Background-Immunoglobulin amyloid light-chain (AL)-related cardiac amyloidosis (CA) has a worse prognosis than either wild-type (ATTRwt) or mutant (ATTRm) transthyretin (TTR) CA. Detailed echocardiographic studies have been performed in AL amyloidosis but not in TTR amyloidosis and might give insight into this difference. We assessed cardiac structure and function and outcome in a large population of patients with CA and compared findings in TTR and AL-related disease. Methods and Results-We analyzed 172 patients with CA (AL amyloidosis, n=80; ATTRm, n=36; ATTRwt, n=56) by standard echocardiography and 2-dimensional speckle-tracking imaging-derived left ventricular (LV) longitudinal (LS), radial, and circumferential strains. Despite a preserved LV ejection fraction (55±12%), LS was severely impaired in CA.Standard measures of LV function and speckle-tracking imaging worsened as wall thickness increased, whereas apical LS was preserved regardless of the pathogenesis of CA and the degree of wall thickening. Compared with ATTRm and AL amyloidosis, ATTRwt was characterized by greater LV wall thickness and lower ejection fraction. LS was more depressed in both ATTRwt and AL amyloidosis (−11±3% and −12±4%, respectively, P=0.54) than in ATTRm (−15±4%, P<0.01 versus AL amyloidosis and ATTRwt). TTR-related causes were favorable predictors of survival, whereas LS and advanced New York Heart Association class were negative predictors. Conclusions-In patients with CA, worsening LV function correlated with increasing wall thickness regardless of pathogenesis.Patients with ATTRwt had a statistically greater wall thickness but lesser mortality than those with AL amyloidosis, despite very similar degrees of LS impairment. This paradox suggests an additional mechanism for LV dysfunction in AL amyloidosis, such as previously demonstrated light-chain toxicity. (Circulation. 2014;129:1840-1849.)

Objectives We sought to test the hypothesis that coronary microvascular function is impaired in subjects with cardiac amyloidosis. Background Effort angina is common in subjects with cardiac amyloidosis even in the absence of epicardial coronary artery disease (CAD). Methods Thirty one subjects were prospectively enrolled in this study including 21 subjects with definite cardiac amyloidosis without epicardial CAD and 10 subjects with hypertensive left ventricular hypertrophy (LVH). All subjects underwent rest and vasodilator stress N-13 ammonia positron emission tomography and 2D echocardiography. Global LV myocardial blood flow (MBF) was quantified at rest and during peak hyperemia, and coronary flow reserve (CFR) was computed (peak stress MBF / rest MBF) adjusting for rest rate pressure product. Results Compared to the LVH group, the amyloid group showed lower rest MBF (0.59 ± 0.15 vs. 0.88 ± 0.23 ml/g/min, P = 0.004), stress MBF (0.85 ± 0.29 vs. 1.85 ± 0.45 vs. ml/min/g, P < 0.0001), CFR (1.19 ± 0.38 vs. 2.23 ± 0.88, P < 0.0001), and higher minimal coronary vascular resistance (111 ± 40 vs. 70 ± 19 mm Hg/mL/g/min, P = 0.004). Of note, almost all amyloid subjects (> 95%) demonstrated significantly reduced peak stress MBF (< 1.3 mL/g/min). In multivariable linear regression analyses, a diagnosis of amyloidosis, increased LV mass and age were the only independent predictors of impaired coronary vasodilator function. Conclusions Coronary microvascular dysfunction is highly prevalent in subjects with cardiac amyloidosis even in the absence of epicardial CAD, and may explain their anginal symptoms. Further study is required to understand whether specific therapy directed at amyloidosis may improve coronary vasomotion in amyloidosis.

BackgroundNumerous digital health interventions have been developed for mental health promotion and intervention, including eating disorders. Efficacy of many interventions has been evaluated, yet knowledge about reasons for dropout and poor adherence is scarce. Most digital health intervention studies lack appropriate research design and methods to investigate individual engagement issues. User engagement and program usability are inextricably linked, making usability studies vital in understanding and improving engagement.ObjectiveThe aim of this study was to explore engagement and corresponding usability issues of the Healthy Body Image Program—a guided online intervention for individuals with body image concerns or eating disorders. The secondary aim was to demonstrate the value of usability research in order to investigate engagement.MethodsWe conducted an iterative usability study based on a mixed-methods approach, combining cognitive and semistructured interviews as well as questionnaires, prior to program launch. Two separate rounds of usability studies were completed, testing a total of 9 potential users. Thematic analysis and descriptive statistics were used to analyze the think-aloud tasks, interviews, and questionnaires.ResultsParticipants were satisfied with the overall usability of the program. The average usability score was 77.5/100 for the first test round and improved to 83.1/100 after applying modifications for the second iteration. The analysis of the qualitative data revealed five central themes: layout, navigation, content, support, and engagement conditions. The first three themes highlight usability aspects of the program, while the latter two highlight engagement issues. An easy-to-use format, clear wording, the nature of guidance, and opportunity for interactivity were important issues related to usability. The coach support, time investment, and severity of users’ symptoms, the program’s features and effectiveness, trust, anonymity, and affordability were relevant to engagement.ConclusionsThis study identified salient usability and engagement features associated with participant motivation to use the Healthy Body Image Program and ultimately helped improve the program prior to its implementation. This research demonstrates that improvements in usability and engagement can be achieved by testing and adjusting intervention design and content prior to program launch. The results are consistent with related research and reinforce the need for further research to identify usage patterns and effective means for reducing dropout. Digital health research should include usability studies prior to efficacy trials to help create more user-friendly programs that have a higher likelihood of “real-world” adoption.