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.

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Kawasaki disease is a common systemic vasculitis of childhood that may result in life-threatening coronary artery abnormalities. Despite an overlap of clinical features with toxic shock syndrome, children with Kawasaki disease generally do not develop shock. We report two cases of older children who presented with a toxic shock-like illness, and were diagnosed with Kawasaki disease when coronary artery abnormalities were found on echocardiography, in keeping with the recently described 'Kawasaki disease shock syndrome'. Clinicians should consider Kawasaki disease in all children presenting with toxic shock and assess for coronary artery damage.

This brief report describes a case of flail anterior tricuspid valve leaflet in a neonate associated with maternal antiphospholipid syndrome. Fetal echocardiography at 27 weeks of gestation showed competent atrioventricular valves with biventricular echogenic chordae. Fetal distress was noted at delivery, and echocardiography showed a flail anterior leaflet of the tricuspid valve with severe regurgitation. Possible causation and implications of maternal antiphospholipid syndrome are discussed.