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Aims The aim of our study is to assess the ability of left atrial (LA) strain values to improve left ventricular and diastolic pressure (LVEDP) non-invasive estimation as compared with traditional echocardiographic indexes in the acute phase of Takotsubo syndrome (TTS) and to predict adverse in-hospital outcomes in this population. Methods and results Consecutive TTS patients were prospectively enrolled. Left ventricular and diastolic pressure was measured at the time of catheterization. Transthoracic echocardiography was performed within 48 h from hospital admission. In-hospital complications (acute heart failure, death from any cause, and life-threatening arrhythmias) were collected. A total of 62 patients were analysed (72.2 ± 10.1 years, female 80%) and in-hospital complications occurred in 25 (40.3%). Left ventricular and diastolic pressure mean value was 24.53 ± 7.92 mmHg. Left atrial reservoir and pump strain values presented higher correlation with LVEDP (r −0.859, P < 0.001 and r −0.848, P < 0.001, respectively) in comparison with E/e ′ ratio, left atrial volume index (LAVi), and tricuspid regurgitation (TR) peak velocity. In addition, at receiver-operating characteristic curve analysis, LA reservoir and pump strain resulted to be better predictors of LVEDP above the mean of our population [0.909 (95% CI 0.818–0.999, P < 0.001) and 0.889 (95% CI 0.789–0.988, P < 0.001)], respectively] as compared with E/e′ ratio, LAVi, and TR peak velocity. Finally, LA reservoir strain resulted to be an independent predictor of worse in-hospital outcomes, together with LVEDP and left ventricular ejection fraction (all P < 0.001). Conclusion In our study, lower LA reservoir and pump strain values were better predictors of LVEDP as compared with traditional echocardiographic indexes in the acute phase of TTS syndrome. Moreover, LA reservoir strain was an independent predictor of adverse in-hospital outcomes.

Introduction: Left ventricular (LV) hypertrophy is the main feature of cardiac involvement in Anderson-Fabry disease (FD), but the right ventricle (RV) is also frequently affected. Previous studies failed to demonstrate an independent association between conventional parameters of RV performance and outcomes in FD. Nevertheless, if RV free wall strain (RV-FWS), assessed by 2D speckle tracking analysis, may provide a better prognostication is currently unknown. Methods: We retrospectively evaluated the association between RV-FWS and the occurrence of cardiovascular events in a cohort of 56 patients with FD. The study endpoint comprises cardiovascular mortality, severe heart failure symptoms, new-onset atrial fibrillation and major arrhythmias requiring device implantation. Results: Reduced RV-FWS, defined by values lower than 23%, was found in 25 (45%) patients. During a median follow-up of 47 months, 16 (29%) patients met the study endpoint. A ROC-curve analysis confirmed the threshold of reduced RV-FWS (

Little is known about prevalence and predictors of myocardial infarction with non-obstructive coronary arteries (MINOCA) in Fabry disease (FD) and hypertrophic cardiomyopathy (HCM). We assessed and compared the prevalence and predictors of MINOCA in a large cohort of HCM and FD patients.In this multicenter, retrospective study we enrolled 2870 adult patients with HCM and 267 with FD. The only exclusion criterion was documented obstructive coronary artery disease. MINOCA was defined according to guidelines. For each patient we collected clinical, ECG and echocardiographic data recorded at initial evaluation.Overall, 36 patients had MINOCA during a follow-up period of 4.5 ± 11.2 years. MINOCA occurred in 16 patients with HCM (0.5%) and 20 patients with FD (7.5%; p lt; 0.001). The difference between the 2 groups was highly significant, also after adjustment for the main clinical, ECG and echocardiographic variables (OR 6.12; 95%CI 2.80-13.3; p lt; 0.001). In the FD population MINOCA occurred in 17 out of 96 patients with left ventricle hypertrophy (LVH, 17.7%) and in 3 out of 171 patients without LVH (1.7%; OR 12.0; 95%CI 3.43-42.3; p lt; 0.001). At multivariable analysis, voltage criteria for LVH at ECG (OR 7.3; 95%CI 1.93-27.7; p = 0.003) and maximal LV wall thickness at echocardiography (OR 1.15; 95%CI 1.05-1.27; p = 0.002) maintained an independent association with MINOCA. No major significant differences were found in clinical, ECG and echocardiographic findings between HCM patients with or without MINOCA.MINOCA was rare in HCM patients, and 6-fold more frequent in FD patients. MINOCA may be considered a red flag for FD and aid in the differential diagnosis from HCM.

