Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology.

Primary malignant cardiac tumors are rare, with a prevalence of about 0.01% among all cancer histotypes. At least 60% of them are primary soft tissue sarcomas of the heart (pSTS-h) that represent almost 1% of all STSs. The cardiac site of origin is the best way to classify pSTS-h as it is directly linked to the surgical approach for cancer removal. Indeed, histological differentiation should integrate the classification to provide insights into prognosis and survival expectancy of the patients. The prognosis of pSTS-h is severe and mostly influenced by the primary localization of the tumor, the difficulty in achieving complete surgical and pharmacological eradication, and the aggressive biological features of malignant cells. This review aims to provide a detailed literature overview of the most relevant issues on primary soft tissue sarcoma of the heart and highlight potential diagnostic and therapeutic future perspectives.

The analysis of left atrial function predicts the severity of functional impairment in chronic heart failure: The FLASH multicenter study.

BACKGROUND: Heart failure (HF) patients present with a variety of symptoms at different stages of the disease, but the underlying pathophysiology still is unclear. Left atrial (LA) function might be tightly related to changes in patients' symptoms, more than morphological and anatomic heart features, measurable by ultrasound imaging technique. This study sought to investigate the correlation between LA function, assessed by Speckle Tracking Echocardiography (STE) and Quality of Life (QoL), assessed by the Minnesota Living with Heart Failure Questionnaire (MLHFQ), in patients with chronic HF. METHODS: Clinically stable HF outpatients (n = 369) were enrolled from 7 different international centres and underwent echocardiographic studies. Patients >75 years old and with atrial fibrillation were excluded. LA strain during reservoir phase (LASr) by STE was measured in all subjects by averaging the 6 atrial segments. LA size was assessed using biplane volume and 4-chamber area acquisition. RESULTS: LASr strongly correlated with both MLHFQ total score (r = -0.87; p < 0.0001). Less significant correlations between MLHFQ and either LA volume or left ventricular global longitudinal strain (LV-GLS) were found (r = 0.28; p = 0.05 and r = 0.30; p = 0.01, respectively). No significant correlation was found between MLHFQ score, LVEF (r = -0.15; p = ns), E/E' ratio (r = 0.19; p = ns), and E/A ratio (r = 0.20; p = ns). Among all echocardiographic parameters analyzed, LASr presented the highest diagnostic accuracy (AUC = 0.74) in predicting a poor QoL (>45), when compared with LV-GLS (AUC = 0.61), LA volume (AUC = 0.54) and E/e' ratio (AUC = 0.51). CONCLUSIONS: In patients with HF, irrespective of etiology, LA function strongly correlates with patients' QoL.

Diagnostic Accuracy of Point-of-Care Lung Ultrasonography and Chest Radiography in Adults With Symptoms Suggestive of Acute Decompensated Heart Failure: A Systematic Review and Meta-analysis.

