Anesthetic management of the hybrid stage 1 procedure for hypoplastic left heart syndrome (HLHS).

Introduction: Despite advances in the surgical and perioperative management of patients with hypoplastic left heart syndrome (HLHS), outcomes for this high-risk group of patients remains suboptimal. The hybrid approach [bilateral pulmonary artery (PA) banding, ductal stenting, balloon atrial septostomy], is an emerging alternative therapy for the management of HLHS, which defers the risks of a major surgical repair until the infants are older. This article will describe our experience providing the anesthetic management of patients undergoing the hybrid procedure.

Methods: After Institutional Review Board approval, we retrospectively reviewed the records of 77 patients who underwent the hybrid procedure as neonates between July 2002 and August 2008. We reviewed both the anesthetic and intensive care records.

Results: The hybrid procedure was performed in 77 patients (31 female and 46 male). The average age of the patients was 11.8 days with an average weight of 2.98 kg. Fentanyl was used for analgesia at an average dose of 5.7 mcg x kg(-1). The average increase in the systolic blood pressure after placement of the right and left PA bands was 11.3 mmHg. The average drop in the systemic saturation after placement of the bands was 7%, with an average postband and stent SaO(2) of 82%. Twenty-one patients received blood transfusion (27.3%) at an average dose of 43.5 ml (14.5 ml x kg(-1)). Forty patients received albumin during the case (51.9%) at an average dose of 23.2 ml (7.7 ml x kg(-1)). Seventeen patients arrived at the hybrid suite already intubated, and no attempt was made to extubate these patients at the end of the case. Thirty-six patients were extubated at the end of the procedure, and a total of 64.9% of patients were extubated within the first 24 h postoperatively. Patients had notably stable hemodynamics throughout the first 24 h in the intensive care unit.

Discussion: Patients undergoing the hybrid procedure have relatively stable intraoperative and early postoperative hemodynamics. The procedure is performed without cardiopulmonary bypass (CPB) and with minimal narcotic and anesthetic exposure. Patients typically do not require blood transfusions or inotropic support and are extubated at either the end of the procedure or within 24 h of ICU admission. In our experience, the anesthetic management of patients undergoing the hybrid procedure is straightforward and requires relatively few interventions when compared to traditional neonatal surgical repairs. Deferring the risks of anesthesia, CPB, hypothermic circulatory arrest, and prolonged postoperative sedation may yield developmental advantages to patients born with HLHS.