Few data are available on the prevalence of right ventricle (RV) systolic dysfunction, assessed including RV strain, and RV to pulmonary artery (PA) coupling in patients with aortic stenosis (AS) submitted to TAVI and the early effect of the procedure. We performed standard and speckle tracking echocardiography in 80 patients with severe AS the day before TAVI and within 48 h after TAVI. In all patients we measured TAPSE/PASP (cut-off for RV-PA uncoupling 0.31) and in 60/80 we were able to analyze RV global longitudinal strain (RV-GLS) and RV free wall strain (RV-FWS). RVFAC and TAPSE were impaired in 8.3% while RV-GLS and RV-FWS in 45% and 33.3% respectively before TAVI. TAPSE/PASP 0.31 was documented in 7/80 patients (8.7%) before TAVI. These subjects differed from patients with TAPSE/PASP ≥ 0.31 for: enlarged left ventricular (LV) end-diastolic and end-systolic volumes (p 0.001), worst LV ejection fraction (p 0.001) and RVFAC (p 0.001), worst RV-GLS and RV-FWS (p = 0.01 and p = 0.03) and bigger right atrium (RA) area (p 0.001). After TAVI, RV systolic function did not improve while PASP significantly decreased (p = 0.005) driving the improvement of TAPSE/PASP (p = 0.01). Patients with TAPSE/PASP improvement (51%) differed from the others for worst pre-TAVI diastolic function (E/e' p = 0.045), RVFAC (p = 0.042), RV-GLS (p = 0.049) and RA area (p = 0.02). RV-GLS unveils RV systolic dysfunction in as much as 45% of patients with AS vs only 8.3% revealed by conventional echocardiography. RV systolic function does not significantly improve early after TAVI while RV-PA coupling does. Patients with lower TAPSE/PASP at baseline have worst LV and RV systolic function as well as larger RA. Patients who improve TAPSE/PASP after TAVI are those with worst diastolic function, RV systolic function and larger RA at baseline.

Symptoms-based detection of SARS-CoV-2 infection is not a substitute for precise diagnostic tests but can provide insight into the likely level of infection in a given population. This study uses symptoms data collected in the Global COVID-19 Trends and Impact Surveys (UMD Global CTIS), and data on variants sequencing from GISAID. This work, conducted in January of 2022 during the emergence of the Omicron variant (subvariant BA.1), aims to improve the quality of infection detection from the available symptoms and to use the resulting estimates of infection levels to assess the changes in vaccine efficacy during a change of dominant variant; from the Delta dominant to the Omicron dominant period. Our approach produced a new symptoms-based classifier, Random Forest, that was compared to a ground-truth subset of cases with known diagnostic test status. This classifier was compared with other competing classifiers and shown to exhibit an increased performance with respect to the ground-truth data. Using the Random Forest classifier, and knowing the vaccination status of the subjects, we then proceeded to analyse the evolution of vaccine efficacy towards infection during different periods, geographies and dominant variants. In South Africa, where the first significant wave of Omicron occurred, a significant reduction of vaccine efficacy is observed from August-September 2021 to December 2021. For instance, the efficacy drops from 0.81 to 0.30 for those vaccinated with 2 doses (of Pfizer/BioNTech), and from 0.51 to 0.09 for those vaccinated with one dose (of Pfizer/BioNTech or Johnson&Johnson). We also extended the study to other countries in which Omicron has been detected, comparing the situation in October 2021 (before Omicron) with that of December 2021. While the reduction measured is smaller than in South Africa, we still found, for instance, an average drop in vaccine efficacy from 0.53 to 0.45 among those vaccinated with two doses. Moreover, we found a significant negative (Pearson) correlation of around −0.6 between the measured prevalence of Omicron in several countries and the vaccine efficacy in those same countries. This prediction, in January of 2022, of the decreased vaccine efficacy towards Omicron is in line with the subsequent increase of Omicron infections in the first half of 2022. IMDEA Networks, Comunidad de Madrid, Spain, the European Union through the European Regional Development Fund (ERDF), the Ministry of Sciences and Innovation, Spain, and individual donations to the CoronaSurveys Project TRUE pub

The hypertrophic cardiomyopathy phenotype encompasses a heterogeneous spectrum of genetic and acquired diseases characterized by the presence of left ventricular hypertrophy in the absence of abnormal cardiac loading conditions. This “umbrella diagnosis” includes the “classic” hypertrophic cardiomyopathy (HCM), due to sarcomere protein gene mutations, and its phenocopies caused by intra‐ or extracellular deposits, such as Fabry disease (FD) and cardiac amyloidosis (CA). All these conditions share a wide phenotypic variability which results from the combination of genetic and environmental factors and whose pathogenic mediators are poorly understood so far. Accumulating evidence suggests that inflammation plays a critical role in a broad spectrum of cardiovascular conditions, including cardiomyopathies. Indeed, inflammation can trigger molecular pathways which contribute to cardiomyocyte hypertrophy and dysfunction, extracellular matrix accumulation, and microvascular dysfunction. Growing evidence suggests that systemic inflammation is a possible key pathophysiologic process potentially involved in the pathogenesis of cardiac disease progression, influencing the severity of the phenotype and clinical outcome, including heart failure. In this review, we summarize current knowledge regarding the prevalence, clinical significance, and potential therapeutic implications of inflammation in HCM and two of its most important phenocopies, FD and CA.

Background Anderson Fabry disease (AFD) is a rare X-linked lysosomal storage disorder caused by deficient activity of α-galactosidase A bringing to intracellular accumulation of globotriaosylceramide (Gb3) in affected tissues, including heart. Progressive cardiac involvement has shown to cause electrocardiographic modifications in these patients. Treatment with enzyme replacement therapy (ERT) or chaperone therapy has demonstrated to decrease Gb3 levels in the heart, but little is known about its influence on ECG evolution. Purpose to gain new insights about ECG evolution in AFD patients on and off specific disease therapy and to assess its potential role in the diagnosis and the follow-up of these patients. Methods we analysed the ECG evolution of a multicentre study cohort of 170 patients with a diagnosis of AFD (64 males 38%, median age 46±15 years) for a median follow-up of 64±48 months, dividing them into patients off (group A, N=63) and on (group B, N= 107) specific therapy. Results the two groups did not differ as regard age at baseline (47±14 vs 44±12 years; p=0,171) but patients off specific disease therapy (group A) showed lower prevalence of male sex [13(21%) vs 51(48%); p= Conclusion We observed ECG progression despite specific disease therapy in 29% of patients of group B showing a potential role of ECG as a marker of cardiac specific response to therapy in these patients. Differently we detected ECG progression in 14% of patients off specific therapy, which is consistent with their less advanced cardiac involvement (lower prevalence of male sex, classic phenotype and lower maximum wall thickness), suggesting that ECG could be an important tool to detect an initial cardiac involvement in these patients even in absence of hypertrophy. The absence of left bundle branch blocks in the two groups compared with a significant prevalence of complete and incomplete right conduction delay could suggest a prevalent involvement of the right bundle branch in these patients and could represent a red flag for the diagnosis of AFD.

