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Obesity is common among individuals with obstructive hypertrophic cardiomyopathy (HCM) and is associated with an increased likelihood of left ventricular outflow tract (LVOT) obstruction. Mavacamten, a novel cardiac myosin inhibitor, was approved for the treatment of symptomatic obstructive hypertrophic cardiomyopathy (HCM) in April 2022. Data regarding the association of body mass index (BMI) and characteristics and treatment outcomes among patients initiated on mavacamten in the real-world are limited. We performed a retrospective study of all patients who completed 36 weeks of mavacamten treatment from May 2022 to December 2023 at a single high volume HCM center of excellence. Baseline demographics, clinical characteristics, and echocardiographic data, including left ventricular ejection fraction (LVEF) and LVOT gradients at rest and with Valsalva, were collected. Descriptive statistics were presented as means (standard deviation [SD]) or as counts and percentages and stratified by BMI category. Individuals with BMI greater than or equal to 30.0 kg/m2 were categorized as obese. Ninety-six patients (54% female) were initiated on mavacamten, 84 patients completed 12 weeks of therapy, and 56 patients completed 36 weeks of therapy. Obese patients were initiated on mavacamten nearly a decade earlier in life compared with non-obese patients (58 [15] vs. 68 [12] years, p<0.001), but duration of HCM diagnosis was similar for both groups (p=0.13). Prevalence of hypertension (p=0.28) and atrial fibrillation (p=0.53) were similar between the two groups; however, obstructive coronary artery disease was significantly more common in the non-obese patients (22% vs. 6%, p=0.026). Similar proportions of patients in the two groups experienced New York Heart Association class III symptoms prior to mavacamten initiation. Baseline LVEF and LVOT gradients at rest and with Valsalva were similar in obese and non-obese patients. Patients in both groups who completed 12 and 36 weeks of mavacamten therapy achieved comparable improvements in obstructive physiology. In this large single center real-world study of patients with symptomatic obstructive HCM, patients with BMI greater than or equal to 30.0 kg/m2 experienced similar favorable hemodynamic outcomes with mavacamten therapy compared with non-obese patients. [ABSTRACT FROM AUTHOR]

Mavacamten, a first-in-class cardiac myosin ATPase inhibitor was approved for the treatment of symptomatic obstructive hypertrophic cardiomyopathy (HCM) in April 2022. Little is known about sex differences in characteristics and treatment outcomes among patients initiated on mavacamten in the post-approval, real-world phase. Retrospective study of all patients who completed 36 weeks of mavacamten from May 2022 to December 2023 at a single center. Baseline demographics, clinical characteristics, and echocardiographic data, including left ventricular ejection fraction (LVEF) and left ventricular outflow tract (LVOT) gradients at rest and with Valsalva, were collected. Descriptive statistics were presented as means (standard deviation [SD]) or as counts and percentages as appropriate and stratified by sex. Ninety-six patients (54% female) were initiated on mavacamten. Females were started on mavacamten at a significantly older mean age (68 [13] vs. 57 [14] years, p<0.001) and were more severely symptomatic, as assessed by New York Heart Association (NYHA) class at baseline, prior to initiating mavacamten (56% female NYHA III vs. 23% male NYHA III, p=0.001). At the baseline echocardiogram, females had a higher mean resting LVOT gradient at rest (67 [45] vs. 43 [28] mm Hg, p=0.003) and with Valsalva (89 [36] vs. 66 [24] mm Hg, p<0.001). Females were less likely to have a care partner (71% vs. 95%, p=0.002) and more likely to live alone (29% vs. 7%, p=0.013). In this large single center real world study of patients with symptomatic obstructive HCM initiated on mavacamten, females were older at HCM diagnosis and suffered from worse symptoms compared with men, similar to the findings of the EXPLORER-HCM trial. We also found that females in this cohort were less likely to have a care partner and more likely to live alone, highlighting an opportunity to structure care interventions that may improve health status and the treatment experience for females. [ABSTRACT FROM AUTHOR]