Importance: Standard tools used to diagnose pulmonary edema in acute decompensated heart failure (ADHF), including chest radiography (CXR), lack adequate sensitivity, which may delay appropriate diagnosis and treatment. Point-of-care lung ultrasonography (LUS) may be more accurate than CXR, but no meta-analysis of studies directly comparing the 2 tools was previously available. Objective: To compare the accuracy of LUS with the accuracy of CXR in the diagnosis of cardiogenic pulmonary edema in adult patients presenting with dyspnea. Data Sources: A comprehensive search of MEDLINE, Embase, and Cochrane Library databases and the gray literature was performed in May 2018. No language or year limits were applied. Study Selection: Study inclusion criteria were a prospective adult cohort of patients presenting to any clinical setting with dyspnea who underwent both LUS and CXR on initial assessment with imaging results compared with a reference standard ADHF diagnosis by a clinical expert after either a medical record review or a combination of echocardiography findings and brain-type natriuretic peptide criteria. Two reviewers independently assessed the studies for inclusion criteria, and disagreements were resolved with discussion. Data Extraction and Synthesis: Reporting adhered to the Cochrane Handbook for Systematic Reviews of Diagnostic Test Accuracy and the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Two authors independently extracted data and assessed the risk of bias using a customized QUADAS-2 tool. The pooled sensitivity and specificity of LUS and CXR were determined using a hierarchical summary receiver operating characteristic approach. Main Outcomes and Measures: The comparative accuracy of LUS and CXR in diagnosing ADHF as measured by the differences between the 2 modalities in pooled sensitivity and specificity. Results: The literature search yielded 1377 nonduplicate titles that were screened, of which 43 articles (3.1%) underwent full-text review. Six studies met the inclusion criteria, representing a total of 1827 patients. Pooled estimates for LUS were 0.88 (95% Cl, 0.75-0.95) for sensitivity and 0.90 (95% Cl, 0.88-0.92) for specificity. Pooled estimates for CXR were 0.73 (95% CI, 0.70-0.76) for sensitivity and 0.90 (95% CI, 0.75-0.97) for specificity. The relative sensitivity ratio of LUS, compared with CXR, was 1.2 (95% CI, 1.08-1.34; P < .001), but no difference was found in specificity between tests (relative specificity ratio, 1.0; 95% CI, 0.90-1.11; P = .96). Conclusions and Relevance: The findings suggest that LUS is more sensitive than CXR in detecting pulmonary edema in ADHF; LUS should be considered as an adjunct imaging modality in the evaluation of patients with dyspnea at risk of ADHF.

Pulmonary congestion evaluated by lung ultrasound predicts decompensation in heart failure outpatients.

BACKGROUND: Pulmonary congestion is the main cause of hospital admission among heart failure (HF) patients. Lung ultrasound (LUS) assessment of B-lines has been recently proposed as a reliable and easy tool for evaluating pulmonary congestion. OBJECTIVE: To determine the prognostic value of LUS in predicting adverse events in HF outpatients. METHODS: Single-center prospective cohort of 97 moderate-to-severe systolic HF patients (53±13years; 61% males) consecutively enrolled between November 2011 and October 2012. LUS evaluation was performed during the regular outpatient visit to evaluate the presence of pulmonary congestion, determined by B-lines number. Patients were followed up for 4months to assess admission due to acute pulmonary edema. RESULTS: During follow-up period (106±12days), 21 hospitalizations for acute pulmonary edema occurred. At Cox regression analysis, B-lines number≥30 (HR 8.62; 95%CI: 1.8-40.1; p=0.006) identified a group at high risk for acute pulmonary edema admission at 120days, and was the strongest predictor of events compared to other established clinical, laboratory and instrumental findings. No acute pulmonary edema occurred in patients without significant pulmonary congestion at LUS (number of B-lines<15). CONCLUSION: In a HF outpatient setting, B-line assessment by LUS identifies patients more likely to be admitted for decompensated HF in the following 4months. This simple evaluation could allow prompt therapy optimization in those patients who, although asymptomatic, carry a significant degree of extravascular lung water. CONDENSED ABSTRACT: Pulmonary congestion is the main cause of hospital admissions among heart failure patients. Lung ultrasound can be used as a reliable and easy way to evaluate pulmonary congestion through assessment of B-lines. In a cohort of heart failure outpatients, a B-lines cutoff≥30 (HR 8.62; 95%CI: 1.8-40.1) identified patients most likely to develop acute pulmonary edema at 120-days.

Stress echo 2020: the international stress echo study in ischemic and non-ischemic heart disease.

