Cardiac amyloidosis is a great masquerader that often results in misdiagnosis of this condition. Early clinical recognition is crucial for timely therapeutic interventions to improve survival in patients with cardiac amyloidosis. Currently, Food and Drug Administration (FDA)-approved medications work best if started early in the disease. Thus, to increase identification, disease awareness, expertise in diagnostic techniques, and a multidisciplinary team approach is essential. The majority of the patients (~90%) in the United States are treated in community hospitals, thus, it would be helpful for these hospitals to have their own designated, comprehensive cardiac amyloidosis center to provide care to the patients who are widespread in the community. Most of these patients are elderly, and it is difficult for these patients to travel long distances to academic amyloid centers. Our manuscript aims to provide a path to the development of cardiac amyloid centers at community hospitals.
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http://dx.doi.org/10.31083/j.rcm2502061 | DOI Listing |
J Clin Med
December 2024
Department of Internal Medicine, Division of Cardiology, Medical University of Vienna, 1090 Wien, Austria.
Variant transthyretin amyloidosis cardiomyopathy (ATTRv-CM) is a rare form of cardiac amyloidosis associated with many possible mutations in the transthyretin gene, presenting as various distinct clinical phenotypes. Among these, the His108Arg mutation is the most prevalent TTR variant in Austria. However, data describing its clinical phenotype are lacking.
View Article and Find Full Text PDFJ Clin Med
December 2024
Pfizer S.L.U., 28108 Madrid, Spain.
Hereditary transthyretin amyloidosis (ATTRv) is an autosomal-dominant systemic disease, where amyloid fibrils accumulate especially in the peripheral and autonomic nervous systems and in the heart. The aim of the present work was to outline the follow-up and type of management received by asymptomatic carriers (ACs) and stage 1 ATTRv patients in Spain. A cross-sectional, non-interventional study was conducted throughout seven experienced hospitals in Spain.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Department of Invasive Cardiology, Medical University of Białystok, 15-089 Białystok, Poland.
Cardiac transthyretin amyloidosis is an underdiagnosed disorder with significant diagnostic difficulties due to its non-specific clinical manifestations. It is caused by the deposition of protein aggregates with an abnormal tertiary structure in the extracellular matrix. Their accumulation leads to the development of hypertrophic and restrictive cardiomyopathy and, at a later stage, heart failure with preserved ejection fraction syndrome.
View Article and Find Full Text PDFBackground: Diagnosis of cardiac amyloidosis (CA) is often missed or delayed due to confusion with other causes of increased left ventricular wall thickness. Conventional transthoracic echocardiographic measurements like global longitudinal strain (GLS) has shown promise in distinguishing CA, but with limited specificity. We conducted a study to investigate the performance of a computer vision detection algorithm in across multiple international sites.
View Article and Find Full Text PDFFront Cardiovasc Med
December 2024
National Key Laboratory for Innovation and Transformation of Luobing Theory, Jinan, China.
Background: Cardiac amyloidosis (CA) is a challenging diagnosis, particularly when the classic signs, such as increased wall thickness in a non-dilated left ventricle (LV), are absent. This makes the diagnosis more difficult in patients with normal LV wall thickness. We present a case of CA without increased wall thickness and without the characteristic granular sparkling echotexture in a non-dilated LV.
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