Search Results (175)

Erdheim–Chester Disease (ECD) constitutes a rare and clinically heterogeneous non-Langerhans cell histiocytosis, characterized by the systemic infiltration of tissues by foamy, lipid-laden histiocytes. These cells typically exhibit an immunophenotypic profile positive for CD68 and negative for CD1a. The disease’s multifaceted presentation, which can span from isolated bone lesions to fulminant multi-organ failure, frequently results in considerable diagnostic delay. In this case-based review, we describe the case of a 58-year-old who presented with a primary complaint of exertional dyspnoea and fatigue. The initial diagnostic evaluation revealed a hemodynamically significant circumferential pericardial effusion and imaging findings suggestive of aortitis. Clinical presentation of ECD depends on the organs and tissues involved, and may range from bone pain to neurological symptoms, endocrine dysfunction, and cardiac involvement. Cardiovascular involvement occurs in at least 40% of ECD patients, although it is frequently underdiagnosed. Cardiac ECD is heterogeneous and may mimic many alternative aetiologies. The infiltration of the right atrioventricular sulcus, right atrial walls, or interatrial septum is one of the most typical cardiac manifestations of ECD. Recognition of pseudo-tumour intra-atrial mass, pericardial involvement, as well as the circumferential encasement of the entire aorta, the so-called coated aorta, are other frequent findings. Diagnosis often requires a multimodal approach, in particular when cardiac symptoms represent the onset of clinical manifestation of ECD. The combined use of computed tomography, fluorodeoxyglucose positron emission tomography, dedicated cardiac and abdominal magnetic resonance imaging, and X-ray of long bones can collectively reveal a constellation of findings diagnostic of ECD. Full article

Background: Hydatid disease, caused primarily by Echinococcus granulosus, remains a significant public health challenge in endemic regions. While hepatic (80–85%) and pulmonary (15–20%) involvements are common, multi-organ dissemination involving rare sites such as the pericardium, diaphragm, and mediastinum occurs in less than 0.1–2% of cases. Case presentation: We present a rare case of a 26-year-old male, a farmer for 10 years, with occupational exposure to dogs and horses, with a personal history of multiple surgically treated abdominal cysts in 2016, admitted after abdominal computed tomography revealed liver cysts greater than 5 cm, as well as mediastinal and diaphragmatic cysts. Histopathological examination of the surgically resected hepatic cyst material confirmed echinococcosis. Serology was also positive for echinococcosis. Echocardiography revealed a pericardial cyst, posterior to the left atrium. Under these circumstances, antiparasitic treatment was initiated by an infectious disease specialist, followed by surgical treatment of the abdominal cysts, confirming the final diagnosis of hydatid disease, and subsequently, surgical treatment of the thoracic hydatid cysts was performed. The postoperative course was complicated by bronchial superinfection with Stenotrophomonas maltophilia, identified from bronchial aspirate culture after extended incubation and managed with trimethoprim–sulfamethoxazole. Conclusions: This case underscores the necessity of lifelong surveillance in hydatid disease, the potential role of postoperative antiparasitic therapy in preventing long-term recurrence, and the vital role of a multidisciplinary team in managing complex, disseminated relapses. Full article

Background: Cardiovascular complications are increasingly recognized in patients undergoing hematopoietic stem cell transplantation (HSCT). Early detection of subclinical myocardial dysfunction may improve risk stratification, and global longitudinal strain (GLS) is emerging as a sensitive marker of early cardiac impairment. Methods: We conducted a single-center observational cohort study including 518 adult patients undergoing autologous (n = 64) or allogeneic (n = 454) HSCT between 2004 and 2025. Baseline cardiovascular risk factors, transplant characteristics, and echocardiographic parameters—including GLS in a subset—were recorded. Abnormal GLS was defined as less negative than −20%. The primary outcome was the occurrence of cardiovascular events (composite of cardiovascular death, myocardial infarction, stroke, atrial fibrillation/flutter, pericardial effusion, pulmonary embolism, and left ventricular systolic dysfunction). Multivariable logistic regression was used to identify independent predictors. Results: Median age was 53 years; 58% were male. Cardiovascular events were predominantly atrial fibrillation, pericardial effusion, and reduced left ventricular function, whereas ischemic events were rare. Over 90% of events occurred within 100 days post-transplant. Multivariable analysis identified older age (OR 1.28 per 10-year increment; 95% CI 1.10–1.48; p = 0.002), chronic kidney disease (OR 2.44; 95% CI 1.18–5.02; p = 0.01), pre-transplant atrial fibrillation (OR 2.12; 95% CI 1.04–4.31; p = 0.03), and abnormal baseline GLS (OR 1.89; 95% CI 1.02–3.52; p = 0.04) as independent predictors. Importantly, the prognostic value of GLS remained significant after excluding clinically insignificant pericardial effusions from the composite endpoint. GLS deterioration during follow-up occurred more frequently in patients receiving reduced-intensity conditioning compared with myeloablative conditioning (25% vs. 12.7%; p = 0.006). Conclusions: Subclinical myocardial dysfunction detected by GLS identifies HSCT recipients at increased cardiovascular risk. These findings support the incorporation of strain imaging into routine pre- and post-transplant cardiovascular evaluation to enable earlier detection and guide targeted interventions. Full article

