentage of LV mass by late gadolinium enhancement (LGE%; hazard ratio [HR], 1.86; 95% CI, 1.58-2.20; P < .001), LV mass index (HR, 1.09; 95% CI, 1.01-1.17; P = .03), LV end-systolic volume index (HR, 1.28; 95% CI, 1.12-1.46; P < .001 ), all per 10-unit increase, history of heart failure at study entry (HR, 2.89; 95% CI, 1.75-4.77; P < .001), and log N-terminal pro-B-type natriuretic peptide (NT-proBNP; HR, 1.41; 95% CI, 1.17-1.70; P < .001) level per log unit, (C index for all, 0.77). An LGE percentage of the LV mass of 9% or higher substantially increased the primary composite event rate (P = .001). The secondary sudden cardiac death and VA risk factor model (in 69 patients) included LGE%, LV mass index, LV ejection fraction, and log(NT-proBNP) (C index, 0.76). Conclusions and Relevance These results provide prospective evidence for incorporating cardiac magnetic resonance and NT-proBNP in the evaluation of patients with hypertrophic cardiomyopathy.
Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease characterized by myocardial hypertrophy and disarray caused by mutations in genes coding for cardiac sarcomeres. HCM is the most common inherited heart disease with a prevalence of 0.2%. Patients with HCM are at an increased risk of potentially lethal ventricular arrhythmias. Prevention of sudden cardiac death is best accomplished by implantation of a cardioverter-defibrillator (ICD) in patients who are at substantial risk of sudden cardiac death due to ventricular arrhythmias. Selection of patients in whom ICD implantation is appropriate is guided by the presence or absence of specific clinical risk factors.
Importance:Current risk prediction guidelines for hypertrophic cardiomyopathy predict only sudden cardiac death and are imperfect, leading to avoidable deaths and unnecessary implantable cardioverter defibrillators. Objective:To combine prospectively collected clinical history, imaging, genetic, and biomarker data to improve risk prediction of adverse events in hypertrophic cardiomyopathy. Design, Setting, and Participants:A total of 2750 patients with hypertrophic cardiomyopathy were prospectively enrolled in the registry-based study from 44 sites in North America and Europe with expertise in hypertrophic cardiomyopathy and cardiac magnetic resonance (CMR) imaging. Participants were enrolled from April 1, 2014, to April 7, 2017. Exposures:Patients underwent a health history questionnaire, blood sampling for biomarkers and genotyping, and contrast-enhanced CMR. Patients were followed up yearly by telephone and through records review regarding event documentation. Main Outcomes and Measures:The predefined composite adjudicated primary end point was time to first event for hypertrophic cardiomyopathy-related deaths; nonfatal sustained ventricular arrhythmias (VAs) requiring cardioversion or defibrillation; and left ventricular (LV) assist device implant or heart transplant. A secondary end point was a composite of sudden cardiac death and nonfatal VA events. The elastic-net method identified the most important predictors. Cox proportional hazards regression assessed associations with time to the first end point. Results:Of the 2750 prospectively enrolled patients, 2698 (98%) had analyzable data after 9 were excluded because they had hypertrophic cardiomyopathy phenocopies and 43 withdrew. Of these remaining patients, 1919 (71%) were male, mean age was 50 years (SD, 11 years), and 423 (16%) were from underrepresented racial and minority groups. The mean follow-up was 6.9 years (SD, 2.1 years). The primary event model in 104 patients included LV scar as a percentage of LV mass by late gadolinium enhancement (LGE%; hazard ratio [HR], 1.86; 95% CI, 1.58-2.20; P < .001), LV mass index (HR, 1.09; 95% CI, 1.01-1.17; P = .03), LV end-systolic volume index (HR, 1.28; 95% CI, 1.12-1.46; P < .001 ), all per 10-unit increase, history of heart failure at study entry (HR, 2.89; 95% CI, 1.75-4.77; P < .001), and log N-terminal pro-B-type natriuretic peptide (NT-proBNP; HR, 1.41; 95% CI, 1.17-1.70; P < .001) level per log unit, (C index for all, 0.77). An LGE percentage of the LV mass of 9% or higher substantially increased the primary composite event rate (P = .001). The secondary sudden cardiac death and VA risk factor model (in 69 patients) included LGE%, LV mass index, LV ejection fraction, and log(NT-proBNP) (C index, 0.76). Conclusions and Relevance:These results provide prospective evidence for incorporating cardiac magnetic resonance and NT-proBNP in the evaluation of patients with hypertrophic cardiomyopathy. Trial Registration:ClinicalTrials.gov Identifier: NCT01915615.
