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Heart failure (HF) therapeutics have advanced significantly over the past few years [...].
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Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy resulting from a mutation in one of several cardiac sarcomeric proteins [...].
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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.
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Doença de Fabry , Linfedema , Masculino , Feminino , Humanos , Doença de Fabry/complicações , Doença de Fabry/epidemiologia , Doença de Fabry/genética , alfa-Galactosidase/genética , Prevalência , Linfedema/etiologia , Linfedema/genética , Sistema de Registros , Progressão da DoençaRESUMO
Viral myocarditis is inflammation of the myocardium secondary to viral infection. The clinical presentation of viral myocarditis is very heterogeneous and can range from nonspecific symptoms of malaise and fatigue in subclinical disease to a more florid presentation, such as acute cardiogenic shock and sudden cardiac death in severe cases. The accurate and prompt diagnosis of viral myocarditis is very challenging. Endomyocardial biopsy is considered to be the gold standard test to confirm viral myocarditis; however, it is an invasive procedure, and the sensitivity is low when myocardial involvement is focal. Cardiac imaging hence plays an essential role in the noninvasive evaluation of viral myocarditis. The current coronavirus disease 2019 (COVID-19) pandemic has generated considerable interest in the use of imaging in the early detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related myocarditis. This article reviews the role of various cardiac imaging modalities used in the diagnosis and assessment of viral myocarditis, including COVID-19-related myocarditis.
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Cardiovascular diseases are the most common cause of death worldwide, with cardiovascular medications being amongst the most common medications prescribed. These medications have diverse effects on the heart, vascular system, as well as other tissues and organ systems. The extra cardiovascular effects have been found to be of use in the treatment of non-cardiovascular diseases and pathologies. Minoxidil is used to manage systemic hypertension with its well-known side effect of hirsutism used to treat alopecia and baldness. Sildenafil was originally investigated as a treatment option for systemic hypertension; however, its side effect of penile erection led to it being widely used for erectile dysfunction. Alpha-1 blockers such as terazosin are indicated to treat systemic hypertension but are more commonly used for benign prostatic hyperplasia and post-traumatic stress disorder. Beta blockers are the mainstay treatment for congestive heart failure and systemic hypertension but have been found useful to help in patients with intention tremors as well as prophylaxis of migraines. Similarly, calcium channel blockers are indicated in medical expulsion therapy for ureteric calculi in addition to their cardiovascular indications. Thiazides are commonly used for treating systemic hypertension and as diuretics. Thiazides can cause hypocalciuria and hypercalcemia. This side effect has led to thiazides being used to treat idiopathic hypercalciuria and associated nephrolithiasis. Spironolactone is commonly utilized in treating heart failure and as a diuretic for edema. It's well described anti-androgen side effects have been used for acne vulgaris and hirsutism in polycystic ovarian syndrome. This review article discusses how the various extracardiovascular effects of commonly used cardiovascular medications are put to use in managing non-cardiovascular conditions.
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Doenças Cardiovasculares , Insuficiência Cardíaca , Hipertensão , Antagonistas Adrenérgicos beta/uso terapêutico , Doenças Cardiovasculares/tratamento farmacológico , Diuréticos/efeitos adversos , Insuficiência Cardíaca/tratamento farmacológico , Hirsutismo/induzido quimicamente , Hirsutismo/tratamento farmacológico , Humanos , Hipertensão/tratamento farmacológico , Masculino , Tiazidas/uso terapêuticoRESUMO
There is increasing experimental and clinical evidence that inflammation appears to play an important role in atherosclerosis and coronary artery disease. Treatment of coronary artery disease currently involves management of cardiovascular risk factors, lipid-lowering strategies and antiplatelet medications. Inflammation seems to be central to the pathogenesis of atherosclerotic plaque development, instability, and rupture seen in coronary artery disease. Colchicine, a well-known and relatively inexpensive drug, has unique anti-inflammatory properties, which is generating considerable interest in its potential role in reducing cardiovascular morbidity and potentially mortality. This review discusses the mechanism of action of colchicine in preventing and treating atherosclerosis as well as the literature from recent clinical studies supporting its use in coronary artery disease.
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Aterosclerose , Colchicina , Doença da Artéria Coronariana , Placa Aterosclerótica , Anti-Inflamatórios/uso terapêutico , Aterosclerose/tratamento farmacológico , Ensaios Clínicos Fase III como Assunto , Doença da Artéria Coronariana/tratamento farmacológico , Humanos , Placa Aterosclerótica/tratamento farmacológico , Ensaios Clínicos Controlados Aleatórios como AssuntoRESUMO
We report a case of a 55-year-old woman with previous coronary artery bypass grafting. She presented with acute ST-segment elevation myocardial infarction secondary to occlusion of the proximal left subclavian artery causing reduced flow in the left internal mammary artery to left anterior descending artery graft. (Level of Difficulty: Intermediate.).