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The aim of this study was to define the variability of maximal wall thickness (MWT) measurements across modalities and predict its impact on care in patients with hypertrophic cardiomyopathy (HCM).Left ventricular MWT measured by echocardiography or cardiovascular magnetic resonance (CMR) contributes to the diagnosis of HCM, stratifies risk, and guides key decisions, including whether to place an implantable cardioverter-defibrillator (ICD).A 20-center global network provided paired echocardiographic and CMR data sets from patients with HCM, from which 17 paired data sets of the highest quality were selected. These were presented as 7 randomly ordered pairs (at 6 cardiac conferences) to experienced readers who report HCM imaging in their daily practice, and their MWT caliper measurements were captured. The impact of measurement variability on ICD insertion decisions was estimated in 769 separately recruited multicenter patients with HCM using the European Society of Cardiology algorithm for 5-year risk for sudden cardiac death.MWT analysis was completed by 70 readers (from 6 continents; 91% with5 years' experience). Seventy-nine percent and 68% scored echocardiographic and CMR image quality as excellent. For both modalities (echocardiographic and then CMR results), intramodality inter-reader MWT percentage variability was large (range -59% to 117% [SD ±20%] and -61% to 52% [SD ±11%], respectively). Agreement between modalities was low (SE of measurement 4.8 mm; 95% CI 4.3 mm-5.2 mm; r = 0.56 [modest correlation]). In the multicenter HCM cohort, this estimated echocardiographic MWT percentage variability (±20%) applied to the European Society of Cardiology algorithm reclassified risk in 19.5% of patients, which would have led to inappropriate ICD decision making in 1 in 7 patients with HCM (8.7% would have had ICD placement recommended despite potential low risk, and 6.8% would not have had ICD placement recommended despite intermediate or high risk).Using the best available images and experienced readers, MWT as a biomarker in HCM has a high degree of inter-reader variability and should be applied with caution as part of decision making for ICD insertion. Better standardization efforts in HCM recommendations by current governing societies are needed to improve clinical decision making in patients with HCM.

Background: Cardiac muscle hypercontractility is a key pathophysiological abnormality in hypertrophic cardiomyopathy, and a major determinant of dynamic left ventricular outflow tract (LVOT) obstruction. Available pharmacological options for hypertrophic cardiomyopathy are inadequate or poorly tolerated and are not disease-specific. We aimed to assess the efficacy and safety of mavacamten, a first-in-class cardiac myosin inhibitor, in symptomatic obstructive hypertrophic cardiomyopathy. Methods: In this phase 3, randomised, double-blind, placebo-controlled trial (EXPLORER-HCM) in 68 clinical cardiovascular centres in 13 countries, patients with hypertrophic cardiomyopathy with an LVOT gradient of 50 mm Hg or greater and New York Heart Association (NYHA) class II–III symptoms were assigned (1:1) to receive mavacamten (starting at 5 mg) or placebo for 30 weeks. Visits for assessment of patient status occurred every 2–4 weeks. Serial evaluations included echocardiogram, electrocardiogram, and blood collection for laboratory tests and mavacamten plasma concentration. The primary endpoint was a 1·5 mL/kg per min or greater increase in peak oxygen consumption (pVO2) and at least one NYHA class reduction or a 3·0 mL/kg per min or greater pVO2 increase without NYHA class worsening. Secondary endpoints assessed changes in post-exercise LVOT gradient, pVO2, NYHA class, Kansas City Cardiomyopathy Questionnaire-Clinical Summary Score (KCCQ-CSS), and Hypertrophic Cardiomyopathy Symptom Questionnaire Shortness-of-Breath subscore (HCMSQ-SoB). This study is registered with ClinicalTrials.gov, NCT03470545. Findings: Between May 30, 2018, and July 12, 2019, 429 adults were assessed for eligibility, of whom 251 (59%) were enrolled and randomly assigned to mavacamten (n=123 [49%]) or placebo (n=128 [51%]). 45 (37%) of 123 patients on mavacamten versus 22 (17%) of 128 on placebo met the primary endpoint (difference +19·4%, 95% CI 8·7 to 30·1; p=0·0005). Patients on mavacamten had greater reductions than those on placebo in post-exercise LVOT gradient (−36 mm Hg, 95% CI −43·2 to −28·1; p2 (+1·4 mL/kg per min, 0·6 to 2·1; p=0·0006), and improved symptom scores (KCCQ-CSS +9·1, 5·5 to 12·7; HCMSQ-SoB −1·8, −2·4 to −1·2; p