Mavacamten, a first-in-class cardiac myosin inhibitor, received FDA approval for the treatment of symptomatic hypertrophic obstructive cardiomyopathy (oHCM) in April 2022. In this study, we sought to evaluate the early effect of mavacamten on cardiac structure and function by echocardiography in a real-world cohort of patients. All patients started on mavacamten at a single center oHCM referral center between June 2022 and March 2023 were included. Baseline clinical characteristics and echocardiographic parameters were collected every 4 weeks from initiation of Mavacamten, up to 20 weeks of follow-up; which included measurements of left ventricular (LV) wall thickness, left ventricular outflow gradient (LVOTg) at rest and with Valsalva, left ventricular ejection fraction (LVEF), right ventricular function, and diastology parameters. Echocardiographic data were compared using Wilcox matched-pairs signed rank test. Of the 44 patients initiated on mavacamten, 45% were female, and the median age was 63 years (IQR 48, 71). All patients were symptomatic with severe dynamic LVOT obstruction on maximally tolerated medical therapy. Baseline demographic, clinical characteristics and echocardiographic parameters are reported in Table 1. The average follow up period was 19±9weeks. Median dose at the end of the follow up period was 5mg. The baseline average resting LVOTg was 60 ± 30 mm Hg, and the baseline average LVOTg with Valsalva was 73 ±27.5 mm Hg. By week 4 of treatment, the average resting LVOTg significantly decreased to 24.5 ± 19 mm Hg and the average LVOTg with Valsalva was 42.8 ±26 mmHg (59% reduction from baseline) (p<0.02). After an average of 19±9 weeks of follow-up, the average resting LVOT gradient significantly decreased to 21±18 mmHg and the average LVOTg with Valsalva decreased to 36±23 mmHg, indicating a 64% decrease from baseline (p<0.02). 27% of patients required at least one reduction in dose per FDA protocol due to a decrease in LVOT gradient to <20 mmHg, with no patient requiring drug cessation due to decline in LVEF. There was no statistically significant difference between LVEF at initiation and at the end of the follow-up period. There was a non-significant trend towards improvement in filling pressures after initiation of mavacamten. Right ventricular function parameters and median inter-ventricular septum thickness were unchanged at the end of the follow-up period. This real-world cohort of patients with oHCM treated with mavacamten confirms the significant decrease in LVOTg observed in clinical trials. The initial effect of drug therapy was seen after the first 4 weeks of treatment and was sustained at longer-term follow up. [ABSTRACT FROM AUTHOR]

Background: Full-thickness skin grafts (FTSGs) are a common repair option on the external ear, but there are few large case series examining graft sublocations, dimensions, and outcomes.Objective: We sought to report our experience with FTSGs for repair of postsurgical defects of the external ear.Methods: We conducted a retrospective review of all FTSGs on the ear performed by 2 surgeons (J. C., 2000-2014; B. C. L., 2007-2014) after clearance by Mohs micrographic surgery at a single institution.Results: A total of 1519 FTSGs on the ear were performed between June 2000 and March 2014. The most common sublocations were the superior helix (38.8%), the crura of the antihelix or scapha (18.9%), and the back of ear/back of helix (15.4%). The overall complication rate was 1.6%, and the most common complication was graft failure (1.2%).Limitations: Data were collected retrospectively from a single institution. Follow-up beyond 3 months was limited. A standardized assessment tool for aesthetic outcomes was not performed.Conclusion: By taking advantage of predictable "pincushioning" and combining with local flaps or cartilage grafts, FTSGs can provide more volumetric replacement than previously described. They reliably preserve the height and complex topography of the ear with a low complication rate. [ABSTRACT FROM AUTHOR]

Purpose To determine whether the Spot Vision Screener effectively detects amblyopia risk factors (ARFs) in patients with developmental disability using the 2013 guidelines of the American Association for Pediatric Ophthalmology and Strabismus (AAPOS). Methods Children with developmental disability presenting for complete pediatric ophthalmologic examination were prospectively enrolled between June 2012 and March 2016. The following data were analyzed: presence of ARFs according to the AAPOS guidelines, refraction, eye alignment, and other ocular pathology. Results A total of 100 children (average age, 5.7 years; range, 2.2- 9.2 years) were included. The prevalence of ARFs in this cohort was 38%. The sensitivity of the Spot Vision Screener in detecting amblyopia risk factors was 84%; the specificity, 62%. The positive predictive value was 58%; the negative predictive value, 86%. Conclusions In our study cohort the Spot Vision Screener provided good sensitivity and adequate specificity for a screening examination. Automated screeners may be useful in screening children with developmental disability. [ABSTRACT FROM AUTHOR]

Abstract: PMOS transistor degradation due to negative bias temperature instability (NBTI) has proven to be a significant concern to present CMOS technologies. This is of particular importance for analog applications where the ability to match device characteristics to a high precision is critical. Analog circuits use larger than minimum device dimensions to minimize the effects of process variation, leaving PMOS NBTI as a possible performance limiter. This paper examines the effect of PMOS-NBTI induced mismatch on analog circuits in a 90nm technology. [Copyright &y& Elsevier]