Death and Cardiac Arrest in U.S. Triathlon Participants, 1985 to 2016: A Case Series.

Background: Reports of race-related triathlon fatalities have raised questions regarding athlete safety.

Objective: To describe death and cardiac arrest among triathlon participants.

Design: Case series.

Identification of Fabry Disease in a Tertiary Referral Cohort of Patients with Hypertrophic Cardiomyopathy.

Background: Fabry disease is an X-linked lysosomal storage disorder caused by the deficient activity of α-galactosidase A due to mutations in the GLA gene, which may be associated with increased left ventricular wall thickness and mimic the morphologic features of hypertrophic cardiomyopathy. Management strategies for these 2 diseases diverge, with Fabry disease-specific treatment utilizing recombinant α-galactosidase A enzyme replacement therapy.

Methods: We studied a prospectively assembled consecutive cohort of 585 patients (71% male) from 2 hypertrophic cardiomyopathy tertiary referral centers by screening for low α-galactosidase A activity in dried blood spots.

Ventricular Tachyarrhythmias in Patients With Hypertrophic Cardiomyopathy and Defibrillators: Triggers, Treatment, and Implications.

Introduction: Triggers and ICD interventions of ventricular arrhythmias in patients with hypertrophic cardiomyopathy (HCM) offer insight into mechanisms and treatment.

Methods And Results: Intracardiac ICD electrograms from 71 HCM patients in the HCM I and II studies were analyzed by three individuals. Rhythms were defined as VF (polymorphic ventricular arrhythmia), VT (monomorphic ventricular tachycardia), and ventricular flutter (VFL; VT ≥ 240 bpm).

Hypertrophic Cardiomyopathy With Left Ventricular Apical Aneurysm: Implications for Risk Stratification and Management.

Background: A previously under-recognized subset of hypertrophic cardiomyopathy (HCM) patients with left ventricular (LV) apical aneurysms is being identified with increasing frequency. However, risks associated with this subgroup are unknown.

Objectives: The authors aimed to clarify clinical course and prognosis of a large cohort of HCM patients with LV apical aneurysms over long-term follow-up.

Lack of Phenotypic Differences by Cardiovascular Magnetic Resonance Imaging in MYH7 (β-Myosin Heavy Chain)- Versus MYBPC3 (Myosin-Binding Protein C)-Related Hypertrophic Cardiomyopathy.

Background: The 2 most commonly affected genes in hypertrophic cardiomyopathy (HCM) are MYH7 (β-myosin heavy chain) and MYBPC3 (β-myosin-binding protein C). Phenotypic differences between patients with mutations in these 2 genes have been inconsistent. Scarce data exist on the genotype-phenotype association as assessed by tomographic imaging using cardiac magnetic resonance imaging.

Demographics and Epidemiology of Sudden Deaths in Young Competitive Athletes: From the United States National Registry.

Background: Sudden deaths in young competitive athletes are tragic events, with high public visibility. The importance of race and gender with respect to sport and the diagnosis and causes of sudden death in athletes has generated substantial interest.

Methods: The US National Registry of Sudden Death in Athletes, 1980-2011, was accessed to define the epidemiology and causes of sudden deaths in competitive athletes.

Relation of Doppler Tissue Imaging Parameters With Heart Failure Progression in Hypertrophic Cardiomyopathy.

Refractory progressive heart failure (HF) is becoming the predominant cause of mortality in nonobstructive hypertrophic cardiomyopathy (HC). To anticipate development of this important and often unpredictable clinical course, we investigated whether left ventricular diastolic dysfunction, assessed by echocardiographic Doppler parameters, could identify a subset of patients with HC without obstruction at rest who would experience progression of HF. Diastolic function parameters, assessed by Doppler tissue imaging (DTI), mitral inflow, and pulmonary venous flow were measured in 274 consecutive adult patients with HC evaluated from 2003 to 2007.

Significance of Late Gadolinium Enhancement at Right Ventricular Attachment to Ventricular Septum in Patients With Hypertrophic Cardiomyopathy.

Cardiovascular magnetic resonance (CMR) with extensive late gadolinium enhancement (LGE) is a novel marker for increased risk for sudden death (SD) in patients with hypertrophic cardiomyopathy (HC). Small focal areas of LGE confined to the region of right ventricular (RV) insertion to ventricular septum (VS) have emerged as a frequent and highly visible CMR imaging pattern of uncertain significance. The aim of this study was to evaluate the prognostic significance of LGE confined to the RV insertion area in patients with HC.

Significance of sarcomere gene mutations analysis in the end-stage phase of hypertrophic cardiomyopathy.

End-stage hypertrophic cardiomyopathy (ES-HC) has an ominous prognosis. Whether genotype can influence ES-HC occurrence is unresolved. We assessed the spectrum and clinical correlates of HC-associated mutations in a large multicenter cohort with end-stage ES-HC.

Significance of left ventricular apical-basal muscle bundle identified by cardiovascular magnetic resonance imaging in patients with hypertrophic cardiomyopathy.

Aims: Cardiovascular magnetic resonance (CMR) has improved diagnostic and management strategies in hypertrophic cardiomyopathy (HCM) by expanding our appreciation for the diverse phenotypic expression. We sought to characterize the prevalence and clinical significance of a recently identified accessory left ventricular (LV) muscle bundle extending from the apex to the basal septum or anterior wall (i.e.

Strategies for assessing the prevalence of cardiovascular sudden deaths in young competitive athletes.

