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The impact of tafamidis on myocardial strain in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) have been barely investigated. We aimed to determine tafamidis-induced changes using serial speckle tracking echocardiography and to identify imaging parameters for specific therapy monitoring.ATTR-CM patients underwent serial TTE with two-dimensional (2 D) speckle tracking imaging. Patients receiving tafamidis free acid 61 mg (Treatment with tafamidis free acid 61 mg in ATTR-CM patients delays the deterioration of LA and LV longitudinal function, resulting in significant clinical benefits compared with natural history. Serial TTE with 2 D speckle tracking imaging may be appropriate for disease-specific therapy monitoring.

We aimed to identify cardiopulmonary long-term effects after severe COVID-19 disease as well as predictors of Long-COVID in a prospective registry. A total of 150 consecutive, hospitalized patients (February 2020 and April 2021) were included six months post hospital discharge for a clinical follow-up. Among them, 49% experienced fatigue, 38% exertional dyspnea and 75% fulfilled criteria for Long-COVID. Echocardiography detected reduced global longitudinal strain (GLS) in 11% and diastolic dysfunction in 4%. Magnetic resonance imaging revealed traces of pericardial effusion in 18% and signs of former pericarditis or myocarditis in 4%. Pulmonary function was impaired in 11%. Chest computed tomography identified post-infectious residues in 22%. Whereas fatigue did not correlate with cardiopulmonary abnormalities, exertional dyspnea was associated with impaired pulmonary function (OR 3.6 [95% CI: 1.2–11], p = 0.026), reduced GLS (OR 5.2 [95% CI: 1.6–16.7], p = 0.003) and/or left ventricular diastolic dysfunction (OR 4.2 [95% CI: 1.03–17], p = 0.04). Predictors of Long-COVID included length of in-hospital stay (OR: 1.15 [95% CI: 1.05–1.26], p = 0.004), admission to intensive care unit (OR cannot be computed, p = 0.001) and higher NT-proBNP (OR: 1.5 [95% CI: 1.05–2.14], p = 0.026). Even 6 months after discharge, a majority fulfilled criteria for Long-COVID. While no associations between fatigue and cardiopulmonary abnormalities were found, exertional dyspnea correlated with impaired pulmonary function, reduced GLS and/or diastolic dysfunction.

Aims Tafamidis improves outcomes in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). However, it is not yet known whether tafamidis affects cardiac amyloid deposition and structural changes in the myocardium. We aimed to determine disease-modifying effects on myocardial amyloid progression and to identify imaging parameters that could be applied for specific therapy monitoring. Methods and results ATTR-CM patients underwent serial cardiac magnetic resonance (CMR) imaging using T1 mapping techniques to derive extracellular volume (ECV). Patients receiving tafamidis 61 mg (n = 35) or 20 mg (n = 15) once daily showed stable measurements at follow-up (FU) {61 mg: 9.0 [interquartile range (IQR) 7.0–11.0] months, 20 mg: 11.0 (IQR 8.0–18.0) months} in left ventricular (LV) ejection fraction (LVEF; 61 mg: 47.6% vs. 47.5%, P = 0.935; 20 mg: 52.4% vs. 52.1%, P = 0.930), LV mass index (LVMI; 61 mg: 110.2 vs. 106.2 g/m2, P = 0.304; 20 mg: 114.5 vs. 115.4 g/m2, P = 0.900), and ECV (61 mg: 47.5% vs. 47.7%, P = 0.861; 20 mg: 56.7% vs. 57.5%, P = 0.759), whereas treatment-naïve ATTR-CM patients (n = 19) had clear signs of disease progression at the end of the observation period [12.0 (IQR 10.0–21.0) months; LVEF: 53.3% vs. 45.7%, P = 0.031; LVMI: 98.9 vs. 106.9 g/m2, P = 0.027; ECV: 49.3% vs. 54.6%, P = 0.023]. Between-group comparison at FU revealed positive effects in tafamidis 61 mg-treated compared to treatment-naïve patients (LVEF: P = 0.035, LVMI: P = 0.036, ECV: P = 0.030), while those treated with 20 mg showed no difference in the above LV measurements when compared with treatment-naïve (P = 0.120, P = 0.287, P = 0.158). However, both treatment groups showed clinically beneficial effects compared to the natural course [61 mg, 6-min walk distance (6-MWD): P = 0.005, N-terminal prohormone of brain natriuretic peptide (NT-proBNP): P = 0.002; 20 mg, 6-MWD: P = 0.023, NT-proBNP: P = 0.003]. Conclusion Tafamidis delays myocardial amyloid progression in ATTR-CM patients, resulting in structural, functional, and clinical benefits compared to the natural course. Serial CMR including measurement of ECV may be appropriate for disease-specific therapy monitoring.