BACKGROUND: Stress echocardiography (SE) has an established role in evidence-based guidelines, but recently its breadth and variety of applications have extended well beyond coronary artery disease (CAD). We lack a prospective research study of SE applications, in and beyond CAD, also considering a variety of signs in addition to regional wall motion abnormalities. METHODS: In a prospective, multicenter, international, observational study design, > 100 certified high-volume SE labs (initially from Italy, Brazil, Hungary, and Serbia) will be networked with an organized system of clinical, laboratory and imaging data collection at the time of physical or pharmacological SE, with structured follow-up information. The study is endorsed by the Italian Society of Cardiovascular Echography and organized in 10 subprojects focusing on: contractile reserve for prediction of cardiac resynchronization or medical therapy response; stress B-lines in heart failure; hypertrophic cardiomyopathy; heart failure with preserved ejection fraction; mitral regurgitation after either transcatheter or surgical aortic valve replacement; outdoor SE in extreme physiology; right ventricular contractile reserve in repaired Tetralogy of Fallot; suspected or initial pulmonary arterial hypertension; coronary flow velocity, left ventricular elastance reserve and B-lines in known or suspected CAD; identification of subclinical familial disease in genotype-positive, phenotype- negative healthy relatives of inherited disease (such as hypertrophic cardiomyopathy). RESULTS: We expect to recruit about 10,000 patients over a 5-year period (2016-2020), with sample sizes ranging from 5,000 for coronary flow velocity/ left ventricular elastance/ B-lines in CAD to around 250 for hypertrophic cardiomyopathy or repaired Tetralogy of Fallot. This data-base will allow to investigate technical questions such as feasibility and reproducibility of various SE parameters and to assess their prognostic value in different clinical scenarios. CONCLUSIONS: The study will create the cultural, informatic and scientific infrastructure connecting high-volume, accredited SE labs, sharing common criteria of indication, execution, reporting and image storage of SE to obtain original safety, feasibility, and outcome data in evidence-poor diagnostic fields, also outside the established core application of SE in CAD based on regional wall motion abnormalities. The study will standardize procedures, validate emerging signs, and integrate the new information with established knowledge, helping to build a next-generation SE lab without inner walls.

Left Atrial Volumes and Function by Three-Dimensional Echocardiography: Reference Values, Accuracy, Reproducibility, and Comparison With Two-Dimensional Echocardiographic Measurements.

BACKGROUND: Our study sought to (1) identify reference values for left atrial (LA) volumes and phasic function indices by 3-dimensional echocardiography (3DE) and compare them with those measured by 2-dimensional echocardiography (2DE) and (2) analyze their relationship with age, sex, body size, and left ventricular function. Accuracy and reproducibility of 3DE and 2DE have been also tested to evaluate the robustness of our data. METHODS AND RESULTS: We obtained maximal, minimal, and preA LA volumes by 3DE and 2DE in 276 healthy volunteers (18-79 years; 57% women). Limits of normality for LA volumes and total LA emptying fraction were larger with 3DE than with 2DE (maximal LA volume: 43 versus 35 mL/m(2); preA LA volume: 31 versus 25 mL/m(2); minimal LA volume: 18 versus 14 mL/m(2); 53 versus 48%, respectively; P<0.001). 3DE LA volumes indexed by body surface area were similar in men and women and increased with age. On multivariable analysis, age, weight, and left ventricular systolic and diastolic function indices resulted as correlates of LA 3DE indices. LA volumes were tightly correlated with cardiac magnetic resonance measurements, yet more underestimated by 2DE versus 3DE (bias±SD: -17±16 versus -7±15 mL, respectively). Among all LA parameters, maximal LA volume and total emptying fraction were the most reproducible, including at test-retest and at expert versus trainee comparisons. CONCLUSIONS: This study provides reference values for LA 3DE volumes and function from a relatively large cohort of healthy subjects with a wide age range. Our data may help clinicians to identify LA remodeling and dysfunction.

European Association of Cardiovascular Imaging/Cardiovascular Imaging Department of the Brazilian Society of Cardiology recommendations for the use of cardiac imaging to assess and follow patients after heart transplantation.

