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.

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.

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.

A Review of Cardiac Surgical Procedures and Their Outcomes for Paediatric Rheumatic Heart Disease in Western Australia.

INTRODUCTION: Surgical intervention is an important treatment modality for advanced rheumatic heart disease (RHD). This study aimed to describe patient characteristics and outcomes from cardiac surgery for RHD in patients referred to the only tertiary paediatric hospital in Western Australia. METHODS: An analysis of patient characteristics and cardiac surgery outcomes in patients with RHD was undertaken, using data from clinical cardiac databases, medical notes, and correspondence from rural outreach clinics. RESULTS: 29 patients (59% female, 97% Aboriginal, Maori or Pacific Islander) underwent 41 valve interventions over 34 cardiac surgeries for RHD between 2000-2018. Median age at first surgery was 12.2 (range 4-16) years. Severe mitral regurgitation (MR) was the most common indication for primary surgery (62%), followed by mixed mitral regurgitation/aortic regurgitation (21%) and severe aortic regurgitation (17%). Mitral valve repair was the most common valve intervention (56%). Two patients had mitral valve replacement (MVR) at first operation, two patients had MVR at second operation and two had MVR at third operation. There was no early mortality. One patient required early (<30 days) reoperation for aortic valve repair failure. Two patients had late reoperations at 3.3 and 6.1 months after the first procedure for MR. Four (14%) patients experienced documented ARF recurrences. Late mortality occurred in 3 (10%) patients, all due to cardiac causes. On last follow-up echocardiogram 5 patients (17%) had moderate MR and none had severe MR. CONCLUSIONS: This is the first study to describe characteristics and outcomes in WA paediatric patients having surgery for RHD. Outcomes are comparable to similar studies, with favourable long-term survival.

Intraoperative Management of Adult Patients on Extracorporeal Membrane Oxygenation: An Expert Consensus Statement From the Society of Cardiovascular Anesthesiologists-Part I, Technical Aspects of Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation (ECMO) is used to support patients with refractory cardiopulmonary failure. Given ECMO's increased use in adults and the fact that many ECMO patients are cared for by anesthesiologists, the Society of Cardiovascular Anesthesiologists ECMO working group created an expert consensus statement that is intended to help anesthesiologists manage adult ECMO patients who are cared for in the operating room. In the first part of this 2-part series, technical aspects of ECMO are discussed, and related expert consensus statements are provided.

Intraoperative Management of Adult Patients on Extracorporeal Membrane Oxygenation: An Expert Consensus Statement From the Society of Cardiovascular Anesthesiologists-Part II, Intraoperative Management and Troubleshooting.

In the second part of the Society of Cardiovascular Anesthesiologists Extracorporeal Membrane Oxygenation (ECMO) working group expert consensus statement, venoarterial (VA) and venovenous (VV) ECMO management and troubleshooting in the operating room are discussed. Expert consensus statements are provided about intraoperative monitoring, anesthetic drug dosing, and management of intraoperative problems in VA and VV ECMO patients.

Birth prevalence of congenital heart defects in Western Australia, 1990-2016.

AIM: To describe the birth prevalence and characteristics of congenital heart defects in a geographically defined Australian population. METHODS: This descriptive, population-based study examined congenital heart defects in live births, stillbirths and pregnancy terminations ascertained by the Western Australian Register of Developmental Anomalies, 1990-2016. Birth prevalence (per 1000 births) was stratified by severity, known cause, maternal and birth characteristics, and primary diagnosis; and prevalence ratios were calculated for Aboriginal versus non-Aboriginal births. Temporal trends in prevalence, diagnosis age and infant mortality were examined. RESULTS: For births 1990-2010 (allowing 6 years for complete case ascertainment by 2016), 6419 cases were identified; prevalence was 11.5 per 1000 births (95% confidence interval (CI), 11.2-11.8). Severe defects were ascertained in 2.5 per 1000 births (95% CI 2.4-2.7). Most cases were liveborn (5842, 91.0%), and 28.9% had other birth defects. Prevalence was slightly higher in Aboriginal births (prevalence ratio 1.1; 95% CI 1.0-1.2); and the infant mortality rate more than doubled (13.4% vs. 5.8%, P < 0.001). Prenatal diagnosis increased over time but, in remote areas, was significantly lower for Aboriginal versus non-Aboriginal cases (3.1% vs. 9.3%; P = 0.008). A cause was identified in 920 cases (14.3%), more often for severe defects (347, 24.4%); 63% of known causes were rare diseases. Congenital heart defects associated with fetal alcohol spectrum disorder were much more common in Aboriginal births (prevalence ratio 82; 95% CI 28-239). CONCLUSIONS: Earlier detection of congenital heart defects and improved survival has occurred over time, although discrepancies between ethnic groups and regions warrant further investigation and strategic action.