Left ventricular (LV) hypertrophy is the main feature of Fabry cardiomyopathy (FC), but the right ventricle (RV) is also frequently affected. Previous studies failed to demonstrate an independent association between RV hypertrophy or conventional parameters of RV function and the occurrence of adverse outcomes in patients with FC. Nevertheless, if RV free wall strain (RV-FWS), assessed by 2D speckle tracking analysis, may provide a better prognostication is currently unexplored. We retrospectively evaluated the association between RV-FWS and the occurrence of cardiovascular events in a cohort of 28 patients with FC. The study endpoint comprises the occurrence of cardiovascular mortality, severe heart failure symptoms, new-onset atrial fibrillation, bradyarrhythmias requiring pacemaker implantation and sustained ventricular tachyarrhythmias. The median value of RV-FWS was 21.4% (interquartile range:17.1-23.9%) and a total of 18 (64%) patients showed reduced RV-FWS ( In Conclusion, impaired RV-FWS was not independently associated with the occurrence of cardiovascular events in FC, confirming previous observations that prognosis is mainly driven by the severity of LV cardiomyopathy.

Background The clinical impact of valvular heart disease (VHD) in adult congenital heart disease (ACHD) patients is unascertained. Aim of our study was to assess the prevalence and clinical impact of severe VHD (S-VHD) in a real-world contemporary cohort of ACHD patients. Methods Patients followed-up at our ACHD Outpatient Clinic were enrolled. Clinical characteristics and echocardiographic data were prospectively entered into a digitalized medical records database. VHD at the first access was evaluated and graded according to VHD guidelines. Clinical data at follow-up were collected. Results A total of 390 patients were included and S-VHD was present in 101 (25.9%) patients. Over a median follow-up time of 26 months (IQR: 12-48), the primary composite endpoint occurred in 76 patients (19.5%). The cumulative primary endpoint-free survival was significantly lower in patients with S-VHD vs patients with non-severe VHD (Log rank p 45 mmHg. Conclusion In ACHD patients, the presence of S-VHD is independently associated with the occurrence of cardiovascular mortality and hospitalization. The prognostic value of S-VHD is incremental above other established prognostic markers.

Background Takotsubo syndrome (TTS) is associated with a non-negligible risk of in-hospital complications. Elevated left ventricular filling pressures (LVFP) showed to predict adverse outcomes in this population. Recently, LA reservoir and LA pump strain, demonstrated a close correlation with increased LVFP in unselected patients. The aim of our study is to assess the ability of LA strain analysis to improve non-invasive estimation of LVFP and to predict IH complications in TTS patients. Methods We prospectively enrolled patients with confirmed TTS diagnosis. LVEDP was assessed invasively at the time of catheterization. Transthoracic echocardiography was performed within 48 hours from hospital admission. In-hospital complications were collected, including occurrence of acute heart failure, death from any cause and life-threatening arrhythmias. Results A total of 62 patients were analysed (72.2±10.1 years, female 80%). In-hospital complications occurred in 25 (40.3%). Patients who experienced IH complications had higher LVEDP and lower LVEF, LA reservoir strain and LA pump strain values compared to patients without IH complications (all p24.5 mmHg in comparison with E/e’, LAVi and tricuspid regurgitation (TR) peak velocity. Moreover, the incorporation of LA strain values in a multivariable model including E/e’ ratio, LAVi and TR peak velocity to predict a LVEDP>24.5 mmHg led to a significant incremental predictive value (p=0.002). Conclusion In patients with TTS, lower LA reservoir and pump strain values correlate with increased LVEDP and improve non-invasive estimation of LVFP. LA strain analysis may be an easy tool to individuate subjects at higher risk of in-hospital complications.

