The hypereosinophilic syndromes (HESs) are characterized by persistent marked eosinophilia (>1500 eosinophils/mm(3)), the absence of a primary cause of eosinophilia (such as parasitic or allergic disease), and evidence of eosinophil-mediated end organ damage. Cardiovascular complications of HES are a major source of morbidity and mortality in these disorders. The most characteristic cardiovascular abnormality in HES is endomyocardial fibrosis. Patients who have an HES also may develop thrombosis, particularly in the cardiac ventricles, but also occasionally in deep veins. Because of the rarity of these disorders, specific guidelines for the management of the cardiac and thrombotic complications of HES are lacking. This article reviews the diagnosis and management of the cardiovascular manifestations of HES.
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http://dx.doi.org/10.1016/j.iac.2007.07.001 | DOI Listing |
Cureus
December 2024
Cardiovascular Surgery, Ayase Heart Hospital, Tokyo, JPN.
Subvalvular aortic stenosis typically manifests at a young age and rarely presents in adulthood. It may cause left ventricular outflow tract stenosis, which requires surgical treatment in severe cases. The coexistence of discrete subvalvular aortic stenosis and quadricuspid aortic valve is a highly unusual finding.
View Article and Find Full Text PDFCureus
December 2024
Pharmacy, Punjab University College of Pharmacy, Lahore, PAK.
Berardinelli-Seip congenital lipodystrophy (BSCL), also known as congenital generalized lipodystrophy (CGL), is an exceptionally rare autosomal recessive disorder marked by a significant deficiency of adipose tissue throughout the body. This lack of adipose tissue, normally found beneath the skin and between internal organs, leads to impaired adipocyte formation and fat storage, causing lipids to accumulate in atypical tissues such as muscles and the liver. The extent of adipose tissue loss directly influences the severity of symptoms, which can include a muscular appearance, increased appetite, bone cysts, marrow fat depletion, acromegalic features, severe insulin resistance, skeletal muscle hypertrophy, hypertrophic cardiomyopathy, hepatic steatosis, hepatomegaly, cirrhosis, and intellectual disability.
View Article and Find Full Text PDFCardiovasc Endocrinol Metab
March 2025
Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Kalaburagi, Karnataka, India.
Hypothyroidism is typically associated with bradyarrhythmias, but can rarely precipitate life-threatening ventricular arrhythmias. We present a case of severe hypothyroidism manifesting as polymorphic ventricular tachycardia (VT). A previously healthy woman in her early 50s presented with an acute onset of breathlessness and on examination had hypotension and tachycardia.
View Article and Find Full Text PDFEur J Case Rep Intern Med
December 2024
Internal Medicine, Holy Family Hospital, Rawalpindi, Pakistan.
Background: Andersen-Tawil syndrome (ATS) is a rare autosomal dominant disorder caused by variants in the gene. It is associated with periodic paralysis, dysmorphic features and cardiac arrhythmias. The syndrome exhibits incomplete penetrance, leading to a broad spectrum of clinical manifestations, making diagnosis challenging.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
December 2024
Department of Thoracic and Cardiovascular Surgery, Chungnam National University Hospital, Daejeon, Republic of Korea.
Torsion of extralobar pulmonary sequestration is a rare congenital anomalous condition that can manifest with various symptoms, including abdominal pain. However, a masslike lesion in the mediastinum can be easily overlooked when the clinician focuses only on abdominal pain. By using magnetic resonance imaging, a few features specific to the torsion of extralobar pulmonary sequestration can be identified, and that information will be helpful.
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