UNASSIGNED: This study aims to elucidate the clinical features observed in cases of pediatric acute myeloid leukemia (AML) initially presenting with cardiac tamponade and to share treatment experiences.
UNASSIGNED: Five pediatric patients were initially diagnosed with AML accompanied by cardiac myeloid sarcoma (MS). The diagnosis was established by examining our hospital records and reviewing pertinent literature from 1990 to July 2023, accessible through MEDLINE/PubMed. We comprehensively assessed the clinical characteristics and treatment modalities employed for these patients.
UNASSIGNED: Five pediatric patients presented with acute symptoms, including shortness of breath, malaise, cough, and fever, leading to their hospitalization. Physical examination revealed irritability, hypoxia, tachypnea, tachycardia, and hypotension. Initial detection utilized chest X-ray or echocardiogram, leading to subsequent diagnoses based on pericardial effusion and/or bone marrow examination. Two patients received chemotherapy at the time of initial diagnosis, one with cytarabine and etoposide, and the other with cytarabine and cladribine. Post-treatment, their bone marrow achieved remission, and over a 2.5-year follow-up, their cardiac function remained favorable. Unfortunately, the remaining three patients succumbed within two weeks after diagnosis, either due to receiving alternative drugs or without undergoing chemotherapy.
UNASSIGNED: This is the first and largest case series of pediatric AML patients with cardiac MS, manifesting initially with cardiac tamponade. It highlights the rarity and high mortality associated with this condition. The critical factors for reducing mortality include identifying clinical manifestations, conducting thorough physical examinations, performing echocardiography promptly, initiating early and timely pericardial drainage, and avoiding cardiotoxic chemotherapy medications.

暂无摘要。

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.
CONCLUSIONS: 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.

暂无摘要。

OBJECTIVE: Acute type A aortic dissection (ATAAD) is a life-threatening cardiovascular disease that requires an effective predictive model to predict and assess a patient\'s risk of death. Our study aimed to construct a model for predicting the risk of 30-day death in patients with ATAAD and the prediction accuracy of the German Registry of Acute Aortic Dissection Type A (GERAADA) Score and the European System for Cardiac Operative Risk Evaluation (EuroSCORE II) was verified.
METHODS: Between June 2019 and June 2023, 109 patients with ATAAD underwent surgical treatment at our hospital (35 in the death group and 74 in the survival group). The differences in image parameters between the two groups were compared. Search for independent predictors and develop models that predict 30-day mortality in patients with ATAAD. GERAADA Score and EuroSCORE II were retrospectively calculated and indicated mortality was assessed using the receiver operating characteristic (ROC) curve.
RESULTS: Logistic regression analysis showed that ascending aortic length and pericardial effusion were independent predictors of death within 30 days in patients with ATAAD. We constructed four models, GERAADA Score (Model 1), EuroSCORE II (Model 2), Model 1, ascending aorta length, and pericardial effusion (Model 3), and Model 2, ascending aorta length, and pericardial effusion (Model 4). The area under the curve (AUC = 0.832) of Model 3 was significantly different from those of Models 1 (AUC = 0.683) and 2 (AUC = 0.599), respectively (p < 0.05, DeLong test).
CONCLUSIONS: Adding ascending aorta length and pericardial effusion to the GERAADA Score can improve the predictive power of 30-day mortality in patients with ATAAD.

Transesophageal echocardiography (TEE) shows pericardial effusion and a gap between the left atrium and the aortic sinus by atrial septal defect occluder.

UNASSIGNED: The right atrial aneurysm is a rare cardiac malformation of unknown origin. It is typically asymptomatic but can occasionally lead to life-threatening and serious complications.
UNASSIGNED: We present a case of a right atrial aneurysm in an eight-year-old child who experienced complications including rupture of the atrial aneurysm, thrombosis, and recurrent large pericardial effusions over a one-month period. Following surgical treatment, the child had a favorable prognosis.
UNASSIGNED: A congenital right atrial aneurysm may manifest as either a widespread enlargement of the right atrium or a localized, smaller sac-like protrusion. In the latter case, diagnosis can be challenging to confirm through transthoracic echocardiography alone, and may require a cardiac computed tomography angiography examination for a definitive diagnosis. For patients experiencing recurrent large volumes of bloody pericardial effusion within one month, and exhibiting no atrial enlargement but showing abnormalities of the atrial wall in echocardiography, it is important to be vigilant about the potential for atrial aneurysm rupture in the heart. Timely treatment is essential to prevent the progression of the condition, which could otherwise result in a poor prognosis.

