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Of 53 individuals with a confirmed diagnosis of Proteus syndrome at our institution from 10\/2001 to 10\/2019, 38 individuals (men, n\u2009=\u200923; average age\u2009=\u200924\u00a0years) underwent cardiothoracic imaging (routine chest CT, CT pulmonary angiography and\/or cardiac MRI). All studies were retrospectively and independently reviewed by two fellowship-trained cardiothoracic readers. Disagreements were resolved by consensus. Differences between variables were analyzed via parametric and nonparametric tests based on the normality of the distribution. The cardiothoracic findings of Proteus syndrome were diverse, but several were much more common and included: scoliosis from bony overgrowth (94%), pulmonary venous dilation (62%), band-like areas of lung scarring (56%), and hyperlucent lung parenchyma (50%). In addition, of 20 individuals who underwent cardiac MRI, 9\/20 (45%) had intramyocardial fat, mostly involving the endocardial surface of the left ventricular septal wall. There was no statistically significant difference among the functional cardiac parameters between individuals with and without intramyocardial fat. Only one individual with intramyocardial fat had mildly decreased function (LVEF\u2009=\u200953%), while all others had normal ejection fraction.<\/jats:p>","DOI":"10.1038\/s41598-021-86029-0","type":"journal-article","created":{"date-parts":[[2021,3,22]],"date-time":"2021-03-22T11:03:43Z","timestamp":1616411023000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Cardiothoracic imaging findings of Proteus syndrome"],"prefix":"10.1038","volume":"11","author":[{"given":"S. Mojdeh","family":"Mirmomen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew E.","family":"Arai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evrim B.","family":"Turkbey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew J.","family":"Bradley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie C.","family":"Sapp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie G.","family":"Biesecker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arlene","family":"Sirajuddin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,3,22]]},"reference":[{"key":"86029_CR1","doi-asserted-by":"crossref","unstructured":"Kinsler, V. 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Andrew E. Arai has research agreements with Siemens Healthineers, Bayer Pharmaceuticals, and Circle Cardiovascular Imaging. Leslie G. Biesecker receives in-kind research support from ArQule Inc (since acquired by Merck, Inc.), is a member of the Illumina ethics advisory board and receives honoraria from Cold Spring Harbor Press. The other authors have no disclosures.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"6577"}}