Prevalence of lymphedema among Anderson-Fabry disease patients: A report from the Fabry registry.

BACKGROUND: Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disease due to a genetic variation in the α-galactosidase A (GLA) gene. As a result, the activity of the α-galactosidase A (AGAL-A) enzyme is reduced or absent, which causes sphingolipid deposition within different body parts. AFD typically manifests with cardiovascular, renal, cerebrovascular, and dermatologic involvement. Lymphedema is caused by sphingolipid deposition within lymphatics. Lymphedema can cause intolerable pain and limit daily activities. Very limited data exist on lymphedema in AFD patients. METHODS: Using data from the Fabry Registry (NCT00196742) with 7671 patients included (44% males and 56% females), we analyzed the prevalence of lymphedema among AFD patients who were ever assessed for lymphedema and studied the age of first reported lymphedema. Additionally, we assessed whether patients received AFD-specific treatment at some point during their clinical course. The data was stratified by gender and phenotype. RESULTS: Our study showed that lymphedema occurred in 16.5% of the Fabry Registry patients who were ever assessed for lymphedema (n = 5487). Male patients when compared to female patient have higher prevalence (21.7% vs 12.7%) and experienced lymphedema at a younger age (median age at first reported lymphedema of 43.7 vs 51.7 years). When compared to other phenotypes, classic phenotype has the highest prevalence of lymphedema with the earliest reported lymphedema. Among those who reported lymphedema, 84.5% received AFD-specific treatment during their clinical course. CONCLUSIONS: Lymphedema is a common manifestation of AFD in both genders, with a tendency to present later in female patients. Recognition of lymphedema can offer an important opportunity for intervention and potential impact on associated morbidity. Additional future studies are needed to characterize the clinical implications of lymphedema in AFD patients and identify additional treatment options for this growing population.

The Roles of Coronary Computed Tomography Angiography in Characterizing Coronary Plaque: Screening, Treatment, and Prevention.

One of the major risk factors for coronary atherosclerosis is the gradual formation and maturation of coronary atherosclerotic plaque (CAP) [...].

In-hospital Outcomes and the Impact of Transfer Status in Nonelective vs Elective Transcatheter Aortic Valve Replacement.

Background: Nonelective transcatheter aortic valve replacement (TAVR) requires additional research to be fully understood. Methods: Using the National Inpatient Sample database (2016-2019), we conducted a retrospective cohort study comparing nonelective vs elective TAVR. The primary outcome of interest was the in-hospital mortality rate among patients undergoing nonelective TAVR, compared to that among patients undergoing elective TAVR. We matched patients in a greedy nearest-neighbor 1:1 model and multivariable logistic regression, which was adjusted for demographics, hospital factors, and comorbidities, and was used to compare mortality in our matched cohort. Results: Each cohort had 4389 patients in each cohort. When adjusted for age, race, sex, and comorbidities, nonelective TAVR patients had 1.99 times higher odds of suffering in-hospital mortality compared to elective admissions (adjusted odds ratio 1.99, 95% confidence interval: 1.42-2.81; P < 0.001). When separated by transfer status, nonelective patients admitted as regular hospital admissions or transferred from other acute-care centres also had higher odds of suffering in-hospital mortality compared to elective admissions. Conclusions: Our findings illustrate that nonelective TAVR patients are a vulnerable population that require additional medical support in the acute-care setting. As the demand for TAVR increases, further discussions regarding access to healthcare in underserved regions, the national physician shortage, and the future of the TAVR industry are imperative.

Contexte: Le remplacement valvulaire aortique par cathéter (RVAC) d'urgence nécessite plus de recherche pour être bien compris. Méthodologie: À partir de la base de données National Inpatient Sample (2016-2019), nous avons réalisé une étude rétrospective de cohortes comparant le RVAC non urgent et le RVAC d'urgence. Celle-ci avait pour principal critère d'évaluation la comparaison du taux de mortalité à l'hôpital chez les patients soumis à un RVAC d'urgence à celui noté chez ceux qui subissent un RVAC non urgent. Nous avons apparié les patients selon le modèle du plus proche voisin, avec un rapport 1:1, et utilisé une régression logistique multivariée, ajustée en fonction des caractéristiques démographiques, des facteurs hospitaliers et des affections concomitantes, pour comparer le taux de mortalité dans les cohortes appariées. Résultats: Chaque cohorte comportait 4 389 patients. Après correction pour tenir compte de l'âge, de l'origine ethnique, du sexe et des affections concomitantes, nous avons constaté que le risque des patients ayant subi un RVAC d'urgence de mourir à l'hôpital était 1,99 fois plus élevé que celui des patients chez qui un RVAC non urgent a été effectué (rapport des risques ajustés : 1,99; intervalle de confiance à 95 % : 1,42 à 2,81; p < 0,001). De plus, les patients chez qui l'intervention a été pratiquée d'urgence courraient également un risque plus élevé de décéder à l'hôpital que ceux soumis à un RVAC non urgent, qu'ils aient été admis directement à l'hôpital ou transférés d'autres centres de soins de courte durée. Conclusions: Nos conclusions montrent que les patients ayant subi un RVAC d'urgence forment une population vulnérable qui requiert un soutien médical supplémentaire dans un milieu de soins de courte durée. Comme la demande pour des RVAC augmente, d'autres discussions sur l'accès aux soins de santé dans les régions mal desservies, la pénurie nationale de médecins et l'avenir de l'industrie du RVAC s'imposent.

