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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 In Fabry cardiomyopathy, left ventricular outflow tract obstruction mimicking hypertrophic cardiomyopathy is a very rare finding, with few cases reported and successfully treated with cardiac surgery. In our population of patients with Fabry disease and severe left ventricular hypertrophy (LVH) at the time of diagnosis, we observed an evolution towards a midventricular obstructive phenotype. Methods and results We present a case series of three classically affected Fabry male patients with significant diagnostic delay and severe cardiac involvement (maximal wall thickness >20 mm) at first evaluation. All patients developed midventricular obstructive form over time despite prompt initiation and optimal compliance to enzyme replacement therapy. The extension and distribution of the LVH, involving the papillary muscles, was the main mechanism of obstruction, unlike the asymmetric septal basal hypertrophy and the mitral valve abnormalities commonly seen as substrate of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy. Conclusions Fabry cardiomyopathy can evolve over time towards a midventricular obstructive form due to massive LVH in classically affected men with significant diagnostic delay and severe LVH before enzyme replacement therapy initiation. This newly described cardiac phenotype could represent an adverse outcome of the disease.

Aims Right ventricular hypertrophy (RVH) is a common finding in Anderson–Fabry disease (AFD), but the prognostic role of right ventricular (RV) involvement has never been assessed. The aim of our study was to evaluate the prognostic significance of RVH and RV systolic function in AFD. Methods and results Forty‐five AFD patients (56% male patients) with extensive baseline evaluation, including assessment of RVH and RV systolic function, were followed‐up for an average of 51.2 ± 11.4 months. RV systolic function was assessed by standard and tissue Doppler echocardiography. Cardiovascular events were defined as new‐onset atrial fibrillation (AF), sustained ventricular arrhythmias, heart failure, or pacemaker/implantable cardioverter defibrillator implantation; renal events were defined as progression to dialysis and/or renal transplantation or significant worsening of glomerular filtration rate; and cerebrovascular events were defined as transient ischaemic attack or stroke. Fourteen patients (31.1%) presented RVH, while RV systolic function was normal in all cases. During the follow‐up period, 13 patients (28.8%, 11 male) experienced 18 major events, including two deaths. Cardiovascular events occurred in eight patients (17.7%). The most common event was pacemaker/implantable cardioverter defibrillator implantation (six patients, 13.3%), followed by AF (three cases, 6.6%). Only one case of worsening New York Heart Association class (from II to III and IV) was observed. Ischaemic stroke occurred in three cases (6.6%). Renal events were recorded in three patients (6.6%). At univariate analysis, several variables were associated with the occurrence of events, including RVH (HR: 7.09, 95% CI: 2.17 to 23.14, P = 0.001) and indexes of RV systolic function (tricuspid annular plane systolic excursion HR: 0.77, 95% CI: 0.62 to 0.96, P = 0.02; and RV tissue Doppler systolic velocity HR: 0.76, 95% CI: 0.61 to 0.93, P = 0.01). At multivariate analysis, proteinuria (HR:8.3, 95% CI: 2.88 to 23.87, P

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.

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.

Heart failure with preserved ejection fraction (HFpEF) is a major public health problem with growing prevalence and poor outcomes, mainly due to the lack of an effective treatment. HFpEF pathophysiology is heterogeneous and complex. Recently a “new paradigm” has been proposed, suggesting that cardiovascular and non-cardiovascular coexisting comorbidities lead to a systemic inflammatory state, perturbing the physiology of the endothelium and the perivascular environment and engaging molecular pathways that ultimately converge to myocardial fibrosis. If inflammation and fibrosis are the “fil rouge” in the heterogeneous spectrum of HFpEF, anti-fibrotic and anti-inflammatory drugs may have a role in its treatment. Pirfenidone is an orally bioavailable drug with antifibrotic and anti-inflammatory properties already approved for the treatment of idiopathic pulmonary fibrosis. Pirfenidone has been recently tested in animal models of myocardial fibrosis with promising results. Here we will review the rationale underlying the potential therapeutic effect of Pirfenidone in HFpEF.

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.

Introduction Many additional tasks in the Stat laboratory (SL) increase the workload. It is necessary to control them because they can affect the service provided by the laboratory. Our aim is to calculate these tasks, study their evolution over a 10 year period, and compare turnaround times (TAT) in summer period to the rest of the year. Materials and methods Additional tasks were classified as "additional test request" and "additional sample". We collected those incidences from the laboratory information system (LIS), and calculated their evolution over time. We also calculated the monthly TAT for troponin for Emergency department (ED) patients, as the difference between the verification and LIS registration time. A median time of 30 minutes was our indicator target. TAT results and tests workload in summer were compared to the rest of the year. Results Over a 10-year period, the technologists in the SL performed 51,385 additional tasks, a median of 475 per month. The workload was significantly higher during the summer (45,496 tests) than the rest of the year (44,555 tests) (P = 0.019). The troponin TAT did not show this variation between summer and the rest of the year, complying always with our 30 minutes indicator target. Conclusion The technicians accomplished a significant number of additional tasks, and the workload kept increasing over the period of 10 years. That did not affect the TAT results.

