背景: 肺动脉高压（PAH）是一种恶性肺血管疾病，对生活质量、运动能力和死亡率有负面影响。本研究旨在调查PAH合并先天性心脏病（PAH-CHD）患者的血清尿酸（UA）水平与疾病严重程度及治疗反应的关系。方法: 本研究纳入了225例CHD患者和40名健康受试者。测量了所有患者的血清UA水平，并对至少接受了PAH特异性药物治疗7±1个月的20名患者进行了UA水平和血流动力学参数的重新评估。结果: PAH-CHD患者的血清UA水平显著高于肺动脉压力正常的CHD患者和正常受试者（347.7±105.7μmol/L vs. 278.3±84.6μmol/L；347.7±105.7μmol/L vs. 255.7±44.5μmol/L，p < 0.05）。在中、高危组的UA水平显著高于低危组（365.6±107.8μmol/L vs. 311.2±82.8μmol/L；451.6±117.6μmol/L vs. 311.2±82.8μmol/L，p < 0.05）。血清UA水平与平均肺动脉压、WHO功能分级、肺血管阻力和NT-proBNP呈正相关（r = 0.343, 0.357, 0.406, 0.398；p < 0.001），与混合静脉血氧饱和度（SvO2）和动脉血氧饱和度（SaO2）呈负相关（r = -0.293, -0.329；p < 0.001）。PAH特异性药物治疗至少6个月后，UA水平从352.7±97.5降至294.4±56.8μmol/L（p = 0.001），平均肺动脉压和肺血管阻力显著降低，心脏指数和混合SvO2显著增加。结论: 血清UA可作为PAH-CHD患者风险分层和PAH特异性药物治疗效果评价的实用经济生物标记物。Copyright © 2023 Luo, Li, Chen, Qiu, Chen, Luo, Chen, Tan and Li.

Background: Pulmonary arterial hypertension (PAH) is a malignant pulmonary vascular disease that negatively impacts quality of life, exercise capacity, and mortality. This study sought to investigate the relationship between serum uric acid (UA) level and the disease severity and treatment response of patients with PAH and congenital heart disease (PAH-CHD). Methods: This study included 225 CHD patients and 40 healthy subjects. Serum UA was measured in all patients, and UA levels and haemodynamic parameters were re-evaluated in 20 patients who had received PAH-specific drug treatment for at least 7 ± 1 month. Results: Serum UA levels were significantly higher in PAH-CHD patients than in CHD patients with a normal pulmonary artery pressure and normal subjects (347.7 ± 105.7 μmol/L vs. 278.3 ± 84.6 μmol/L; 347.7 ± 105.7 μmol/L vs. 255.7 ± 44.5 μmol/L, p < 0.05). UA levels in the intermediate and high risk groups were significantly higher than those in the low-risk group (365.6 ± 107.8 μmol/L vs. 311.2 ± 82.8 μmol/L; 451.6 ± 117.6 μmol/L vs. 311.2 ± 82.8 μmol/L, p < 0.05). Serum UA levels positively correlated with mean pulmonary arterial pressure, WHO functional class, pulmonary vascular resistance, and NT-proBNP (r = 0.343, 0.357, 0.406, 0.398; p < 0.001), and negatively with mixed venous oxygen saturation (SvO2) and arterial oxygen saturation (SaO2) (r = -0.293, -0.329; p < 0.001). UA significantly decreased from 352.7 ± 97.5 to 294.4 ± 56.8 μmol/L (p = 0.001) after PAH-specific drug treatment for at least 6 months, along with significant decreases in mean pulmonary arterial pressure and pulmonary vascular resistance and increases in cardiac index and mixed SvO2. Conclusion: Serum UA can be used as a practical and economic biomarker for risk stratification and the evaluation of PAH-specific drug treatment effects for patients with PAH-CHD.Copyright © 2023 Luo, Li, Chen, Qiu, Chen, Luo, Chen, Tan and Li.