Patients with hyperlipidemia are at high risk for developing a fatty liver. The fatty liver index (FLI) is a noninvasive and well-established method for the estimation of a fatty liver. However, little is known about the metabolic characterization of nondiabetic treated patients with hyperlipidemia who have different risk levels for a fatty liver.In this study, 74 nondiabetic patients with hyperlipidemia were divided into 3 groups according to their fatty liver index. A comparison of metabolic characteristics was done. These characteristics included intima media thickness (IMT) and nutritional habits, which were further divided into FLI subgroups with low, intermediate, and high risk for a fatty liver.Patients with hyperlipidemia, with a high risk for a fatty liver (FLI ≥ 60), had subclinical elevations in parameters of carbohydrate metabolism (insulin, fasting plasma glucose, C-peptide) including a higher insulin resistance (quantitative insulin sensitivity check index, QUICKI) compared to lower FLI groups. These patients also presented a higher risk for a metabolic syndrome (p = 0.018), as well as an adverse lipid profile (e.g., high-density lipoprotein [HDL] cholesterol, triglycerides [TG]-HDL ratio). FLI group 3 was characterized by significantly lower levels of omega-3 fatty acids (p = 0.048).The fatty liver index relates to diabetes-specific parameters and an adverse lipid profile and is an appropriate index for risk evaluation of metabolic syndrome.

Objective: In the long term, diabetes mellitus is potentially associated with the occurrence of microvascular damage. This study sought to assess whether a history of prior gestational diabetes mellitus is associated with long-term effects on the women’s microcirculation. Methods: Within the scope of a long-term follow-up of the ‘Viennese Post-Gestational Diabetes Project’, women with prior gestational diabetes mellitus as well as women with previous pregnancy but with no history of gestational diabetes mellitus (controls) were enrolled in this cross-sectional study. Microvascular function was assessed by post-occlusive reactive hyperaemia using laser Doppler fluxmetry. Baseline perfusion, biological zero, peak perfusion, time to peak and recovery time were recorded and compared between both groups. Results: Microvascular function was assessed in 55 women with prior gestational diabetes mellitus (46.1 ± 4.6 years) and 32 women with previous pregnancy but without prior gestational diabetes mellitus (42.9 ± 5.3 years). The mean period of time between delivery and the assessment of microvascular function was 16.2 ± 5.2 years in women with prior gestational diabetes mellitus group and 14.2 ± 4.8 years in controls. Regarding microvascular function, baseline perfusion, biological zero, peak perfusion, time to peak and recovery time did not differ between women with prior gestational diabetes mellitus and controls (all p > 0.05). Conclusion: In the long term, microvascular function appears not to be impaired in women with prior gestational diabetes mellitus.

Background— In incipient Raynaud phenomenon, nailfold capillaroscopy and autoantibody tests are obtained to screen for an emerging connective tissue disease. Whether the presence of abnormal nailfold capillaries and autoantibodies are related to mortality in patients with incipient Raynaud phenomenon is not known. Methods and Results— In 2958 consecutive patients (78% women, median age 45 years) with incipient Raynaud phenomenon without previously known connective tissue disease, nailfold capillaroscopy and laboratory tests for antinuclear antibodies (ANA) and ANA subsets were obtained at initial presentation. During a median follow-up period of 9.3 years, 227 women (9.9% of female patients) and 129 men (20% of male patients) with Raynaud phenomenon died. In comparison with a demographically matched standard population, survival was poorer in patients with Raynaud phenomenon (log-rank test P P Conclusions— In Raynaud phenomenon, male sex, age, and serum creatinine are related to mortality. Abnormal nailfold capillaries and autoantibodies are associated with an increase in all-cause mortality in female patients, but not in male patients with Raynaud phenomenon.

