Blast from the Past: Acute Myeloid Leukemia Presenting with Cardiac Tamponade.

Acute Myeloid Leukemia (AML) is a life-threatening illness that requires prompt diagnosis and often immediate treatment. It can present in a variety of manners but most commonly is associated with fevers, fatigue, shortness of breath, or infection. Extramedullary leukemia is a less common finding upon initial presentation, but includes dermatologic manifestations, including leukemia cutis, and rarely, large mass-like presentations known as myeloid sarcomas. While leukemic infiltration of organ systems is a well-described phenomenon, cardiac tamponade is a rare form of presentation. Herein we describe a 58-year-old man with a recent hospitalization for idiopathic cardiac tamponade who re-presented to the hospital with worsening dyspnea and fevers. He was found to have a recurrent pericardial effusion with features concerning for tamponade, as well as worsening thrombocytopenia and macrocytic anemia. Bone marrow biopsy revealed 24% myeloblasts, confirming the diagnosis of AML. Notably, his cardiac symptoms improved with treatment of his leukemia. To our knowledge, this is one of only a few cases of AML with cardiac tamponade as the initial presentation.

Successful Visceral and Parietal Pericardiectomy for Refractory Effusive-Constrictive Pericarditis: A Challenging Case Report.

BACKGROUND Effusive-constrictive pericarditis (ECP) is an uncommon clinical syndrome characterized by the coexistence of pericardial effusion and constriction involving the visceral pericardium. This differs from constrictive pericarditis, which presents with thickening of the pericardium without effusions. Specific diagnostic criteria of ECP include the failure of right atrial pressure to decrease by 50% or reach a new level below 10 mmHg after normalization of intrapericardial pressure. CASE REPORT We present the case of a 32-year-old obese man with multiple comorbidities who initially presented with flu-like symptoms and pleural effusion with development of constrictive-like symptoms. Despite undergoing numerous pericardiocentesis and appropriate medical management, the patient's condition failed to improve, leading to the likely diagnosis of effusive-constrictive pericarditis. Cultures of pericardial fluid revealed E. -faecium, which required multiple antimicrobial therapy. Despite infection, the exact etiology of ECP remained unknown and likely idiopathic. Common causes of ECP include idiopathic, tuberculosis, cardiac surgery complications, radiation, or neoplasia. Ultimately, the patient underwent a pericardiectomy involving the visceral and parietal pericardium, resulting in hemodynamic stability and resolution of symptoms. CONCLUSIONS This case highlights the challenges in diagnosing and managing ECP, emphasizing the importance of considering surgical intervention in refractory cases. ECP initially presents as a pericardial effusion, often addressed through pericardiocentesis; however, in a small subset of patients, sustained symptoms and altered hemodynamics persist following pericardiocentesis, necessitating further evaluation and management. The success of pericardiectomy in our patient highlights the potential efficacy of surgical intervention in improving outcomes for patients with ECP.

Patterns and organ treatment response of Erdheim-Chester disease with cardiac involvement.

OBJECTIVE: To evaluate the heart response of Erdheim-Chester disease (ECD) through continuous follow-up within our large cohort, for which there is a lack of understanding. METHODS: We conducted a retrospective analysis of clinical data from patients with ECD with cardiac involvement diagnosed at our centre between January 2010 and August 2023. We assessed the heart response by integrating pericardial effusion and metabolic responses. RESULTS: A total of 40 patients were included, with a median age of 51.5 years (range: 29-66) and a BRAFV600E mutation rate of 56%. The most common imaging manifestations observed were pericardial effusion (73%), right atrium (70%) and right atrioventricular sulcus infiltration (58%). Among 21 evaluable patients, 18 (86%) achieved a heart response including 5 (24%) complete response (CR) and 13 (62%) partial response (PR). The CR rate of pericardial effusion response was 33%, while the PR rate was 56%. Regarding the cardiac mass response, 33% of patients showed PR. For cardiac metabolic response, 32% and 53% of patients achieved complete and partial metabolic response, respectively. There was a correlation between pericardial effusion response and cardiac metabolic response (r=0.73 (95% CI 0.12 to 0.83), p<0.001). The median follow-up was 50.2 months (range: 1.0-102.8 months). The estimated 5-year overall survival was 78.9%. The median progression-free survival was 59.4 months (95% CI 26.2 to 92.7 months). Patients who received BRAF inhibitors achieved better heart response (p=0.037) regardless of treatment lines. CONCLUSION: We pioneered the evaluation of heart response of ECD considering both pericardial effusion and cardiac metabolic response within our cohort, revealing a correlation between these two indicators. BRAF inhibitors may improve heart response, regardless of the treatment lines.

