Cardiac lymphoma with early response to chemotherapy: A case report and review of the literature.

Cardiac tumors are rare and benign masses account for the most part of the diagnosis. When malignant cancer is detected, primary or secondary cardiac lymphoma are quite frequent. Cardiac lymphoma may present as an intra or peri-cardiac mass or, rarely, it may diffusely infiltrate the myocardium. Although often asymptomatic, patients can have non-specific symptoms. Acute presentations with cardiogenic shock, unstable angina, or acute myocardial infarction are also described. Modern imaging techniques can help the clinicians not only in the diagnostic phase but also during administration of chemotherapy. A multidisciplinary counseling and serial multi-parametric assessment (echocardiography, cardiac troponin) seem to be the most effective approach to prevent possible fatal complications (i.e., cardiac rupture). Currently, only chemo- and radiotherapy are available options for treatment, but the prognosis remains poor. This is a case of secondary cardiac lymphoma presenting as a mediastinal mass with large infiltration of the heart and the great vessels with a good improvement after only one cycle of chemotherapy. It demonstrates the importance of an early diagnosis to modify the natural history of the disease.

Active cancer and cardiac surgery: Possible scenarios in patient decision-making.

Heart disease and cancer are often found simultaneously in the same patient, and may require cardiac and non-cardiac surgery. Cancer may be part of the past medical history; in other cases the presence of an active malignancy makes the clinical management more complex. No general evidence-based recommendations are available to help in the decision-making process. Because of the lack of specific guidelines we provided a series of possible scenarios describing not unusual cases. We focused on cases where the concomitant presence of heart disease and active malignancies involved a multidisciplinary team. Four real patients with active cancer referred to our Center were assessed. Three of them had valve disease requiring cardiac surgery. Defining the timing of surgery and choosing the surgical approach required a careful and comprehensive evaluation. In the last case, the complicated balance between the thrombotic and the hemorrhagic risk involved difficult decision. Several critical points, which characterized the management of this kind of patients, were identified. In particular, the hemodynamic status, the type and stage of the tumor, the need for cancer therapy, as well as the comorbidities of the patient, had to be taken into account. This narrative review shows the importance of submitting every challenging case to the assessment of a multidisciplinary team, which involves different clinical figures, in order to guarantee the most comprehensive evaluation. When clinical management deviates from the general recommendations, an individualized approach should be used.

Stage A Heart Failure: Modern Strategies for an Effective Prevention.

Stage A heart failure (HF) patients do not show HF symptoms or any structural heart disease but are at risk of HF development. Cardiovascular risk factors (hypertension, diabetes, metabolic syndrome, sedentary lifestyle, poor diet, and exposure to cardiotoxic agents) characterize subjects affected by stage A HF. It is essential to identify these subjects early and ensure that, despite being asymptomatic, they grasp the importance of undertaking correct lifestyle and therapeutic interventions. A careful stratification of asymptomatic subject's risk profile is needed to adopt proper preventive strategies and to set individualized therapeutic targets that avoid progression to advanced stages of HF.

Congestion in Patients with Advanced Heart Failure: Assessment and Treatment.

Heart failure (HF) is characterized by frequent hospital admissions due to acute decompensation and shortened life span with a progressive clinical course leading to an advanced stage where traditional therapies become ineffective. Due to aging of the population and improved therapies, only a small of proportion of patients with advanced HF are candidates for surgical treatments, such as mechanical circulatory support or heart transplantation. In most cases, prompt identification and management of congestion is paramount to improving symptoms and quality of life and avoiding progression to severe multiorgan dysfunction and death.

Direct Oral Anticoagulants for the Treatment of Left Ventricular Thrombus-A New Indication? A Meta-summary of Case Reports.

Left ventricular thrombus (LVT) can be a consequence of cardiac diseases such as heart failure with reduced ejection fraction and acute myocardial infarction. Currently, the guidelines recommend the use of warfarin for the treatment of this condition. However, there are increasing reports of patients with LVTs being treated with direct oral anticoagulants (DOACs), for several reasons. We set out to review the available literature to assess the safety and the efficacy of this approach. We analyzed 52 cases, extrapolated by 34 papers contained in literature, focusing on the characteristics of patients, treatment, outcome, and follow-up. Rivaroxaban was the most commonly used DOAC, followed by apixaban. The diagnosis of LVT and the follow-up were mainly performed by transthoracic echocardiography. The thrombus resolved in 45 patients (92%) of 49 (there are no data available regarding the outcome of 3 patients) and failed to resolve in 4 patients treated with DOACs. The resolution occurred in a median of 32 days. DOACs are shown to be a reasonable and valid option for the treatment of LVT. Our study provides a rationale for a prospective randomized controlled trial.

