Association between cardiovascular autonomic neuropathy and left ventricular hypertrophy in young patients with congenital generalized lipodystrophy

Background: Congenital generalized lipodystrophy (CGL) is a rare disorder characterized by the absence of subcutaneous adipose tissue, severe insulin resistance, diabetes mellitus, and cardiovascular complications, including cardiac autonomic neuropathy (CAN), left ventricular hypertrophy (LVH), and...

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Detalhes bibliográficos
Autores: Ponte, Clarisse Mourão Melo, Fernandes, Virgínia Oliveira, Liberato, Christiane Bezerra Rocha, Montenegro, Ana Paula Dias Rangel, Batista, Lívia Aline, Gurgel, Maria Helane Costa, Karbage, Lia Beatriz de Azevedo Souza, Vasconcelos, Izabella Tamira Galdino Farias, d’Alva, Catarina Brasil, Montenegro Júnior, Renan Magalhães
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2019
País:Brasil
Recursos:Universidade Federal do Ceará (UFC)
Repositorio:Repositório Institucional da Universidade Federal do Ceará (UFC)
Idioma:inglés
OAI Identifier:oai:repositorio.ufc.br:riufc/46566
Acesso em linha:http://www.repositorio.ufc.br/handle/riufc/46566
Access Level:acceso abierto
Palavra-chave:Cardiopatias
Heart Diseases
Lipodistrofia
Lipodystrophy
Lipodistrofia Generalizada Congênita
Lipodystrophy, Congenital Generalized
Descrição
Resumo:Background: Congenital generalized lipodystrophy (CGL) is a rare disorder characterized by the absence of subcutaneous adipose tissue, severe insulin resistance, diabetes mellitus, and cardiovascular complications, including cardiac autonomic neuropathy (CAN), left ventricular hypertrophy (LVH), and atherosclerosis. The present study aimed to access the association between CAN parameters and cardiovascular abnormalities in CGL patients. Methods: A cross-sectional study was conducted with 10 CGL patients and 20 healthy controls matched for age, sex, BMI, and pubertal stage. We evaluated clinical, laboratory, and cardiovascular parameters—left ventricular mass index (LVMI), interventricular septum thickness (IVS), systolic and diastolic function determined by two-dimensional transthoracic echocardiography; carotid intimal media thickness (cIMT); and cQT interval. Heart rate variability (HRV) was evaluated by spectral analysis components—high frequency (HF), low frequency (LF), very low frequency (VLF), LF/ HF ratio, and total amplitude spectrum (TAS)—and cardiovascular reflexes tests (postural hypotension test, respiratory, orthostatic and Valsalva coefficients). Results: In CGL group, four patients (40%) had LVH and diastolic dysfunction. HF component (parasympathetic control) was lower in LVH patients. CGL patients presented higher values of cIMT and cQT interval than heathy subjects. Inverse association between LVMI and LF (p = 0.011), IVS and LF (p = 0.007), and cIMT and leptin (p < 0.001) were observed, even after adjustments by HOMA-IR, A1c, and blood pressure. In CGL group, there were associations between LMVI and HF component (IC95%: − 1.000; − 00.553), LVMI and TAS (IC95%: − 1.000; − 0.012), and IVS and HF component (IC95%: − 1.000; − 0.371). Conclusion: The association between increased LV mass and parameters of HRV provides possible speculations about the involvement of CAN in the pathophysiology of the cardiac complications, including LVH, in patients with CGL.