Congenital pericardial agenesis (CPA) is a rare anomaly that is often considered a benign incidental finding but may present with nonspecific symptoms and mimic structural heart disease. Its clinical relevance remains incompletely defined, particularly regarding the distinction between a harmless anatomical variant and a clinically significant condition. We present a retrospective two-center case series of four patients with imaging-confirmed CPA, combined with a narrative review of the literature aiming to evaluate the clinical spectrum, diagnostic challenges, and management implications of CPA. The clinical presentation of our patients was heterogeneous, ranging from incidental findings to chest discomfort and dyspnea. In all cases, initial echocardiography suggested alternative diagnoses, including right ventricular cardiomyopathy, atrial septal defect, or pericardial disease, leading to diagnostic uncertainty. Definitive diagnosis was established using multimodality imaging, particularly cardiac magnetic resonance and computed tomography, which demonstrated characteristic features such as cardiac levoposition and interposition of lung parenchyma. Three patients had complete left pericardial agenesis and one had a partial defect. All patients were managed conservatively, without complications during follow-up. Full article

Background/Objectives: In individuals with a genetic predisposition, acute rheumatic fever (ARF) can manifest as arthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum. It occurs after a latent period of 1–3 weeks of untreated upper respiratory tract infections caused by group A beta-hemolytic streptococci. The presence and severity of carditis determine the prognosis for ARF. Carditis manifests as pancarditis, and although all patients have pericarditis, not all experience a pericardial effusion. Patients with severe carditis exhibit pericardial effusion more frequently. The physiopathology of ARF remains unclear, specifically which patients will experience carditis, arthritis, or chorea. However, the Turkish population has fully clarified the physiopathology and clinical features of Familial Mediterranean fever (FMF), a common rheumatic disease. In the Turkish population, the heterozygous positivity rate for the FMF gene mutation is 15–35%. For these reasons, we examined the presence of FMF gene mutations in our patients to determine whether there is a correlation between the clinical course of ARF and the FMF gene mutation. Methods: The study included 60 patients with arthritis (n = 11), carditis (n = 26), or both (n = 23), as well as 60 healthy controls. These pediatric patients underwent screening for mutations in exons 2 and 10 of the MEFV gene. Results: There was no statistically significant difference between the patient and control groups in terms of the incidence of MEFV gene mutations in exon 10. However, in patients with ARF, the exon 2 E148Q variant was significantly more common than in the control group. Conclusions: This study suggests a relationship between certain clinical manifestations of ARF and MEFV gene mutations in children. Full article

Pembrolizumab is standard adjuvant therapy for high-risk clear cell renal cell carcinoma, but serositis is an uncommon immune-related adverse event that may mimic recurrence or infection. We report a 55-year-old man who achieved no evidence of disease after nephrectomy and metastasectomy and developed anasarca, large bilateral pleural effusions, mild ascites, peripheral eosinophilia, and a small pericardial effusion after six cycles of adjuvant pembrolizumab. Pleural fluid was exudative and contained 20% eosinophils. Cytology showed inflammatory cells without evidence of malignancy; bacterial, mycobacterial, and fungal studies were negative; and mildly elevated adenosine deaminase did not support tuberculosis. Cardiac function and natriuretic peptides were preserved. Pembrolizumab was discontinued, thoracentesis and corticosteroids were administered, and symptoms, eosinophilia, renal function, and albumin improved rapidly. Follow-up through March 2026 showed no oncologic progression, although some residual pleural and abdominal fluid persisted alongside imaging findings suggestive of portal-hypertension physiology, which may have contributed to residual fluid but did not explain the eosinophilic pleural syndrome. In a targeted literature review, effusion eosinophil data were infrequently reported. This case highlights a likely underrecognized eosinophilic pleural-fluid phenotype within pembrolizumab-associated polyserositis and supports routine differential cell counts in drained serosal fluid when immune-related serositis is suspected. Full article