Objective To assess the relationship of age and body mass index (BMI) to skin temperature and perfusion in patients with primary Raynaud's phenomenon (RP) compared with controls. Methods Patients with RP as well as age- and sex-matched controls underwent external cold provocation by exposure to 20°C water for 1 minute. Before and after cold provocation, skin temperature and skin perfusion were measured. Results Twenty-six patients with RP (20 women and 6 men; median age 41.9 years) and 22 controls (17 women and 5 men; median age 42.9 years) were studied. In RP patients, cold exposure led to a median change in skin temperature of −7% (interquartile range [IQR] −13.1, −4.1) and to a median change in skin perfusion of −26.4% (IQR −36.2, 2.9). In controls, skin temperature changed by −15.7% (IQR −18.3, −11.6) and skin perfusion by −33% (IQR −53.3, −1.1) upon cold exposure. In patients with RP, age and BMI were related to skin temperature (for age, r = 0.683, P < 0.0001; for BMI r = 0.657, P < 0.0001) and skin perfusion (for age, r = 0.595, P = 0.002; for BMI, r = 0.653, P < 0.0001), while no association was found in controls. The cold-induced decrease in skin temperature was inversely related to age (r = −0.518, P = 0.003) and BMI (r = −0.662, P < 0.0001) in patients with RP; correlations were not observed in controls. The cold-induced change in skin perfusion was not related to age or BMI in either group. Conclusion The cold-induced decrease in skin temperature is related to age and BMI in patients with RP but not in controls. Further studies are needed to clarify the pathophysiology of digital ischemia in primary RP.

We thank Dr Boulon and Dr Constans for their interest in our study reporting the relation of abnormal nailfold capillaries and autoantibodies to mortality in patients with Raynaud phenomenon.1 As correctly highlighted by Dr Boulon and Dr Constans, a complete microscopic scan of each nailfold is essential to not missing single nailfold capillary aberrations. In our study, the entire nailfold capillary fields of fingers 2 to 5 of both hands were completely scanned. In contrast to Dr Boulon and Dr Constans, however, we used a ×250 to ×300 magnification, which complies with previously published studies on nailfold capillaroscopy (×200 to ×600 magnification).2,3 The advantage of a greater magnification during nailfold …

Objective. The aim of this study was to evaluate sex specific differences of metabolic and clinical characteristics of treated hyperlipidemic men and women (HL-men and HL-women).Methods. In this study vascular and metabolic characteristics of 35 HL-women and 64 HL-men were assessed. In addition a sex specific analysis of metabolic and nutritional habits of HL-patients with prediabetes (HL-IGR) was done.Results. HL-women were older and had favourable concentrations of high density lipoprotein cholesterol (HDL-cholesterol), triglycerides (TG), and triglyceride/HDL-cholesterol ratio (TG/HDL-ratio) but were also shown to have higher concentrations of lipoprotein-a compared to HL-men. HL-men were characterized as having higher levels of liver-specific parameters and body weight as well as being more physically active compared to HL-women. Brain natriuretic peptide (pro-BNP) was higher in HL-women than HL-men, while no differences in metabolic syndrome and glycemic parameters were shown. HL-IGR-women were also older and still had a better profile of sex specific lipid parameters, as well as a lower body weight compared to HL-IGR-men. No differences were seen in vascular parameters such as the intima media thickness (IMT).Conclusion. HL-women were older and had overall more favourable concentrations of lipid parameters and liver enzymes but did not differ regarding vascular morphology and insulin sensitivity compared to HL-men of comparable body mass index (BMI).