Prognostic role of tricuspid annular plane systolic excursion/pulmonary artery systolic pressure ratio in patients hospitalized for acute heart failure.

BACKGROUND: The role of TAPSE/PASP, a measurement of right ventricular to pulmonary artery coupling, in patients hospitalized for acute heart failure (AHF) is poorly described. OBJECTIVES: To evaluate the prognostic impact of TAPSE/PASP in AHF. METHODS: This retrospective single-center study included patients hospitalized for AHF between January 2004 and May 2017. TAPSE/PASP was evaluated as a continuous variable and as tertiles according to its value on admission. The main outcome was the composite of 1-year all-cause death or heart failure hospitalization. RESULTS: A total of 340 patients were included [mean age 68.8 ±â€Š11.8 years; 76.2% men, mean left ventricular ejection fraction (LVEF) 30.4 ±â€Š13.3%]. Patients with lower TAPSE/PASP had more comorbidities and a more advanced clinical profile, and received higher doses of intravenous furosemide in the first 24 h. There was a significant, linear, inverse relationship between TAPSE/PASP values and the incidence of the main outcome (P = 0.003). In two multivariable analyses including clinical (model 1), biochemical and imaging parameters (model 2) TAPSE/PASP ratio was independently associated with the primary end point [model 1: hazard ratio 0.813, 95% confidence interval (CI) 0.708-0.932, P = 0.003; model 2: hazard ratio 0.879, 95% CI 0.775-0.996, P = 0.043]. Patients with TAPSE/PASP greater than 0.47 mm/mmHg had a significantly lower risk of the primary end point (model 1: hazard ratio 0.473, 95% CI 0.277-0.808, P = 0.006; model 2: hazard ratio 0.582, 95% CI 0.355-0.955, P = 0.032; both compared with TAPSE/PASP <0.34 mm/mmHg). Similar findings were observed for 1-year all-cause mortality. CONCLUSION: TAPSE/PASP on admission demonstrated a prognostic value among patients with AHF.

Multimodality imaging in the diagnostic management of concomitant aortic stenosis and transthyretin-related wild-type cardiac amyloidosis.

Severe aortic stenosis (AS) is the most common valvular heart disease with a prevalence rate of more than 4% in 75-year-old people or older. Similarly, cardiac amyloidosis (CA), especially "wild-type transthyretin" (wTTR), has shown a prevalence rate ranging from 22% to 25% in people older than 80 years. The detection of the concomitant presence of CA and AS is challenging primarily because of the similar type of changes in the left ventricle caused by AS and CA, which share some morphological characteristics. The aim of this review is to identify the imaging triggers in order to recognize occult wtATTR-CA in patients with AS, clarifying the crucial step of the diagnostic process. Multimodality imaging methods such as echocardiography, cardiac magnetic resonance, cardiac computed tomography, and DPD scintigraphy will be analyzed as part of the available diagnostic workup to identify wtATTR-CA early in patients with AS.

Left atrial disease and left atrial reverse remodelling across different stages of heart failure development and progression: a new target for prevention and treatment.

The left atrium is a dynamic chamber with peculiar characteristics. Stressors and disease mechanisms may deeply modify its structure and function, leading to left atrial remodelling and disease. Left atrial disease is a predictor of poor outcomes. It may be a consequence of left ventricular systolic and diastolic dysfunction and neurohormonal and inflammatory activation and/or actively contribute to the progression and clinical course of heart failure through multiple mechanisms such as left ventricular filling and development of atrial fibrillation and subsequent embolic events. There is growing evidence that therapy may improve left atrial function and reverse left atrial remodelling. Whether this translates into changes in patient's prognosis is still unknown. In this review we report current data about changes in left atrial size and function across different stages of development and progression of heart failure. At each stage, drug therapies, lifestyle interventions and procedures have been associated with improvement in left atrial structure and function, namely a reduction in left atrial volume and/or an improvement in left atrial strain function, a process that can be defined as left atrial reverse remodelling and, in some cases, this has been associated with improvement in clinical outcomes. Further evidence is still needed mainly with respect of the possible role of left atrial reverse remodelling as an independent mechanism affecting the patient's clinical course and as regards better standardization of clinically meaningful changes in left atrial measurements. Summarizing current evidence, this review may be the basis for further studies.

Aortic Valve Stenosis and Cardiac Amyloidosis: A Misleading Association.

The association between aortic stenosis (AS) and cardiac amyloidosis (CA) is more frequent than expected. Albeit rare, CA, particularly the transthyretin (ATTR) form, is commonly found in elderly people. ATTR-CA is also the most prevalent form in patients with AS. These conditions share pathophysiological, clinical and imaging findings, making the diagnostic process very challenging. To date, a multiparametric evaluation is suggested in order to detect patients with both AS and CA and choose the best therapeutic option. Given the accuracy of modern non-invasive techniques (i.e., bone scintigraphy), early diagnosis of CA is possible. Flow-charts with the main CA findings which may help clinicians in the diagnostic process have been proposed. The prognostic impact of the combination of AS and CA is not fully known; however, new available specific treatments of ATTR-CA have changed the natural history of the disease and have some impact on the decision-making process for the management of AS. Hence the relevance of detecting these two conditions when simultaneously present. The specific features helping the detection of AS-CA association are discussed in this review, focusing on the shared pathophysiological characteristics and the common clinical and imaging hallmarks.

Assessment of right ventricle in pulmonary arterial hypertension with three-dimensional echocardiography and cardiovascular magnetic resonance.

AIM: To correlate 3-D Echo and CMR RV parameters and to verify whether they are similarly related to the clinical conditions of patients with pulmonary arterial hypertension (PAH), a disease in which the RV plays a crucial prognostic role. METHODS: We enrolled 34 consecutive PAH patients followed by our PAH clinics. All patients underwent a 3-D Echo and CMR assessment of RV volumes and functions in the same day. The presence or absence of correlation between major findings was investigated; functional RV parameters were also analyzed in relation to 6-min walking test (6MWT) results and BNP/Nt-proBNP plasma levels. Twenty-four subjects served as controls. RESULTS: Good agreement was found between 3-D Echo and CMR measures of RV volumes [RV-end-diastolic volume (r = 0.72, P < 0.0001), RV-end-systolic volume (ESV) (r = 0.80, P < 0.0001)] and function [RV-EF (r = 0.73, P < 0.0001), RV-ESV/SV (r = 0.83, P = 0.001)] for all the subjects of the study. These correlations were stronger in PAH patients than in control subjects. Importantly, 3-D Echo and CMR RV-EF and RV to pulmonary arterial coupling (RV-ESV/SV) similarly correlated with BNP/Nt-proBNP levels and with functional capacity measured at 6MWT in the PAH patients group. CONCLUSIONS: 3-D Echo demonstrated a significant agreement with CMR in the assessment of RV volume and function in PAH patients. Both techniques showed a similar correlation with clinical and prognostic parameters. The use of 3-D Echo should be amply boosted in the real-world clinical evaluation of PAH patients.

Acute pulmonary embolism in COVID-19 disease: Preliminary report on seven patients.

There is some evidence that Covid 19 pneumonia is associated with prothrombotic status and increased risk of venous thromboembolic events (deep venous thrombosis and pulmonary embolism). Over a two-week period we admitted in our Unit 25 patients with Covid-19 pneumonia, of these pulmonary embolism was diagnosed using computed tomography angiography in 7. We report on clinical and biochemical features of these patients. They were all males, with a mean age of 70.3 years (range 58-84); traditional risk factors for venous thromboembolism were identified in the majority of patients with pulmonary embolism, however not differently from those without pulmonary embolism. Clinical presentation of pulmonary embolism patients was usually characterized by persistence or worsening of respiratory symptoms, with increasing oxygen requirement. D-dimer levels were several fold higher than the upper threshold of normal; in patients in whom PE was recognized during hospital stay, a rapid and relevant increase of D-dimer levels was observed. Computed tomographic findings ranged from massive acute pulmonary embolism to a segmental or sub-segmental pattern; furthermore, thrombosis of sub-segmental pulmonary arteries within lung infiltrates were occasionally seen, suggesting local mechanisms. Six out of 7 patients were treated with unfractionated or low molecular weight heparin with clinical benefit within few days; one patient needed systemic thrombolysis (death from hemorrhagic complication).