Fetal cardiac teratoma and pericardiocentesis: A case report.

Fetal pericardial teratomas are rare. They present with pericardial effusion and hydrops. The definitive management is postnatal resection of the tumor. The exact antenatal management is not known due to its rarity. We present a case of fetal pericardial teratoma with pericardial tamponade. Pericardiocentesis performed at 31 weeks significantly relieved the venous compression, leading to resolution of hydrops and prolonging the gestational age for the definitive management.

Pericardial Effusion After Left Atrial Appendage Closure: Timing, Predictors, and Clinical Impact.

BACKGROUND: Pericardial effusion (PE) is the most common serious left atrial appendage closure (LAAC) complication, but its mechanisms, time course, and prognostic impact are poorly understood. OBJECTIVES: This study sought to assess the frequency, timing, predictors and clinical impact of PE after LAAC. METHODS: Data on consecutive patients undergoing percutaneous LAAC between 2009 and 2022 were prospectively collected including the 1-year follow-up. Both single (Watchman 2.5/FLX, Boston Scientific) and double (Amplatzer Cardiac Plug or Amulet, St. Jude Medical/Abbott) LAAC devices were used. An imaging core laboratory adjudicated the PEs and categorized them as early (≤7 days) and late (8-365 days). Logistic regression analysis was used to identify predictors of early and overall PE. RESULTS: Of 1,023 attempted LAAC procedures, PE was observed in 44 (4.3%) patients; PE was categorized as early in 34 (3.3%) and late in 10 (0.9%) patients. The majority of PEs occurred within 6 hours after LAAC (n = 25, 56.8%) and were clinically relevant (n = 28, 63.6%). Independent predictors of early PE were double-closure left atrial appendage devices (adjusted OR: 8.20; 95% CI: 1.09-61.69), female sex (adjusted OR: 3.41; 95% CI: 1.50-7.73), the use of oral anticoagulation (OAC) at baseline (adjusted OR: 2.60; 95% CI: 1.11-6.09), and advanced age (adjusted OR: 1.07; 95% CI: 1.01-1.23), whereas female sex and OAC at baseline remained independent predictors of overall PE. CONCLUSIONS: In this large LAAC registry, PE was observed in <1 in 20 patients and usually occurred within 6 hours after procedure. The majority of early PEs were clinically relevant and occurred in the Amplatzer Cardiac Plug/Amulet procedures. Independent predictors included the use of double-closure devices, female sex, OAC at baseline, and advanced age. (LAAC-registry: Clinical Outcome After Echocardiography-guided LAA-closure; NCT04628078).

Descriptions of Etiology, Clinical Course, and Prognosis of Patients Presenting with Pericardial Effusion.

Pericardial effusion (PE) presentation varies from an incidental finding to a life-threatening situation; thus, its etiology and clinical course remain unknown. The aim of the present study was to retrospectively investigate these factors.We analyzed 171 patients (0.4%) who presented with PE among 34,873 patients who underwent echocardiography between 2011 and 2021 at our hospital. Clinical and prognostic information was retrieved from electronic medical records. The primary endpoints were all-cause death, hospitalization due to heart failure (HF), and other cardiovascular events such as cardiovascular death, acute coronary syndrome, elective percutaneous coronary intervention, and stroke.The etiologies of PE were as follows: idiopathic (32%), HF-related (18%), iatrogenic (11%), cardiac surgery-related (10%), radiation therapy-related (9%), malignancy (8%), pericarditis/myocarditis (8%), myocardial infarction-related (2%), and acute aortic dissection (2%). Patients with idiopathic/HF etiology were more likely to be older than the others.During a mean follow-up period of 2.5 years, all-cause death occurred in 21 patients (12.3%), cardiovascular events in 10 patients (5.8%), and hospitalization for HF in 24 patients (14.0%). All-cause death was frequently observed in patients with malignancy (44% per person-year). Cardiovascular events were mostly observed in patients with radiation therapy-related and malignancy (8.6% and 7.3% per person-year, respectively).The annual incidence of hospitalization for HF was the highest in patients with HF-related (25.1% per person-year), followed by radiation therapy-related (10.4% per person-year).This retrospective study is the first, to the best of our knowledge, to reveal the contemporary prevalence of PE, its cause, and outcome in patients who visited a cardiovascular hospital in an urban area of Japan.

Acute Pericarditis: Rapid Evidence Review.

