IMPORTANCE Strategies for reliable selection of high-risk patients with hypertrophic cardiomyopathy (HCM) for prevention of sudden cardiac death (SCD) with implantable cardioverter/defibrillators (ICDs) are incompletely resolved. OBJECTIVE To assess the reliability of SCD prediction methods leading to prophylactic ICD recommendations to reduce the number of SCDs occurring in patients with HCM. DESIGN, SETTING, AND PARTICIPANTS In this observational longitudinal study, 2094 predominantly adult patients with HCM consecutively evaluated over 17 years in a large HCM clinical center were studied. All patients underwent prospective ICD decision making relying on individual major risk markers derived from the HCM literature and an enhanced American College of Cardiology/American Heart Association (ACC/AHA) guidelines-based risk factor algorithm with complete clinical outcome follow-up. Data were collected from June 2017 to February 2018, and data were analyzed from February to July 2018. MAIN OUTCOMES AND MEASURES Arrhythmic SCD or appropriate ICD intervention for ventricular tachycardia or ventricular fibrillation. RESULTS Of the 2094 study patients, 1313 (62.7%) were male, and the mean (SD) age was 51 (17) years. Of 527 patients with primary prevention ICDs implanted based on 1 or more major risk markers, 82 (15.6%) experienced device therapy-terminated ventricular tachycardia or ventricular fibrillation episodes, which exceeded the 5 HCM-related SCDs occurring among 1567 patients without ICDs (0.3%), including 2 who declined device therapy, by 49-fold (95% CI, 20-119; P = .001). Cumulative 5-year probability of an appropriate ICD intervention was 10.5% (95% CI, 8.0-13.5). The enhanced ACC/AHA clinical risk factor strategy was highly sensitive for predicting SCD events (range, 87%-95%) but less specific for identifying patients without SCD events (78%). The C statistic calculated for enhanced ACC/AHA guidelines was 0.81 (95% CI, 0.77-0.85), demonstrating good discrimination between patients who did or did not experience an SCD event. Compared with enhanced ACC/AHA risk factors, the European Society of Cardiology risk score retrospectively applied to the study patients was much less sensitive than the ACC/AHA criteria (34% [95% CI, 22-44] vs 95% [95% CI, 89-99]), consistent with recognizing fewer high-risk patients. CONCLUSIONS AND RELEVANCE A systematic enhanced ACC/AHA guideline and practice-based risk factor strategy prospectively predicted SCD events in nearly all at-risk patients with HCM, resulting in prophylactically implanted ICDs that prevented many catastrophic arrhythmic events in this at-risk population.

BackgroundThe relation of sex to clinical presentation and course in hypertrophic cardiomyopathy (HCM) remains incompletely resolved. We assessed differences in clinical outcomes between men and women within our large HCM cohort.Methods and ResultsOf 2123 consecutive patients, a minority (38%) were women who were diagnosed with HCM at older ages or referred for subspecialty evaluation later than men (50±19 versus 44±16 and 55±18 versus 49±16; P<0.001). Women more commonly developed advanced New York Heart Association class III/IV symptoms (53% versus 35% in men; P<0.001), predominantly secondary to outflow obstruction. While end‐stage heart failure with systolic dysfunction (ejection fraction <50%) was similar in men (5% versus 4% in women; P=0.33), women were 3‐fold more likely to develop heart failure with preserved systolic function (7.5% versus 2.6%; P=0.002). Sudden death events terminated by defibrillator therapy were similar in women (0.9%/year) versus men (1.0%/year; hazard ratio, 0.92; 95% CI, 0.6–1.5; P=0.73). HCM mortality was uncommon, with identical rates in both sexes (0.3%/year; hazard ratio, 1.5; 95% CI, 0.7–3.4;, P=0.25). Age‐adjusted all‐cause mortality also did not differ between women and men (1.7% versus 1.3%/year; hazard ratio, 1.32; 95% CI, 0.92–1.91; P=0.13).ConclusionsSurvival was not less favorable in women with HCM. Contemporary treatments including surgical myectomy to reverse heart failure and defibrillators to prevent sudden death, were effective in both sexes contributing to low mortality. However, despite more frequent outflow obstruction, women with HCM are underrecognized and referred to centers later than men, often with more advanced heart failure. Greater awareness of HCM in women should lead to earlier diagnosis and treatment, with implications for improved quality of life.

