Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Long-distance transport of neonates with transposition of the great arteries for the arterial switch operation: A 26-year Western Australian experience.

AIM: There is evidence that outcomes of complex paediatric cardiac procedures including the arterial switch operation (ASO) for transposition of the great arteries (TGA) are improved when performed at higher volume centres. While in utero transport for surgery is considered ideal, antenatal detection rates of TGA are low. Long-distance transport of post-natally diagnosed neonates has the potential to destabilise the patient's clinical condition. Since 1986, many neonates with TGA have been transported interstate from Perth to Melbourne or Brisbane for ASO surgery. The aim of this study was to review the Western Australian experience of interstate transport of newborns with TGA for ASO, noting transport complications and comparing the early mortality of these patients with published outcomes of the ASO from Royal Children's Hospital (RCH), Melbourne. METHOD: In this retrospective cohort study, we reviewed the neonatal and cardiology databases and medical records to identify infants with TGA born between 1986 and 2011 and requiring ASO surgery during the neonatal period. RESULTS: Over 26 years, 80 neonates were transferred interstate for ASO surgery. Twelve infants required ventilation, 36 needed prostaglandin (prostaglandin E1) infusion and 3 inotropic support. There was no mortality during transport and there was a single early post-operative death. This early mortality of 1.2% compares favourably with the RCH mortality of 2.8% from a recently published review of early outcomes for ASO. CONCLUSIONS: When in utero transport is not possible, long-distance transport of neonates with TGA can be safely undertaken, with no evidence of increased transport mortality/ major morbidity or higher early surgical mortality.

Impact of mitral regurgitation on left ventricular remodeling and function in children with rheumatic heart disease.

The impact of mitral regurgitation (MR) from pediatric rheumatic heart disease (RHD) and its effect on left ventricular (LV) remodeling and function following surgical intervention is uncertain. The objective is to explore the impact of mitral valve (MV) surgeries on myocardial mechanics, remodeling and function and identify pre-operative predictors of post-operative dysfunction which may contribute to the optimal timing of intervention. A retrospective review of echocardiographic data was performed of eighteen pediatric patients with RHD (median 9yrs, IQR 6-12) who underwent MV surgery. Echocardiograms pre-operatively and a median of 13.5 months (IQR 10.2-15) following intervention were compared to controls. Pre-operative LV end-diastolic indexed volumes (LVEDVi) were significantly increased compared to controls and remained persistently larger post-operatively. LV ejection fraction (LVEF) (pre 62.6% ± 6.1, post 51.7% ± 9.7, p = 0.002), and global longitudinal strain (GLS) (pre - 24.3 ± 4.1, post - 18.2 ± 2.6, p < 0.001) decreased post-operatively at mid-term follow-up. Pre-operative LVEDVi was a significant predictor of post-operative LVEF, with a cut-off of ≥ 102 ml/m2 associated with LV dysfunction (LVEF < 55%; sensitivity 70%, specificity 75%). Pre-operative LVEDVi also negatively correlated with GLS (r = - 0.58, p = 0.01). LV dimensions and volumes remain persistently larger than controls while LV function decreases post-surgical alleviation of MR in paediatric RHD. Pre-operative LVEDVi predicted post-operative LV dysfunction and utilising LV indexed volumes in directing timing of surgical planning should be considered. Further studies are required to investigate whether timely alleviation of MR before significant LV dilatation and remodeling occur may substantially prevent LV dysfunction and improve outcomes.

Bilateral aortico-atrial tunnels.

This report describes the investigation, diagnosis, and surgical correction of two aortico-atrial tunnels running from the noncoronary sinus of Valsalva to both the left and right atria in an asymptomatic 8-year-old boy.

Benefits of an international working exchange in pediatric cardiology.