Acute pericarditis is defined as inflammation of the pericardium and occurs in approximately 4.4% of patients who present to the emergency department for nonischemic chest pain, with a higher prevalence in men. Although there are numerous etiologies of pericarditis, most episodes are idiopathic and the cause is presumed to be viral. Diagnosis of pericarditis requires at least two of the following criteria: new or worsening pericardial effusion, characteristic pleuritic chest pain, pericardial friction rub, or electrocardiographic changes, including new, widespread ST elevations or PR depressions. Pericardial friction rubs are highly specific but transient, and they have been reported in 18% to 84% of patients with acute pericarditis. Classic electrocardiographic findings include PR-segment depressions; diffuse, concave, upward ST-segment elevations without reciprocal changes; and T-wave inversions. Transthoracic echocardiography should be performed in all patients with acute pericarditis to characterize the size of effusions and evaluate for complications. Nonsteroidal anti-inflammatory drugs are the first-line treatment option. Glucocorticoids should be reserved for patients with contraindications to first-line therapy and those who are pregnant beyond 20 weeks' gestation or have other systemic inflammatory conditions. Colchicine should be used in combination with first- or second-line treatments to reduce the risk of recurrence. Patients with a higher risk of complications should be admitted to the hospital for further workup and treatment.

Symptomatic Pericardial Effusion Associated With Graves' Disease in a Pediatric Patient.

Pericarditis is a well-known complication of hypothyroidism. Although pericarditis and pericardial effusions have been reported as rare complications of hyperthyroidism in adults, they are rarely reported in the pediatric population. In this case report, we describe a 12-year-old, previously healthy girl with nighttime chest pain, dyspnea, tachycardia, and abnormal thyroid function studies consistent with hyperthyroidism who was found to have pericarditis and pericardial effusion requiring pericardiocentesis.

[Repeated Pericardial Effusion Leading to the Diagnosis of Synovial Sarcoma:Report of a Case].

The patient is a 76-year-old man. His chief complaint of chest pain led to a diagnosis of pericardial effusion of unknown cause, and pericardial drainage was performed. On the 30th day, chest pain appeared again. Echocardiography revealed a pericardial fluid reaccumulation and a substantial mass in the pericardial space. Surgical drainage was performed to find the cause. A hematoma/mass was present on the epicardium. The pericardial sac was filled with hematoma. The hematoma was removed, but part of the mass infiltrated close to the anterior descending branch of the left coronary artery, and removal of that part was abandoned. The intrapericardial hematoma and epicardium were submitted to pathology leading to the diagnosis of synovial sarcoma. The patient was discharged home 14 days after surgery.

Screening Tests Predicting Cancer Metastasis in the Etiology of Pericardial Effusion: HALP Score and PNI. / Testes de Triagem Prevendo Metástase de Câncer na Etiologia do Derrame Pericárdico: HALP Score e PNI.

BACKGROUND: Cancer screening is absolutely necessary in patients with pericardial effusion, given that cancer is one of the most serious diseases in the etiology of pericardial effusion. In previous studies, it was stated that the systemic immune-inflammation index (SII); the prognostic nutrition index (PNI); and the hemoglobin, albumin, lymphocyte, platelet (HALP) score can produce scores related to cancer. OBJECTIVES: This study began considering that these scoring systems could predict cancer in the etiology of patients with pericardial effusion. METHODS: This study produced a retrospective analysis of patients who underwent pericardiocentesis between 2006 and 2022. Pericardiocentesis was performed in a total of 283 patients with moderate-to-large pericardial effusion or pericardial tamponade within the specified period. HALP, PNI, and SII scores were calculated according to the peripheral venous blood taken before the pericardiocentesis procedure. The statistical significance level was set at p<0.05. RESULTS: The HALP score proved to be 0.173 (0.125-0.175) in cancer patients and 0.32 (0.20-0.49) in non-cancer patients (p<0.001). The PNI score proved to be 33.1±5.6 in cancer patients and 39.8±4.8 in non-cancer patients (p<0.001). CONCLUSION: The HALP score and PNI proved to be easy and fast cancer screening tests that can predict cancer metastasis in the etiology of patients with pericardial effusion.