Background Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder that have gained attention due to the availability of therapeutic options. Disease specific therapy (DST), either by enzyme replacement therapy or oral pharmacological chaperone, is the mainstay for AFD treatment. Although its widespread use, few data are available on the electrocardiographic variations associated with DST. Purpose To evaluate ECG findings and variations in AFD according to time duration of DST, comparing patients under long-term therapy with naïve patients starting therapy during follow-up. Methods One-hundred-seventy-nine AFD patients, ≥18 years old, with 2 readable ECGs, were recruited in the present multicentre study cohort. Two patients were excluded due to pacemaker (PM) implantation. Only patients on DST (n=107) were considered for final cohort and divided into 2 groups according to therapy duration: Group A (n=42) included patients treated for ≥12 months at the time of first evaluation, whereas Group B patients (n=65) started therapy during follow-up. Results Group A and Group B had not significant difference in terms of age at presentation (48[39-60] vs 48[36-56]years; p=0.856) and maximal wall thickness (13[11-15] vs 13[11-18]mm; p=0.090) whereas they differed for male prevalence (61% vs 38%; p=0.029) and classic phenotype (86% vs 29%; p During the follow-up (57[60-28] months for Group A vs 70[37-85] months for Group B; p=0.152), both groups developed electrocardiographic alterations (38% vs 23%; p=0.127). Specifically, in GroupA, 4 (10%) patients presented AF, 1 (2%) AVB, 7 (17%) complete or incomplete RBBB, 4 (10%) LAFB, 1 (2%) LVH and 8 (19%) repolarization abnormalities. In Group B, 2 (3%) developed AF, 1 (2%) AVB, 7 (11%) complete or incomplete RBBB, 2(3%) LVH and 11(17%) repolarization abnormalities; none developed LAFB. Conclusions In this AFD cohort, both patients on chronic DST (Group A) and patients who started treatment during follow-up (Group B) developed ECG alterations. Treatment status didn't affect considerably the developing of ECG abnormalities and DST did not prevent ECG changes. ECG alterations during the follow-up were more frequent in Group A (38% vs 23%), mainly composed by classic phenotype and male patients, thus supporting a prompt start of therapy at an early stage.

Multiple COVID-19 diagnosis methods based on information collected from patients have been proposed during the global pandemic crisis, with the aim of providing medical staff with quick diagnosis tools to efficiently plan and manage the limited healthcare resources. In general, these methods have been developed to detect COVID-19 positive cases from a particular combination of reported symptoms, and have been evaluated using datasets extracted from different studies with different characteristics. On the other hand, the University of Maryland, in partnership with Facebook, launched the Global COVID-19 Trends and Impact Survey (UMD-CTIS), the largest health surveillance tool to date that has collected information from 114 countries/territories since April 2020. This survey captured various individual features including gender, age groups, self-reported symptoms, isolation measures, and mental health status, among others. In this paper, we compare the performance of different proposed COVID-19 diagnosis methods using the information collected by UMD-CTIS, for the years 2020 and 2021, in five countries: Brazil, Canada, Germany, Japan, and South Africa. The evaluation of these methods with homogeneous data across countries and years provides a solid and consistent comparison among them.

2D speckle tracking echocardiography (2DSTE) is superior to standard echocardiography in the assessment of subtle right ventricle (RV) systolic dysfunction. In this study we aimed to: 1) test the hypothesis that 2DSTE may unveil subtle RV systolic dysfunction in patients with Fabry disease; 2) investigate whether the physiologic difference between the 3-segment (RV-FWS) and the 6-segment (RV-GLS) RV strain (∆RV strain) is preserved in Fabry patients.Standard echocardiography and 2DSTE were performed in 49 Fabry patients and 49 age- and sex-matched healthy controls. Fabry patients were divided in two groups according to the presence/absence of left ventricular hypertrophy (LVH+: left ventricular wall thickness 12 mm, 49% of total Fabry patients). RV systolic function assessed by standard echocardiography was normal in the majority of Fabry patients (92%) while RV-GLS and RV-FWS were impaired in about 40%. RV-GLS and RV-FWS were significantly worse in patients LVH+ vs LVH- and vs controls (RV-GLS: LVH+ vs LVH-: -18.4 ± -4.3% vs -23.8 ± -3.1% p0.001; LVH+ vs controls: -18.4 ± -4.3% vs -23.9 ± -2.8% p0.001; RV-FWS: LVH+ vs LVH-: -21.8 ± -5.3% vs -26.7 ± -3.8% p = 0.002, LVH+ vs controls -21.8 ± -5.3% vs -26.8 ± -3.9% p0.001). No difference was found between LVH- patients and controls in both RV-GLS (p = 0.65) and RV-FWS (p = 0.79). ∆RV strain was similar among the groups.In Fabry cardiomyopathy impaired RV-GLS and RV-FWS is a common finding, while RV strain is preserved in Fabry patients without overt cardiac involvement. The physiologic difference between RV-FWS and RV-GLS is maintained in Fabry patients, regardless of the presence of cardiomyopathy.

Objectives This study aimed to compare, in a cohort of critically ill children with biventricular anatomy and no cardiovascular shunt, cardiac output (CO) and cardiac index (CI) assessed by echocardiography and a continuous pulse-contour method, MostCareUP, to measure the differences between these techniques (biasCO and biasCI), and their association with clinical variables. Design Retrospective study. Setting Tertiary pediatric cardiac intensive care unit. Participants Children admitted to the pediatric cardiac intensive care unit who underwent echocardiography with CO measurement. Interventions None. Measurements and Main Results Thirty-five patients were included. BiasCO was –0.02 (0.26) L/min (percentage error 36%). BiasCI was 0.07 (0.34) L/min/m2 (percentage error 18%). Biases and percentage errors were higher in 24 nonsupervised echocardiographies. A negative biasCO (overestimation by MostCareUP) was associated with post-surgical status (v cardiomyopathy), higher systolic arterial pressure, and spontaneous breathing (v intubation). When only absolute values were considered, biasCONONEG correlated with age, weight, arterial pressure, and heart rate, whereas biasCINONEG was associated with a femoral arterial cannula, no use of inotropes, and the absence of mechanical ventilation. After adjustment, biasCONONEG remained independently associated with patients’ body weight(p = 0.0001). BiasCINONEG showed a nonlinear relationship with weight below 20 kg and above 40 kg. Conclusions Children with extreme low or high weights, those who are extubated, and those with a femoral cannula carry the highest bias. When younger patients are considered, CI should be evaluated instead of CO, because biases are better highlighted by indexing data on body surface area. In children, both echocardiography and MostCareUP may be responsible of inaccurate CO/CI assessment.

