A combined stage 1 and 2 repair for hypoplastic left heart syndrome: anaesthetic considerations

Therapy of hypoplastic left heart syndrome (HLHS) consists of the staged Norwood procedure or cardiac transplantation. Stenting the ductus arteriosus and subsequent banding of the pulmonary arteries allows the combination of neoaortic reconstruction with the establishment of a bidirectional cavopulmonary connection (combined stage 1 and 2 procedure) in a later session. We report the anaesthetic management in eight infants ranging from 107 to 195 days undergoing a combined stage 1 and 2 procedure. Nonselective pulmonary vasodilators and nitric oxide were needed in all cases to improve oxygen saturation in the postbypass period. Phosphodiesterase inhibitors and epinephrine were required in all patients for inotropic support during and after weaning off cardiopulmonary bypass. The procedure was successful in seven patients. One patient died intraoperatively because of right heart failure. The physiological changes of this new surgical strategy for palliation of HLHS offers a challenge for the anaesthetist primarily in the early postbypass period.