Background Impaired glucose regulation (IGR) and hyperlipidemia (HL) are associated with an increased risk of developing a cardiovascular disease. Hyperlipidemic patients were shown to bear a greater risk for an increased intima media thickness (IMT). However little is known about differences between treated hyperlipidemic patients (HL) with normal (NGR) or impaired (IGR) glucose regulation. Methods We performed a cross-sectional study, involving 96 non-diabetic HL patients with IGR (fasting plasma glucose of ≥ 100 mg/dl and

Purpose: Vascular ultrasound (US) allows the analysis of vascular strain by speckle-tracking. This study sought to assess the extent to which vas cular strain varies between different segments of the arterial tree. Furthermore, this study aimed to investigate the reproducibility of vascular strain determination as well as of the components that contribute to the variance of vascular strain measurements in different vascular beds. Materials and Methods: Speckle-tracking was used to determine the vascular strain of the abdominal aorta (AA), the common carotid artery (CCA), the common femoral (CFA) and the popliteal artery (PA) of healthy adults. Intra- and interday reproducibility and the components of variance of vascular strain of the respective arteries were determined. Results: A total of 589 US clips obtained in 10 healthy adults (7 males, 28.3 ± 3.2 years) were analyzable. Vascular strain was 7.2 ± 3.0 % in the AA, 5.7 ± 2.1 % in the CCA, 2.1 ± 1.1 % in the CFA and 1.9 ± 1.1 % in the PA. The intraday coefficients of variation of vascular strain were 6.2 % (AA), 3.9 % (CCA), 3.3 % (CFA) and 6.1 % (PA), and the interday coefficients of variation were 5.9 % (AA), 8.4 % (CCA), 10 % (CFA) and 4.6 % (PA). The variance of vascular strain mainly depended on the investigated vessel and subject. Individual DUS clips, the day of examination and the (right/left) body side (in paired arteries) had no impact on the variance of vascular strain. Conclusion: Vascular strain substantially varies between different sites of the arterial tree. Speckle-tracking by DUS allows the reliable determination of vascular strain at different arterial sites.

To assess the relationship of age and body mass index (BMI) to skin temperature and perfusion in patients with primary Raynaud's phenomenon (RP) compared with controls.Patients with RP as well as age- and sex-matched controls underwent external cold provocation by exposure to 20 °C water for 1 minute. Before and after cold provocation, skin temperature and skin perfusion were measured.Twenty-six patients with RP (20 women and 6 men; median age 41.9 years) and 22 controls (17 women and 5 men; median age 42.9 years) were studied. In RP patients, cold exposure led to a median change in skin temperature of -7% (interquartile range [IQR] -13.1, -4.1) and to a median change in skin perfusion of -26.4% (IQR -36.2, 2.9). In controls, skin temperature changed by -15.7% (IQR -18.3, -11.6) and skin perfusion by -33% (IQR -53.3, -1.1) upon cold exposure. In patients with RP, age and BMI were related to skin temperature (for age, r = 0.683, P0.0001; for BMI r = 0.657, P0.0001) and skin perfusion (for age, r = 0.595, P = 0.002; for BMI, r = 0.653, P0.0001), while no association was found in controls. The cold-induced decrease in skin temperature was inversely related to age (r = -0.518, P = 0.003) and BMI (r = -0.662, P0.0001) in patients with RP; correlations were not observed in controls. The cold-induced change in skin perfusion was not related to age or BMI in either group.The cold-induced decrease in skin temperature is related to age and BMI in patients with RP but not in controls. Further studies are needed to clarify the pathophysiology of digital ischemia in primary RP.

Background Microcirculatory function can be assessed by postocclusive reactive hyperaemia (PORH) using laser Doppler fluxmetry. Previous studies have shown that PORH reveals microvascular damage at an early stage. In particular, at younger ages, PORH might depend on age and gender. To implement PORH into a larger scale of clinical studies, one has to be aware of the influence of age and gender on microcirculation. The aim of this study was to assess the impact of age and gender on microcirculatory function during adolescence. Materials and methods Within the scope of an epidemiological project, 896 children and adolescents underwent assessment of PORH by laser Doppler fluxmetry. Microcirculatory parameters during PORH (baseline perfusion, biological zero, peak perfusion, time to peak perfusion and recovery time) were analysed in relation to age (by tertiles) and gender. Results Baseline perfusion, biological zero and peak perfusion were lower in children/adolescents in the upper age tertile (12·3–18·1 years) than in the middle (9·8–12·2 years) and lower (4·3–9·7 years) age tertiles (P