[An initial experience of endotherine-1 dual-receptor blockade in the treatment of sustained pulmonary hypertension early after congenital cardiac surgery in infancy]

Abstract

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-month-old girl, BOS is a possible alternate in the treatment of the postoperative sustained PH as reported in a various type of chronic PH.