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Cardiac manifestations in systemic sclerosis (SSc) are variable and are associated with a poor prognosis, frequently resulting in impaired right ventricular function and heart failure. A high proportion of patients with SSc experience pulmonary arterial hypertension (PAH), interstitial lung disease, or myocardial involvement, all of which can lead to exercise intolerance. In this context, cardiopulmonary exercise testing (CPET) is a useful tool for diagnosing exercise intolerance, elucidating its pathophysiology, and assessing its prognosis. CPET can also identify patients with SSc at higher risk of developing PAH. Despite its utility, current guidelines for CPET do not include the evaluation of patients with SSc, nor do standard SSc management guidelines consider CPET in the clinical work-up. This review summarizes the development, supporting evidence, and application of CPET in assessing cardiac involvement in patients with SSc.

Transcatheter valve procedures have become a cornerstone in the management of patients with valvular heart disease and high surgical risk, especially for aortic stenosis and mitral and tricuspid regurgitation. Cardiopulmonary exercise testing (CPET) is generally considered the gold standard for objectively quantifying functional capacity, providing a comprehensive evaluation of the human body's performance, particularly in patients with heart failure (HF). Its accurate assessment is valuable for exploring the pathogenetic mechanisms implicated in HF-related functional impairment. It is also useful for objectively staging the clinical severity and the prognosis of the disease. The improvement in functional capacity after transcatheter valve procedures may be clinically relevant and may provide prognostic information, even in this setting. However, it remains to be fully determined as data on the topic are limited. This review aims to summarize the available evidence on the usefulness of CPET to assess functional improvement in patients undergoing transcatheter valve procedures.

Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTD). Its early diagnosis is essential to start effective treatment. In the present paper, we aimed to evaluate the role of plasma osteopontin (OPN) as a candidate biomarker of PAH in a cohort of CTD patients. OPN is a pleiotropic protein involved in inflammation and fibrogenesis and, therefore, potentially promising in this specific clinical context. We performed a cross-sectional observational study on a cohort of 113 CTD patients (females N = 101, 89.4%) affected by systemic sclerosis N = 88 (77.9%), mixed connective tissue disease N = 10 (8.8%), overlap syndrome N = 10 (8.8%) or undifferentiated connective tissue disease N = 5 (4.4%). CTD-PAH patients showed significantly higher OPN plasma values than patients with CTD alone (241.0 (188.8–387.2) vs. 200.7 (133.5–281.6) ng/mL; p = 0.03). Although OPN levels were directly correlated with age and inversely with glomerular filtration rate, they remained associated with PAH at multivariate analysis. In conclusion, OPN was significantly associated with PAH among patients with CTD, suggesting it may have a role as a non-invasive disease biomarker of PAH.

The early diagnosis of pulmonary arterial hypertension (PAH) is a major determinant of prognosis in patients affected by connective tissue diseases (CTDs) complicated by PAH. In the present paper we investigated the diagnostic accuracy of cardiopulmonary exercise testing (CPET) in this specific setting. We recorded clinical and laboratory data of 131 patients who underwent a CPET at a pulmonary hypertension clinic. Out of them, 112 (85.5%) had a diagnosis of CTDs; 8 (6.1%) received a diagnosis of CTDs-PAH and 11 (8.4%) were affected PH of different etiology. Among CPET parameters the following parameters showed the best diagnostic performance for PAH: peak volume of oxygen uptake (VO2; AUC: 0.845, CI95% 0.767–0.904), ratio between ventilation and volume of exhaled carbon dioxide (VE/VCO2 slope; AUC: 0.888, CI95%: 0.817–0.938) and end-tidal partial pressures (PetCO2; AUC: 0.792, CI95%: 0.709–0.861). These parameters were comparable among CTDs-PAH and PH of different etiology. The diagnostic performance was even improved by creating a composite score which included all the three parameters identified. In conclusion, CPET is a very promising tool for the stratification of risk of PAH among CTDs patients; the use of composite measures may improve diagnostic performance.