AimThe purpose of this study is, first to present a 10-year monitoring of postanalytical turnaround time (TAT) adapted to different clinicians and patient situations, second to evaluate and analyse the indicators results during that period of time, and finally to show a synthetic appropriate indicator to be included in the balanced scorecard management system.MethodsTAT indicator for routine samples was devised as the percentage of certain key tests that were verified before a specific time on the phlebotomy day. A weighted mean synthetic indicator was also designed. They were calculated for inpatients at 15:00 and 12:00 and for primary care patients only at 15:00. The troponin TAT of emergency department patients, calculated as the difference between the troponin verification and registration time, was selected as the stat laboratory TAT indicator.ResultsThe routine and stat TAT improved along the 10-year study period. The synthetic indicator showed the same trend.ConclusionsThe implementation of systematic and continuous monitoring over years, promoted a continuous improvement in TAT which will probably benefit patient outcome and safety.

Background The eradication of errors regarding patients' identification is one of the main goals for safety improvement. As clinical laboratory intervenes in 70% of clinical decisions, laboratory safety is crucial in patient safety. We studied the number of Laboratory Information System (LIS) demographic data errors registered in our laboratory during one year. Methods The laboratory attends a variety of inpatients and outpatients. The demographic data of outpatients is registered in the LIS, when they present to the laboratory front desk. The requests from the primary care centers (PCC) are made electronically by the general practitioner. A manual step is always done at the PCC to conciliate the patient identification number in the electronic request with the one in the LIS. Manual registration is done through hospital information system demographic data capture when patient's medical record number is registered in LIS. Laboratory report is always sent out electronically to the patient's electronic medical record. Daily, every demographic data in LIS is manually compared to the request form to detect potential errors. Results Fewer errors were committed when electronic order was used. There was great error variability between PCC when using the electronic order. Conclusions LIS demographic data manual registration errors depended on patient origin and test requesting method. Even when using the electronic approach, errors were detected. There was a great variability between PCC even when using this electronic modality; this suggests that the number of errors is still dependent on the personnel in charge of the technology.

Introduction: Preanalytical control and monitoring continue to be an important issue for clinical laboratory professionals. The aim of the study was to evaluate a monitoring system of preanalytical errors regarding not suitable samples for analysis, based on different indicators; to compare such indicators in different phlebotomy centres; and finally to evaluate a single synthetic preanalytical indicator that may be included in the balanced scorecard management system (BSC). Materials and methods: We collected individual and global preanalytical errors in haematology, coagulation, chemistry, and urine samples analysis. We also analyzed a synthetic indicator that represents the sum of all types of preanalytical errors, expressed in a sigma level. We studied the evolution of those indicators over time and compared indicator results by way of the comparison of proportions and Chi-square. Results: There was a decrease in the number of errors along the years (P < 0.001). This pattern was confirmed in primary care patients, inpatients and outpatients. In blood samples, fewer errors occurred in outpatients, followed by inpatients. Conclusion: We present a practical and effective methodology to monitor unsuitable sample preanalytical errors. The synthetic indicator results summarize overall preanalytical sample errors, and can be used as part of BSC management system.

SUMMARY Background: A correct preanalytical phase procedure is critical to get an adequate sample and consequently to achieve the most reliable laboratory results, promoting patient safety. Continuous laboratory staff changes create the need to establish improvement strategies to reduce the error risk. The objective was to show how the numbers of preanalytical errors related to unsuitable samples in a hospital setting decrease following two improvement strategies related to new technology and educational actions and how their effects were measured by monitoring indicators. Methods: Samples were drawn by the laboratory and other hospital departments’ nurses without previous patient appointment, therefore, prior tube preparation was not available before the phlebotomy. Corrective measures for these activities were established: educational program for nurses and a system of custom labels, which correlate each laboratory test in the Laboratory Information System (LIS) with the corresponding tube. Three phases were defined based on the implementation dates of the improvement actions to be assessed. The set of indicators designed to monitor the improvement related to clotted, hemolyzed, insufficient, and uncollected samples. Data were collected and indicators calculated from the LIS using a data warehouse application. Patient satisfaction with respect to phlebotomy was also measured annually using a scoring survey. Results: There was a reduction in all types of preanalytical sample errors related to the improvement strategies adopted. The indicators demonstrated that the unavailable, insufficient, and clotted samples decreased between two- and three-fold, whereas hemolysis errors benefited more from these improvement strategies. Patient satisfaction with the laboratory and phlebotomy procedures improved over the past several years as based on the annual satisfaction surveys. Conclusions: The educational program for nursing personnel is relevant and important as can be seen in the decrease of sample errors and the resulting quality improvement. The custom label system minimizes the potential oversight of forgetting to draw a tube, which happens frequently when operating without appointments, by printing the labels according to requested tests. Detection, identification, and monitoring of the error and implementing strategies to improve preanalytical quality reduces error numbers and thereby improves patient safety and health system outcomes. (Clin. Lab. 2012;58:911-917. DOI: 10.7754/Clin.Lab.2012.111111)