Risk Factors for Peri-Procedural Arterial Ischaemic Stroke in Children with Cardiac Disease.

Improved survival of children with congenital heart disease has led to increasing focus on neurodevelopmental outcome, as close to half of the infants undergoing cardiac surgery are affected by neurodevelopmental disability. Stroke is particularly important as it frequently results in permanent neurologic sequelae. The aim of this study was to investigate risk factors for peri-procedural arterial ischaemic stroke (AIS) in children with cardiac disease.

No difference between aspirin and warfarin after extracardiac Fontan in a propensity score analysis of 475 patients.

Objectives: The most effective method of long-term thromboprophylaxis after the Fontan procedure is not clear. We compared the rates of thromboembolic events between patients receiving aspirin and warfarin after an extracardiac conduit Fontan procedure in a bi-national registry.

Methods: All patients who underwent an extracardiac conduit Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified.

Arterial ischemic stroke in children with cardiac disease.

Objective: To describe the spectrum of cardiac disorders, timing in relation to interventional procedures, and outcome in children with cardiac disease and arterial ischemic stroke (AIS).

Methods: Children younger than 18 years with cardiac disease and radiologically confirmed AIS admitted to the Royal Children's Hospital Melbourne between 1993 and 2010 were retrospectively identified using ICD-9 and ICD-10 searches.

Results: Seventy-six children with cardiac disease and radiologically confirmed AIS were identified with the median age at diagnosis of 5 months (interquartile range 0-58).

Ten-year outcomes of Fontan conversion in Australia and New Zealand demonstrate the superiority of a strategy of early conversion.

Objective: To investigate the benefits of a strategy of early Fontan conversion.

Methods: Using the Australia and New Zealand Fontan Registry, retrospective analysis of their long-term follow-up data was performed.

Results: Between 1990 and 2014, a total of 39 patients underwent surgical conversion in 6 centres at a median age of 23.

Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery.

Objectives: To identify determinants of adverse outcomes in patients with atrial isomerism.

Methods: Determinants of survival were analyzed for the group as a whole as well as separately for left and right atrial isomerism.

Results: There were 78 cases with right and 104 with left atrial isomerism.

Medium-term outcomes of bovine jugular vein graft and homograft conduits in children.

Background: The bovine jugular vein (Contegra) conduit has been described as an alternative to the homograft for right ventricle (RV) to pulmonary artery (PA) connection. We assessed the outcomes of Contegra conduits and homografts at a single institution.

Methods: We conducted a retrospective review of children (n = 249) who underwent RV-to-PA conduit (Contegra or homograft) implantation from 2001 to 2011.

New engineering treatment of bovine pericardium confers outstanding resistance to calcification in mitral and pulmonary implantations in a juvenile sheep model.

Objective: To conduct a test of noninferiority for CardioCel (Admedus, Brisbane, Australia), a chemically engineered bovine pericardium over autologous pericardium treated intraoperatively with glutaraldehyde in a chronic juvenile sheep model of pulmonary valve (PV) and mitral valve (MV) reconstruction.

Methods: We replaced the posterior leaflet of the MV and of 1 PV cusp with patches in ewes aged 10 months. There were 2 groups: CardioCel (n = 6) and control (n = 4).

Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand.

Background: The life expectancy of patients undergoing a Fontan procedure is unknown.

Methods And Results: Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975-1995), 271 lateral tunnels (1988-2006), and 532 extracardiac conduits (1997-2010).

Repair of partial atrioventricular septal defect: a 37-year experience.

Objectives: Partial atrioventricular septal defect (pAVSD) is routinely repaired with a low mortality. However, limited data are available on the long-term follow-up of these patients. The current study was designed to determine long-term survival and morbidity of a large cohort of patients operated on at a single institution.

End-to-side repair for aortic arch lesions offers excellent chances to reach adulthood without reoperation.

Background: The purpose of this study was to determine the rate of reinterventions from birth to adulthood after end-to-side anastomosis technique for interrupted and hypoplastic aortic arches. Multicenter data have shown that more than half of patients require arch reintervention in the 2 decades after repair.

Methods: The follow-up of 170 consecutive patients undergoing end-to-side repair through sternotomy for interrupted aortic arch (95) or coarctation (75) in one institution between 1985 and 2012 was reviewed.

Impact of Blalock-Taussig shunt size on tricuspid regurgitation in hypoplastic left heart syndrome.

Background: The prognosis for hypoplastic left heart syndrome (HLHS) has greatly improved over the past years, but there is still a significant risk of interstage mortality. Tricuspid valve regurgitation (TR) is known to be a risk factor for interstage mortality. We hypothesized that a modified Blalock-Taussig (BT) shunt with a smaller diameter would lead to a reduction in ventricular volume loading with a consequent reduction in TR.

The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes.

Objectives: To identify factors associated with hospital and long-term outcomes in a binational cohort of extracardiac conduit (ECC) Fontan recipients.

Methods: All patients who underwent an ECC Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified, and perioperative, follow-up, echocardiographic and reintervention data collected. Risk factors for early and late mortality, failure and adverse outcomes were analysed.

Surgical palliation in patients with a single ventricle and dextrocardia.

Background: Dextrocardia is found in a significant proportion of patients undergoing a single-ventricle repair. Surgical outcomes in this cohort are unclear.

Methods: The records of 41 consecutive patients with single-ventricle physiology and dextrocardia presenting in a single institution from 1990 to 2008 were reviewed.

Low risk of pulmonary valve implantation after a policy of transatrial repair of tetralogy of Fallot delayed beyond the neonatal period: the Melbourne experience over 25 years.