Colchicine has emerged as a prominent anti-inflammatory candidate in cardiovascular medicine, supported by a hierarchy of evidence spanning chronic and acute coronary syndromes, post-myocardial infarction care, revascularization, atrial fibrillation, pericardial disease, heart failure, peripheral arterial disease, and mechanistic translational models. Across this literature, the most mature study architecture and the strongest clinical support are derived from completed randomized trials in chronic coronary disease and secondary prevention, where colchicine has been shown to prevent major cardiovascular events (MACEs) when added to standard of care. The clearest clinical benefits are the reduction in non-fatal ischemic events in atherosclerotic disease, prevention of recurrent pericarditis and postoperative atrial fibrillation, and attenuation of inflammatory and plaque-related markers. By contrast, mixed or lower-tier evidence renders its application less consistent in acute coronary syndromes, ST-elevation MI (STEMI), percutaneous coronary intervention (PCI)-related hard outcomes, and heart failure, while a definitive mortality benefit has not been demonstrated. Overall, colchicine is best understood as a targeted clinical adjunct whose value depends heavily on precise indication, timing, dose, gastrointestinal tolerability, and the maturity of the supporting evidence. Full article

Background: Rheumatic diseases (RDs) are associated with increased cardiovascular morbidity, including a 40% higher risk of atrial fibrillation (AF). While ablation has become the cornerstone of rhythm control, its safety in patients with rheumatic diseases remains poorly defined. Methods: Adults with a primary admission diagnosis of AF catheter ablation from 2016 to 2022 were identified using the National Inpatient Sample. We excluded patients with other forms of supraventricular tachycardia, pacemaker/defibrillator procedures, and atrioventricular junction ablations. Sociodemographic, clinical characteristics, and outcomes were compared between groups. Multivariate logistic regression adjusted for age, race, sex, and potential comorbid confounders was used to assess for independent associations. Results: A weighted total of 48,855 patients were included, 2.5% of which had RD. These patients were predominantly female, older, and had higher rates of renal dysfunction, hypertension, heart failure, history of stroke, ischemic heart disease, heart failure, and obstructive sleep apnea (all p < 0.001). Patients with RD had higher complication rates (12.9% vs. 8.8%, p < 0.001); specifically, bleeding (p < 0.001), infection (p = 0.008), pericardial (p = 0.003), and respiratory complications (p < 0.001). RDs were not found to be an independent predictor of complications, though there was a trend towards more complications (odds ratio 1.43, 95% confidence interval 0.97–2.11, p = 0.070). Conclusions: Patients with RD undergoing AF ablation were older, female, and had higher rates of comorbidities. This translated to higher unadjusted periprocedural complications in patients with rheumatic diseases. While RDs were not independently associated with adverse outcomes, a trend towards increased complications was observed. Full article

Bisphenols may negatively impact human health, including the heart and circulatory system. It is crucial to determine the presence of these xenobiotics in biological samples, including pericardial fluid. Pericardial fluid was collected from patients with acute coronary syndromes (ACS), and with coronary artery disease during coronary artery bypass surgery. Bisphenol residues were identified and quantified in samples from 15 patients. Quantitative analysis of bisphenols in the samples was performed by LC–MS/MS on a triple quadrupole (QqQ) mass spectrometer and electrospray ionization (ESI−/ESI+) was applied in the negative and positive ion modes, respectively. The procedure was successfully applied to the biomonitoring of free forms of 14 bisphenols in pericardial fluid. After statistical examination of the relationships between the selected variables, it was concluded that while male subjects demonstrated higher Body Mass Index (BMI), longer procedural times, and earlier troponin release, female subjects exhibited later but more pronounced increases in CK and TrI, suggesting differences in kinetics and physiological response. Full article

Acute aortic dissection (AAD) is a life-threatening cardiovascular emergency characterized by important sex-related differences in presentation, management, and outcomes. Although women account for a smaller proportion of cases, they typically present at older ages and more frequently exhibit atypical symptoms, hemodynamic instability, and complications such as pericardial effusion or tamponade, contributing to diagnostic delays and higher pre-hospital mortality. Beyond clinical factors, biological differences may influence disease expression in women. Menopause-associated vascular aging, hormonal modulation of extracellular matrix remodeling, and pregnancy-related hemodynamic and connective tissue changes may alter aortic wall integrity and susceptibility to dissection. Notably, women often experience dissection at smaller absolute aortic diameters, highlighting the potential importance of body-size indexing in risk stratification and surgical thresholds. In type A AAD, women are less likely to undergo extensive surgical repair in some cohorts, and although contemporary in-hospital mortality differences are narrowing, long-term survival disparities may persist. In type B AAD, women are more frequently managed conservatively, while outcomes following thoracic endovascular aortic repair appear broadly comparable between sexes. Pregnancy and the postpartum period represent particularly vulnerable windows, especially among patients with underlying heritable aortopathies. Greater awareness of sex-specific biological and clinical characteristics, incorporation of indexed aortic dimensions, and improved multidisciplinary management strategies are essential to optimize outcomes for women with acute aortic dissection. Full article

