OBJECTIVES (1) To measure the global shift in the metabolome in hypoxemic versus non-hypoxemic infants with congenital heart disease; (2) To identify metabolites and metabolic pathways that are altered in hypoxemia. STUDY DESIGN Analysis of serum samples obtained prior to cardiopulmonary bypass from 82 infants ≤120 days old with congenital heart disease requiring surgery at Children's Hospital Colorado. Infants were divided into groups based on pre-operative oxygen saturations: non-hypoxemic (>92%), mild hypoxemia (85-92%), and severe hypoxemia (<85%). Tandem mass spectrometry was used to analyze 165 targeted metabolites. Partial least squares discriminant analysis and t-tests were used to determine differences among metabolic profiles and individual metabolites respectively. RESULTS The broad metabolic fingerprint of neonates or older infants did not vary by degree of hypoxemia. There were 12 individual metabolites that differed between hypoxemic and non-hypoxemic neonates, including lower methylmalonic acid (p = 2.44 × 10-4), glutamate (p = 0.001), and hypoxanthine (p = 0.003), and higher thymine (p = 8.67 × 10-4) and myo-inositol (p = 0.014) seen in hypoxemic neonates. Individual metabolites did not vary significantly between older infants with or without hypoxemia. CONCLUSIONS We did not find evidence supporting global metabolic changes associated with cyanotic congenital heart disease in neonates or older infants. However, specific metabolites did discriminate between hypoxemic and non-hypoxemic neonates. These include methylmalonic acid, as well as several metabolites known to change in hypoxia-reoxygenation states (hypoxanthine) and chronic hypoxemic states (glutamate, thymine, myo-inositol) and may represent specific metabolic changes triggered by hypoxemia among neonates with cyanotic congenital heart disease.

中文翻译：

---

与先天性心脏病新生儿和婴儿低氧血症相关的代谢物变化。

目标 (1) 测量低氧血症与非低氧血症先天性心脏病婴儿代谢组的总体变化；(2) 识别低氧血症中改变的代谢物和代谢途径。研究设计 在科罗拉多州儿童医院对 82 名≤120 天的先天性心脏病婴儿进行体外循环前获得的血清样本分析。根据术前氧饱和度将婴儿分为几组：非低氧血症（>92%）、轻度低氧血症（85-92%）和严重低氧血症（<85%）。串联质谱法用于分析 165 种靶向代谢物。偏最小二乘判别分析和 t 检验分别用于确定代谢谱和单个代谢物之间的差异。结果新生儿或较大婴儿的广泛代谢指纹没有因低氧血症程度而异。有 12 种单独的代谢物在低氧血症和非低氧血症新生儿之间存在差异，包括较低的甲基丙二酸 (p = 2.44 × 10-4)、谷氨酸 (p = 0.001) 和次黄嘌呤 (p = 0.003)，以及较高的胸腺嘧啶 (p = 8.67 × 10-4）和肌醇（p = 0.014）见于低氧新生儿。有或没有低氧血症的较大婴儿之间的个体代谢物没有显着差异。结论 我们没有发现支持新生儿或较大婴儿紫绀型先天性心脏病相关的全球代谢变化的证据。然而，特定的代谢物确实可以区分低氧血症和非低氧血症新生儿。这些包括甲基丙二酸，