Background: Echocardiographic Pulmonary to Left Atrial Ratio (ePLAR) represents an accurate and sensitive non-invasive tool to estimate the trans-pulmonary gradient. The prognostic value of ePLAR in hospitalized patients with COVID-19 remains unknown. We aimed to investigate the predictive value of ePLAR on in-hospital mortality in patients with COVID-19. Methods: One hundred consecutive patients admitted to two Italian institutions for COVID-19 undergoing early (<24 h) echocardiographic examination were included; ePLAR was determined from the maximum tricuspid regurgitation continuous wave Doppler velocity (m/s) divided by the transmitral E-wave: septal mitral annular Doppler Tissue Imaging e′-wave ratio (TRVmax/E:e′). The primary outcome measure was in-hospital death. Results: patients who died during hospitalization had at baseline a higher prevalence of tricuspid regurgitation, higher ePLAR, right-side pressures, lower Tricuspid Annular Plane Systolic Excursion (TAPSE)/ systolic Pulmonary Artery Pressure (sPAP) ratio and reduced inferior vena cava collapse than survivors. Patients with ePLAR > 0.28 m/s at baseline showed non-significant but markedly increased in-hospital mortality compared to those having ePLAR ≤ 0.28 m/s (27% vs. 10.8%, p = 0.055). Multivariate Cox regression showed that an ePLAR > 0.28 m/s was independently associated with an increased risk of death (HR 5.07, 95% CI 1.04–24.50, p = 0.043), particularly when associated with increased sPAP (p for interaction = 0.043). Conclusions: A high ePLAR value at baseline predicts in-hospital death in patients with COVID-19, especially in those with elevated pulmonary arterial pressure. These results support an early ePLAR assessment in patients admitted for COVID-19 to identify those at higher risk and potentially guide strategies of diagnosis and care.

Background: Pulmonary hypertension (PH) is a life-threatening complication of connective tissue diseases (CTD); in this study, we aimed at investigating the potential role of inducible co-stimulator (ICOS) and its ligand (ICOS-L) as biomarkers of PH in CTD. Materials and Methods: We recruited 109 patients: 84 CTD patients, 13 patients with CTD complicated by pulmonary arterial hypertension (PAH), and 12 subjects with PAH alone. All recruited patients underwent a complete clinical and instrumental assessment along with quantitative measurement of serum ICOS and ICOS-L. Results: Independently of the underlying cause, patients with PAH were older and had a lower glomerular filtration rate. Interestingly, patients with both CTD-related and CTD-unrelated PAH had higher ICOS and ICOS-L serum concentrations than CTD patients (0.0001 for both). When compared to CTD patients, those affected by CTD-PAH showed higher ICOS (440 (240–600) vs. 170 (105–275) pg/mL, p = 0.0001) and ICOS-L serum concentrations (6000 (4300–7000) vs. 2450 (1500–4100) pg/mL; p = 0.0001). In a logistic regression, ICOS and ICOS-L were associated with a diagnosis of PAH, independently from age, gender, and renal function. The corresponding receiver operating characteristic (ROC) curves demonstrated a good diagnostic performance for both ICOS and ICOS-L. Conclusions: ICOS and ICOS-L are increased in patients with PAH, irrespectively from the underlying cause, and represent promising candidate biomarkers for the diagnostic screening for PAH among CTDs patients.

Distal radial artery (dRA) is a novel vascular access site in interventional cardiology. We evaluated the use of dRA as alternative approach to standard forearm radial artery (fRA) for invasive blood pressure monitoring in Intensive Cardiac Care Unit (ICCU). This is a single-center, randomized, noninferiority trial. Patients admitted in ICCU needing invasive blood pressure monitoring were randomly allocated to dRA or fRA access site (1:1 ratio). Primary endpoint was noninferiority of dRA in the final catheterization success rate. Secondary endpoints were: first attempt success rates; arterial catheterization time; catheterization-related quality of pain; incidence of complications. A total of 250 patients were enrolled (125 in each arm). Final success rate was 95.2% in the dRA group versus 96.8% in the fRA arm (p <0.001 for noninferiority). First attempt success rates were 59.2% with dRA and 70.4% with fRA (p = 0.12). There was no difference in arterial catheterization time and catheterization-related quality of pain between the 2 arms. Entry-site complications were reduced with dRA (6.7% vs 17.4% in the fRA group; p = 0.013); this was mainly driven by decreased incidence of hematoma (0.8% vs 6.6%; p = 0.020). A numerically lower occurrence of arterial occlusion was observed with dRA (0.8% vs 4.9%; p = 0.06). In conclusion, in ICCU patients, the use of dRA to invasively monitor blood pressure is noninferior to fRA for catheterization success rates and may reduce entry-site bleeding. •Distal radial artery (dRA) is a novel vascular access site in interventional cardiology.•We explored the potential role of an invasive arterial pressure monitoring via the dRA access in intensive cardiac care unit (ICCU) patients.•dRA showed a noninferior catheterization success compared to forearm radial artery (fRA) in ICCU patients.•Entry-site complications were lower with dRA, especially fewer hematomas.

