Hypoplastic left heart syndrome in context.

Hypoplastic left heart syndrome is a rare condition requiring major cardiac surgery during the neonatal period to sustain life, with subsequent procedures culminating in completion of the Fontan circulation - the common pathway for all 'single ventricle' conditions. Algorithms for care of these children are now well defined with predictable medium-term outcomes with the majority achieving a Fontan circulation. Hypoplastic left heart syndrome is one of a group of conditions that require complex surgery as a neonate and require a similar perioperative approach. Antenatal diagnosis is common in this patient subgroup, and there is a significant body of work that can be drawn on to inform parental choice.

Five-year analysis of operative mortality and neonatal outcomes in congenital heart disease.

BACKGROUND: We sought to compare overall mortality with neonatal outcomes over a five year period to define risk factors for mortality and service development priorities. METHODS: A retrospective cohort study of surgical outcomes following repair or palliation of structural congenital heart defects January 2005-2010. We defined mortality according to contemporary international guidelines and classified surgical procedures using the Risk Adjustment in Congenital Heart Surgery (RACHS-1) score. The effect of age and weight at operation on mortality and annual variation in case-complexity and surgeon case-mix were assessed. Subgroup analysis was performed in patients who were ≤30 days at operation (neonates). RESULTS: Overall mortality within 30 days of operation or prior to hospital discharge was 1.3 and 1.9%, respectively. Mortality was higher in neonates (6.8%) and low birth weight infants (≤2.5kg) (12.1%). Mortality was similar in bypass versus non-bypass procedures (odds ratio 0.74, p=0.425). Annual mortality rates were consistent despite a marked increase in case-complexity. Neonates overall required longer periods of intensive care support and were more likely to suffer serious complications compared to older children. Age, weight and RACHS-1 score were independent risk factors for mortality on multivariate analysis. In neonates undergoing bypass procedures, only RACHS-1 score was a significant risk factor. CONCLUSIONS: This study provides an accurate and contemporary audit of mortality risk associated with congenital heart surgery. Outcomes compare favourably to international benchmarks but highlight the risks of morbidity and mortality associated with neonatal cardiac surgery.

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.