Primary cardiac angiosarcoma is a rare malignant soft-tissue sarcoma derived from vascular endothelial cells or lymphatic endothelial cells, with a high malignancy, poor prognosis, and a lack of effective medical therapy. This article reports on two patients with primary cardiac angiosarcoma who received first-line treatment with multi-targeted anti-angiogenic agent, anlotinib monotherapy. The treatment rapidly controlled pleural and pericardial effusion, significantly reduced the tumor, improved symptoms, and showed satisfactory recent efficacy. This indicates that anlotinib offers a new first-line treatment option for advanced primary cardiac angiosarcoma.

BACKGROUND: Programmed cell death 1 (PD-1) inhibitors can induce various adverse reactions associated with immunity, of which cardiotoxicity is a serious complication. Limited research exists on the link between PD-1 inhibitor use and pericardial effusion (PE) occurrence and outcomes.
METHODS: We conducted a retrospective study at the First Affiliated Hospital of Xi\'an Jiaotong University from 2017 to 2019, comparing cancer patients who developed PE within 2 years after PD-1 inhibitor therapy to those who did not. Our primary outcome was the all-cause mortality rate at one year. We applied the Kaplan-Meier method for survival analysis. Multivariate logistic regression was utilized to identify PE risk factors, adjusting for potential confounders.
RESULTS: A total of 91 patients were finally included, of whom 39 patients had PE. Compared to non-PE group, one-year all-cause mortality was nearly 5 times higher in PE group (64.10% vs. 13.46%, P < 0.001). Patients who developed PE within 2 years of taking PD-1 inhibitors were significantly associated with increased all-cause mortality compared with those who did not (HR: 6.26, 95%CI: 2.70-14.53, P < 0.001). Multivariable logistic regression showed that use of sintilimab (OR: 14.568, 95%CI: 3.431-61.857, P < 0.001), history of lung cancer (OR: 15.360, 95%CI: 3.276-72.017, P = 0.001), and history of hypocalcemia (OR: 7.076, 95%CI: 1.879-26.649, P = 0.004) were independent risk factors of PE development in patients received PD-1 inhibitors therapy.
CONCLUSIONS: In cancer patients receiving PD-1 inhibitors, PE was associated with higher one-year mortality. Use of sintilimab, and history of lung cancer or hypocalcemia were linked to PE occurrence.

OBJECTIVE: Systemic lupus erythematosus (SLE) is a multisystem-involved, highly heterogeneous autoimmune disease with diverse clinical manifestations. We report an extremely rare case of SLE with severe diffuse myocardial hypertrophy.
METHODS: The patient\'s echocardiography and cardiac magnetic resonance imaging (CMR) results indicated diffuse myocardial hypertrophy. After excluding coronary atherosclerosis, hypertensive cardiomyopathy, drug toxicity, and other causes, the patient was diagnosed with SLE-specific cardiomyopathy. Medications such as hormones, antimalarials, immunosuppressants, and biologics were administered.
RESULTS: Ancillary test results were as follows: hs-cTnI: 0.054 ng/mL (0-0.016); NTproBNP: 1594.0 pg/mL (<150); A contrast-enhanced CMR revealed the diffuse thickening of the left ventricular wall with multiple abnormal enhancements, reduced left ventricular systolic and diastolic function, and moderate amount of pericardial effusion. Endomyocardial myocardial biopsy was performed, showing cardiomyocyte hypertrophy and degeneration, and no changes in myocarditis or amyloidosis. The pathology viewed by electron microscopy showed increased intracellular glycogen in the myocardium, and no hydroxychloroquine-associated damage in the myocardium. The 24-h ambulatory blood pressure and contrast-enhanced computed tomography of coronary arteries were normal. The diagnosis of SLE-specific cardiomyopathy was clear. The myocardial hypertrophy showed reversible alleviation following treatment with high-dose corticosteroids. CMR results before and after treatment were as follows: interventricular septum, pretreatment (28) versus post-treatment (22) mm; left ventricular inferior wall, pretreatment (18-21) versus post-treatment (12-14) mm; left ventricular lateral wall, pretreatment (17-18) versus post-treatment (10-12) mm; pericardial effusion (left ventricular lateral wall), pretreatment (25) versus post-treatment (12) mm; left ventricular ejection fraction, pretreatment (38.9%) versus post-treatment (66%).
CONCLUSIONS: Myocardial hypertrophy may be an important sign of active and prognostic assessment in SLE diagnosis and management. Similarly, when encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to common causes.