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Disease frequency is reportedly lower in women than men, but sex-related differences have not been well established. We conducted a systematic literature review (SLR), based on PRISMA-P guidelines and registered with PROSPERO, to assess whether the epidemiology and clinical presentation of ATTR-CM differ between women and men. MEDLINE, Embase, and Cochrane databases and selected conference proceedings were searched (August 16, 2019) to identify observational and clinical studies reporting sex-specific data for patients with wild-type or hereditary ATTR-CM. Of 193 publications satisfying final eligibility criteria, 69 studies were included in our pooled analysis. Among the 4669 patients with ATTR-CM analyzed, 791 (17%) were women, including 174 (9%), 366 (29%), and 251 (18%) in studies of wild-type, hereditary, and undefined ATTR-CM, respectively. Data available on disease characteristics were limited and very heterogeneous, but trends suggested some cardiac structural\/functional differences, i.e., lower interventricular septal and posterior wall thickness and left ventricular (LV) end diastolic diameter, and higher LV ejection fractions, in women versus men across ATTR-CM subtypes. Because LV wall thickness &gt;\u200912\u00a0mm is generally the suggested threshold for ATTR-CM diagnosis in both sexes, smaller cardiac anatomy in women with the disease may lead to underdiagnosis. Additional research and studies are needed to elucidate potential disparities between sexes in ATTR-CM frequency, clinical characteristics, and underlying biological mechanisms. This study was registered within the International Prospective Register of Systematic Reviews (PROSPERO) database of the University of York (CRD42019146995).<\/jats:p>","DOI":"10.1007\/s10741-020-10010-8","type":"journal-article","created":{"date-parts":[[2020,8,14]],"date-time":"2020-08-14T00:07:48Z","timestamp":1597363668000},"page":"35-45","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":60,"title":["Transthyretin amyloid cardiomyopathy in women: frequency, characteristics, and diagnostic challenges"],"prefix":"10.1007","volume":"26","author":[{"given":"Marianna","family":"Bruno","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Casta\u00f1o","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arianna","family":"Burton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justin L.","family":"Grodin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,8,14]]},"reference":[{"issue":"7","key":"10010_CR1","doi-asserted-by":"publisher","first-page":"398","DOI":"10.1038\/nrcardio.2010.67","volume":"7","author":"C Rapezzi","year":"2010","unstructured":"Rapezzi C, Quarta CC, Riva L, Longhi S, Gallelli I, Lorenzini M, Ciliberti P, Biagini E, Salvi F, Branzi A (2010) Transthyretin-related amyloidoses and the heart: a clinical overview. 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Bruno, Casta\u00f1o, and Burton are full-time employees of Pfizer and may hold Pfizer stock and\/or stock options. Dr. Grodin has received funding as a consultant for Pfizer and Eidos.Medical writing support for this article was provided by Donna McGuire of Engage Scientific Solutions and was funded by Pfizer. Sara Lucas, PhD, and Catherine Rolland, PhD, are employees of Envision Pharma Group and paid consultants to Pfizer in connection with the development of the SLR report that forms the basis of this manuscript. They were not compensated for their role in the review of this manuscript.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"All the authors provided consent for the publication of this article.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}}]}}