Lundell K H, Sabel K G, Eriksson B O
Department of Paediatrics, Jönköping Central Hospital, Sweden.
Acta Paediatr. 1999 Jul;88(7):718-23. doi: 10.1080/08035259950168982.
Growth retardation is common in infants with congenital heart defects. The aim of this study was to investigate whether growth retardation or type of heart defect in infants with congenital heart defects is related to disturbances in lipid metabolism. Sixteen infants with ventricular septal defects and six infants with transposition of the great arteries were given an intravenous load of lipid emulsion (Intralipid 20 mg/ml) corresponding to 0.5 g fat/kg body weight for 5 min after fasting for 8 h. Blood samples were drawn immediately before the infusion and 3, 20, 60, 120 and 240 min after the infusion was completed. Plasma concentrations of triglycerides (TG), free fatty acids (FFA), ketones, lactate, pyruvate, alanine, glycerol and glucose were determined. The fatty acid patterns in the TG and FFA fractions were measured using gas chromatography. Severe growth retardation in infants with defects of these kinds was correlated to higher fasting and maximum levels of linoleic acid in plasma FFA. The maximum levels of linoleic acid in the TG fraction were positively correlated to weight SD score, and maximum glycerol levels were higher in the most growth-retarded infants, indicating faster intravascular lipolysis. Linoleic acid in the TG fraction was still elevated at 120 and 240 min after the lipid load. Some differences between the cyanotic and VSD groups could be noted. These indicate decreased metabolic capacity to utilize released FFA in the cyanotic group. Infants with cyanotic heart defects also had higher lactate and alanine levels compared to infants with VSD. Our results support the hypothesis that lipid metabolism is disturbed in infants with congenital heart defects.
生长发育迟缓在先天性心脏病患儿中很常见。本研究的目的是调查先天性心脏病患儿的生长发育迟缓或心脏缺陷类型是否与脂质代谢紊乱有关。对16例室间隔缺损婴儿和6例大动脉转位婴儿在禁食8小时后给予静脉注射脂质乳剂（英脱利匹特20mg/ml），剂量相当于0.5g脂肪/kg体重，持续5分钟。在输注前以及输注完成后3、20、60、120和240分钟立即采集血样。测定血浆甘油三酯（TG）、游离脂肪酸（FFA）、酮体、乳酸、丙酮酸、丙氨酸、甘油和葡萄糖的浓度。使用气相色谱法测量TG和FFA组分中的脂肪酸模式。患有这类缺陷的婴儿出现严重生长发育迟缓与血浆FFA中较高的空腹和最高水平的亚油酸相关。TG组分中亚油酸的最高水平与体重标准差评分呈正相关，生长发育最迟缓的婴儿中甘油的最高水平更高，表明血管内脂解更快。脂质负荷后120和240分钟时，TG组分中的亚油酸仍处于升高状态。可以注意到青紫型和室间隔缺损组之间存在一些差异。这些差异表明青紫型组利用释放的FFA的代谢能力下降。与室间隔缺损婴儿相比，患有青紫型先天性心脏病的婴儿乳酸和丙氨酸水平也更高。我们的结果支持先天性心脏病患儿脂质代谢紊乱这一假说。
