A Machine Learning-Derived Score to Effectively Identify Heart Failure With Preserved Ejection Fraction.

Background: The diagnosis of heart failure with preserved ejection fraction (HFpEF) in the clinical setting remains challenging, especially in patients with obesity.

Objectives: This study aimed to identify novel predictors of HFpEF well suited for patients with obesity.

Methods: We performed a retrospective analysis of a well-characterized cohort of patients with obesity with HFpEF (n = 404; mean body mass index [BMI] 36.

B cell-mediated antigen presentation promotes adverse cardiac remodeling in chronic heart failure.

Cardiovascular disease remains the leading cause of death worldwide. A primary driver of cardiovascular mortality is ischemic heart failure, a form of cardiac dysfunction that can develop in patients who survive myocardial infarction. Acute cardiac damage triggers robust changes in the spleen with rapid migration of immune cells from the spleen to the heart.

B cell-mediated antigen presentation promotes adverse cardiac remodeling in chronic heart failure.

Cardiovascular disease remains the leading cause of death worldwide. A primary driver of cardiovascular mortality is ischemic heart failure, a form of cardiac dysfunction that can develop in patients who survive myocardial infarction. Acute cardiac damage triggers robust changes in the spleen with rapid migration of immune cells from the spleen to the heart.

Myocardial B cells have specific gene expression and predicted interactions in dilated cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy.

Introduction: Growing evidence from animal models indicates that the myocardium hosts a population of B cells that play a role in the development of cardiomyopathy. However, there is minimal data on human myocardial B cells in the context of cardiomyopathy.

Methods: We integrated single-cell and single-nuclei datasets from 45 healthy human hearts, 70 hearts with dilated cardiomyopathy (DCM), and 8 hearts with arrhythmogenic right ventricular cardiomyopathy (ARVC).

Myocardial B cells have specific gene expression and predicted interactions in Dilated Cardiomyopathy and Arrhythmogenic Right Ventricular Cardiomyopathy.

Introduction: Growing evidence from animal models indicates that the myocardium hosts a population of B cells that play a role in the development of cardiomyopathy. However, there is minimal data on human myocardial B cells in the context of cardiomyopathy.

Methods: We integrated single-cell and single-nuclei datasets from 45 healthy human hearts, 70 hearts with dilated cardiomyopathy (DCM), and 8 hearts with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC).

Serum Proteomic Analysis of Peripartum Cardiomyopathy Reveals Distinctive Dysregulation of Inflammatory and Cholesterol Metabolism Pathways.

Background: The pathophysiology of peripartum cardiomyopathy (PPCM) and its distinctive biological features remain incompletely understood. High-throughput serum proteomic profiling, a powerful tool to gain insights into the pathophysiology of diseases at a systems biology level, has never been used to investigate PPCM relative to nonischemic cardiomyopathy.

Objectives: The aim of this study was to characterize the pathophysiology of PPCM through serum proteomic analysis.

Myocardial Immune Cells: The Basis of Cardiac Immunology.

The mammalian heart is characterized by the presence of striated myocytes, which allow continuous rhythmic contraction from early embryonic development until the last moments of life. However, the myocardium contains a significant contingent of leukocytes from every major class. This leukocyte pool includes both resident and nonresident immune cells.

Myocarditis in Patients With Idiopathic Inflammatory Myopathies: Clinical Presentation and Outcomes.

Objective: To determine the clinical phenotype and outcomes of patients with idiopathic inflammatory myopathies (IIMs) and myocarditis.

Methods: Using the Johns Hopkins Myositis Center Research Registry, we identified 31 adult patients with IIM-out of a total of 3082 with confirmed or suspected muscle disease-with an encounter code of myocarditis from 2004 to 2021. Of these, 14 adult patients with IIM were adjudicated to have clinical myocarditis.

COVID-19 and Myocarditis: Review of Clinical Presentations, Pathogenesis and Management.

There are four main myocarditis presentations identified in the context of severe acute respiratory coronavirus 2 (SARS-CoV-2): myocarditis associated with acute coronavirus disease 2019 (COVID-19) infection, post-acute COVID-19 syndrome, multisystem inflammatory syndrome, and vaccination-associated myocarditis. This article reviews the clinical features and current management strategies for each of these presentations. The overall prevalence of myocarditis is considered to be rare, although accurate estimation is affected by heterogeneity in diagnostic criteria and reporting, as well as infrequent use of gold-standard diagnostic endomyocardial biopsy.

Large Atrial Septal Aneurysm Associated with Secundum Atrial Septal Defect.

• A structured multimodality approach is imperative in evaluating interatrial anatomy. • Secundum ASDs may be associated with ASA. • Interatrial shunts may be missed, especially when aneurysms are large.

Coronary computed tomography angiography in patients with stable coronary artery disease.

The treatment of coronary artery disease (CAD), which is defined by stable anatomical atherosclerotic and functional alterations of epicardial vessels or microcirculation, focuses on managing intermittent angina symptoms and preventing major adverse cardiovascular events with optimal medical therapy. When patients with known CAD present with angina and no acute coronary syndrome, they have historically been evaluated with a variety of noninvasive stress tests that utilize electrocardiography, radionuclide scintigraphy, echocardiography, or magnetic resonance imaging for determining the presence and extent of inducible myocardial ischemia. Patient event-free survival, however, is largely driven by the coronary atherosclerotic disease burden, which is not directly assessed by functional testing.

Pulmonary Manifestations of GATA2 Deficiency.

Background: GATA2 deficiency is a genetic disorder of hematopoiesis, lymphatics, and immunity caused by autosomal dominant or sporadic mutations in GATA2. The disease has a broad phenotype encompassing immunodeficiency, myelodysplasia, leukemia, and vascular or lymphatic dysfunction as well as prominent pulmonary manifestations.

Research Question: What are the pulmonary manifestations of GATA2 deficiency?

Study Design And Methods: A retrospective review was conducted of clinical medical records, diagnostic imaging, pulmonary pathologic specimens, and tests of pulmonary function.

Mediastinal and Disseminated Mycobacterium kansasii Disease in GATA2 Deficiency.

Rationale: Mycobacterium kansasii usually causes chronic pulmonary infections in immunocompetent patients. In contrast, disseminated M. kansasii disease is commonly associated with advanced human immunodeficiency virus infection, but is reported infrequently in other immunocompromised patients.

Persistent nodal histoplasmosis in nuclear factor kappa B essential modulator deficiency: Report of a case and review of infection in primary immunodeficiencies.

In the absence of acquired or secondary immunosuppression, mutations causing failure to properly activate the IL-12/IFN-γ pathway, NF-κB, or STAT3 should be excluded in patients presenting with severe histoplasmosis.

Association of GATA2 Deficiency With Severe Primary Epstein-Barr Virus (EBV) Infection and EBV-associated Cancers.

Background: Most patients infected with Epstein-Barr virus (EBV) are asymptomatic, have nonspecific symptoms, or have self-limiting infectious mononucleosis. EBV, however, may result in severe primary disease or cancer.

Methods: We report EBV diseases associated with GATA2 deficiency at one institution and describe the hematology, virology, and cytokine findings.