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Fabry disease (FD) is a rare X chromosome‐linked disorder and can be easily misdiagnosed. Here, we report the case of a 69‐year‐old male patient with FD who developed heart failure and showed extremely high pulmonary artery pressure. His initial symptom was recurrent atrial fibrillation. The left and right atrial inner diameters were large, and the ventricular wall was thick. Gene analysis which showed GLA c.215T>C p.Met72Thr mutation and single photon emission computed tomography indicated the diagnosis of FD with coronary microvascular dysfunction. The patient was prescribed anti‐heart failure drugs, including vericiguat. Following the treatment, his heart function and microvascular perfusion significantly improved, which might be due to the beneficial effects of vericiguat. [ABSTRACT FROM AUTHOR]

Aims: A high red blood cell distribution width (RDW) at admission or discharge is associated with a worse prognosis in hospitalized patients with heart failure (HF), and the prognostic value of the in‐hospital change in RDW (∆RDW) remains debatable. Methods and results: We included 5514 patients with critical illness and HF from the MIMIC‐IV database. The ΔRDW was calculated by the RDW at discharge minus that at admission. Clinical outcomes included all‐cause mortality at 90 day, 180 day, and 1 year after discharge. The median age of the patients was 73.91 years, and 46.37% were women. Kaplan–Meier curve and Cox regression analyses were used to examine the association between the ΔRDW and all‐cause mortality at different time points. A multivariable Cox proportional hazard model showed that the ΔRDW (per 1% increase) was independently associated with all‐cause mortality at 90 day, 180 day, and 1 year after adjusting for confounding factors (hazard ratio [HR] = 1.17, 95% confidence interval [CI] = 1.13–1.21, P < 0.001; HR = 1.17, 95% CI = 1.14–1.20, P < 0.001; and HR = 1.18, 95% CI = 1.15–1.20, P < 0.001, respectively). Restricted cubic splines showed a non‐linear relationship between the ΔRDW and the risk of clinical outcomes. High ΔRDW was associated with a high risk of mortality at different time points. A subgroup analysis showed that this positive association remained consistent in pre‐specified subgroups. Conclusions: Our study suggests that an increased RDW during hospitalization is independently associated with short‐ or long‐term all‐cause mortality in critical‐ill patients with HF. [ABSTRACT FROM AUTHOR]

This paper investigates the seismic behavior of a seismic-damaged double-deck viaduct frame pier (DVFP) strengthened with CFRP and enveloped steel, four strengthened DVFP specimens with different degrees of initial damage were tested under quasi-static cyclic loading. Based on the test results, the hysteretic behavior, the stiffness and strength degradation, crack propagation, and failure mechanism were firstly analyzed. Then, the damage indexes of the tested specimens were calculated with different models to evaluate the seismic strengthening performance. Results of this study show that CFRP and enveloped steel strengthening could effectively improve the strength and ductility of pre-damaged DVFPs. The ultimate load, the failure displacement and the displacement ductility of the moderately damaged specimen after being strengthened were found to increase by 120.74%, 35% and 32.33%, respectively. For the severely damaged specimens with CFRP and enveloped steel strengthening, the figures were 105.36%, 25.98% and 31.41%, respectively. The research results can provide reference for the hybrid strengthening application of seismic-damaged DVFP. [ABSTRACT FROM AUTHOR]

We analyzed the differences in knowledge spillover effect between industries by constructing an econometric model. In the model, we measured the relation degree and validity between influencing factors and their influence on knowledge spillover. The result indicates that there is a convergent pattern in the steady state of enterprise knowledge spillover and imitation structure, which formed the screw type advancement of innovation and imitation. Knowledge spillover is influenced by such factors as R&D, trade and traffic condition, labor force mobility, enterprise knowledge absorbency, market mechanism flexibility, time lag of knowledge spillover, and changes of factory site besides trade cost and space-time span. Enterprise knowledge absorbency is proportionate to knowledge spillover. When enterprise knowledge storage quantity disparity is equal to enterprise knowledge absorbency, the knowledge spillover effect is greatest. The time lag of knowledge spillover and the knowledge degeneration rate display inverse ratio with knowledge spillover effect. [ABSTRACT FROM AUTHOR]

Background: Few studies had examined the role of ABO blood groups on CAD in hypertensive patients with different blood pressure (BP) controls. Methods: A total of 2708 patients with primary hypertension (HTN) were consecutively enrolled and underwent coronary angiography (CAG) due to angina-like chest pain. The severity of coronary artery stenosis was assessed by Gensini score (GS). Patients were divided into two groups due to results of CAG: HTN with CAD (n = 2185) and HTN without CAD (n = 523). Poor BP control was defined as systolic BP (SBP) ≥ mean in the study. Multivariable regression analysis was used to determine the potential impact of ABO blood groups on risk of the presence and severity of CAD. Results: Compared to HTN without CAD group, the percentage of A blood group was statistically higher and O blood group was significantly lower in HTN with CAD group. Moreover, percentage of the angiography-proven CAD was higher in A blood group than that in non-A blood group (p < 0.05). After adjusting for confounding factors, A blood group was independently associated with CAD (odds ratio (OR): 1.422; 95% confidence interval (CI): 1.017–1.987; p = 0.039) and GS (β = 0.055, p = 0.046) in patients with poor BP control. Conclusions: A blood group was an independent risk factor for the presence and severity of CAD in hypertensive patients with poor BP control. [ABSTRACT FROM AUTHOR]

Aims: Previous studies report that blood pressure (BP) variability is associated with increased risk of adverse outcomes in patients diagnosed with cardiovascular disease. However, studies have not fully explored this association in patients with heart failure with preserved ejection fraction (HFpEF). This study sought to explore the association between visit-to-visit variability (VVV) of BP and clinical outcomes in patients with HFpEF., Methods and Results: A total of 1988 patients (mean age of 67.73 ± 9.22, 51.7% female) from the Treatment of Preserved Cardiac Function Heart Failure with an Aldosterone Antagonist (TOPCAT) trial were included in this study. BP-VVV was determined by standard deviation (SD) of mean systolic BP (SBP-SD) from six measurements (baseline and months 1, 2, 4, 8, and 12) during the first 12 months after randomization. Mean on-treatment SBP during the first 12 months was 127.77 ± 10.42 mmHg, and the median of SBP-SD was 8.15 mmHg. A total of 192 (9.7%) patients met the primary outcome during the subsequent median follow-up of 35.16 months, including a composite of cardiovascular death, heart failure hospitalization, or aborted cardiac arrest. Multiple Cox regression analysis showed that SBP-SD was independently associated with the increased risk of the primary outcome after adjusting for age, gender, method of BP measurement, treatment, renal function and common co-morbidities, and the mean SBP during the first 12 months [hazard ratio (HR) for fourth vs. first quartile, 1.63; 95% confidence interval (CI), 1.07-2.49; P = 0.024]. Analysis showed that SBP-SD as continuous variable was associated with a 23% increase in the risk of primary outcome (HR 1.23, 95% CI 1.06-1.43; P = 0.006)., Conclusions: The findings of the current study show that high SBP-VVV in patients with HFpEF is associated with an increased risk of adverse outcomes independent of the mean on-treatment SBP., (© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)