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

ANZ J Surg. 2015 Jun;85(6):466-71. doi: 10.1111/ans.12103. Epub 2013 Feb 19.

Abstract

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.

Keywords: cardiac surgery; congenital heart disease; hypoplastic left heart syndrome; neonate; outcome.

Publication types

  • Evaluation Study

MeSH terms

  • Female
  • Humans
  • Hypoplastic Left Heart Syndrome / mortality
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant, Newborn
  • Male
  • Norwood Procedures* / methods
  • Palliative Care* / methods
  • Postoperative Care / methods
  • Program Evaluation
  • Regional Medical Programs*
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome