Effects of Bosentan in Treatment of Severe Pulmonary Hypertension Related to Congenital Heart Disease / 昆明医科大学学报

ABSTRACT

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.