医学
危险分层
疾病
心脏淀粉样变性
重症监护医学
淀粉样变性
病理
作者
Claudio Rapezzi,Alberto Aimo,Matteo Serenelli,Andrea Barison,Giuseppe Vergaro,Claudio Passino,Giorgia Panichella,Gianfranco Sinagra,Marco Merlo,Marianna Fontana,Julian Gillmore,Candida Cristina Quarta,Mathew S Maurer,Michelle M Kittleson,Pablo Garcia-Pavia,Michele Emdin
标识
DOI:10.1016/j.jacc.2022.01.036
摘要
Over the last year, 5 national or international scientific societies have issued documents regarding cardiac amyloidosis (CA) to highlight the emerging clinical science, raise awareness, and facilitate diagnosis and management of CA. These documents provide useful guidance for clinicians managing patients with CA, and all include: 1) an algorithm to establish a diagnosis; 2) an emphasis on noninvasive diagnosis with the combined use of bone scintigraphy and the exclusion of a monoclonal protein; and 3) indications for novel disease-modifying therapies for symptomatic CA, either with or without peripheral neuropathy. Nonetheless, the documents diverge on specific details of diagnosis, risk stratification, and treatment. Highlighting the similarities and differences of the documents by the 5 scientific societies with respect to diagnosis, risk stratification, and treatment offers useful insight into the knowledge gaps and unmet needs in the management of CA. An analysis of these documents, therefore, highlights “gray zones” requiring further investigation. • Clinical guidance documents on CA have been issued by 5 professional societies during the past year. • These are generally aligned on a diagnostic algorithm, noninvasive diagnosis based on combining bone scintigraphy and exclusion of a monoclonal proteins, and indications for disease-modifying therapies for symptomatic patients. • Divergence on certain aspects of diagnosis, risk stratification, and clinical management highlights areas requiring further investigation.
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