Early Results for Management of Hypoplastic Left Heart Syndrome in a New Program

Introduction: Surgery for hypoplastic left heart syndrome (HLHS) has been routinely offered in NSW since 2009, prior to which selected cases were performed. We report early outcomes for this program. Methods: We performed an audit of outcomes using prospectively collected data. Only cases with true HLHS were included. Mortality to second stage and operative mortality after second stage surgery were the primary endpoints. In addition to reporting of the entire series, data are presented for the periods prior to and after January 2009, ending March 2011. Patients with birth weight <2.5 kg, additional major syndrome or malformation, restriction of or intact atrial septum, moderate RV dysfunction or tricuspid regurgitation or pulmonary venous anomalies were grouped as ‘high risk’ vs. ‘standard risk’ patients according to convention. Results: Thirty-five patients with HLHS were identified. Thirty-two had an antenatal diagnosis and three underwent non-operative palliation. Twenty-nine underwent Norwood surgery, 11 were considered high risk. Overall survival to second stage was 79%; 94% for standard risk and 55% for high risk groups, including two that had intact atrial septum, both died after surgery. There were two deaths after second stage surgery (10%) and one late death. Conclusions: Early outcomes indicate that standard risk patients have a predictable and satisfactory outcome compared to other reported outcomes. In addition to the development of technical aspects of care, management of HLHS requires significant resources and provision of social supports. Service development priorities include addressing the modifiable risk factors present in high risk patients and reducing resource utilisation.