BACKGROUND:We performed an individual patient data analysis of COMBINE AF to assess differences in 14 clinically relevant outcomes between patients with paroxysmal (PAF) vs. non-PAF. METHODS:Cox-proportional-hazards models stratified by trial and adjusted for CHA2DS2-VASc elements were constructed. Sensitivity analyses were performed across subgroups. RESULTS:Among 71,466 patients with AF, 16,609 (23 %) had PAF. The overall follow-up period was 157,225 patient-years (median 2.2 years). Compared with non-PAF patients, PAF patients were more likely to be women (43 % vs 35 %), have prior coronary artery disease (35 % vs 31 %), and use aspirin (41 % vs 32 %), but were less likely to be Asian (12 % vs 15 %) or have CHA₂DS₂-VASc ≥4 (59 % vs 60 %) (all p<0.01). In adjusted analyses, PAF was associated with a lower risk of stroke/systemic embolic event (HR, 0.81; 95 % CI, 0.73-0.90; p<0.001) and all-cause death (HR, 0.81; 95 % CI, 0.75-0.86; p<0.001), but higher risk of major or clinically relevant non-major bleeding (HR, 1.07; 95 % CI, 1.02-1.13; p=0.005). Risk of myocardial infarction was numerically higher in PAF (HR, 1.15; 95 % CI, 1.00-1.32; p=0.057). Major bleeding risk was similar between groups (HR, 1.04; 95 % CI, 0.96-1.12; p=0.36). Findings were consistent across subgroups, trials, anticoagulant types, and sensitivity analyses. CONCLUSIONS:Compared to non-PAF, adjusted risks in PAF patients were lower for stroke/systemic embolic event and all-cause death, and higher for major or clinically relevant non-major bleeding and myocardial infarction.These findings highlight clinically important differences in outcomes by AF type that may have clinical applications for AF patients.
Sports cardiology is a cardiovascular subspecialty that cares for competitive athletes and highly active individuals with cardiovascular risk factors and/or disease. Exercise can lead to physiological, structural, and electrical cardiac remodeling while also contributing to maladaptive changes, including arrhythmias. These nuances have contributed to the emergence of sports electrophysiology as a specialized extension within sports cardiology, dedicated to understanding arrhythmias, including best practices in arrhythmia management in this patient group. Although the diagnosis and treatment of arrhythmias in athletes often parallel standard electrophysiological practices, there are differences and factors to be taken into account. Examples include exercise type and intensity; potential performance effects and side effects of antiarrhythmic therapies; timing and indications for catheter ablation; device selection and programming for cardiac implantable electronic devices when applicable; interpretation of cardiovascular testing; and return-to-play guidance. In this state-of-the-art review, athletic electrical remodeling, clinical considerations for arrhythmia management in athletes, device-related matters, and future directions for the evolving field of sports electrophysiology are discussed. This document builds on recent clinical references by providing practical clinical guidance for electrophysiologists and cardiovascular clinicians who care for athletic patients with arrhythmias and formally introduces the concept of "sports electrophysiology" within sports cardiology as a novel discipline.
Hypertrophic cardiomyopathy (HCM) in pregnancy presents unique hemodynamic and arrhythmic challenges despite generally low maternal mortality. This JACC State-of-the-Art Review synthesizes current evidence on preconception risk stratification, genetic counseling, antepartum surveillance, arrhythmia management, and delivery and postpartum care. Key considerations include assessing left ventricular outflow tract obstruction, systolic function, and arrhythmia burden and applying CARPREG II (Cardiac Disease in Pregnancy Study II)/modified World Health Organization classifications to guide monitoring intensity. Pregnancy is typically well tolerated in low-risk women, whereas severe obstruction or systolic dysfunction confers high maternal-fetal risk. Multidisciplinary pregnancy heart team management remains essential to optimize outcomes and support informed decision making across the reproductive continuum.
AIM:The American College of Cardiology/American Heart Association Scientific Statement, "Clinical Considerations for the Care of the Tactical Athlete With Cardiovascular Abnormalities," was written to provide guidance and education for clinicians caring for the tactical athlete (ie, firefighters, law enforcement officers, military) with cardiovascular disease or risk for cardiovascular disease, and for the organizations overseeing the care and wellness of these athletes. The considerations are shaped by the interaction between occupational demands and fit for full duty assessments, including risk discussions about how a cardiovascular event in a tactical athlete could impact teammates' well-being, community safety, and overall mission success. METHODS:This scientific statement is organized into 11 sections focused on cardiovascular disease processes and other topics that are relevant when considering the potential risks and benefits of performing tasks specific to the tactical athlete. Task forces, comprised of experts in tactical athlete domains, sports cardiology, and the respective topics covered, were assigned to each section, and specific "Clinical Considerations Tables" for clinicians to reference were prepared. Comprehensive literature reviews and an emphasis on tactical athlete-focused data, as available, were integral in the writing of all clinical considerations presented. The framework mirrors that of the recently published, "Clinical Considerations for Competitive Sports Participation for Athletes With Cardiovascular Abnormalities: A Scientific Statement From the American Heart Association and American College of Cardiology." STRUCTURE:The specific sections in this document include: Section 1: Tactical Tasks Classification; Section 2: The Tactical Athlete Preparticipation Cardiac Evaluation; Section 3: Ethical and Legal Aspects of Tactical Clinical Management; Section 4: Genetic Cardiomyopathies; Section 5: Myocarditis and Other Acquired Cardiac Conditions; Section 6: Congenital Heart Disease; Section 7: Aortopathy, Bicuspid Aortic Valve, and Spontaneous Coronary Artery Dissection; Section 8: Syncope, SCA, Arrhythmias, and Devices; Section 9: Cardiac Channelopathies; Section 10: Older Tactical Athlete; Section 11: Environmental Exposures, PED/S, and Additional Cardiac Conditions and Considerations. Each section provides a summary detailing the rationale for key clinical considerations and the respective Clinical Considerations Table(s).
