OBJECTIVES: The current Australian epidemiology of Kawasaki disease (KD) is poorly defined. Previous enhanced surveillance (1993–1995) estimated an incidence of 3.7/100 000 <5 years. METHODS: We identified all patients hospitalized in Western Australia (current population ∼2.4 million) 1979 through 2009 with a discharge diagnosis of KD. We reviewed demographic, clinical, laboratory, and echocardiographic data from individual patient files and derived age-specific population estimates. KD diagnosis was made using standard criteria. RESULTS: There were 353 KD cases, with incomplete KD in 34 (9.6%). Male to female ratio was 1.7:1 and median age was 3.8 years (interquartile range 12–60 months). Fifty (18.1%) patients were Asian. Mean annual incidence increased from 2.82 per 100 000 children aged <5 years (95% confidence interval, 1.93–3.99) in 1980 to 1989, to 7.96 (6.48–9.67) in 1990 to 1999, to 9.34 (7.72–11.20) in 2000 to 2009. The highest incidence was 15.7 in 2005. A total of 293 children (83%) received intravenous immunoglobulin and 331 (95.4%) aspirin. Of 282 children who completed echocardiographic studies, 47 (16.7%) had coronary artery (CA) ectasia/dilatation and 19 (6.8%) had CA aneurysms; male gender was significantly associated with CA abnormalities. CONCLUSIONS: KD epidemiology in Western Australia mirrors that of other industrialized, predominantly European-Caucasian populations. The rising incidence likely reflects both improved ascertainment and a real increase in disease burden. The current Australian incidence is threefold higher than previously reported and similar to the United Kingdom. The CA outcomes, which include the pre-intravenous immunoglobulin era, are comparable to those reported elsewhere.

Cancer therapy related cardiac dysfunction (CTRCD) is an area of increasing focus, particularly during the survivorship period, for paediatric, adolescent and adult cancer survivors. With the advent of immunotherapy and targeted therapy, there is a new set of mechanisms from which paediatric and young adult patients with cancer may suffer cardiovascular injury. Furthermore, cardiovascular disease is the leading cause of morbidity and mortality in the survivorship period. The recently established Australian Cardio‐Oncology Registry is the largest and only population‐based cardiotoxicity database of paediatric and adolescent and young adult oncology patients in the world, and the first paediatric registry that will document cardiotoxicity caused by chemotherapy and novel targeted therapies using a prospective approach. The database is designed for comprehensive data collection and evaluation of the Australian practice in terms of diagnosis and management of CTRCD. Using the Australian Cardio‐Oncology Registry critical clinical information will be collected regarding predisposing factors for the development of CTRCD, the rate of subclinical left ventricular dysfunction and transition to overt heart failure, further research into protectant molecules against cardiac dysfunction and aid in the discovery of which genetic variants predispose to CTRCD. A health economic arm of the study will assess the cost/benefit of both the registry and cardio‐oncology clinical implementation. Finally, an imaging arm will establish if exercise cardiac magnetic resonance imaging and VO2 max testing is a more sensitive predictor of cardiac reserve in paediatric and adolescent and young adult oncology patients exposed to cardiac toxic therapies.

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Background The Technical Performance Score (TPS) developed by Boston Children’s Hospital showed surgical outcomes correlate with adequacy of technical repair when implemented on pre-discharge echocardiograms. We applied this scoring system to intraoperative imaging in a tertiary UK congenital heart surgical centre. Methods After a period of training, intraoperative TPS (epicardial and/or transesophageal echocardiography) was instituted. TPS was used to inform intraoperative discussions and recorded on a custom-made database using the previously published scoring system. After a year, we reviewed the feasibility, results and relationship between the TPS and mortality, extubation time and length of stay. Results From 01 September 2015 to 04 July 2016, there were 272 TPS procedures in 251 operations with 208 TPS recorded. Seven patients had surgery with no documented TPS, three had operations with no current TPS score template available. Patients left the operating theatre with TPS optimal in 156 (75%), adequate 34 (16%) and inadequate 18 (9%). Of those with an optimal score on leaving theatre, ten had more than one period of cardiopulmonary bypass. All four deaths <30 days after surgery (1.9%) had optimal TPS. There was a statistically significant difference in extubation times in the RACHS category 4 patients (3 days vs 5 days, P < 0.05) and in PICU and total length of stay in the RACHS category three patients (2 and 8 days vs 12.5 and 21.5 days respectively) if leaving theatre with an inadequate result. Conclusions Application of intraoperative TPS is feasible and provides a way of objectively recording intraoperative imaging assessment of surgery. An ‘inadequate’ TPS did not predict mortality but correlated with a longer ventilation time and longer length of stay compared to those with optimal or adequate scores.

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