Objectives: The study sought to evaluate the late outcomes of a policy of transatrial repair delayed beyond the neonatal period.

Background: Long-term outcomes of transatrial repair of tetralogy of Fallot are unknown.

Methods: The records of 675 consecutive patients undergoing a transatrial repair of tetralogy of Fallot between 1980 and 2005 were reviewed, their follow-up updated and survival confirmed from national death registries.

Intersurgeon variability in long-term outcomes after transatrial repair of tetralogy of Fallot: 25 years' experience with 675 patients.

Objective: To compare the long-term reoperation rates among surgeons performing transatrial repair of tetralogy of Fallot.

Methods: The data set of 675 patients undergoing transatrial repair of tetralogy of Fallot at 1 institution from 1980 to 2005 was analyzed for intersurgeon differences in the reoperation rates.

Results: A follow-up period >15 years was available for 5 surgeons, allowing for comparison (541 patients; >80 patients/surgeon).

Trends in Fontan surgery and risk factors for early adverse outcomes after Fontan surgery: the Australia and New Zealand Fontan Registry experience.

Objectives: This study examined changes in practice and analyzed risk factors for adverse early outcomes after Fontan surgery through use of a binational, population-based registry.

Methods: Demographic, preoperative, and perioperative data were collected from all participating institutions of the Australia and New Zealand Fontan Registry. Patient and operative characteristics were analyzed with multivariable logistic regression for impact on early mortality, early Fontan failure (death, takedown, or mechanical support), effusions (prolonging hospital stay >30 days or requiring surgical reintervention), and stay longer than 30 days.

Outcomes after operations for bicuspid aortic valve disease in the pediatric population.

Background: Outcomes after operations for bicuspid aortic valve disease in pediatric patients were determined.

Methods: Between 1977 and 2011, 146 consecutive patients underwent surgical repair of bicuspid aortic valve. Median age at operation was 207 days (range, 5 days to 16 years).

Abnormal left ventricular diastolic function at late follow-up after repair of total anomalous pulmonary venous drainage: the impact of altered ventricular loading in utero.

Background: Assessment of diastolic function has not been described after repair of total anomalous pulmonary venous drainage (TAPVD), but studies of exercise capacity demonstrate impaired performance in this population despite normal systolic function. We postulated that diastolic impairment might contribute to this finding.

Methods: We analyzed echocardiographic variables from 28 patients with repaired TAPVD and compared these with data from 32 healthy controls (normals) and 21 subjects with repaired transposition of the great arteries (TGA).

Outcomes of patients born with single-ventricle physiology and aortic arch obstruction: the 26-year Melbourne experience.

Background: To review the long-term outcomes of patients born with single-ventricle physiology and aortic arch obstruction.

Methods: Follow-up of 70 consecutive neonates undergoing single-ventricle palliation and arch repair, excluding hypoplastic left heart syndrome, between 1983 and 2008, was reviewed. Dominant arch anomalies were coarctation (n = 48), interrupted arch (n = 10), and hypoplastic arch alone (n = 12).

Favourable anatomy after end-to-side repair of interrupted aortic arch.

Objective: To evaluate cardiovascular outcomes in patients with aortic arch repair and their possible correlation with arch geometry.

Methods: Ten patients who underwent end-to-side repair for aortic arch interruption (IAA), older than 10 years were compared to a cohort of 10 post coarctation (CoA) repair patients matched for age, sex and age at repair. Mean age at operation was 9.

Outcomes of repair of complete atrioventricular septal defect in the current era.

Objectives: We sought to evaluate the surgical outcomes of the repair of complete atrioventricular septal defects (cAVSDs) in our institution in the current era.

Methods: From 2000 to 2011, 138 patients underwent definitive repair of cAVSD. Repair was performed using a two-patch technique in 92.

Surgical valvotomy and repair for neonatal and infant congenital aortic stenosis achieves better results than interventional catheterization.

Objectives: This study sought to compare outcomes after surgical valvuloplasty and balloon dilation of the aortic valve in neonates and infants.

Background: Surgical techniques of aortic valve repair have improved and there is today controversy on the best approach to treat neonatal congenital aortic valve stenosis.

Methods: Retrospective review of data and follow-up of 123 consecutive neonates and infants (35 females, 88 males) undergoing intervention for congenital aortic stenosis.

Association of maternal and nutrient supply line factors with DNA methylation at the imprinted IGF2/H19 locus in multiple tissues of newborn twins.

Epigenetic events are crucial for early development, but can be influenced by environmental factors, potentially programming the genome for later adverse health outcomes. The insulin-like growth factor 2 (IGF2)/H19 locus is crucial for prenatal growth and the epigenetic state at this locus is environmentally labile. Recent studies have implicated maternal factors, including folate intake and smoking, in the regulation of DNA methylation at this locus, although data are often conflicting in the direction and magnitude of effect.

MCAR is not necessary for the complete cases to constitute a simple random subsample of the target sample.

Missing data is the norm rather than the exception in complex epidemiological studies. Complete-case analyses, which discard all subjects with some data values missing, are known to be valid under the very restrictive assumption that the response mechanism is missing completely at random (MCAR). While conditions weaker than MCAR are known under which estimators of regression coefficients are unbiased, one often comes across the view in the literature that MCAR is necessary for the complete cases to form a simple random subsample of the target sample.

Ross procedure in children: 17-year experience at a single institution.

Background: The Ross procedure in children carries substantial mortality and reoperation rate. Aortic root dilatation is of concern. To prevent dilatation of the neoaortic root, but permit normal growth, we began to apply an absorbable poly-(p-dioxanone)-filaments (PDS) band at the sino-tubular (ST)-junction.