Surgical Management of Extensive Perinatal Myocardial Infarction.

Extensive perinatal myocardial infarction caused by coronary artery thrombosis is extremely rare and has a dismal prognosis. We report a 3.5-kg neonate who presented at birth with an extensive myocardial infarction caused by aortic root and left main coronary artery thrombus after an emergency cesarean section.

Impact of arch reobstruction and early hypertension on late hypertension after coarctation repair.

Objectives: Late hypertension after coarctation repair is associated with high mortality, but its risk factors remain unclear. This study aims to determine early and late postoperative risk factors for late hypertension after coarctation repair.

Methods: A cross-sectional study including transthoracic echocardiogram and 24-h blood pressure (BP) monitoring was performed in 144 patients aged ≥10 years with previous coarctation repair.

Mid-term outcomes of congenital mitral valve surgery: Shone's syndrome is a risk factor for death and reintervention.

Objectives: To study the recent trends and outcomes of congenital mitral valve surgery in children.

Methods: From 2008 to 2014, 84 procedures in 66 consecutive patients (41 procedures in 31 patients with mitral stenosis and 43 procedures in 35 patients with mitral regurgitation) were retrospectively evaluated. The mean age at surgery was 4.

Extubation Failure Is Associated With Increased Mortality Following First Stage Single Ventricle Reconstruction Operation.

Objectives: To identify the prevalence, causes, risk factors, and outcomes associated with extubation failure following first stage single ventricle reconstruction surgery.

Design: Retrospective cohort analysis of neonates who underwent a first stage single ventricle reconstruction operation. Extubation failure was defined as endotracheal reintubation within 48 hours of first extubation attempt.

Determinants of Adverse Outcomes After Systemic-To-Pulmonary Shunts in Biventricular Circulation.

Background: Systemic-to-pulmonary shunts are useful palliative procedures, although many teams have been deterred by high mortality and morbidity. We aimed to identify predictors of adverse outcomes after shunts in biventricular lesions.

Methods: From 2004 to 2014, 173 children had shunt procedures.

Inspiratory Muscle Training Is Associated With Improved Inspiratory Muscle Strength, Resting Cardiac Output, and the Ventilatory Efficiency of Exercise in Patients With a Fontan Circulation.

Background: Patients with a Fontan circulation have reduced exercise capacity and respiratory muscle strength. Inspiratory muscle training (IMT) improves exercise capacity and quality of life in adults with heart failure. We assessed whether 6 weeks of a home-based program of IMT improves inspiratory muscle strength and the ventilatory efficiency of exercise in adolescent patients with a Fontan circulation.

Chylothorax following paediatric cardiac surgery: a case-control study.

Objectives: The aims of this study were to investigate risk factors for the development of postoperative chylothorax following paediatric congenital heart surgery and to investigate the impact of a management guideline on management strategies and patient outcome.

Methods: All patients with chylothorax following cardiac surgery at the Royal Children's Hospital, Melbourne, over a 48-month period beginning in January 2008 were identified. A control group, matched for age, date of surgery, and sex, was identified.

Neonatal Ebstein Anomaly: A 30-year Institutional Review.

This study aimed to review our 30-year, single-center experience of neonates admitted with Ebstein anomaly. Between January 1985 and August 2015, 80 neonates with Ebstein anomaly were managed. The primary outcome measures were early and late survival, freedom from reoperation, and functional status.

Involvement of patients and parents in research undertaken by the Australian and New Zealand Fontan Registry.

Research that is closely connected with the population it is studying and in which the translational value to healthcare is high is a laudable goal, but it is not often achieved. The Australian and New Zealand Fontan Registry has developed a model for involving patients and parents of children with a Fontan circulation in its research. The model involves consumer participation in the overarching Steering Committee, and has set in place multiple channels of communication allowing the early dissemination of research findings before peer-reviewing, and consumer feedback at all levels of the research.

Successful atrioventricular valve repair improves long-term outcomes in children with unbalanced atrioventricular septal defect.