In-hospital and long-term mortality for acute heart failure: analysis at the time of admission to the emergency department.

AIMS: Acute heart failure (AHF) leads to a drastic increase in mortality and rehospitalization. The aim of the study was to identify prognostic variables in a real-life population of AHF patients admitted to the emergency department with acute shortness of breath. METHODS AND RESULTS: We evaluated potential predictors of mortality in 728 consecutive patients admitted to the emergency department with AHF. Possible predictors of all-cause and cardiovascular (CV) mortality were investigated by Cox and Fine and Gray models at multivariable analysis. Among the 728 patients, 256 died during the entire follow-up, 142 of these due to CV cause. The 1 year mortality rate was 20%, with the highest risk of death during the index hospitalization (with 8% estimate in-hospital mortality at 30 days). A higher risk of events during the index hospitalization was more evident for the CV deaths, for which we found a cumulative 1 year incidence of 12% with a cumulative incidence in the first 30 days of hospitalization of about 5%. At multivariable analysis, age (P < 0.001), New York Heart Association (NYHA) class IV vs. I-II-III (P = 0.001), systolic blood pressure (P < 0.001), non-cardiac co-morbidities (≥3 vs. 0, P = 0.05), oxygen saturation (P = 0.03), serum creatinine (P < 0.001), and left ventricular ejection fraction (LVEF) (40-49% vs. <40%, P = 0.004; ≥50% vs. <40%, P = 0.003) were independent predictors of all-cause mortality during the entire follow-up. Age (P = 0.03), systolic blood pressure (P = 0.01), oxygen saturation (P = 0.03), serum creatinine (P = 0.02), and LVEF (40-49% vs. <40%, P = 0.03; ≥50% vs. <40%, P = 0.004) were independent predictors of CV mortality during the entire follow-up. NYHA class IV vs. I-II-III (P < 0.001), serum creatinine (P = 0.01), and LVEF (40-49% vs. <40%, P = 0.02; ≥50% vs. <40%, P < 0.001) remained independent predictors for in-hospital death, while only serum creatinine (P = 0.04), LVEF (40-49% vs. <40%: 0.32, P = 0.04; ≥50% vs. <40%, P < 0.001), and NYHA class vs. I-II-III (P = 0.02) remained predictors for in-hospital CV mortality. CONCLUSIONS: In this real-life cohort of patients with AHF, the results showed a similar mortality rate comparing with other analysis and with the most important registries. Age, NYHA class IV, systolic blood pressure, creatinine levels, sodium levels, and ejection fraction were independent predictors of 1 year mortality, while LVEF <40% was the only predictor of both all-cause mortality and CV mortality.

Atrial septal defects, supravalvular aortic stenosis and syndromes predisposing to aneurysm of large vessels.

Atrial septal defect is a persistent interatrial communication. It is the second most common congenital heart defect and is detected in 1:1500 live births. Clinical course is variable and depends on the size of the malformation. Clinical diagnosis is based on patient history, physical and instrumental examination. Atrial septal defect is frequently sporadic, but familial cases have been reported. The disease has autosomal dominant inheritance with reduced penetrance, variable expressivity and genetic heterogeneity. Supravalvular aortic stenosis is a congenital narrowing of the lumen of the ascending aorta. It has an incidence of 1:20000 newborns and a prevalence of 1:7500. Clinical diagnosis is based on patient history, physical and instrumental examination. Supravalvular aortic stenosis is either sporadic or familial and has autosomal dominant inheritance with reduced penetrance and variable expressivity. It is associated with mutations in the ELN gene. Syndromes predisposing to aneurysm of large vessels is a group of inherited disorders that may affect different segments of the aorta. They may occur in isolation or associated with other genetic syndromes. Clinical symptoms are highly variable. Familial thoracic aortic aneurysm and dissection accounts for ~20% of all cases of aneurysms. The exact prevalence is unknown. Clinical diagnosis is based on medical history, physical and instrumental examination. Genetic testing is useful for confirming diagnosis of these syndromes and for differential diagnosis, recurrence risk evaluation and prenatal diagnosis in families with a known mutation. Most syndromes predisposing to aneurysm of large vessels have autosomal dominant inheritance with reduced penetrance and variable expressivity.