This report describes a 1-year exchange between members of two pediatric cardiology centers: one in Canada and one in Australia. Five cardiologists participated in sequence, fully engaging in the activities of the host department. The motivation of the exchange was broadly educational including clinical experience, shared expertise, teaching, and research collaboration. Structured debriefing confirmed the value of the exchange. In addition to the experience of working in a different medical system, eight research papers were developed, with two research projects ongoing as well as subsequent exchanges of nursing and technical personnel. Interchange between two academic departments can add strength to both and allow development of new skills and research activity.

The Fontan procedure: contemporary techniques have improved long-term outcomes.

BACKGROUND: To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection. METHODS AND RESULTS: Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution. There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were preoperative elevated pulmonary artery pressures (P=0.002) and common atrioventricular valve (P=0.04). Fontan was taken down during hospital stay in 7 patients. A mean of 12+/-6 years of follow-up was obtained in the 257 nonforeign Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%). Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P=0.004). Nine pts required heart transplantation (8 atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P=0.02). Freedom from Fontan failure (death, take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac conduits, no death, SVT, or failure was observed. CONCLUSIONS: The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less arrhythmia and are likely to have failure of their Fontan circulation postponed.

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. One-third (220 of 675) had undergone previous palliation. Median age at repair was 2 years in the first 8 years, and 1 year from 1988 onward. A transannular incision was performed in 75% of cases and autologous pericardium was the material used to patch this incision in 92% of cases. RESULTS: There were 7 hospital deaths (1%). Eight patients died during follow-up (2 sudden unexpected and 6 noncardiac deaths). Mean follow-up was 11.7 ± 6.3 years. Twenty-five years' survival was 97% (95% confidence interval [CI]: 95% to 98%). Twenty-five years' freedom from implantation of a valved conduit was 84.6% (95% CI: 77.8% to 89.5%). By multivariable analysis, prior palliation and younger age at repair were predictive of implantation of a valved conduit (hazard ratio: 2.4, 95% CI: 1.3 to 4.6, p = 0.008; hazard ratio: 0.70, 95% CI: 0.50 to 0.96, p = 0.03, respectively). CONCLUSIONS: During long-term follow-up, transatrial repair of tetralogy of Fallot was associated with a minimal risk of sudden death and low rate of reintervention for right ventricular dilation and residual outflow tract obstruction.

A national prospective surveillance study of acute rheumatic fever in Australian children.

BACKGROUND: Acute rheumatic fever (ARF) is an important cause of heart disease in Indigenous people of northern and central Australia. However, little is known about ARF in children across all Australian population groups. This national prospective study was conducted to determine patterns of disease, and populations and regions at highest risk. METHODS: The Australian Paediatric Surveillance Unit surveillance model was used to collect data on children with ARF across Australia. Children up to 15 years of age were included if they had an ARF episode diagnosed between October 1, 2007 and December 31, 2010 that met the case definition. RESULTS: ARF was identified in 151 children: 131 Indigenous Australians, 10 non-Indigenous Australians, 8 Pacific Islanders and 1 African (1 unknown). Common presenting features were joint symptoms, fever and carditis. Sydenham chorea was reported in 19% of children. Aseptic monoarthritis was a major manifestation in 19% of high-risk children. Seven non-Indigenous Australian children presented with classic, highly specific features compared with 23% of high-risk children, suggesting that subtle presentations of ARF are being missed in non-Indigenous children. Recent sore throat was reported in 33% of cases, including 25% of remote Indigenous children. There were delays in presentation to care and referral to higher-level care across urban/rural and remote areas. CONCLUSIONS: ARF may be more common than previously thought among low-risk children. These data should prompt an awareness of ARF diagnosis and management across all regions, including strategies for primary prevention. There should be renewed emphasis on treatment of sore throat in high-risk groups.