Background: Atrioventricular valve regurgitation is a significant cause of morbidity and mortality in patients with unbalanced atrioventricular septal defect. However, knowledge of the outcomes of atrioventricular valve repair in children with unbalanced atrioventricular septal defect and univentricular physiology is limited.

Methods: We conducted a retrospective review of all patients with unbalanced atrioventricular septal defect treated with single-ventricle palliation who underwent atrioventricular valve surgery at The Royal Children's Hospital.

Late-Term Gestation Is Associated With Improved Survival in Neonates With Congenital Heart Disease Following Postoperative Extracorporeal Life Support.

Objective: Several population-based studies have shown that gestational age 39-40 weeks at birth is associated with superior outcomes in various pediatric settings. A high proportion of births for neonates with congenital heart disease occur before 39 weeks. We aimed to assess the influence of late-term gestation (39-40 wk) on survival in neonates requiring extracorporeal life support following surgery for congenital heart disease.

Adolescent and parent perspectives prior to involvement in a Fontan transition program.

Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program.

Hospital Inpatient Costs for Single Ventricle Patients Surviving the Fontan Procedure.

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.

Are more extensive procedures warranted at the time of aortic arch reoperation?

Objectives: To determine the early and late outcomes of patients undergoing aortic arch reoperations.

Methods: The follow-up of 70 patients undergoing a second arch operation (excluding univentricular physiology) between 1979 and 2015 was reviewed. Median age at initial arch operation and second operation was 9 days (interquartile range: 5-35) and 10 months (interquartile range: 3-64), respectively.

Elevated sympathetic activity, endothelial dysfunction, and late hypertension after repair of coarctation of the aorta.

Background: There is a high prevalence of late hypertension after coarctation repair. The relative contribution of elevated sympathetic tone and endothelial dysfunction to its development is unknown. This study aims to investigate the neural profile of coarctation patients including muscle sympathetic nerve activity testing to directly measure sympathetic nervous activity.

Towards the goal of achieving a normal duration and quality of life after Fontan operation: Creation of the International Fontan Interest group (I-FIG), an international collaborative initiative dedicated to improving outcomes.

A growing population of patients are now surviving the Fontan procedure and face an uncertain future and a clear burden of disease. The best medical therapy and follow-up modalities for these patients have not yet been identified, indications for transplantation are unclear and we have not been successful in mechanically supporting their circulation. There has only been a limited number of trials to address these issues.

Ventricular assist device support in patients with single ventricles: the Melbourne experience.

Objectives: The capacity and limitations of ventricular assist device (VAD) support in single-ventricle physiology remains poorly understood. We aimed to review our experience in the use of VAD support in the single-ventricle circulation to determine its feasibility in this population.

Methods: We reviewed our experience with VAD support in patients with single ventricles over the past 25 years.

Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: A 19-year experience.

Objective: The surgical indication, timing, strategy, and surgical technique for anatomic correction of congenitally corrected transposition of the great arteries are challenging. We evaluated the long-term results at The Royal Children's Hospital Melbourne.

Methods: Review of 32 successive anatomic corrections between 1996 and 2015.

The ordeal of left atrioventricular valve replacement in children under 1 year of age.

Objectives: This study reviews the outcomes of children under 1 year of age who had left atrioventricular valve (LAVV) replacement (LAVVR) in one centre and explores the benefits of an innovative approach for LAVVR in very small patients.

Methods: Thirteen consecutive patients operated for LAVV replacement between 1997 and 2016 were reviewed retrospectively. Indication for surgery was regurgitation in 7, stenosis in 5 and both stenosis and regurgitation in 1.

Hospital costs and cost implications of co-morbid conditions for patients with single ventricle in the period through to Fontan completion.

Background: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed.

Methods: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions.

Closing the gap in paediatric ventricular assist device therapy with the Berlin Heart EXCOR® 15-ml pump.

Objectives: The Berlin Heart EXCOR ® (EXCOR) paediatric ventricular assist device is used worldwide for mechanical support of infants and small children with end-stage heart failure. A clinically important gap between the smallest EXCOR blood pump (10 ml) and the next larger size (25 ml) limited the choice of pump size in patients with a body surface area (BSA) between 0.33 and 0.