Anaesthetic management and outcomes after noncardiac surgery in patients with hypoplastic left heart syndrome: a retrospective review.

Context: Noncardiac surgery in patients with hypoplastic left heart syndrome has been associated with significant morbidity and mortality in case reports and small series.

Objective: A retrospective study to review the anaesthetic care and outcomes of patients with hypoplastic left heart syndrome undergoing noncardiac surgery.

Design: The medical records of patients undergoing anaesthesia for noncardiac surgery were reviewed, including anaesthesiology records, operative notes, admission history, physical examination records and discharge summaries. Data were collected on patient characteristics, co-morbidities, surgical procedure, anaesthetic and monitoring techniques, intraoperative and postoperative complications and admission status.

Setting: A tertiary medical centre with a high volume of congenital heart disease.

Participants: Seventy-three procedures performed in 40 patients with hypoplastic left heart syndrome undergoing noncardiac surgery between July 2002 and May 2008.

Results: Thirty-three procedures were performed on an outpatient basis without invasive monitoring or complications. Adverse events occurred in 11 (15%) cases, including cardiovascular and respiratory instability, airway obstruction and postoperative stridor, with 13 (18%) patients admitted to the ICU postoperatively.

Conclusion: Given the high incidence of adverse events in this patient population, it is imperative that perioperative care be individualised based on the presence of known risk factors such as the stage of palliation, residual cardiac disease and severity of planned surgery.