The cohort of long-term survivors of heart transplant is expanding, and the assessment of these patients requires specific knowledge of the surgical techniques employed to implant the donor heart, the physiology of the transplanted heart, complications of invasive tests routinely performed to detect graft rejection (GR), and the specific pathologies that may affect the transplanted heart. A joint EACVI/Brazilian cardiovascular imaging writing group committee has prepared these recommendations to provide a practical guide to echocardiographers involved in the follow-up of heart transplant patients and a framework for standardized and efficient use of cardiovascular imaging after heart transplant. Since the transplanted heart is smaller than the recipient's dilated heart, the former is usually located more medially in the mediastinum and tends to be rotated clockwise. Therefore, standard views with conventional two-dimensional (2D) echocardiography are often difficult to obtain generating a large variability from patient to patient. Therefore, in echocardiography laboratories equipped with three-dimensional echocardiography (3DE) scanners and specific expertise with the technique, 3DE may be a suitable alternative to conventional 2D echocardiography to assess the size and the function of cardiac chambers. 3DE measurement of left (LV) and right ventricular (RV) size and function are more accurate and reproducible than conventional 2D calculations. However, clinicians should be aware that cardiac chamber volumes obtained with 3DE cannot be compared with those obtained with 2D echocardiography. To assess cardiac chamber morphology and function during follow-up studies, it is recommended to obtain a comprehensive echocardiographic study at 6 months from the cardiac transplantation as a baseline and make a careful quantitation of cardiac chamber size, RV systolic function, both systolic and diastolic parameters of LV function, and pulmonary artery pressure. Subsequent echocardiographic studies should be interpreted in comparison with the data obtained from the 6-month study. An echocardiographic study, which shows no change from the baseline study, has a high negative predictive value for GR. There is no single systolic or diastolic parameter that can be reliably used to diagnose GR. However, in case several parameters are abnormal, the likelihood of GR increases. When an abnormality is detected, careful revision of images of the present and baseline study (side-by-side) is highly recommended. Global longitudinal strain (GLS) is a suitable parameter to diagnose subclinical allograft dysfunction, regardless of aetiology, by comparing the changes occurring during serial evaluations. Evaluation of GLS could be used in association with endomyocardial biopsy (EMB) to characterize and monitor an acute GR or global dysfunction episode. RV size and function at baseline should be assessed using several parameters, which do not exclusively evaluate longitudinal function. At follow-up echocardiogram, all these parameters should be compared with the baseline values. 3DE may provide a more accurate and comprehensive assessment of RV size and function. Moreover, due to the unpredictable shape of the atria in transplanted patients, atrial volume should be measured using the discs' summation algorithm (biplane algorithm for the left atrium) or 3DE. Tricuspid regurgitation should be looked for and properly assessed in all echocardiographic studies. In case of significant changes in severity of tricuspid regurgitation during follow-up, a 2D/3D and colour Doppler assessment of its severity and mechanisms should be performed. Aortic and mitral valves should be evaluated according to current recommendations. Pericardial effusion should be serially evaluated regarding extent, location, and haemodynamic impact. In case of newly detected pericardial effusion, GR should be considered taking into account the overall echocardiographic assessment and patient evaluation. Dobutamine stress echocardiography might be a suitable alternative to routine coronary angiography to assess cardiac allograft vasculopathy (CAV) at centres with adequate experience with the methodology. Coronary flow reserve and/or contrast infusion to assess myocardial perfusion might be combined with stress echocardiography to improve the accuracy of the test. In addition to its role in monitoring cardiac chamber function and in diagnosis the occurrence of GR and/or CAV, in experienced centres, echocardiography might be an alternative to fluoroscopy to guide EMB, particularly in children and young women, since echocardiography avoids repeated X-ray exposure, permits visualization of soft tissues and safer performance of biopsies of different RV regions. Finally, in addition to the indications about when and how to use echocardiography, the document also addresses the role of the other cardiovascular imaging modalities during follow-up of heart transplant patients. In patients with inadequate acoustic window and contraindication to contrast agents, pharmacological SPECT is an alternative imaging modality to detect CAV in heart transplant patients. However, in centres with adequate expertise, intravascular ultrasound (IVUS) in conjunction with coronary angiography with a baseline study at 4-6 weeks and at 1 year after heart transplant should be performed to exclude donor coronary artery disease, to detect rapidly progressive CAV, and to provide prognostic information. Despite the fact that coronary angiography is the current gold-standard method for the detection of CAV, the use of IVUS should also be considered when there is a discrepancy between non-invasive imaging tests and coronary angiography concerning the presence of CAV. In experienced centres, computerized tomography coronary angiography is a good alternative to coronary angiography to detect CAV. In patients with a persistently high heart rate, scanners that provide high temporal resolution, such as dual-source systems, provide better image quality. Finally, in patients with insufficient acoustic window, cardiac magnetic resonance is an alternative to echocardiography to assess cardiac chamber volumes and function and to exclude acute GR and CAV in a surveillance protocol.

