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目的评价非选择性内皮素受体拮抗剂(波生坦)治疗6个月以下婴儿围术期先天性心脏病肺动脉高压的临床效果。方法按世界卫生组织(WHO)肺动脉高压分级(FC)将60例年龄小于6个月的先天性心脏病(分流)相关性肺动脉高压患者分为两组,对照组:30例围术期接受常规治疗[包括:地高辛口服0.01mg/(kg.d),1次/d;安体舒通口服3mg/(kg.d),3次/d;卡托普利口服1.5mg/(kg.d),3次/d];治疗组:30例在常规治疗基础上加用波生坦(术前6.25mg,2次/d,1周;术后12.5mg,2次/d,应用1~4周)口服治疗。观察两组患者肺动脉收缩压、运动耐量和机械辅助通气时间的变化,评估药物的临床效果。结果治疗组用药后1周肺动脉收缩压较对照组明显改善(63.43±7.37mm Hg vs.69.11±6.35mm Hg,t=3.197,P=0.002),术后1周治疗组肺动脉压较对照组持续下降(42.42±8.13mm Hg vs.58.93±7.79mm Hg,t=8.030,P=0.001);治疗组术后机械辅助通气时间和运动耐量(30ml奶液喂养时间)均明显优于对照组。结论波生坦用于治疗6个月以下的小婴儿是安全的,先天性心脏病(分流)相关的肺动脉高压患者围术期使用波生坦能显著降低肺动脉压,改善心功能及运动耐量,缩短术后机械辅助通气时间。
Objective To evaluate the clinical effect of nonselective endothelin receptor antagonist (bosentan) in the treatment of pulmonary hypertension in congenital heart disease infants under 6 months old. Methods Sixty patients with congenital heart disease (shunt) related pulmonary hypertension who were less than 6 months old were divided into two groups according to the World Health Organization (WHO) pulmonary hypertension classification (FC). The control group: 30 patients underwent routine perioperative Diazepam was orally administered orally at a dose of 0.01 mg / (kg · d) once / d; spironolactone was administered orally at 3 mg / (kg · d) for 3 times daily; captopril was administered orally at 1.5 mg / kg .D), 3 times / d]; treatment group: 30 cases were treated with bosentan (6.25mg, 2 times / d, 1 week; 12.5mg, 2 times / d after operation, 1 to 4 weeks) oral treatment. The pulmonary artery systolic pressure, exercise tolerance and mechanical ventilation time were observed in two groups to evaluate the clinical effect of the drug. Results Compared with the control group, the systolic pressure of pulmonary artery in the treatment group was significantly improved at 1 week (63.43 ± 7.37mm Hg vs.69.11 ± 6.35mm Hg, t = 3.197, P = 0.002). The pulmonary arterial pressure (42.42 ± 8.13mm Hg vs.58.93 ± 7.79mm Hg, t = 8.030, P = 0.001). The mechanical ventilation time and exercise tolerance (30ml milk feeding time) in the treatment group were significantly better than those in the control group. CONCLUSION Bosentan is safe for the treatment of infants younger than 6 months. Bosentan perioperatively associated with congenital heart disease (shunt) significantly reduces pulmonary arterial pressure, improves cardiac function and exercise tolerance, Shorten the postoperative mechanical ventilation time.