Clinical profile of paediatric acute rheumatic fever and rheumatic heart disease in Western Australia: 1987 to 2020.

AIM: To describe the clinical profile of acute rheumatic fever (ARF) presentations to paediatric cardiology tertiary services in Western Australia (WA). METHODS: A retrospective clinical audit of individuals with confirmed ARF referred to the only paediatric tertiary cardiac service in WA (1 January 1987 to 31 December 2020). Comparisons between inpatient, outpatient, remote and non-remote groups were assessed. RESULTS: Four hundred seventy-one episodes of ARF in 457 individuals (235 male; median age = 8 years) met clinical criteria. The majority were Aboriginal and Torres Strait Islander children (91.2%), with 62.1% living in remote areas. The number of ARF and rheumatic heart disease (RHD) diagnoses per year increased from 1987 to 2017 with notable peaks in 2013 and 2017. The average annual incidence of tertiary-referred ARF in WA of 4-15-year-olds from 1987 to 2020 was 4.96 per 100 000. ARF features included carditis (59.9%), chorea (31%), polyarthritis (30%) and polyarthralgia (24.2%). RHD was evident in 61.8% of cases and predominantly manifested as mitral regurgitation (55.7%). Thirty-four children (7.4%) with severe RHD underwent valvular surgery. 12% had at least one recurrent ARF episode. Remote individuals had more than double the rate of recurrence compared to non-remote individuals (P = 0.0058). Compared to non-remote episodes, remote presentations had less polyarthritis (P = 0.0022) but greater proportions of raised ESR (P = 0.01), ASOT titres (P = 0.0073), erythema marginatum (P = 0.0218) and severe RHD (P = 0.0133). CONCLUSION: The high proportion of Aboriginal and Torres Strait Islander Australians affected by ARF/RHD in WA reflects the significant burden of disease within this population. Children from remote communities were more likely to present with concurrent severe RHD. Our study reinforces the persisting need to improve primary and secondary ARF initiatives in rural and remote communities.

3D Printed Cardiac Models as an Adjunct to Traditional Teaching of Anatomy in Congenital Heart Disease-A Randomised Controlled Study.

INTRODUCTION: Three-dimensional (3D) printed cardiac models are increasingly being used for medical education, simulation and training, communication, surgical planning and research. Given the complexities of congenital cardiac anatomy, 3D printing is well suited as an adjunct to traditional teaching methods. This study aims to explore the influence of 3D printed cardiac models as a teaching aid for nurses and paediatric trainees. We hypothesise that using 3D models as an adjunct to didactic teaching methods improves knowledge and confidence levels of participants, regardless of their cardiology experience. METHOD: A prospective randomised study was performed recruiting paediatric nurses and doctors at a tertiary paediatric hospital. All participants undertook traditional congenital cardiac teaching describing normal cardiac anatomy and seven congenital lesions of increasing complexity (atrial septal defect, ventricular septal defect, vascular ring, partial anomalous pulmonary venous return, tetralogy of Fallot, transposition of the great arteries, and double outlet right ventricle). The intervention group received an additional recorded demonstration while handling 3D printed models of a normal heart and the same lesions. Pre- and post-intervention assessments were completed using a subjective Likert-scale questionnaire and objective multiple-choice examination. RESULTS: A total of 73 health practitioners (30 cardiac nurses and 43 paediatric trainees) were included. Subjective knowledge and confidence levels substantially improved in the intervention group (both p<0.001), with no differences observed in the control group. Greater improvement in both subjective and objective post-test scores was observed in the intervention group. A pronounced difference between pre- and post-teaching objective examination scores was found in both groups (p=0.002), with larger improvements observed in the intervention group. The mean score in the intervention group after teaching increased by 4.27 (21.4% improvement), as opposed to 2.28 (11.4% improvement) in the control group. There was no difference in pre-test score or post-test improvement based on previous cardiology experience. DISCUSSION: Three-dimensional (3D) printed cardiac models, when used as an adjunct to traditional teaching methods, substantially improve knowledge and confidence levels of health professionals on a range of congenital cardiac lesions. These models enhance the learners' educational experience and understanding of cardiac anatomy by overcoming the limitation of two-dimensional representations of 3D structures.

Left ventricular remodelling in rheumatic heart disease - trends over time and implications for follow-up in childhood.

