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Early Left Ventricular Systolic Dysfunction Detected by Two-Dimensional Speckle-Tracking Echocardiography in Young Patients with Congenital Generalized Lipodystrophy

Authors Liberato CBR, Olegario NBC, Fernandes VO, Montenegro APDR, Lima GECP, Batista LAA, Martins LV, Penaforte-Saboia JG, Liberato ILR, Lopes LF, d'Alva CB, Furtado FLB, Lima RLDM, Nóbrega LHC, Lima JG, Montenegro Junior RM

Received 8 October 2019

Accepted for publication 6 December 2019

Published 13 January 2020 Volume 2020:13 Pages 107—115


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Dr Antonio Brunetti

Christiane Bezerra Rocha Liberato, 1, 2 Natália Bitar da Cunha Olegario, 1, 2 Virginia Oliveira Fernandes, 1–3 Ana Paula Dias Rangel Montenegro, 2 Grayce Ellen da Cruz Paiva Lima, 1, 2 Lívia Aline de Araújo Batista, 1, 2 Lívia Vasconcelos Martins, 1, 2 Jaquellyne Gurgel Penaforte-Saboia, 1, 2 Ivan Lucas Rocha Liberato, 2 Larissa Ferreira Lopes, 2 Catarina Brasil d’Alva, 1, 2 Frederico Luís Braz Furtado, 1, 2 Ricardo Luiz De Medeiros Lima, 4 Lucia Helena Coelho Nóbrega, 4 Josivan Gomes Lima, 4 Renan Magalhães Montenegro Junior 1–3 Brazilian Group for the Study of Inherited and Acquired Lipodystrophies (BRAZLIPO)

1Department of Clinical Medicine, Federal University of Ceará, Fortaleza, Brazil; 2Clinical Research Unit, Walter Cantidio University Hospital, Federal University of Ceará, Fortaleza, Brazil; 3Department of Community Health, Federal University of Ceará, Fortaleza, Brazil; 4Department of Clinical Medicine, Onofre Lopes University Hospital, Federal University of Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil

Correspondence: Renan Magalhães Montenegro Junior Rua Professor Costa Mendes, 1608, Fortaleza, Ceará 60416-200, Brazil
Tel +55 85 3366-8600
Fax +55 85 3366-8619

Purpose: Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by the absence of functional adipocytes resulting in ectopic lipid storage, metabolic disorders and early cardiovascular disease. Two-dimensional speckle-tracking (2D-STE) allows the detection of early abnormalities in myocardial function. We aimed to evaluate myocardial deformation in a large sample of CGL patients using 2D-STE.
Patients and Methods: A cross-sectional study of 22 patients with CGL and 22 healthy subjects, matched for sex and age, was conducted from 2013 to 2018. All participants had undergone standard conventional echocardiography (ECHO) and 2D-STE. Determination of blood glucose, lipids, insulin, and leptin were performed in all CGL patients.
Results: In the CGL group the mean age was 14.6± 10.7 years where 68.2% (n=15) were younger than 18 years old. All the patients had hypoleptinemia, 95.4% (21/22) low HDL-c, 86.4% (19/22) hypertriglyceridemia, 68.2% (15/22) diabetes, 50% (11/22) hepatic steatosis, 41% (9/22) insulin resistance, 41% (9/22) hypercholesterolemia, and 18.2% (4/22) hypertension. ECHO showed that 36.6% (8/22) of CGL patients presented diastolic dysfunction, 31.8% (7/22) left ventricular hypertrophy (LVH), 27.3% (6/22) increased left atrial volume index (LAVI), and 18.2% (4/22) increased left ventricular systolic diameter (LVDS) but normal ejection fraction (EF), whether using 2D-STE, 68.2% (15/22) of CGL patients showed abnormal global longitudinal strain (GLS) (p< 0.01), and in almost LV segments. Positive association between abnormal GLS and A1c (r=0.57, p=0.005), glucose (r=0.5, p=0.018) and basal insulin (r= 0.69, p= 0.024), and negative association with leptin (r = − 0.51, p = 0.005) were found in these patients.
Conclusion: The 2D-STE revealed precocious left ventricular systolic dysfunction in a young CGL population with normal systolic function by ECHO. Early exposure to common metabolic abnormalities as insulin resistance, hyperglycemia, and hypoleptinemia must be involved in myocardial damage in these patients.

Keywords: Berardinelli-Seip congenital lipodystrophy, global longitudinal strain, cardiac function, early detection

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