Background/objectives: Prevalence/incidence of sudden death due to cardiovascular disease in young competitive athletes has become an important part of the debate over the most effective and practical preparticipation screening strategies for this population. Since event reporting is not mandatory, identification of cases has been achieved largely through publicly available data and internet searches. The accuracy of this methodology has not been studied and deserves scrutiny.

Risk of sudden death and outcome in patients with hypertrophic cardiomyopathy with benign presentation and without risk factors.

Patients with hypertrophic cardiomyopathy (HC) are reported to have a mortality rate of about 1.0% per year, and those patients without sudden death risk factors and with no or mild symptoms are generally considered to have a benign clinical presentation. However, the risk of sudden death and the outcome in this latter subgroup have not been investigated systematically and remain unresolved.

Left atrial remodeling in hypertrophic cardiomyopathy and susceptibility markers for atrial fibrillation identified by cardiovascular magnetic resonance.

In hypertrophic cardiomyopathy (HC), atrial fibrillation (AF) is an important determinant of clinical deterioration due to heart failure or embolic stroke. This study characterizes left atrial (LA) structural and functional parameters to establish markers predictive of AF risk, using cardiovascular magnetic resonance (CMR) imaging. We studied 427 consecutive patients with HC in sinus rhythm with CMR (age 44±18 years), including 41 who developed clinically overt AF after study entry (2.

Incidence and causes of sudden death in U.S. college athletes.

Objectives: The goal of this study was to reliably define the incidence and causes of sudden death in college student-athletes.

Background: The frequency with which cardiovascular-related sudden death occurs in competitive athletes importantly influences considerations for pre-participation screening strategies.

Methods: We assessed databases (including autopsy reports) from both the U.

Obesity and its association to phenotype and clinical course in hypertrophic cardiomyopathy.

Objectives: This study sought to assess the impact of body mass index (BMI) on cardiac phenotypic and clinical course in a multicenter hypertrophic cardiomyopathy (HCM) cohort.

Background: It is unresolved whether clinical variables promoting left ventricular (LV) hypertrophy in the general population, such as obesity, may influence cardiac phenotypic and clinical course in patients with HCM.

Methods: In 275 adult HCM patients (age 48 ± 14 years; 70% male), we assessed the relation of BMI to LV mass, determined by cardiovascular magnetic resonance (CMR) and heart failure progression.

Risk stratification and outcome of patients with hypertrophic cardiomyopathy >=60 years of age.

Background: Hypertrophic cardiomyopathy (HCM) is prominently associated with risk for sudden death and disease progression, largely in young patients. Whether patients of more advanced age harbor similar risks is unresolved, often creating clinical dilemmas, particularly in decisions for primary prevention of sudden death with implantable defibrillators.

Methods And Results: We studied 428 consecutive HCM patients presenting at ≥60 years of age and followed for 5.

Incidence of cardiovascular sudden deaths in Minnesota high school athletes.

Background: Sudden death (SD) in young competitive athletes due to cardiovascular disease is an important community issue with relevance to designing effective screening initiatives. However, the frequency with which these tragic events occur importantly affects considerations for selecting the most appropriate screening strategy.

Objective: To determine the incidence and causes of cardiovascular SDs in Minnesota high school athletes.

Increasing survival rate from commotio cordis.

Background: Commotio cordis events due to precordial blows triggering ventricular fibrillation are a cause of sudden death (SD) during sports and also daily activities. Despite the absence of structural cardiac abnormalities, these events have been considered predominantly fatal with low survival rates.

Objective: To determine whether expected mortality rates for commotio cordis have changed over time, associated with greater public visibility.

Ventricular tachycardia/fibrillation early after defibrillator implantation in patients with hypertrophic cardiomyopathy is explained by a high-risk subgroup of patients.

Background: Implantable cardioverter-defibrillator (ICD) studies in patients with coronary artery disease report higher risk of ventricular tachycardia/fibrillation (VT/VF) early post-implant, potentially related to local proarrhythmic effects of ICD leads.

Objective: To characterize early and long-term risk of ICD discharge for VT/VF in a large hypertrophic cardiomyopathy (HCM) cohort.

Methods: By using HCM multicenter registry data, we compared long-term risk of VT/VF subsequent to an early post-implant period (a priori defined as within 3 months of implant) between patients with or without VT/VF within 3 months after ICD implantation.

Significance of false negative electrocardiograms in preparticipation screening of athletes for hypertrophic cardiomyopathy.

Preparticipation screening of athletes with 12-lead electrocardiography has been promoted for the detection of asymptomatic cardiovascular disease, particularly hypertrophic cardiomyopathy (HC). Although false-positive electrocardiographic (ECG) results for HC are well recognized in athlete screening, expected false-negative rates are unknown. The aim of this study was to characterize the rate of false-negative ECG findings in a cohort of young asymptomatic patients with phenotypically expressed HC, defined by cardiovascular magnetic resonance, using the 2010 European Society of Cardiology recommended ECG criteria for the identification of suspected heart disease in trained athletes.

Sickle cell trait associated with sudden death in competitive athletes.

Sickle cell trait (SCT; hemoglobin AS) occurs in 8% of African Americans and although typically benign has been associated with sudden death in military recruits during intense physical activity. However, the role of SCT in the deaths of trained athletes is less well documented or acknowledged. The 31-year United States Sudden Death in Athletes Registry was interrogated to determine the frequency, epidemiology, and clinical profile of SCT-related death in a large population of competitive athletes.