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Background To observe the clinical efficacy of bosentan in severe pulmonary hypertension related to congenital heart disease (CHD-PAH) Methods Five patients with severe CHD-PAH patients receiving bosentan therapy,pulmonary artery pressure,pulmonary vascular resistance (PVR),6-min walk test,right ventricular end-systolic diameter (RVSD) changes were observed and statistically analyzed after six months medication.