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.

The heart beads program.

From July 2008 through June 2009, 760 infants and children with cardiac conditions were admitted to a pediatric hospital in Australia with approximately 360 cardiac surgical procedures performed.This was the first experience in hospital for many of these children, with diagnoses signaling the beginning of a long and arduous journey. These children undergo multiple treatments and procedures,as well as multiple admissions for further surgeries. Procedures in any regard can cause stress and anxiety, especially in children who often have limited understanding and so little control over what happens to them (Lau, 2002).A heart center for children exists at the hospital with the aim of providing a consistent experience for children with cardiac conditions as they move through the different hospital environments, from preadmission clinic to operating theaters to the pediatric intensive care unit (PICU), and then on to the cardiac ward. The Heart Beads Program was developed within a context of person-centered care to enrich the experience of children with cardiac conditions by providing them with distinctive beads specific to each procedure, treatment, or event in recognition of their experiences and endurance (McCormack et al., 2008). This column focuses on the process of starting the program and on preliminary responses from staff, children, and families.