Fetal and post-natal diagnosis of major congenital heart disease: implications for medical and psychological care in the current era.

AIM: The fetal or post-natal diagnosis of major congenital cardiac abnormality has important medical and psychological consequences. METHODS: We reviewed infants who underwent cardiac surgery in the first year of life at the Heart Centre for Children, The Children's Hospital at Westmead during 2009. The aims of this study were to: (i) examine the key features of cardiac diagnosis and clinical outcome, and (ii) consider how these data can inform priorities for the delivery of clinical services. RESULTS: Over the 12-month study period, a first cardiac surgical procedure was performed on 195 infants, with 85 infants (44%) diagnosed in the antenatal period. Of the total sample, a subset of 90 babies (46%) underwent their first procedure in the neonatal period, with 62% having had a fetal diagnosis. Major intracardiac lesions including truncus arteriosus (100%), single ventricular lesions (83%), pulmonary atresia with ventricular septal defect (78%) and transposition of the great arteries (53%) were diagnosed prior to birth. Improved haemodynamic stability at initial presentation was found in those with a fetal diagnosis. The overall mortality rate for all patients was 6.1% at 12 months, with a higher mortality in infants with single ventricle. CONCLUSIONS: The contemporary paradigm of care for infants with major congenital heart disease requires a multidisciplinary approach to care, with improvements in clinician-clinician and clinician-family communication, and psychological support and education for families. Changes in the allocation of resources are required to meet this model of best practice.

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.