Early outcomes from a new regional programme for the surgical management of hypoplastic left heart syndrome

Background: Early survival and quality of outcome after surgery for hypoplastic left heart syndrome (HLHS) are influenced by patient-specific factors, the quality of surgery and perioperative care. Some skills are common to the care of other complex neonatal presentations but integrating this expertise is a key challenge for new programmes. We began offering surgery for HLHS from 2006 and provided a regional service from January 2009 and report early outcomes. Methods: Prospectively collected data for neonates with HLHS undergoing surgical palliation from January 2006 until June 2011 were analysed. Standard definitions of high-risk and standard-risk presentations were utilized. Results: Thirty neonates underwent surgical palliation of HLHS with a modified Norwood procedure with an overall survival to stage II palliation of 80%. A total of 46.7% of our patients were categorized as high-risk, mostly on the basis of low birth weight. Survival to stage II palliation was 100% in standard-risk patients and 57.1% in the high-risk group. Conclusion: Outcomes for this new programme are comparable to reported outcomes demonstrating the feasibility of integrating a new complex procedure within an existing multidisciplinary unit handling large volumes of other complex neonatal work. Excellent outcomes can be achieved in standard-risk patients. Outcomes in the high-risk group may be improved by alternative approaches and rigorous case selection.