Cardiac Alterations in Patients with Familial Lipodystrophy

Familial lipodystrophy is a rare genetic condition in which individuals have, besides metabolic changes and body fat deposits, a type of cardiomyopathy that has not been well studied. Many of the patients develop cardiovascular changes, the most commonly reported in the literature being the expressi...

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Bibliographic Details
Published in:Arquivos brasileiros de cardiologia 2020-02, Vol.114 (2), p.305-312
Main Authors: Romano, Minna Moreira Dias, Chacon, Paula Ananda Inês, Ramalho, Fernanda Naira Zambelli, Foss, Maria Cristina, Schmidt, André
Format: Article
Language:eng ; por
Subjects:
Adipose Tissue - physiopathology
CARDIAC & CARDIOVASCULAR SYSTEMS
Cardiomegaly - diagnostic imaging
Cardiomegaly - etiology
Cardiomegaly - physiopathology
Cardiomyopathy, Hypertrophic - diagnostic imaging
Cardiomyopathy, Hypertrophic - etiology
Cardiomyopathy, Hypertrophic - physiopathology
Cardiomyopathy, Hypertrophic, Magnetic Resonance Imaging/trends
Humans
Hypertrophy, Left Ventricular
Lipodystrophy, Congenital Generalized - complications
Lipodystrophy, Congenital Generalized - diagnostic imaging
Lipodystrophy, Congenital Generalized - physiopathology
Lipodystrophy, Familial Partial - complications
Lipodystrophy, Familial Partial - diagnostic imaging
Lipodystrophy, Familial Partial - physiopathology
Lipodystrophy, Familial Partial/genetics
Magnetic Resonance Imaging
Metabolic Diseases/complications
Review
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Description
Summary:Familial lipodystrophy is a rare genetic condition in which individuals have, besides metabolic changes and body fat deposits, a type of cardiomyopathy that has not been well studied. Many of the patients develop cardiovascular changes, the most commonly reported in the literature being the expression of a type of hypertrophic cardiomyopathy. This article, presented as a bibliographic review, reviews the clinical and cardiovascular imaging aspects in this scenario of cardiomyopathy in a rare metabolic disease, based on the latest scientific evidence published in the area. Despite the frequent association of congenital lipodystrophy and ventricular hypertrophy described in the literature, the pathophysiological mechanisms of this cardiomyopathy have not yet been definitively elucidated, and new information on cardiac morphological aspects is emerging in the aegis of recent and advanced imaging methods, such as cardiac magnetic resonance.
ISSN:0066-782X
1678-4170
1678-4170
DOI:10.36660/abc.20190016