The five-point Likert scale for dyspnea can properly assess the degree of pulmonary congestion and predict adverse events in heart failure outpatients.

OBJECTIVES: Proper assessment of dyspnea is important in patients with heart failure. Our aim was to evaluate the use of the 5-point Likert scale for dyspnea to assess the degree of pulmonary congestion and to determine the prognostic value of this scale for predicting adverse events in heart failure outpatients. METHODS: We undertook a prospective study of outpatients with moderate to severe heart failure. The 5-point Likert scale was applied during regular outpatient visits, along with clinical assessments. Lung ultrasound with ≥15 B-lines and an amino-terminal portion of pro-B-type natriuretic peptide (NT-proBNP) level >1000 pg/mL were used as a reference for pulmonary congestion. The patients were then assessed every 30 days during follow-up to identify adverse clinical outcomes. RESULTS: We included 58 patients (65.5% male, age 43.5 ± 11 years) with a mean left ventricular ejection fraction of 27 ± 6%. In total, 29.3% of these patients had heart failure with ischemic etiology. Additionally, pulmonary congestion, as diagnosed by lung ultrasound, was present in 58% of patients. A higher degree of dyspnea (3 or 4 points on the 5-point Likert scale) was significantly correlated with a higher number of B-lines (p = 0.016). Patients stratified into Likert = 3-4 were at increased risk of admission compared with those in class 1-2 after adjusting for age, left ventricular ejection fraction, New York Heart Association functional class and levels of NT-proBNP >1000 pg/mL (HR = 4.9, 95% CI 1.33-18.64, p = 0.017). CONCLUSION: In our series, higher baseline scores on the 5-point Likert scale were related to pulmonary congestion and were independently associated with adverse events during follow-up. This simple clinical tool can help to identify patients who are more likely to decompensate and whose treatment should be intensified.

The five-point Likert scale for dyspnea can properly assess the degree of pulmonary congestion and predict adverse events in heart failure outpatients

OBJECTIVES: Proper assessment of dyspnea is important in patients with heart failure. Our aim was to evaluate the use of the 5-point Likert scale for dyspnea to assess the degree of pulmonary congestion and to determine the prognostic value of this scale for predicting adverse events in heart failure outpatients. METHODS: We undertook a prospective study of outpatients with moderate to severe heart failure. The 5-point Likert scale was applied during regular outpatient visits, along with clinical assessments. Lung ultrasound with ≥15 B-lines and an amino-terminal portion of pro-B-type natriuretic peptide (NT-proBNP) level >1000 pg/mL were used as a reference for pulmonary congestion. The patients were then assessed every 30 days during follow-up to identify adverse clinical outcomes. RESULTS: We included 58 patients (65.5% male, age 43.5±11 years) with a mean left ventricular ejection fraction of 27±6%. In total, 29.3% of these patients had heart failure with ischemic etiology. Additionally, pulmonary congestion, as diagnosed by lung ultrasound, was present in 58% of patients. A higher degree of dyspnea (3 or 4 points on the 5-point Likert scale) was significantly correlated with a higher number of B-lines (p = 0.016). Patients stratified into Likert = 3-4 were at increased risk of admission compared with those in class 1-2 after adjusting for age, left ventricular ejection fraction, New York Heart Association functional class and levels of NT-proBNP >1000 pg/mL (HR = 4.9, 95% CI 1.33-18.64, p = 0.017). CONCLUSION: In our series, higher baseline scores on the 5-point Likert scale were related to pulmonary congestion and were independently associated with adverse events during follow-up. This simple clinical tool can help to identify patients who are more likely to decompensate and whose treatment should be intensified. .