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by immune dysregulation and systemic inflammation, with the cardiovascular (CV) system representing a major yet frequently under-recognized target. Cardiac involvement spans from subclinical myocardial inflammation to overt pericardial disease, myocarditis, valvular abnormalities, coronary microvascular dysfunction, and accelerated atherosclerosis. Given that CV disease remains a leading cause of morbidity and mortality in SLE, early detection of silent cardiac injury is crucial. Aim: This review aims to provide a comprehensive and clinically oriented overview of CV involvement in SLE, focusing on the role of multimodal cardiac imaging in the detection, characterization, and risk stratification of cardiac abnormalities, as well as its potential implications for clinical management and preventive strategies. Methods: This narrative review is based on a structured, non-systematic search of PubMed (2013–2026), combining the term “systemic lupus erythematosus” with imaging-related keywords including “transthoracic echocardiography,” “cardiac magnetic resonance,” and “cardiac computed tomography.” English-language studies in adult populations were screened and selected according to clinical relevance, methodological robustness, and contribution to understanding SLE-related cardiac involvement. Discussion: Multimodal cardiac imaging plays a central role in the evaluation of SLE-related cardiac disease. Transthoracic echocardiography (TTE) represents the first-line modality for the assessment of ventricular function, pericardial disease, and valvular abnormalities, while deformation imaging enables the detection of subtle myocardial dysfunction. Cardiac magnetic resonance (CMR) provides comprehensive tissue characterization, allowing differentiation between active inflammation and chronic fibrosis. Cardiac computed tomography (cCT) identifies subclinical coronary atherosclerosis and high-risk plaque features, whereas nuclear imaging techniques offer insight into inflammatory activity and microvascular dysfunction. Conclusions: An integrated, imaging-based approach enables early diagnosis, refined CV risk stratification, longitudinal monitoring, and personalized therapeutic strategies. Multimodal imaging thus represents a key pillar of precision medicine in lupus-associated CV disease. Full article

Background and Clinical Significance: Mixed-phenotype acute leukemia (MPAL) is a rare hematologic malignancy characterized by the co-expression of myeloid and lymphoid markers and is associated with poor prognosis. Myeloid sarcoma (MS), particularly in the mediastinum, is an uncommon extramedullary manifestation and is rarely reported in association with MPAL. Case Presentation: We report a rare case of mediastinal MS with biphenotypic features and pericardial extension occurring concurrently with MPAL, highlighting diagnostic challenges, therapeutic strategies, and long-term outcomes. We describe the clinical course, diagnostic workup, treatment, and follow-up of a 21-year-old woman who presented with cardiac tamponade secondary to a mediastinal mass. Histopathology and immunophenotyping established the diagnosis of mediastinal MS associated with MPAL (B/myeloid, NOS). Management included surgical cytoreduction, intensive induction chemotherapy, and consolidation with allogeneic hematopoietic stem cell transplantation (allo-HSCT) from an unrelated donor. Fertility preservation with oocyte retrieval, in vitro fertilization (IVF), and embryo cryopreservation was performed prior to conditioning. A focused literature review of MPAL cases with extramedullary involvement was conducted. The patient achieved complete remission following induction therapy and underwent allo-HSCT. Despite the historically poor prognosis of mediastinal MS and MPAL, she remains in sustained complete remission 13 years after diagnosis. A literature review identified only eight reported cases of MPAL with extramedullary disease, with mediastinal involvement described in a single case and allo-HSCT performed in only two patients. Conclusions: This case illustrates a rare presentation of MPAL with mediastinal myeloid sarcoma and cardiac tamponade, demonstrating that aggressive multimodal therapy including allo-HSCT may achieve durable remission even in high-risk presentations. Early multidisciplinary management and consideration of fertility preservation are essential in young patients. Full article