Aims To perform a comparative analysis of right ventricle (RV) myocardial mechanics, assessed by 2D speckle-tracking echocardiography (2D-STE), between patients with Fabry disease and patients with sarcomeric disease. Methods and results Patients with Fabry cardiomyopathy (FC) (n = 28) were compared with patients with sarcomeric hypertrophic cardiomyopathy (HCM), matched for degree of left ventricle hypertrophy (LVH) and demographic characteristics (n = 112). In addition, patients with Fabry disease and no LVH [phenotype-negative carriers of pathogenic α-galactosidase gene mutations (GLA LVH-)] (n = 28) were compared with age and sex-matched carriers of sarcomeric gene mutations without LVH [Phenotype-negative carriers of pathogenic sarcomeric gene mutations (Sarc LVH-)] (n = 56). Standard echocardiography and 2D-STE were performed in all participants. Despite a subtle impairment of RV global longitudinal strain (RV-GLS) was common in both groups, patients with FC showed a more prominent reduction of RV free wall longitudinal strain (RV-FWS) and lower values of difference between RV-FWS and RV-GLS (ΔRV strain), in comparison to individuals with HCM (P < 0.001 and P = 0.002, respectively). RV-FWS and ΔRV strain demonstrated an independent and additive value in discriminating FC from HCM, over the presence of symmetric LVH, systolic anterior motion of the mitral valve and RV hypertrophy. Similar results were found in GLA LVH- patients: they had worse RV-FWS and lower values of ΔRV strain as compared to Sarc LVH- patients (both P < 0.001). Conclusion Patients with FC show a specific pattern of RV myocardial mechanics, characterized by a larger impairment of RV-FWS and lower ΔRV strain in comparison to patients with HCM, which may be helpful in the differential diagnosis between these two diseases.

Funding Acknowledgements Type of funding sources: None. Background The right ventricle-pulmonary artery (RV-PA) coupling is a strong prognostic marker in several clinical settings, but only few studies focused on its role in repaired Tetralogy of Fallot (rToF) with pulmonary regurgitation (PR). Aim of this study was to assess whether differences exist in RV-PA coupling, estimated as by echocardiography, between patients with rToF and PR with (i-PVR) or without (ni-PVR) indication for pulmonary valve replacement (PVR). 3 Materials and Methods The study population included 40 rToF patients allocated to two groups: 20 i-PVR and 20 ni-PVR; 40 healthy controls were also included. All subjects underwent echocardiogram, while Cardiac magnetic resonance (CMR) was available in 27/40 rToF patients. RV-PA coupling was assessed by echocardiographic TAPSE/PASP and RV stroke volume/RV end systolic volume (RVSV/RVESV) by CMR. Results TAPSE was similar in i-PVR vs ni-PVR (19.0 ± 3.4 vs 18.8 ± 2.7 mm, p = 0.85) while RV-PA coupling was significantly worse in i-PVR vs ni-PVR (TAPSE/PASP 0.8 ± 0.3 vs 1.1 ± 0.5 mm/mmHg, p = 0.009) as well as in i-PVR vs controls (p = 0.02) while there was no difference between ni-PVR and controls (p = 0.29). CMR data confirmed the echo results, with a significant difference in RV-PA coupling between i-PVR and ni-PVR (RVSV/RVESV 0.9 ± 0.2 vs 1.2 ± 0.3 mL/min/mL, p = 0.01). Conclusions This study shows the presence of worse RV-PA uncoupling, despite normal RV systolic function, in rToF patients with indication to PVR. RV-PA coupling could be a sensitive marker of a progressive maladaptive RV response to long-standing volume overload in rToF prior to the onset of clinical symptoms and RV systolic dysfunction. Abstract Figure. example of an i-PVR patient

The objective of this study is to compare hemodynamic and echocardiographic findings between valve-in-valve (VIV) and native-valve (NV) patients submitted to transcatheter aortic valve implantation (TAVI) due to pure aortic regurgitation (AR).Patients with severe AR are surgically treated with variable postinterventional left-ventricular (LV) "reverse remodeling." TAVI might be considered in selected AR patients.Twenty-eight patients with pure severe AR caused by either degenerated bioprosthesis or NV disease were successfully treated by TAVI at our institution. LV catheterization before and after TAVI and echocardiography before, after (24-72 h), and at follow-up (3-12 months) were performed.Baseline clinical, hemodynamic, and echocardiographic characteristics were comparable between the two study groups, except for a younger age, higher proto-diastolic LV pressure, and higher LV end-systolic diameter in the NV group. At catheterization, an immediate hemodynamic impact of TAVI in both groups was noticed, with a trend toward better postprocedural residual regurgitation index and significantly lower LV dP/dT values (666.0 ± 177.9 vs. 883.5 ± 259.7 mmHg/s, p = 0.04) in VIV. At echocardiography, both NV and VIV patients showed favorable (early and sustained) post-TAVI echocardiographically detectable reverse remodeling. VIV patients also showed more pronounced early reduction in indexed LV end-diastolic volume (68.1 ± 27.4 vs. 86.5 ± 28.9 ml/mSuccessful TAVI induces a striking hemodynamic impact with major structural (reverse remodeling) consequences in patients with pure AR caused by both bioprosthesis degeneration or NV disease. In the immediate postrelease phase, VIV patients might exhibit a more pronounced early LV contractile and structural benefit.