Primary Cardiac Sarcoma: Angiosarcoma Compressing the Right Coronary Artery.

Cardiac tumors are uncommon and can be classified as either primary benign, primary malignant, or metastatic. Cardiac tumors have a wide range of presentations, which can lead to delays in diagnosis and treatment. Primary cardiac tumors can also affect nearby structures, and there have been a few reported cases of coronary artery involvement with various underlying causes. In this case report, we describe a patient with a primary cardiac sarcoma (angiosarcoma) that had spread to other parts of the body and caused occlusion of the right coronary artery.

Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy resulting from a mutation in one of several cardiac sarcomeric proteins [...].

Novel Device Therapies for Heart Failure.

Heart failure (HF) therapeutics have advanced significantly over the past few years [...].

Impact of transfer from non-acute care centers on clinical outcomes in patients with congestive heart failure.

Study objective: To compare the clinical outcomes in patients with congestive heart failure who are transferred to an acute care hospital from non-acute care centers with patients who are admitted as regular hospital admissions. Design: This was a retrospective cohort study. Setting: We utilized the National Inpatient Sample database from 2016 to 2018. Participants: Our cohort consisted of hospitalized patients who were at least 18 years old with a primary diagnosis of congestive heart failure. Interventions: These patients were either transferred from non-acute centers or presented as regular hospital admissions. Main outcome measurements: We matched patients in a greedy nearest neighbor 1:1 model with caliper set at 0.2. Multivariable logistic regression, adjusted for age, sex, race and comorbidities, was used to compare mortality in our matched cohort. Results: This study included 35,010 non-acute care transfers and 951,189 regularly admitted patients. Compared to patients who were not transferred, non-acute care transfers were older, predominantly female, White and less racially diverse. After matching, there were 6689 patients in each cohort. When adjusted for age, race, sex and comorbidities, non-acute care transfers with congestive heart failure had 2.20 times higher odds of suffering in-hospital mortality compared to regular, non-transferred admissions (aOR 2.20, 95 % CI: 1.85-2.61; p < 0.001). Conclusion: Our findings illustrate that non-acute care transfers are a vulnerable population that require additional medical support in the acute care setting.

Cardiac Anesthesia Intraoperative Interpretation Accuracy of Transesophageal Echocardiograms: A Review of the Current Literature and Meta-Analysis.

Background: In the United States, echocardiography is an essential component of the care of many cardiac patients. Recently, increased attention has been given to the accuracy of interpretation of cardiac-based procedures in different specialties, amongst them the field of cardiac anesthesiology and primary echocardiographers for transesophageal echocardiogram (TEE). The purpose of this study was to assess the TEE skills of cardiac anesthesiologists in comparison to primary echocardiographers, either radiologists or cardiologists. In this systematic review, we evaluated available current literature to identify if cardiac anesthesiologists interpret TEE procedures at an identical level to that of primary echocardiographers. Methods: A PRISMA systematic review was utilized from PubMed from the years 1952-2022. A broad keyword search of "Cardiology Anesthesiology Echocardiogram" and "Echocardiography Anesthesiology" to identify the literature was used. From reviewing 1798 articles, there were a total of 9 studies included in our systematic review, 3 of which yielded quantitative data and 6 of which yielded qualitative data. The mean accuracy from each of these three qualitative studies was calculated and used to represent the overall accuracy of cardiac anesthesiologists. Results: Through identified studies, a total of 8197 TEEs were interpreted by cardiac anesthesiologists with a concordance rate of 84% to the interpretations of primary echocardiographers. Cardiac anesthesiologists had a concordance rate of 83% when compared to radiologists. On the other hand, cardiac anesthesiologists and cardiologists had a concordance rate of 87% in one study and 79% in another study. Conclusion: Based on these studies, cardiac anesthesiologists are shown to interpret TEEs similarly to that of primary echocardiographers. At this time, there is no gold standard to evaluate the accuracy of TEE readings. One way to address this is to individually assess the TEE interpretation of anesthesiologists and primary echocardiographers with a double-blind study.