FUNDAMENTO: A triagem do câncer é absolutamente necessária em pacientes com derrame pericárdico, pois o câncer é uma das doenças mais graves em sua etiologia. Estudos anteriores indicaram que o índice de inflamação imunológica sistêmica (IIS), o índice prognóstico nutricional (PNI) e o escore de hemoglobina, albumina, linfócitos e plaquetas (HALP) podem ser escores relacionados ao câncer. OBJETIVOS: Este estudo foi iniciado considerando que esses sistemas de pontuação poderiam prever o câncer na etiologia de pacientes com derrame pericárdico. MÉTODOS: Os pacientes submetidos à pericardiocentese entre 2006 e 2022 foram analisados retrospectivamente. A pericardiocentese foi realizada em um total de 283 pacientes com derrame pericárdico ou tamponamento cardíaco de moderado a grande no período especificado. Os índices de HALP, PNI e IIS foram calculados do sangue venoso periférico retirado antes do procedimento de pericardiocentese. O nível de significância estatística foi aceito em p<0,05. RESULTADOS: O escore HALP foi de 0,173 (0,125-0,175) em pacientes com câncer. Detectou-se que em pacientes não oncológicos o escore foi de 0,32 (0,20-0,49; p<0,001). O escore de PNI foi de 33,1±5,6 em pacientes com câncer. Detectou-se que em pacientes não oncológicos o escore foi 39,8±4,8 (p<0,001). CONCLUSÃO: Os escores HALP e PNI são testes de triagem de câncer fáceis e rápidos que podem prever metástases de câncer na etiologia de pacientes com derrame pericárdico.

Acute pericarditis as the presenting symptom of a case of oesophageal carcinoma.

Oesophageal carcinoma is a globally prevalent form of cancer. Patients with advanced disease often experience progressive dysphagia and weight loss as initial symptoms, but pericarditis is an uncommon presentation. This study describes a young man who presented with pericarditis and was diagnosed with oesophageal squamous cell carcinoma. The patient's diagnosis came after presenting with intermittent chest pain. His diagnostic tests included an ECG showing ST elevation, echocardiography showing pericardial effusion and elevated inflammatory markers. His imaging tests revealed a neoplastic lesion in the lower oesophagus with metastases. He was initially treated as a case of pericarditis, followed by palliative chemotherapy for his cancer. Pericarditis, as the initial presentation of oesophageal carcinoma, is rare. There have only been 19 cases reported and published in the literature. Treatment depends on the stage of the disease. This case emphasises the importance of considering malignancy in unusual presentations of pericarditis, especially in young patients.

Multisystem inflammatory syndrome in children (MIS-C) post-COVID-19 in Iran: clinical profile, cardiac features, and outcomes.

BACKGROUND: In April 2020, an association between multisystem inflammatory syndromes (MIS-C) was observed in children with severe acute respiratory syndrome coronavirus infection (SARS-CoV-2). Most patients had heart involvement alone, and most patients had pericardial effusion. This study aimed to express and emphasize cardiac involvement in pediatric patients with respiratory symptoms who were diagnosed with COVID-19. METHODS: This study was conducted in July 2021 in Kerman province, Southeastern Iran, during a notable surge in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections. The study included 904 pediatric patients diagnosed with COVID-19. Data collection involved a comprehensive assessment of clinical symptoms and manifestations. Patients with fever lasting more than five days were admitted to the hospital. Echocardiography was utilized for cardiac involvement diagnosis, with 47 patients undergoing this diagnostic procedure. RESULTS: Of the 904 patients, most of them had high fevers (74%). Fifty-five patients had a fever for more than five days and were hospitalized. Of the 47 patients who underwent echocardiography, 45 (81%) had heart involvement. In 75% of patients, pericardial effusion was the only cardiac involvement. Patients with pericardial effusion were treated with dexamethasone up to 3 mg every 8 h for 72 h. CONCLUSIONS: MIS-C has a wide range of clinical symptoms. In cases where the fever is prolonged and there are gastrointestinal symptoms, physicians have clinical suspicion to diagnose this syndrome. Most cases of pericardial effusion are alone and improve with treatment with glucocorticosteroids.

Chylopericardium following esophagectomy: a case report and systematic review.

BACKGROUND: Chylopericardium is a rare condition characterized by the accumulation of chyle in the pericardial space. It is most commonly caused by thoracic duct injury. Chylopericardium following esophagectomy is extremely rare but can cause life-threatening complications. This report presents a case of chylopericardium post-esophagectomy, resulting in cardiac tamponade and cardiac arrest. A systematic literature review was also conducted to facilitate the understanding of this rare condition. CASE PRESENTATION: A 41-year-old male was admitted to our hospital with intermediate to highly differentiated squamous cell carcinoma of the mid-thoracic esophagus (clinical T4NxM0). He underwent thoracoscopic-laparoscopic esophagectomy with cervical anastomosis. On postoperative day 1, patient had a cardiac arrest secondary to cardiac tamponade, requiring emergency ultrasound-guided drainage. The drained fluid was initially serous but became chylous after the administration of enteral nutritional emulsion. As a result of significant daily pericardial drainage, patient subsequently underwent thoracic duct ligation. The amount of drainage was substantially reduced post-thoracic duct ligation. Over a period of 2 years and 7 months, patient recovered well and tolerated full oral diet. A comprehensive literature review was conducted and 4 reported cases were identified. Among these cases, three patients developed pericardial tamponade secondary to chylopericardium post-esophagectomy. CONCLUSION: Chylopericardium is a rare but serious complication post-esophagectomy. Prompt echocardiography and thorough pericardial fluid analysis are crucial for diagnosis. Thoracic duct ligation has been shown to be an effective management approach for this condition.