BACKGROUND: Rheumatic heart disease (RHD) is the most common form of acquired heart disease worldwide. In RHD, volume loading from mitral regurgitation leads to left ventricular (LV) dilatation, increased wall stress, and ultimately LV dysfunction. Improved understanding of LV dynamics may contribute to refined timing of intervention. We aimed to characterize and compare left ventricular remodelling between rheumatic heart disease (RHD) severity groups by way of serial echocardiographic assessment of volumes and function in children. METHODS: Children with RHD referred to Perth Children's Hospital (formally Princess Margaret Hospital) (1987-2020) were reviewed. Patients with longitudinal pre-operative echocardiograms at diagnosis, approximately 12 months and at most recent follow-up, were included and stratified into RHD severity groups. Left ventricular (LV) echocardiographic parameters were assessed. Adjusted linear mixed effect models were used to compare interval changes. RESULTS: 146 patients (median age 10 years, IQR 6-14 years) with available longitudinal echocardiograms were analysed. Eighty-five (58.2%) patients had mild, 33 (22.6%) moderate and 28 (19.2%) severe RHD at diagnosis. Mean duration of follow-up was 4.6 years from the initial diagnosis. Severe RHD patients had significantly increased end-systolic volumes (ESV) and end-diastolic volumes (EDV) compared to mild/moderate groups at diagnosis (severe versus mild EDV mean difference 27.05 ml/m2, p < 0.001, severe versus moderate EDV mean difference 14.95 ml/m2, p = 0.006). Mild and moderate groups experienced no significant progression of changes in volume measures. In severe RHD, LV dilatation worsened over time. All groups had preserved cardiac function. CONCLUSIONS: In mild and moderate RHD, the lack of progression of valvular regurgitation and ventricular dimensions suggest a stable longer-term course. Significant LV remodelling occurred at baseline in severe RHD with progression of LV dilatation over time. LV function was preserved across all groups. Our findings may guide clinicians in deciding the frequency and timing of follow-up and may be of clinical utility during further reiterations of the Australia and New Zealand RHD Guidelines.

Myopericarditis following COVID-19 vaccination in adolescent triplets.

Multiple studies have reported myocarditis and pericarditis after the Pfizer-BioNTech coronavirus disease 2019 messenger ribonucleic acid vaccine. We describe male adolescent triplets who presented with myopericarditis within one week following vaccine administration.

A Single-Centre Retrospective Review of Modified Blalock-Taussig Shunts: A 22-Year Experience.

INTRODUCTION: This single-centre retrospective study explores demographics and outcomes of patients who underwent a modified Blalock-Taussig shunt (MBTS) over a 22-year period. The predominant surgical approach in this study is a lateral thoracotomy, in contrast to a midline sternotomy. Risks and outcomes of this approach are compared with national and international literature. MATERIALS AND METHODS: Demographic, anatomical, clinical, surgical and outcome data of all patients who underwent a MBTS between 2000 and 2022 were collected and analysed, excluding Norwood procedures, which are not performed at this institution. Short- and long-term morbidity and mortality is described. RESULTS: Over the 22-year study period, 185 MBTS were performed in 162 patients, at a median age of 16 days (interquartile range [IQR] 5-59 days) and weight of 3.47 kg (IQR 3-4.25 kg, minimum weight 2 kg). Of these, 79% of patients had a biventricular circulation. Cardiac diagnoses included both univentricular and biventricular anatomy; tetralogy of Fallot (TOF) (36%), transposition of the great arteries/ventricular septal defect/pulmonary stenosis (TGA/VSD/PS) (11%), pulmonary atresia with intact ventricular septum (PA/IVS) (23%), pulmonary atresia with ventricular septal defect (PA/VSD) (14%), other (16%). The most common size of MBTS was 4 mm (71%); 93% were performed via a lateral thoracotomy. There were 47 cases of major operative morbidity, which did not differ significantly with cardiac diagnosis. Overall all-cause mortality was 13.5%. Early operative mortality was 4.3%. Mortality varied with cardiac diagnosis, 6% with TOF and 19% with PA/IVS. There was no era effect on mortality rates, however a lower frequency of major morbidity (23% vs 7%, p=0.03) was observed in the most recent third of the study period. Risk factors for shunt reintervention or mortality included weight <2.5 kg (HR=2.79 [1.37, 5.65], p=0.005), and pre- (HR=3.31 [1.86, 5.9], p<0.001) or postoperative lactic acidosis (HR=1.37 [1.25,1.5], p<0.001). These rates are comparable to those in the literature, with the predominant approach a midline sternotomy. CONCLUSION: Mortality rates and risk factors for adverse outcomes are comparable to those previously reported for both univentricular and biventricular groups. These results highlight that outcomes of MBTS performed via lateral thoracotomy are comparable to those by midline sternotomy as reported in the literature. Operating via the lateral approach may be advantageous as it avoids the complications of a midline sternotomy.

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.

Pre- and post-intervention study examining immunisation rates, documentation, catch-up delivery and the impact of a dedicated immunisation service at a tertiary paediatric hospital.

AIM: To explore immunisation rates and catch-up delivery to children admitted to hospital before and after an immunisation service was commenced. METHODS: This pre- and post-intervention study examined 300 admissions prior to (cohort 1) and 300 following (cohort 2) the introduction of an immunisation service. Immunisation rates, documentation, catch-up delivery and accuracy of the Australian Immunisation Register (AIR) were examined. RESULTS: On admission, 75% (cohort 1) and 89% (cohort 2) were up-to-date with immunisations. Immunisation history was documented in the medical record in 78% and requirement for catch-up documented in 10%. AIR was incorrect in one-third of cases. By 3 months following discharge, 28% (cohort 1) and 64% (cohort 2) of patients were immunised. CONCLUSIONS: Children admitted to hospital have lower immunisation rates than the national average. Documentation was poor, opportunities for catch-up were missed and AIR is error-prone. Catch-up rates increased following the introduction of an immunisation service.

Flail anterior tricuspid valve leaflet in a neonate: association with maternal antiphospholipid syndrome.

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.