ObjectivesTo evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).MethodsIn this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed.ResultsShort PR interval, prolonged QRS duration, right bundle branch block (RBBB), R in augmented vector left (aVL) ≥1.1 mV and inferior ST depression independently predicted AFD diagnosis. A point-by-point ECG score was then derived with the following diagnostic performances: c-statistic 0.80 (95% CI 0.74 to 0.86) for discrimination, the Hosmel-Lemeshow χ2 6.14 (p=0.189) for calibration, sensitivity 69%, specificity 84%, positive predictive value 82% and negative predictive value 72%. After bootstrap resampling, the mean optimism was 0.025, and the internal validated c-statistic for the score was 0.78.ConclusionsStandard ECG can help to differentiate AFD from HCM while investigating unexplained left ventricular hypertrophy. Short PR interval, prolonged QRS duration, RBBB, R in aVL ≥1.1 mV and inferior ST depression independently predicted AFD. Their systematic evaluation and the integration in a multiparametric ECG score can support AFD diagnosis.

There are limited data about the intraprocedural haemodynamic study performed immediately before and after transcatheter aortic valve implantation (TAVI) in patients with severe aortic stenosis (AS). We aimed to evaluate the acute haemodynamic impact of TAVI in patients with severe AS and to investigate invasive and non-invasive parameters predicting all-cause mortality.A total of 245 consecutive AS patients (43% male, mean age 80.3 ± 7.3 years) undergoing TAVI were enrolled. Intraprocedural left heart catheterization (LHC) and echocardiogram before and after TAVI were performed. The clinical endpoint was the death for any cause. LHC after TAVI revealed significant changes in aortic and left ventricular (LV) pressures, including indexes of intrinsic myocardial contractility and diastolic function such as positive dP/dT (1128.9 ± 398.7 vs. 806.3 ± 247.2 mmHg/s, P ˂ 0.001) and negative dP/dT (1310.7 ± 431.1 vs. 1075.1 ± 440.8 mmHg/s, P ˂ 0.001). Post-TAVI echo showed a significant reduction in LV end-diastolic (P = 0.036) and end-systolic (P ˂ 0.001) diameters, improvement in LV ejection fraction (from 55 ± 12% to 57.2 ± 10.5%, P ˂ 0.001), and pulmonary artery systolic pressure (42.1 ± 14.2 vs. 33.1 ± 10.7 mmHg, P 0.001). After a mean follow-up time interval of 24 months, 47 patients died. Post-TAVI significant aortic regurgitation at echocardiography was the only independent predictor of mortality (hazard ratio 5.592, confidence interval 1.932-16.184, P = 0.002).Left heart catheterization performed immediately before and after prosthesis release offers a unique insight in the assessment of LV adaptation to severe AS and the impact of TAVI on LV, catching changes in indexes of intrinsic contractility and myocardial relaxation. Aortic regurgitation assessed by echocardiography was the only independent predictor of mortality in patients undergoing TAVI.

Introduction: Having accurate and timely data on active COVID-19 cases is challenging, since it depends on the availability of an appropriate infrastructure to perform tests and aggregate their results. In this work, we propose alternative methods to assess the number of active cases of COVID-19. Methods: We consider a case to be active if it is infectious, and we propose three methods to estimate the number of active infectious cases of COVID-19 using (1) official publications based on PCR testing, (2) fatalities using the case fatality ratio, and (3) three public surveys from the Delphi Group at Carnegie Mellon University, the University of Maryland, and our self-developed survey entitled Coronasurveys. Results: We show that public surveys are a viable option in countries with reduced testing capacity or infrastructures.

