波生坦治疗先心病合并重度肺动脉高压的疗效观察

摘要

Objective To observe the clinical efficacy of bosentan in treatment of severe pulmonary hypertension related to congenital heart disease (CHD-PAH) .Methods 5 patients with severe CHD-PAH patients received bosentan therapy, then pulmonary artery pressure, pulmonary vascular resistance (PVR), 6min walk test,right ventricular end-systolic diameter (RVSD) changes were observed and statistically analyzed after six months medication. Results Pulmonary arterial systolic pressure (sPAP) was significanfly decreased from (96±11) mmHg to (86±10) mmHg, <0.01.pulmonary arterial diastolic pressure (dPAP) was significanfly decreased from (56±10) mmHg to (46±9) mmHg ( <0.01),pulmonary arterial mean pressure (mPAP) was significanfly decreased from (73 ±11) mmHg to (59 ±10) mmHg ( <0.05), pulmonary vascular resistance was significanfly decreased from (17.8±1.9) Wood to (13.1±1.7) Wood (<0.01) . 6min walk test was improved from (136±40) m to (198±55) m, <0.01.right ventricular end-systolic diameter significanfly decreased from (40±5) mm to (36±6) mm after 6 months therapy ( <0.05) . Conclusion Bosentan can decrease pulmonary arterial systolic pressure, improve exercise tolerance, improve right ventricular function in patients with severe CHD-PAH.%目的：观察波生坦治疗先心病合并重度肺动脉高压的临床疗效．方法5例先心病合并重度肺动脉高压患者服用波生坦治疗，观察服药6月后肺动脉压、肺血管阻力、6 min步行试验、右室收缩末期内径变化情况并进行统计学分析．结果服用波生坦后（1）肺动脉收缩压从（96±11） mmHg降为（86±10） mmHg （＜0.01），肺动脉舒张压（56±10） mmHg降为（46±9） mmHg （＜0.01）．肺动脉平均压（73±11） mmHg降为（59±10） mmHg （＜0.05）．肺血管阻力（17.8±1.9） Wood降为（13.1±1.7） Wood （＜0.01）；（2）6 min步行试验从（136±40） m改善为（198±55） m （＜0.01）；（3）右室收缩末期内径从（40±5） mm减为（36±6） mm （＜0.05）．结论波生坦可降低先心病合并重度肺动脉高压患者的肺动脉压，提高患者的运动耐量，改善右室功能.