Multisystem Erdheim-Chester disease presenting with pericardial effusion confirmed by the effusion cytology specimen.

Erdheim-Chester disease (ECD) is a rare histiocytosis characterized by the foamy CD68+CD1a- histiocytes infiltrating multiple organs and tissues. ECD might be asymptomatic or present with variable manifestations. The diagnosis of ECD requires characteristic radiological findings and pathological features. Herein, we described a 52-year-old female patient who was admitted to our hospital for recurrent pericardial effusion for two months. She has a medical history of papillary thyroid carcinoma (PTC) and underwent a total thyroidectomy two years before admission. The radiological findings suggested a potential diagnosis of ECD. Cytological analysis of the effusion cytology specimen revealed CD68+CD1a- histiocytes, confirming the ECD diagnosis. The BRAF V600E mutation was identified in the histiocytes, prompting the administration of vemurafenib, a BRAF inhibitor. After two months of standard-dose vemurafenib treatment, the disease was well controlled with pericardial effusion regression.

Pericardiocentesis versus window formation in malignant pericardial effusion: trends and outcomes.

OBJECTIVES: Malignant pericardial effusion (MPE) in patients with cancer is associated with poor prognosis. This study aimed to compare clinical outcomes in patients with cancer who underwent pericardiocentesis versus pericardial window formation. METHODS: In the present study, 765 consecutive patients with cancer (mean age 58.4 years, 395 men) who underwent pericardial drainage between 2003 and 2022 were retrospectively analysed. All-cause death and MPE recurrence were compared based on the drainage method (pericardiocentesis vs pericardial window formation) and time period (period 1: 2003-2012; period 2: 2013-2022). RESULTS: Pericardiocentesis was performed in 639 (83.5%) patients and pericardial window formation in 126 (16.5%). There was no difference in age, sex distribution, proportion of metastatic or relapsed cancer, and chemotherapy status between the pericardiocentesis and pericardial window formation groups. Difference was not found in all-cause death between the two groups (log-rank p=0.226) regardless of the period. The pericardial window formation group was associated with lower MPE recurrence than the pericardiocentesis group (6.3% vs 18.0%, log-rank p=0.001). This advantage of pericardial window formation was more significant in period 2 (18.1% vs 1.3%, log-rank p=0.005). In multivariate analysis, pericardial window formation was associated with lower MPE recurrence (HR: 0.31, 95% CI: 0.15 to 0.63, p=0.001); younger age, metastatic or relapsed cancer, and positive malignant cells in pericardial fluid were associated with increased recurrence. CONCLUSION: In patients undergoing pericardial drainage for MPE, pericardial window formation showed mortality outcomes comparable with pericardiocentesis and was associated with lower incidence of MPE recurrence.

Cardiac Tuberculosis: A Case Series from Ethiopia, Italy, and Uganda and a Literature Review.

Extrapulmonary tuberculosis (TB) is estimated to account for up to 20% of active cases of TB disease, but its prevalence is difficult to ascertain because of the difficulty of diagnosis. Involvement of the heart is uncommon, with constrictive pericarditis being the most common cardiac manifestation. Diagnostic research for cardiac disease is frequently lacking, resulting in a high mortality rate. In addition to direct cardiac involvement, instances of cardiac events during antitubercular therapy are described. This case series describes five cases of TB affecting the heart (cardiac TB) from Italy and high-burden, low-income countries (Ethiopia and Uganda), including a case of Loeffler syndrome manifesting as myocarditis in a patient receiving antitubercular therapy. Our study emphasizes how cardiac TB, rare but important in high-burden areas, is a leading cause of pericardial effusion or pericarditis. Timely diagnosis and a comprehensive approach, including imaging and microbiological tools, are crucial. Implementing high-sensitivity methods and investigating alternative samples, such as detection of tuberculosis lipoarabinomannan or use of the GeneXpert assay with stool, is recommended in TB control programs.