二维斑点追踪超声心动图检测先天性全身性脂肪营养不良年轻患者的早期左心室收缩功能障碍
Early Left Ventricular Systolic Dysfunction Detected by Two-Dimensional Speckle-Tracking Echocardiography in Young Patients with Congenital Generalized Lipodystrophy.
作者信息
Liberato Christiane Bezerra Rocha, Olegario Natália Bitar da Cunha, Fernandes Virginia Oliveira, Montenegro Ana Paula Dias Rangel, Lima Grayce Ellen da Cruz Paiva, Batista Lívia Aline de Araújo, Martins Lívia Vasconcelos, Penaforte-Saboia Jaquellyne Gurgel, Liberato Ivan Lucas Rocha, Lopes Larissa Ferreira, d'Alva Catarina Brasil, Furtado Frederico Luís Braz, Lima Ricardo Luiz De Medeiros, Nóbrega Lucia Helena Coelho, Lima Josivan Gomes, Montenegro Junior Renan Magalhães
机构信息
Department of Clinical Medicine, Federal University of Ceará, Fortaleza, Brazil.
Clinical Research Unit, Walter Cantidio University Hospital, Federal University of Ceará, Fortaleza, Brazil.
出版信息
Diabetes Metab Syndr Obes. 2020 Jan 13;13:107-115. doi: 10.2147/DMSO.S233667. eCollection 2020.
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.
目的
先天性全身脂肪营养不良（CGL）是一种罕见的常染色体隐性疾病，其特征是缺乏功能性脂肪细胞，导致脂质异位储存、代谢紊乱和早期心血管疾病。二维斑点追踪技术（2D-STE）可检测心肌功能的早期异常。我们旨在使用2D-STE评估大量CGL患者的心肌变形情况。
患者与方法
2013年至2018年对22例CGL患者和22名年龄及性别匹配的健康受试者进行了一项横断面研究。所有参与者均接受了标准的传统超声心动图（ECHO）检查和2D-STE检查。对所有CGL患者进行了血糖、血脂、胰岛素和瘦素的测定。
结果
CGL组的平均年龄为14.6±10.7岁，其中68.2%（n=15）年龄小于18岁。所有患者均有低瘦素血症，95.4%（21/22）高密度脂蛋白胆固醇（HDL-c）降低，86.4%（19/22）高甘油三酯血症，68.2%（15/22）糖尿病，50%（11/22）肝脂肪变性，41%（9/22）胰岛素抵抗，41%（9/22）高胆固醇血症，18.2%（4/22）高血压。ECHO显示，36.6%（8/22）的CGL患者存在舒张功能障碍，31.8%（7/22）左心室肥厚（LVH），27.3%（6/22）左心房容积指数（LAVI）增加，18.2%（4/22）左心室收缩直径（LVDS）增加但射血分数（EF）正常，无论使用2D-STE与否，68.2%（15/22）的CGL患者显示整体纵向应变（GLS）异常（p<0.01），且几乎在所有左心室节段均如此。在这些患者中发现GLS异常与糖化血红蛋白（A1c）（r=0.57，p=0.005）、血糖（r=0.5，p=0.018）和基础胰岛素（r = 0.69，p= 0.024）呈正相关，与瘦素（r = -0.51，p = 0.005）呈负相关。
结论
2D-STE显示，在ECHO检查收缩功能正常的年轻CGL人群中存在早熟的左心室收缩功能障碍。早期暴露于胰岛素抵抗、高血糖和低瘦素血症等常见代谢异常情况必定与这些患者的心肌损伤有关。
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