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. RESULTS: From 1977 to 2009, 123 consecutive neonates (<30 days) and infants (31 days to 1 year) underwent relief of congenital aortic stenosis. Median age at procedure was 27 days (6 to 76 days). Twenty-year survival was 80 ± 7%. Fifty-four patients required a re-intervention and freedom from re-intervention was 55 ± 6% at 10 years and 40 ± 6% at 20 years. By multivariate analysis, having the relief of stenosis by balloon valvuloplasty and undergoing initial treatment as a neonate were predictive of re-intervention. Freedom from re-intervention at 5 years was 27% after balloon valvuloplasty versus 65% after surgery. At latest follow-up, an additional 16 patients had moderate or severe stenosis and 8 had regurgitation. Freedom from re-intervention or stenosis was 39 ± 5% at 15 years. By multivariate analysis, balloon valvuloplasty (p < 0.001) and treatment as a neonate (p = 0.003) were again predictive of stenosis or re-intervention. Thirty-five patients ultimately needed a valve replacement. Significant predictor of the requirement of valve replacement was unicuspid aortic valve (p < 0.001). Freedom from valve replacement was 55 ± 7% at 20 years. CONCLUSIONS: Surgical valvuloplasty remains the best approach to treat neonates and infants with congenital aortic stenosis. After surgery, a higher proportion of patients remain free of re-intervention than after interventional catheterization and the relief of their stenosis lasts longer.

Long-term outcomes after atrioventricular valve operations in patients undergoing single-ventricle palliation.

BACKGROUND: Outcomes after atrioventricular (AV) valve operations in patients with functional single ventricles are unclear. METHODS: From 1988 to 2010, 76 consecutive patients with single ventricles underwent AV valve operations for regurgitation at a single institution. Five replacements, 66 repairs, and 5 valve closures were performed at a median age of 1 year (range, 1 day-14 years) on 43 tricuspid, 9 mitral, and 24 common AV valves. RESULTS: Hospital mortality was 17% (13/76). The follow-up was 100% complete. There were 15 late deaths. There were 48 survivors with a mean follow-up of 8.3±6 years. One- and 10-year Kaplan-Meier survival after AV valve operations was 72% (95% confidence interval [CI], 60%-81%) and 61% (95% CI, 48%-71%), respectively. Independent predictors of overall mortality were presence of a common AV valve (p=0.03), requirement for postoperative mechanical circulatory support (p=0.02), and timing of valve operations between initial palliation and performance of a bidirectional cavopulmonary shunt (BCPS) (p=0.047). Ten-year freedom from valve reoperation and from thromboembolic events of hospital survivors was 56% (95% CI, 38%-70%) and 70% (95% CI, 56%-80%), respectively. At last follow-up, 11 of 48 surviving patients (23%) had moderate to severe regurgitation, and pacemaker implantation was required in 6 patients. Only 34 patients reached the stage of Fontan completion. CONCLUSIONS: AV valve regurgitation is a considerable burden for the patient with a single ventricle, especially when appearing at a young age. A quarter of patients died within the first year after operation, and they had considerable morbidity in terms of reoperation, thromboembolic events, and pacemaker implantation. Their chances of reaching Fontan completion seemed decreased.

Outcomes of the arterial switch operation for transposition of the great arteries: 25 years of experience.

BACKGROUND: Studies on long-term outcomes of the arterial switch operation (ASO) for transposition of the great arteries (TGA) are uncommon. Thus, we sought to determine the long-term outcomes for patients after ASO performed at a single institution over a 25-year period. METHODS: From 1983 to 2009, 618 patients underwent the ASO for TGA and were reviewed retrospectively. RESULTS: Overall early mortality was 2.8%. Risk factors for early death on multivariate analysis were resection of left ventricular outflow tract obstruction at time of ASO (p = 0.001), weight less than 2.5 kg at time of ASO (p < 0.001), associated aortic arch obstruction (p = 0.043), and the need for postoperative extracorporeal membrane oxygenation (p < 0.001). Mean follow-up time was 10.6 years (range 2 months to 26.1 years). Late mortality was 0.9%. Reintervention was significantly higher (p < 0.001) in patients with ventricular septal defect or arch obstruction versus those without them (25.2% and 23.4% vs 5.9% at 15- year follow-up). Risk factors for late reintervention were left ventricular outflow tract obstruction at time of ASO (p < 0.001) and a greater circulatory arrest time (p < 0.001). Freedom from at least moderate neoaortic valve regurgitation for the entire cohort was 98.7% (95% confidence interval 96.8 to 99.5%) at 20 years. Mild neoaortic regurgitation was seen in 25.6% of patients at mean follow-up. All patients were free of arrhythmia and heart failure symptoms at last follow-up. CONCLUSIONS: The ASO can be performed with good long-term results. Patients with associated ventricular septal defect and aortic arch obstruction warrant close follow-up.

Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance.

OBJECTIVES: This study examined survival after surgical palliation in children with single-ventricle physiology. BACKGROUND: Contemporary surgical outcomes for the entire population of newborns undergoing single-ventricle palliation are unclear. METHODS: In a single-center review of 499 consecutive patients undergoing univentricular palliation from 1990 to 2008, predictors of mortality were determined using multivariate risk analysis, stratified for each post-operative stay and interim states. RESULTS: After 2000, the population comprised more patients with dominant right ventricle (66% vs. 36%) and hypoplastic left heart syndrome (HLHS) (47% vs. 13%). Median age at bidirectional cavopulmonary shunt (BCPS) decreased from 15 months (10 to 22 months) before 2000 to 4 months (3.3 to 9 months) thereafter. Survival rates at 1, 5, and 10 years were, respectively, 82% (95% confidence interval [CI]: 79% to 85%), 74% (95% CI: 70% to 78%), and 71% (95% CI: 67% to 75%). Throughout the study, atrioventricular valve regurgitation (hazard ratio [HR]: 1.8; p = 0.008), not having transposition (HR: 2.0; p = 0.013), and heterotaxia (HR: 2.0; p = 0.026) were predictors of mortality. The most potent risk factor was right ventricular (RV) dominance (HR: 2.2; p = 0.001) because of its impact before BCPS. HR for death in patients with RV dominance went from 2.8 (95% CI: 1.4 to 5.7; p = 0.005) before BCPS to 1.0 (95% CI: 0.5 to 2.1; p = 0.98) thereafter. Survival of patients with RV dominance, adjusted for the risk factors noted here, improved over the study period (p = 0.001). CONCLUSIONS: Considerable mortality is still observed during the first years of life among patients with single ventricle. RV dominance is the most important risk factor for death but only before BCPS.

Outcomes of the arterial switch operation in patients with Taussig-Bing anomaly.

BACKGROUND: The arterial switch operation (ASO) is associated with poorer outcomes in patients with Taussig-Bing anomaly (TBA) compared with transposition of the great arteries (TGA). We describe the outcomes after ASO in patients with TBA at a single institution. METHODS: Between 1983 and 2009, 57 patients with TBA underwent the ASO at the Royal Children's Hospital in Melbourne. RESULTS: Hospital mortality was 5.3% (3 of 57). Larger weight at operation (p=0.015), pulmonary artery banding prior to ASO (p=0.049) and concurrent pulmonary artery banding (p=0.049) were risk factors of early death. Actuarial survival was 94% at 15 years. Follow-up was 84% complete with a mean follow-up of 9.8±6.7 years (range, 6 days to 19.1 years). There was no late mortality. Reintervention was required in 24.4% (11 of 45). Longer cross-clamp time (p=0.027) was a risk factor for reintervention. Freedom from reintervention was 75.3% at 15 years. After ASO, 2.2% (1 of 45) presented with sub-neopulmonary obstruction and 13.3% (6 of 45) had moderate or more neoaortic insufficiency (neo-AI). Surgery prior to ASO was a risk factor for sub-neopulmonary obstruction (p=0.049) and moderate or more neo-AI (p=0.016). Freedom from moderate or more neo-AI was 91.1% at 10 years. CONCLUSIONS: Early mortality has improved over time with no mortality occurring in the last decade. Although patients are doing well on late follow-up, many patients require reintervention and show progression of neo-AI. Close long-term follow-up is warranted as patients are likely to require further reintervention in the second decade after TBA repair.