Introduction: Hypertension is strongly linked to obesity. Blood pressure variability (BPV), a novel BP phenotype with potential clinical significance, may also be associated with increased adiposity. We aimed to investigate the relationship between body fat distribution and average BP and BPV in the short- and long-term. Methods: Using data from the Dallas Heart Study, we assessed within-visit (short-term) and visit-to-visit (long-term) BPV (average real variability [ARV], defined as the average of absolute differences of BP between successive BP measures) during a 5-month follow-up period (2000-2002). Five BP measures were taken at each of the 3 visits (two in-office and one in-home survey). The associations of visceral (VAT) and abdominal subcutaneous adipose tissue (SAT) quantified by MRI and lower body subcutaneous fat (LBF) by DEXA with BP phenotypes were assessed using multivariable-adjusted linear regression. Results: 2595 participants with mean age 44 years; 54 % women; 68 % non-White; mean BMI 29 kg/m2, mean SBP 127 mmHg and DBP 79 mmHg were included. The short-term ARV of SBP at home and office, and long-term ARV of SBP over 3 visits were 4.0±2.4 mm Hg, 4.1±2.5 mmHg, and 9.8±7.1 mmHg, respectively. VAT, SAT, and LBF were not associated with short-term ARV of SBP, while higher VAT was associated with lower long-term ARV of SBP (Table). VAT was strongly associated with average SBP in the short- and long-term, while higher LBF was associated with lower average SBP in the short- and long-term. Conclusions: Greater VAT is associated with persistently higher short- and long-term average BP with lower variability over visits. The presence of persistently elevated BP, coupled with lower BPV, associated with visceral adiposity may impose higher cardiac workload and consequently increase cardiac hypertrophy and risk for heart failure.

Objective: While the global epidemic of diabetes mellitus in adults is well documented, there are few reliable estimates of the prevalence of diabetes and prediabetes in adolescents in the United States. We conducted systematic hemoglobin A1C (HbA1C) screening in a very large adolescent blood donor population. Methods: A screening program was implemented in which HbA1C was measured at the time of blood donation among 31,546 volunteer high-school blood donors, 16-19 years of age, who participated in school blood drives conducted by Carter BloodCare, the largest independent blood program in Texas, between 2011 and 2013. The main study measures were the prevalence of HbA1C in the prediabetes range, 5.7-6.4% (39-46 mmol/mol) and in the diabetes range, ≥ 6.5% (≥ 48 mmol/mol), in the overall cohort as well as stratified by sex, race/ethnicity, and sociodemographic characteristics. Results: In the overall cohort, the prevalence of HbA1C in the prediabetes range was 11.0% (95% CI 10.7, 11.4), and the prevalence of HbA1C in the diabetes range was 0.53% (95% CI, 0.46, 0.62). The prevalence of HbA1C ≥ 5.7% (including prediabetes and diabetes) was higher in boys compared with girls (15.7% vs.7.9%, PTable ). Conclusions: HbA1C values in the prediabetes or diabetes range are common among adolescent blood donors, particularly among boys and ethnic minorities. Blood donation programs can be used as a valuable portal for public health screening, and may provide the opportunity for targeted public health interventions, triaging of donors to medical care, and research programs for community-based participatory interventions.