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Objective The objective of this study was to determine whether there remain developmental differences between term infants at 3 years of age following major non-cardiac surgery (NCS) and cardiac surgery (CS) compared with healthy control infants in New South Wales (NSW), Australia. Study Design Between 2006 and 2008, term infants who required NCS or CS within the first ninety days of life were enrolled in a prospective population-based study. Their developmental outcome was then compared with a cohort of healthy term infants. Infants initially assessed at 1 year of age were then re-assessed at 3 years of age using the Bayley scales of infant and toddler development (version-III) Results Of the 539 term infants assessed at 1 year of age, 417 returned for the 3-year assessment, with 378 complete assessments. The mean scores for the infants who underwent CS (P < 0.001) were significantly lower in all subscales of the assessment compared with the controls, while the mean scores for the infants who underwent NCS were significantly lower in three of the subscales (P < 0.05). The infants who underwent CS scored significantly lower in four of the subscales (P < 0.05), compared with the infants who underwent NCS. Conclusion The second phase of this unique population-based study provides further data on the outcomes of infants who underwent major NCS and CS. Major surgery in infants continues to be associated with developmental delay at 3 years of age compared with control infants; however the majority of the delay is mild. The risk remains higher in CS group with the pattern and severity of delay similar to that observed in the first study.

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.

Aim: To describe cardiac surgery, survival and outcomes for low-birthweight (≤2500 g) infants undergoing surgery for congenital heart disease. Methods: Using data from a prospectively collected population-based database of admissions to neonatal intensive care units in New South Wales and the Australian Capital Territory, we identified all low-birthweight infants undergoing cardiac surgery between 1992 and 2001. Infants with only a persistent ductus arteriosus were excluded. Two-year cardiac and neurodevelopmental outcome data were sought from hospital medical records. Results: A total of 121 low-birthweight infants underwent cardiac surgery, of whom 34% had a congenital syndrome or non-cardiac birth defect. Most (81%) underwent a palliative surgical procedure in the neonatal period. There were 19 early (15.7%) and 19 late deaths giving a 2-year mortality of 31%. Factors associated with mortality included birthweight below 1500 g (P = 0.006), low weight at surgery (P = 0.028) and Apgar score at 1 min (P = 0.019). No single factor predicted 30-day mortality. By 2 years of age, 27 (33% of survivors) were known to have neurodevelopmental delay. Although 22 children are known to be developing normally, the neurodevelopmental status of 34 children was not known. Conclusions: These surgical data were comparable to previous single-institution studies. This group had a high risk of disability due to prematurity, low birthweight and associated conditions. There is a need to prospectively assess and manage neurodevelopmental outcomes in this group.

The objective of this study was to determine whether there remain developmental differences between term infants at 3 years of age following major non-cardiac surgery (NCS) and cardiac surgery (CS) compared with healthy control infants in New South Wales (NSW), Australia.Between 2006 and 2008, term infants who required NCS or CS within the first ninety days of life were enrolled in a prospective population-based study. Their developmental outcome was then compared with a cohort of healthy term infants. Infants initially assessed at 1 year of age were then re-assessed at 3 years of age using the Bayley scales of infant and toddler development (version-Of the 539 term infants assessed at 1 year of age, 417 returned for the 3-year assessment, with 378 complete assessments. The mean scores for the infants who underwent CS (P0.001) were significantly lower in all subscales of the assessment compared with the controls, while the mean scores for the infants who underwent NCS were significantly lower in three of the subscales (P0.05). The infants who underwent CS scored significantly lower in four of the subscales (P0.05), compared with the infants who underwent NCS.The second phase of this unique population-based study provides further data on the outcomes of infants who underwent major NCS and CS. Major surgery in infants continues to be associated with developmental delay at 3 years of age compared with control infants; however the majority of the delay is mild. The risk remains higher in CS group with the pattern and severity of delay similar to that observed in the first study.

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.

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.

The fetal or post-natal diagnosis of major congenital cardiac abnormality has important medical and psychological consequences.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.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.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.

A neonate requiring major surgery in 2006 has a greater prospect of survival than ever before. Increasingly, however, there is awareness that critical illness may affect later neurodevelopment. Pre-existing conditions in addition to the physiologic stresses associated with cardiac and general surgery are implicated but remain unavoidable in the case of significant structural abnormalities such as transposition of the great arteries or congenital diaphragmatic hernia. For those affected by neurodevelopmental impairment, there is a significant cost to the child, family and society. Current research focuses on the preventable causes of brain injury, before, during and after the intervention, and the rate of impairment in apparently uncomplicated procedures. In contrast to the quantity of neurodevelopmental outcome data following cardiac surgery, there remain few outcome studies dealing with non-cardiac surgery despite such intervention being two to three times more common. There appear to be compelling clinical and economic arguments for the instigation of formalised population-based developmental assessments for all infants undergoing major surgery.