Unusual Presentation of Thrombotic Thrombocytopenic Purpura With Non-ST-Elevation Myocardial Infarction.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disease characterized by disseminated thrombus formation in the arterioles and capillaries. Patients usually present with weakness, subtle mental changes, fever, and acute kidney injury. Cardiac symptoms, such as chest pain or arrhythmia, have been reported but were rarely the sole presenting symptom. We report the case of a 68-year-old woman with acute non-ST-elevation myocardial infarction who was found to have TTP. Prompt diagnosis of TTP is essential because traditional approaches to manage an acute coronary event, inclusive of dual antiplatelet therapy and percutaneous coronary intervention, might be contraindicated due to an increased risk of bleeding. Early administration of steroids and urgent initiation of plasmapheresis to improve platelet count would be crucial initial steps in the management of these patients.

A single-center retrospective study on the incidence and clinical significance of the electrocardiographic "Triangular QRS-ST-T Waveform" pattern*.

BACKGROUND: The triangular QRS-ST-T waveform is a rare presentation of ST-segment elevation acute myocardial infarction associated with a poor in-hospital prognosis. OBJECTIVE: To evaluate the incidence and clinical implications of the QRS-ST-T waveform pattern. METHODS: Clinical data from non-pregnant adult patients who presented as STEMI activations at a single institution between 2017 and 2021 were reviewed. Patients who met electrocardiographic criteria for triangular QRS-ST-T waveform - a giant wave from the fusion of the QRS complex, the ST-segment, and the T-wave - were included in the study. RESULTS: There were 417 STEMI activations, eight (1.9%) of which fulfilled the criteria for the triangular QRS-ST-T waveform pattern on electrocardiography. Coronary angiography was performed in five of these patients, four of whom demonstrated a significant lesion to the left anterior descending artery. Three patients did not undergo angiography secondary to hemodynamic instability. Seven of the patients in our study experienced cardiogenic shock requiring vasopressor, inotropic, and/or mechanical support. Only two patients survived to discharge; one was successfully bridged to coronary artery bypass grafting via intra-aortic balloon pump, while the other underwent a staged percutaneous coronary intervention. CONCLUSIONS: The triangular QRS-ST-T waveform pattern is a rare ECG finding that may indicate hyper-acute STEMI and is an ominous sign of impending hemodynamic instability. Patients who survived received prompt aggressive therapeutic management.

Clinical Characteristics and Implications of Bradycardia in COVID-19 Patients Treated with Remdesivir: A Single-Center Retrospective Cohort Study.

BACKGROUND AND OBJECTIVES: Remdesivir is an antiviral drug used to treat coronavirus disease 2019 (COVID-19) with a relatively obscure cardiac effect profile. Previous studies have reported bradycardia associated with remdesivir, but few have examined its clinical characteristics. The objective of this study was to investigate remdesivir associated bradycardia and its associated clinical characteristics and outcomes. METHODS: This is a single-institution retrospective study that investigated bradycardia in 600 patients who received remdesivir for treatment of COVID-19. A total of 375 patients were included in the study after screening for other known causes of bradycardia (atrioventricular [AV] nodal blockers). All patients were analyzed for episodes of bradycardia from when remdesivir was initiated up to 5 days after completion, a time frame based on the drug's putative elimination half-life. Univariate and multivariate statistical tests were conducted to analyze the data. RESULTS: The mean age of the sample was 56.63 ± 13.23 years. Of patients who met inclusion criteria, 49% were found to have bradycardia within 5 days of remdesivir administration. Compared to the cohort without a documented bradycardic episode, patients with bradycardia were significantly more likely to experience inpatient mortality (22% vs 12%, p = 0.01). The patients with bradycardia were found to have marginally higher serum D-dimer levels (5.2 vs 3.4 µg/mL, p = 0.05) and were more likely to undergo endotracheal intubation (28% vs 14%, p = 0.008). Male sex, hyperlipidemia, and bradycardia within 5 days of completing remdesivir were significant predictors of inpatient mortality. No significant differences in length of stay were found. CONCLUSIONS: Bradycardia that occurs during or shortly after remdesivir treatment in COVID-19 patients may be associated with an increased rate of in-hospital mortality. However, COVID-19 and its cardiac complications cannot be excluded as potential contributors of bradycardia in the present study. Future studies are needed to further delineate the cardiac characteristics of COVID-19 and remdesivir.