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Introduction Hyper-cytokinemia is a dreaded complication of severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection and an important predictor of mortality in coronavirus disease 2019 (COVID-19). The current evidence at best is still ambiguous for use of tocilizumab in cytokine storm in COVID-19. Moreover, the factors that are associated with beneficial response from tocilizumab are unknown in COVID-19. We aimed to study the clinical outcomes especially mortality vis-à-vis clinical and laboratory characteristics of patients administered tocilizumab and identify predictors of mortality benefits amongst deceased vs recovered COVID-19 patients. Methods The present study is a retrospective observation of the demographic, clinical, and biological data of all the consecutive patients treated with tocilizumab for COVID-19 pneumonia at the COVID tertiary care centre from July 2020 to October 2020 at Ahmedabad, India. We compared the deceased group with those who recovered/discharged and evaluated patient-level demographics, clinical attributes, and laboratory investigations available to identify subgroups in whom tocilizumab reduced mortality. Results Of the 112 patients included, the mean (SD) age was 56.84 ± 13.56 years and 80 (71.4%) were male. There were 97 (86.6%) patients in the survivors and 15 (13.39%) in the deceased group. Deceased were older than the recovered group (mean: 66.14, SD: 14.41 vs mean: 55.36, SD: 12.98; p=0.04). Hypertension (33.03%) was the commonest comorbidity observed. Mortality was significantly higher in patients with cancer and type-2 diabetes (p=0.05 and p=0.01, respectively). Level of D-dimer and lactate dehydrogenase (LDH) showed trends towards significance as a predictor of mortality (p=0.07 and p=0.08, respectively) not reaching significance. D-dimer level > 5,000 nanograms per millilitre (ng/mL) was the significant predictor of subsequent deaths (p<0.0001). Fourteen patients reported adverse events of tocilizumab. Patients who developed in-hospital complications (such as septic or vasodilatory shock and/or sepsis, acute kidney injury, multiorgan dysfunction) had significantly higher mortality (p<0.0001, p=0.009, and p=0.03, respectively). Conclusion Tocilizumab might be more beneficial in younger patients without sepsis/ septic shock, acute kidney injury, multiorgan dysfunction, and who were non-ventilated. The predictors of mortality amongst Asian Indians treated with tocilizumab were older patients, the presence of type-2 diabetes, cancer, in-hospital complication (such as acute kidney injury, sepsis/septic shock, multiorgan dysfunction), higher D-dimer > 5,000 ng/mL. A larger study with pre-defined inclusion cutoffs of these variables may aid in defining patient's characteristics of Asian Indians who may benefit from tocilizumab in COVID-19.

IMPORTANCEThere was a shift in patient volume from in-person to video telemedicine visits during the COVID-19 pandemic. OBJECTIVE To determine the concordance of provisional diagnoses established at a video telemedicine visit with diagnoses established at an in-person visit for patients presenting with a new clinical problem. DESIGN, SETTING, AND PARTICIPANTS This is a diagnostic study of patients who underwent a video telemedicine consultation followed by an in-person outpatient visit for the same clinical problem in the same specialty within a 90-day window. The provisional diagnosis made during the video telemedicine visit was compared with the reference standard diagnosis by 2 blinded, independent medical reviewers. A multivariate logistic regression model was used to determine factors significantly related to diagnostic concordance. The study was conducted at a large academic integrated multispecialty health care institution (Mayo Clinic locations in Rochester, Minnesota; Scottsdale and Phoenix, Arizona; and Jacksonville, Florida; and Mayo Clinic Health System locations in Iowa, Wisconsin, and Minnesota) between March 24 and June 24, 2020. Participants included Mayo Clinic patients residing in the US without age restriction. Data analysis was performed from December 2020 to June 2021. EXPOSURES New clinical problem assessed via video telemedicine visit to home using Zoom Care Anyplace integrated into Epic. MAIN OUTCOMES AND MEASURES Concordance of provisional diagnoses established over video telemedicine visits compared against a reference standard diagnosis. RESULTS There were 2393 participants in the analysis. The median (IQR) age of patients was 53 (37-64) years; 1381 (57.7%) identified as female, and 1012 (42.3%) identified as male. Overall, the provisional diagnosis established over video telemedicine visit was concordant with the in-person reference standard diagnosis in 2080 of 2393 cases (86.9%; 95% CI, 85.6%-88.3%). Diagnostic concordance by International Statistical Classification of Diseases and Related Health Problems, TenthRevision chapter ranged from 64.7% (95% CI, 42.0%-87.4%) for diseases of the ear and mastoid process to 96.8% (95% CI, 94.7%-98.8%) for neoplasms. Diagnostic concordance by medical specialty ranged from 77.3% (95% CI, 64.9%-89.7%) for otorhinolaryngology to 96.0% (92.1%-99.8%) for psychiatry. Specialty care was found to be significantly more likely than primary (continued) Key Points Question How concordant to an in-person diagnosis are provisional diagnoses established at a video telemedicine visit for patients presenting with a new clinical problem? Findings In this diagnostic study of 2393 patients who underwent a video telemedicine consultation followed by an in-person outpatient visit for the same clinical problem in the same specialty within a 90-day window, the provisional diagnosis established over video telemedicine visit matched the in-person reference standard diagnosis in 86.9% of cases. Meaning These findings suggest that video telemedicine visits yield a high deg...