乳児開心術後の急性期遺残肺高血圧に対するエンドセリン-1 受容体拮抗薬bosentan hydrate の使用経験

We report an initial experience of successful application of bosentan(BOS), a endotheline-1 dual-receptor blocker, in the treatment of sustained pulmonary hypertension(PH)early after surgical correction of congenital heart disease. Two infants(5 and 9-months-old girls undergone repair of complete atrioventricular septal defect and closure of ventricular septal defect, respectively)suffered from PH-relating symptoms after extubation despite oral sildenafil(SIL)treatment. After administration of BOS(1.5 mg/kg/day), the symptoms were dramatically relieved and SIL was uneventfully discontinued. Although BOS caused a transient increment of liver transaminase in the 5-years-old girl, BOS is a possible alternate in the treatment of the postoperative sustained PH as reported in a various type of chronic PH.