Cardiothoracic imaging findings of Proteus syndrome

Abstract In this work, we sought to delineate the prevalence of cardiothoracic imaging findings of Proteus syndrome in a large cohort at our institution. Of 53 individuals with a confirmed diagnosis of Proteus syndrome at our institution from 10/2001 to 10/2019, 38 individuals (men, n = 23; average...

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Autores principales:	S. Mojdeh Mirmomen, Andrew E. Arai, Evrim B. Turkbey, Andrew J. Bradley, Julie C. Sapp, Leslie G. Biesecker, Arlene Sirajuddin
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/6ca8f786b3a44395b78077068c90752e
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spelling	oai:doaj.org-article:6ca8f786b3a44395b78077068c90752e2021-12-02T16:36:05ZCardiothoracic imaging findings of Proteus syndrome10.1038/s41598-021-86029-02045-2322https://doaj.org/article/6ca8f786b3a44395b78077068c90752e2021-03-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-86029-0https://doaj.org/toc/2045-2322Abstract In this work, we sought to delineate the prevalence of cardiothoracic imaging findings of Proteus syndrome in a large cohort at our institution. Of 53 individuals with a confirmed diagnosis of Proteus syndrome at our institution from 10/2001 to 10/2019, 38 individuals (men, n = 23; average age = 24 years) 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 = 53%), while all others had normal ejection fraction.S. Mojdeh MirmomenAndrew E. AraiEvrim B. TurkbeyAndrew J. BradleyJulie C. SappLeslie G. BieseckerArlene SirajuddinNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q S. Mojdeh Mirmomen Andrew E. Arai Evrim B. Turkbey Andrew J. Bradley Julie C. Sapp Leslie G. Biesecker Arlene Sirajuddin Cardiothoracic imaging findings of Proteus syndrome
description	Abstract In this work, we sought to delineate the prevalence of cardiothoracic imaging findings of Proteus syndrome in a large cohort at our institution. Of 53 individuals with a confirmed diagnosis of Proteus syndrome at our institution from 10/2001 to 10/2019, 38 individuals (men, n = 23; average age = 24 years) 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 = 53%), while all others had normal ejection fraction.
format	article
author	S. Mojdeh Mirmomen Andrew E. Arai Evrim B. Turkbey Andrew J. Bradley Julie C. Sapp Leslie G. Biesecker Arlene Sirajuddin
author_facet	S. Mojdeh Mirmomen Andrew E. Arai Evrim B. Turkbey Andrew J. Bradley Julie C. Sapp Leslie G. Biesecker Arlene Sirajuddin
author_sort	S. Mojdeh Mirmomen
title	Cardiothoracic imaging findings of Proteus syndrome
title_short	Cardiothoracic imaging findings of Proteus syndrome
title_full	Cardiothoracic imaging findings of Proteus syndrome
title_fullStr	Cardiothoracic imaging findings of Proteus syndrome
title_full_unstemmed	Cardiothoracic imaging findings of Proteus syndrome
title_sort	cardiothoracic imaging findings of proteus syndrome
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/6ca8f786b3a44395b78077068c90752e
work_keys_str_mv	AT smojdehmirmomen cardiothoracicimagingfindingsofproteussyndrome AT andrewearai cardiothoracicimagingfindingsofproteussyndrome AT evrimbturkbey cardiothoracicimagingfindingsofproteussyndrome AT andrewjbradley cardiothoracicimagingfindingsofproteussyndrome AT juliecsapp cardiothoracicimagingfindingsofproteussyndrome AT lesliegbiesecker cardiothoracicimagingfindingsofproteussyndrome AT arlenesirajuddin cardiothoracicimagingfindingsofproteussyndrome
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Cardiothoracic imaging findings of Proteus syndrome

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