Percutaneous edge-to-edge mitral valve (MV) repair is extensively used in different pathological MV conditions. Randomized controlled trials have evaluated the role of this technique in both primary (organic) and secondary (functional) mitral regurgitation (MR). Furthermore, recent analyses of these studies have shown the relevance of echocardiographic patient selection in the functional setting of MR, differentiating proportionate MR from disproportionate MR according to the degree of the effective regurgitant orifice area (EROA) related to the left ventricular volume. The haemodynamic impact of MR cannot be univocally measured by echocardiography alone and the aim of our study was to determine how invasive LAP monitoring during percutaneous edge-to-edge MV repair can predict long-term procedural success on top of the echocardiographic assessment by introducing the VCX INDEX and identifying haemodynamic variables with direct influence on filling pressures. The VCX INDEX, reflecting the impact of MR, is calculated by dividing the difference between v wave (ventricular systole in the left atrial pressure, LAP, or in the pulmonary capillary wedge pressure, PCWP, waveform) and the mean minimum LAP or mean minimum PCWP (mean between minimum LAP or minimum PCWP, x wave, and a/c wave) by systolic arterial pressure (SAP): (v wave – mean minimum LAP or mean minimum PCWP)/SAP. 85 patients at our centres underwent invasive intracardiac pressure monitoring either measuring LAP during percutaneous edge-to-edge MV repair or PCWP during right heart catheterization. Median VCX INDEX was 0.1 (Q1 0.05, Q3 0.16). The study population was further analysed according to the echocardiographic aetiology of MR: in the organic MR subgroup median VCX INDEX was 0.08 (Q1 0.05, Q3 0.14), in the functional proportionate MR subgroup median VCX INDEX was 0.07 (Q1 0.03, Q3 0.13) and in the functional disproportionate MR subgroup median VCX INDEX was 0.11 (Q1 0.06, Q3 0.19). 20 patients were deemed inoperable by the Heart Team and no further intervention was performed, while 65 patients underwent percutaneous edge-to-edge MV repair with MitraClip device and VCX INDEX was recalculated after the procedure. Median post-MitraClip VCX INDEX was 0.04 (Q1 0.02, Q3 0.07) and a subanalysis based on the echocardiographic MR aetiology was repeated: median post-MitraClip VCX INDEX was 0.02 in the organic MR subgroup (Q1 0.01, Q3 0.05), 0.03 in the functional proportionate MR subgroup (Q1 0.02, Q3 0.07) and 0.05 in the functional disproportionate MR subgroup (Q1 0.03, Q3 0.07). Median VCX INDEX in patients who did not undergo MitraClip implantation was 0.07 (Q1 0.04, Q3 0.12). The variation of VCX INDEX when comparing pre- and post-procedural invasive pressure assessment gives an insight of MitraClip’s favourable haemodynamic effect in terms of VCX INDEX reduction in the treated subgroup of the study and how the intervention has a comparable haemodynamic impact between different echocardiographic MR aetiologies. Further studies are needed to explore the incremental diagnostic role in the decision-making process as well as the prognostic value of the VCX INDEX in patients undergoing percutaneous edge-to-edge MV repair.

Aims Few data are available on the prevalence of right ventricle (RV) systolic dysfunction, assessed including RV strain, and RV to pulmonary artery (PA) coupling in patients with aortic stenosis (AS) submitted to TAVI and the early effect of the procedure. Methods We performed standard and speckle tracking echocardiography in 80 patients with severe AS the day before TAVI and within 48 h after TAVI. In all patients we measured TAPSE/PASP (cut-off for RV-PA uncoupling 0.31) and in 60/80 we were able to analyse RV global longitudinal strain (RV-GLS) and RV free wall strain (RV-FWS). Results RV-FAC and TAPSE were impaired in 8.3% while RV-GLS and RV-FWS in 45% and 33% before TAVI. TAPSE/PASP0.31 for: enlarged left ventricular (LV) end-diastolic and end-systolic volumes (P Conclusions RV-GLS unveils RV systolic dysfunction in as much as 45% of patients with AS vs. only 8.3% revealed by conventional echocardiography. RV systolic function does not significantly improve early after TAVI while RV-PA coupling does. Patients with lower TAPSE/PASP at baseline have worst LV and RV systolic function as well as larger RA. Patients who improve TAPSE/PASP after TAVI are those with worst diastolic function, RV systolic function and larger RA at baseline.

Background Progressive right heart chambers dilatation is frequent in the adult congenital heart disease (ACHD) population. We evaluated the immediate and mid-term response of right heart chambers to surgery performed in adulthood for lesions associated with right heart chambers enlargement. Methods Thirty-six adult patients with lesions associated with right heart chambers enlargement submitted to surgery were studied . We collected echocardiographic data of right ventricle (RV) mid-diameter, right atrial volume indexed, RV systolic pressure, and tricuspid annular plane systolic excursion (TAPSE) prior to surgery (T0), at 2 to 5 days (T1), and 3 to 6 months (T2) after surgery. Results At T1, we observed a significant decrease of RV mid-diameter (47.2 ± 8.4 vs. 39.6 ± 7.4 mm, P Conclusions Positive right heart chambers remodeling occurs as early as in the immediate post-operative period in most ACHD patients operated for lesions associated with right heart chambers enlargement.

AIM: To summarize our experience on the implementation of a telemedicine service dedicated to adult congenital heart disease (ACHD) patients during the lockdown for the first wave of COVID-19. METHODS: This is a prospective study enrolling all ACHD patients who answered a questionnaire dedicated telematic cardiovascular examination. RESULTS: A total of 289 patients were enrolled, 133 (47%) were male, 25 (9%) were affected by a genetic syndrome. The median age was 38 (29-51) years, whereas the median time interval between the last visit and the telematic follow-up was 9.5 (7.5-11.5) months. Overall, 35 patients (12%) reported a worsening of fatigue in daily life activity, 17 (6%) experienced chest pain, 42 (15%) had presyncope and 2 (1%) syncope; in addition, 28 patients (10%) presented peripheral edema and 14 (5%) were orthopneic. A total of 116 (40%) patients reported palpitations and 12 had at least one episode of atrial fibrillation and underwent successful electrical (8) or pharmacological (4) cardioversion. One patient was admitted to the emergency department for uncontrolled arterial hypertension, five for chest pain, and one for heart failure. Two patients presented fever but both had negative COVID-19 nasal swab. CONCLUSION: During the COVID-19 pandemic, the use of telemedicine dramatically increased and here we report a positive experience in ACHD patients. The postpandemic role of telemedicine will depend on permanent regulatory solutions and this early study might encourage a more systematic telematic approach for ACHD patients.