Myocarditis and inflammatory cardiomyopathy are inflammatory diseases of the heart muscle that can have both infectious and non-infectious causes. They can be caused by an unresolved viral infection or other infection, or they can be autoimmune, toxic, or allergic in nature. The specific identification of the pathogen and/or confirmation of inflammation can only be achieved through direct tissue analysis using endomyocardial biopsy (EMB), as neither detection of the virus nor assessment of the quality and intensity of the inflammation is possible using non-invasive methods. Accordingly, the removal and analysis of an EMB is considered the diagnostic gold standard in international guidelines and statements. The sudden onset of atypical angina pectoris and initially exertion-dependent dyspnea, as well as arrhythmias, pericardial effusion, and progressive symptoms of heart failure, indicate an acute inflammatory process of the myocardium. In addition, nonspecific symptoms such as fatigue and reduced physical performance may also occur. Diagnostic evaluation includes an electrocardiogram (ECG), cardiac imaging, and laboratory tests. The analysis of the EMB is crucial for a definitive diagnosis and thus for the initiation of an etiology-based, specific and personalized therapy. This includes histological and immunohistochemical inflammation diagnostics as well as molecular virological diagnostics. These enable both the detection of viruses and the assessment of transcriptional virus activity. New analyses using metagenomic next generation sequencing (NGS) techniques provide insights of enormous diagnostic and therapeutic relevance. This applies both to the spectrum of detectable pathogens and to the possibility of confirming transcriptional viral activity. In addition, gene expression profiling enables the differentiation of specific forms of myocardial inflammation (e.g., giant cell myocarditis, cardiac sarcoidosis, and eosinophilic myocarditis) and reduces the influence of “sampling errors” in focal inflammatory processes. The treatment of heart failure or ventricular arrhythmias is always symptomatic according to general evidence-based guidelines. In severe cases, mechanical circulatory support or even a heart transplant may be necessary. Patients with histologically confirmed myocardial inflammation or intramyocardial viral infection can be offered specific, causal, and personalized therapy. These patients can be successfully treated with immunosuppressive or antiviral therapy, which significantly improves the prognosis of the disease. Full article

Objective and Background: With the increasing use of catheter ablation for tachyarrhythmias, continuous evaluation of in-hospital complications is essential. This study aimed at analyzing complications associated with catheter ablation for atrial fibrillation (AF), atrial flutter (AFL), and ventricular tachycardia (VT) using nationwide administrative data. Methods: We conducted a retrospective multicentric data analysis from large German ablation centers between 2018 and 2023. Patients were identified using ICD and OPS codes for AF, AFL, and VT regarding predefined in-hospital complications: mortality, stroke, pericardial tamponade, pulmonary embolism, and vascular complications requiring intervention. Results: Among 19,258 ablation procedures from 11 centers, AF was most common (n = 12,241), followed by AFL (n = 5582) and VT (n = 1435). Major complications occurred in 2.2% (n = 433) of cases. VT ablations had the highest complication rate (9.8%), followed by AF (1.6%) and AFL (1.7%). Pericardial tamponade occurred in 0.9% patients, most commonly in VT ablations (4.0%). Vascular complications requiring intervention were reported in 1.1%, while stroke (0.3%) and pulmonary embolism (0.05%) were rare. In-hospital mortality was highest in VT patients (2.4%), compared to AF (0.08%) and AFL (0.13%). Higher AFL mortality as compared to AF was associated with older age and more comorbidities. Upon exploratory analysis, no statistical association between hospital volume and complication rates could be seen. Conclusions: In this multicenter analysis, catheter ablation was associated with a low overall complication rate. VT ablations carried the highest risk, highlighting the impact of structural heart disease and comorbidities. Full article

Background: SARS-CoV-2 infection, its late complications, and SARS-CoV-2 vaccines are known to cause pericardial effusion. We sought to investigate the influence of the COVID-19 outbreak on trends in pericardiocentesis. Methods: We performed a retrospective population study including all >18 years patients undergoing pericardiocentesis in a single tertiary hospital between January 2018 and April 2022. The effusion characteristics and patient outcomes were compared between patients admitted before and after the COVID-19 outbreak. Results: 92 patients underwent pericardiocentesis cases during the COVID-19 period compared to 65 patients during the pre-COVID-19 period (χ² = 3.07, p = 0.0796). Only 15% of the post-COVID-19 outbreak cases were related to COVID-19 infection or vaccine. In-hospital mortality was numerically higher during the post-COVID-19 group (7.7% vs. 14.4%), but this difference did not reach statistical significance (p = 0.22). The 90-day mortality was also similar between groups. Conclusions: A numerical, yet statistically insignificant increase in pericardiocentesis was observed following the COVID-19 outbreak. We assume this observation cannot be attributed solely to the virus and vaccines per se. Neglect of other chronic diseases, social distancing, and widespread availability of point-of-care ultrasound may have contributed to this observation. Full article