Cardiac amyloidosis: do not forget to look for it. (25th March 2020)

• Record Type: Journal Article
• Authors: Porcari, Aldostefano; Falco, Luca; Lio, Veronica; Merlo, Marco; Fabris, Enrico; Bussani, Rossana; Sinagra, Gianfranco
• Is Part Of: European heart journal supplements. Volume 22(2020)Supplement E
• Page Start: E142
• Page End: E147
• Publication Date: 2020-03-25
• Subjects: Cardiac amyloidosis -- Diagnosis -- Epidemiology -- Myocardial scintiscan -- Heart failure; Cardiology -- Periodicals; Cardiology -- Europe -- Periodicals; 616.12005
• DOI: 10.1093/eurheartj/suaa080
• Languages: English
• ISSNs: 1520-765X

Abstract

Abstract: Amyloidosis is a systemic disease due to buildup of protein material in the extracellular space, which can affect the heart, mainly in its light chain and transtyretin forms. Historically this condition has been considered very uncommon, and it was certainly under-diagnosed. Today is well known that in certain group of patients its prevalence is, indeed, very high (25% in patients over the age of 80 years; 32% in patients over 75 years with heart failure and preserved systolic function, and 5% in post-mortem series of hypertrophic cardiomyopathy). Some genetically determined form of transthyretin amyloidosis are quite common in certain populations, such as Caribbean origin African-Americans. The wide spectrum of signs, symptoms, and first-level tests often overlapping among various other conditions, represent a diagnostic challenge for the clinical cardiologist. The opportunity to reach the diagnosis with non-invasive testing (first and foremost scintiscan with bone markers), as well as encouraging results of newer classes of drugs, raised the interest in this condition, so far burdened by an ominous prognosis. Early diagnosis of amyloidosis should always be guided by clinical suspicion but should also be supported by a multidisciplinary approach, aimed at optimizing the prognosis of the condition. Despite the newer drugs now available, a late diagnosis affect negatively the prognosis, and the opportunity to implement disease-modifying therapies (e.g. liver transplant in ATTR, or bone marrow transplant in AL) able to cure or at least delay the progression of the disease.