The world is suffering from a pandemic called COVID-19, caused by the SARS-CoV-2 virus. National governments have problems evaluating the reach of the epidemic, due to having limited resources and tests at their disposal. This problem is especially acute in low and middle-income countries (LMICs). Hence, any simple, cheap and flexible means of evaluating the incidence and evolution of the epidemic in a given country with a reasonable level of accuracy is useful. In this paper, we propose a technique based on (anonymous) surveys in which participants report on the health status of their contacts. This indirect reporting technique, known in the literature as network scale-up method, preserves the privacy of the participants and their contacts, and collects information from a larger fraction of the population (as compared to individual surveys). This technique has been deployed in the CoronaSurveys project, which has been collecting reports for the COVID-19 pandemic for more than two months. Results obtained by CoronaSurveys show the power and flexibility of the approach, suggesting that it could be an inexpensive and powerful tool for LMICs., Comment: Presented at The KDD Workshop on Humanitarian Mapping, San Diego, California USA, August 24, 2020

Introduction Multiple Myeloma (MM) is a hematological neoplasia originating from plasma cells, which accounts almost 1% of all oncologic malignancies. The median age of patients at diagnosis is about 65 years old and over. In this age group, cardiovascular (CV) diseases often co-exist, increasing the risk of adverse events related to MM treatment. A comprehensive search on the main educational platforms was performed and high-quality original articles and reviews were included. Areas covered Patients affected by MM are at risk for heart failure, uncontrolled systemic hypertension, accelerated ischemic heart disease, arterial/venous thromboembolism and arrhythmias. These complications may be due to the effects of chemotherapy on the CV system, which may play on pre-existing risk factors, and amyloid deposition at cardiac level. Expert opinion This review provides an updated overview of the spectrum of CV diseases which may affect MM patients, highlighting possible treatment strategies according to the latest recommendations. Cooperation between onco-hematologist and cardiologist is crucial in managing this population, in particular for adequate risk assessment, early diagnosis of CV complications and proper treatment.

Background The COVID-19 pandemic has posed a huge challenge to healthcare systems and their personnel worldwide. The study of the impact of SARS-CoV-2 infection among healthcare workers (HCW), through prevalence studies, will let us know viral expansion, individuals at most risk and the most exposed areas in healthcare organizations. The aim of this study is to gauge the impact of SARS-CoV-2 pandemic in our hospital workforce and identify groups and areas at increased risk. Methods and findings This is a cross-sectional and incidence study carried out on healthcare workers based on molecular and serological diagnosis of SARS-CoV-2 infection. Of the 3013 HCW invited to participate, 2439 (80.9%) were recruited, including 674 (22.4%) who had previously consulted at the Occupational Health Service (OHS) for confirmed exposure and/or presenting symptoms suggestive of COVID-19. A total of 411 (16.9%) and 264 (10.8%) healthcare workers were SARS-CoV-2 IgG and rRT-PCR positive, respectively. The cumulative prevalence considering all studies (IgG positive HCW and/or rRT-PCR positive detection) was 485 (19.9%). SARS-CoV-2 IgG-positive patients in whom the virus was not detected were 221 (9.1%); up to 151 of them (68.3%) did not report any compatible symptoms nor consult at the OHS for this reason. Men became more infected than women (25% vs 18.5%, p = 0.0009), including when data were also classified by age. COVID-19 cumulative prevalence among the HCW assigned to medical departments was higher (25.2%) than others, as well as among medical staff (25.4%) compared with other professional categories (p Conclusions The global impact of the COVID-19 pandemic on HCW of our centre has been 19.9%. Doctors and medical services personnel have had the highest prevalence of SARS-CoV-2 infection, but many of them have not presented compatible symptoms. This emphasizes the performance of continuous surveillance methods of the most exposed health personnel and not only based on the appearance of symptoms.

In this report, we present a rare case of severe mitral regurgitation due to isolated mitral valve chordal rupture without valve leaflet prolapse in a patient with Fabry cardiomyopathy. This finding could be due to subvalvular apparatus storage of glycosphingolipids rather than fibro-elastic deficiency, underlying how close cardiological follow-up of Fabry patients must be comprehensive and not only focused on left ventricular hypertrophy and arrhythmias. We feel that our finding could be of interest for the readership of your Journal.

Pulmonary hypertension (PH) in patients with hypertrophic cardiomyopathy (HCM) has been investigated in a small number of studies. Purpose of this study was to assess the prevalence and its association with outcome in a population of consecutive HCM outpatients.We retrospectively analyzed data of 361 consecutive HCM outpatients in whom echocardiographic measurements of pulmonary artery systolic pressure (PASP) were available at initial and most recent evaluation. Four different clinical groups were specifically investigated: patients without left ventricular outflow tract obstruction (group A, 165), with obstruction (group B, 126), patients diagnosed at the age≥65 (group C, 50) and patients with end stage (ES) HCM (group D, 20).PH was identified in 41 (11.4%) of the 361 patients at initial evaluation while it has been recognized in 25 (7,8% [1.1%/year]) during a median follow-up of 3.4years. Analysis of subgroups showed that prevalence of PH increased from patient group A to D (8%, group A, 19%, group B, 28% group C, 70%, group D, respectively, p0,01). During follow-up, patients with PH showed a significant higher HCM-related mortality (p=0.01) and morbidity (p0.001) as compared with those without PH, but in multivariable analysis, PH resulted an independent risk factor only for HCM-related morbidity (HR=2.50, 95% CI 1.08-5.79, p=0.03).PH affects a significant proportion of patients with HCM. Its prevalence varies according to different clinical profiles. It is associated with an unfavorable clinical outcome and is an independent predictor of morbidity.