Anesthetic management of the standard Norwood stage I procedure for hypoplastic left heart syndrome

Objective To retrospectively analyse the anaesthetic management of Norwood Stage Ⅰ. Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood Stage I procedure. They were all boys. Age at surgeries ranged from 29 to 75 days with median 36 days, and weight from 2.57-3.50 kg with median of 3.13 kg. The first three cases were received intravenous prostaglandin E1 before they were sent to the operation theatre and were under mechanical ventilation. They were received emergent operations because of unstable hemodynamic situation. The other two cases were relatively stable without mechanical ventilation and were received restrict surgery. All 5 cases received the standard Norwood Stage Ⅰprocedure under intravenous-inhalation balance-general anaesthesia with cardiopulmonary bypass. The technique of deep hypothermia and circulatory arrest were used in all five cases. Results The fourth case died from low cardiac output syndrome after cardiopulmonary bypass. The other 4 cases were transferred to the paediatric intensive care unit after withdrawal from bypass. One of the four cases died after 32 h after surgery. Conclusion The standard Norwood Stage Ⅰ procedure is aquite complex procedure, which demands multidisplinary cooperation, to palliatively correct HLHS. We retrospect the experiences of the anesthetic management in our centre and hope it will be helpful to decrease the mortality and morbidity in relatively short period. Key words: Heart defects, congenital; Hypoplastic left heart syndrome; Norwood procedures; Anesthesia