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Waldenström Macroglobulinemia-Induced Cardiac Amyloid Light Chain Amyloidosis.
Ochsner J
January 2024
Division of Cardiology, Baylor Scott & White Medical Center, Temple, TX.
Waldenström macroglobulinemia is a rare cancer of plasma cells characterized by the excessive production of immunoglobulin M (IgM). IgM-associated systemic amyloid light chain (AL) amyloidosis is a rare complication of Waldenström macroglobulinemia, characterized by the misfolding of lambda light chains that deposit in various organs, including the heart. We describe a case of progressive nonischemic cardiomyopathy secondary to Waldenström macroglobulinemia and IgM-associated AL amyloidosis that was refractory to medical therapy and highlight the challenges in diagnosis and management.
View Article and Find Full Text PDFPediatric eosinophilic granulomatosis with polyangiitis and intracardiac thrombus: A case report.
SAGE Open Med Case Rep
December 2024
Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, AB, Canada.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis marked by eosinophilia and extravascular granulomas, predominantly affecting the respiratory tract. This report details a unique EGPA case in a 6-year-old girl with extensive cardiac involvement, featuring an atypical intracardiac mass suggestive of endomyocardial fibrosis and a concomitant thrombus. The clinical course unfolded in three phases: an initial prodrome with asthma; subsequent peripheral hypereosinophilia; and ultimately systemic vasculitis.
View Article and Find Full Text PDFFour cardiomyopathy patients with a heterozygous DSG2 p.Arg119Ter variant.
Hum Genome Var
December 2024
Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Osaka, 565-0871, Japan.
DSG2, encoding desmoglein-2, is one of the causative genes of arrhythmogenic cardiomyopathy. We previously identified a homozygous DSG2 p.Arg119Ter stop-gain variant in a patient with juvenile-onset cardiomyopathy and advanced biventricular heart failure.
View Article and Find Full Text PDFStructural heart disease in the tropics: A comprehensive review.
Curr Probl Cardiol
December 2024
Internal Medicine Study Program, Department of Internal Medicine, Faculty of Medicine, Universitas Airlangga, Surabaya 60132, Indonesia; Department of Internal Medicine, Dr. Soetomo General Academic Hospital, Surabaya 60286, Indonesia.
Structural heart disease (SHD) remains a significant global health challenge, disproportionately impacting populations in tropical regions where the burden of infectious diseases, limited healthcare infrastructure, and socio-economic disparities exacerbate the issue. The tropics are uniquely affected by conditions such as rheumatic heart disease (RHD), endomyocardial fibrosis, tropical cardiomyopathies, and pericardial diseases, often resulting from or complicated by endemic infections like malaria, dengue, tuberculosis, and parasitic diseases. Moreover, Human Immunodeficiency Virus-Associated Cardiac Disease (HIVAC) represents an emerging concern in regions with high HIV prevalence, adding complexity to the interplay between infectious and structural cardiac conditions.
View Article and Find Full Text PDFRheumatic Heart Disease and Endomyocardial Fibrosis: A Complex Novel Case of Heart Failure.
JACC Case Rep
November 2024
Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA.
Rheumatic heart disease (RHD) and endomyocardial fibrosis (EMF) are major causes of cardiac disease in low-income countries. We present a case of a patient with mitral stenosis and restrictive cardiomyopathy, initially attributed to severe RHD, but with disease progression despite valve replacement, likely secondary to previously undiagnosed EMF.
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