Background The PRAISE (PRedicting with Artificial Intelligence riSk aftEr acute coronary syndrome) score is a machine learning‐based model for predicting 1‐year adverse cardiovascular or bleeding events in patients with acute coronary syndrome (ACS). Its role in predicting arrhythmic complications in ACS remains unknown. Methods Atrial fibrillation (AF) and ventricular arrhythmias (VA) were recorded by continuous electrocardiographic monitoring until discharge in a cohort of 365 participants with ACS prospectively enrolled. We considered two separate timeframes for VA occurrence: ≤ 48 and > 48 h. The objective was to evaluate the ability of the PRAISE score to identify ACS patients at higher risk of in‐hospital arrhythmic complications. Results ROC curve analysis indicated a significant association between PRAISE score and risk of both AF (AUC 0.89, p = 0.0001; optimal cut‐off 5.77%) and VA (AUC 0.69, p = 0.0001; optimal cut‐off 2.17%). Based on these thresholds, high/low AF PRAISE score groups and high/low VA PRAISE score groups were created, respectively. Patients with a high AF PRAISE score more frequently developed in‐hospital AF (19% vs. 1%). Multivariate analysis showed a high AF PRAISE score risk as an independent predictor of AF (HR 4.30, p = 0.016). Patients with high VA PRAISE scores more frequently developed in‐hospital VA (25% vs. 8% for VA ≤ 48 h; 33% vs. 3% for VA > 48 h). Multivariate analysis demonstrated a high VA PRAISE score risk as an independent predictor of both VA ≤ 48 h (HR 2.48, p = 0.032) and VA > 48 h (HR 4.93, p = 0.014). Conclusion The PRAISE score has a comprehensive ability to identify with high specificity those patients at risk for arrhythmic events during hospitalization for ACS. PRAISE score is a machine learning‐derived tool with a comprehensive capability to predict arrhythmic complications. The score is easy to use and performs well, and a high score can identify with great specificity those patients at risk for ventricular and atrial arrhythmias during hospitalization. ACS, acute coronary syndrome; AF, atrial fibrillation; AUC, area under the curve; h, hours; PCI, percutaneous coronary intervention; PRAISE, PRedicting with Artificial Intelligence riSk aftEr acute coronary syndrome; VA, ventricular arrhythmias

Abstract Background Myocardial infiltration by primary cardiac neoplasm is a rare entity, providing diagnostic and therapeutic challenges. The pathological spectrum includes more frequently benign forms. Refractory heart failure, pericardial effusion, and arrhythmias due to infiltrative mass are the most common clinical manifestations. Case summary We describe the case of a 35-year-old man complaining of shortness of breath and weight loss in the last 2 months. A previous acute myeloid leukaemia treated with allogenic bone marrow transplant was reported. Transthoracic echocardiography revealed an apical thrombus in the left ventricle, with inferior and septal hypokinesia conditioning a mildly reduced ejection fraction, circumferential pericardial effusion, and abnormal right ventricular thickening. Cardiac magnetic resonance confirmed diffuse thickening of the right ventricular free wall due to myocardial infiltration. Positron emission tomography showed the presence of neoplastic tissue with increased metabolic activity. A pericardiectomy was performed showing a widespread cardiac neoplastic infiltration. Histopathological analysis done on right ventricular pathological samples obtained during cardiac surgery revealed the presence of a rare and aggressive cardiac anaplastic T-cell non-Hodgkin lymphoma. Few days after the operation, the patient developed refractory cardiogenic shock and unluckily died before initiating an adequate antineoplastic therapy. Discussion Primary cardiac lymphoma is not frequent, and the lack of specific symptoms makes the diagnosis extremely challenging and often limited to autopsy findings. Our case highlights the importance of an appropriate diagnostic algorithm, requiring non-invasive multimodality assessment imaging and then invasive cardiac biopsy. This approach may allow an early diagnosis and an adequate therapy for this otherwise fatal pathology.