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Electrocardiograms (ECGs) remain a foundational pillar of cardiovascular diagnostics, providing rapid, non-invasive diagnosis and being universally accessible to all clinicians. An ECG captures the electrical signals of the heart via a standard 12-lead configuration, offering insights into arrhythmias, conduction delays, ischemic injury, structural remodeling, and systemic pathologies with cardiac implications. This review presents a structured framework for ECG interpretation by discussing general approaches to rate, rhythm, axis, intervals, and repolarization dynamics, and by outlining both cardiac and non-cardiac conditions associated with ECG abnormalities. We explore the accelerating pace of innovations in artificial intelligence (AI) for ECG analysis. Deep learning algorithms now rival and, in select domains, surpass expert clinicians in detecting left ventricular systolic and diastolic dysfunction, hypertrophic obstructive cardiomyopathy, and acute myocardial infarction. The integration of AI-enhanced ECG interpretation enables earlier disease recognition, refined risk stratification, and optimized clinical decision-making across acute and chronic care settings. This review systematically guides readers through ECG interpretation, linking fundamental principles with nuanced clinical patterns using AI to enhance accurate diagnosis and improve patient outcomes across a wide range of cardiovascular conditions.
BACKGROUND:Over the past decades, hypertrophic cardiomyopathy has become a contemporary treatable disease. However, limited data exist on the global trends of implantable cardioverter defibrillator (ICD) utilization and its impact on mortality/morbidity burden reduction.METHODS:Electronic databases were systematically searched up to March 2024 for studies reporting on ICD utilization rates in hypertrophic cardiomyopathy. A random effects model was used to pool study estimates across time-era, geographic region, and age group. Primary outcome was global trends in ICD utilization. Secondary outcomes included trends of sudden cardiac death, appropriate/inappropriate shocks, and ICD-related complications.RESULTS:In total, 234 studies (N=92 500, 514 748 patient-years) met inclusion criteria. Mean age was 46.2 (12.4) years and 37.49% were women. A total of 12 139 patients (16.43%) received an ICD over 429 766 person-years of follow-up, with an ICD implantation rate of 2.79%/y ([95% CI, 2.35%-3.32%] I-2=97.80%). Rates of ICD implantation steadily increased over time from 1990 (1.09%) to 2021 (4.01%; P=0.002), with noticeable geographic variation (P=0.008). The overall rate of appropriate ICD discharges and ICD-related complications was 3.44%/y ([95% CI, 3.08%-3.84%] I-2=88.40%) and 1.98%/y ([95% CI, 1.52%-2.59%] I-2=90.44%), respectively, with no significant trend over time. The overall rate of inappropriate discharges was 3.58%/y ([95% CI, 3.08%-4.16%] I2=88.03%), and declined significantly over time (P=0.044). There was a significant decline in the rates of sudden cardiac death from 1990 (0.84%/y) to 2020 (0.31%/y).CONCLUSIONS:Dramatic increases in ICD utilization have occurred, representing a 3.7-fold increase, with appropriate therapies occurring in 3.44%/y. In parallel a significant reduction in sudden cardiac death was observed, but there are insufficient data to demonstrate that a causative relationship exists. Geographic disparities in ICD utilization were evident, highlighting the need to improve access to specialized care for patients with hypertrophic cardiomyopathy. Geographic disparities in ICD utilization were evident, highlighting the need to improve access to specialized care for patients with hypertrophic cardiomyopathy.REGISTRATION:URL: https://www.crd.york.ac.uk/PROSPERO/; Unique identifier: CRD42023407126
This American Heart Association/American College of Cardiology scientific statement on clinical considerations for competitive sports participation for athletes with cardiovascular abnormalities or diseases is organized into 11 distinct sections focused on sports-specific topics or disease processes that are relevant when considering the potential risks of adverse cardiovascular events, including sudden cardiac arrest, during competitive sports participation. Task forces comprising international experts in sports cardiology and the respective topics covered were assigned to each section and prepared specific clinical considerations tables for practitioners to reference. Comprehensive literature review and an emphasis on shared decision-making were integral in the writing of all clinical considerations presented.