Suspected Lower Extremity Ischemia After End-to-Side Femoral Arterial Grafting for VA-ECMO.

Lower extremity ischemic complications are frequently encountered after femoral cannulation for venoarterial extracorporeal membrane oxygenation (VA-ECMO). Many are attributed to mechanical obstruction of distal arterial blood flow related to intraluminal positioning of the arterial cannula. Routine use of distal perfusion catheters is ineffective at eliminating the development of these serious complications. Side- arm grafting instead of direct arterial cannulation is suggested as an alternative. Here, the authors present a case wherein a patient developed suspected lower extremity ischemia from hyperperfusion after femoral VA-ECMO cannulation during use of an arterial side- arm graft, calling into question the benefits of this cannulation strategy.

Intraoperative Management of Adult Patients on Extracorporeal Membrane Oxygenation: an Expert Consensus Statement From the Society of Cardiovascular Anesthesiologists-Part I, Technical Aspects of Extracorporeal Membrane Oxygenation.

Extracorporeal membrane oxygenation (ECMO) is used to support patients with refractory cardiopulmonary failure. Given ECMO's increased use in adults and the fact that many ECMO patients are cared for by anesthesiologists, the Society of Cardiovascular Anesthesiologists ECMO working group created an expert consensus statement that is intended to help anesthesiologists manage adult ECMO patients who are cared for in the operating room. In the first part of this 2-part series, technical aspects of ECMO are discussed, and related expert consensus statements are provided.

Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed During Childhood: Results From a National Population-Based Study.

BACKGROUND: Late survival and symptomatic status of children with hypertrophic cardiomyopathy have not been well defined. We examined long-term outcomes for pediatric hypertrophic cardiomyopathy. METHODS: The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0-10 years of age) diagnosed with cardiomyopathy between 1987 and 1996. The primary study end point was time to death or cardiac transplantation. RESULTS: There were 80 patients with hypertrophic cardiomyopathy, with a median age at diagnosis of 0.48 (interquartile range, 0.1, 2.5) years. Freedom from death/transplantation was 86% (95% confidence interval [CI], 77.0-92.0) 1 year after presentation, 80% (95% CI, 69.0-87.0) at 10 years, and 78% (95% CI, 67.0-86.0) at 20 years. From multivariable analyses, risk factors for death/transplantation included symmetrical left ventricular hypertrophy at the time of diagnosis (hazard ratio, 4.20; 95% CI, 1.60-11.05; P=0.004), Noonan syndrome (hazard ratio, 2.88; 95% CI, 1.02-8.08; P=0.045), higher posterior wall thickness z score (hazard ratio, 1.45; 95% CI, 1.22-1.73; P<0.001), and lower fractional shortening z score (hazard ratio, 0.84; 95% CI, 0.74-0.95; P=0.005) during follow-up. Nineteen (23%) subjects underwent left ventricular myectomy. At a median of 15.7 years of follow-up, 27 (42%) of 63 survivors were treated with ß-blocker, and 13 (21%) had an implantable cardioverter-defibrillator. CONCLUSIONS: The highest risk of death or transplantation for children with hypertrophic cardiomyopathy is within 1 year after diagnosis, with low attrition rates thereafter. Many subjects receive medical, surgical, or device therapy.

A population pharmacokinetic study of benzathine benzylpenicillin G administration in children and adolescents with rheumatic heart disease: new insights for improved secondary prophylaxis strategies.

BACKGROUND: Benzathine benzylpenicillin G (BPG) is recommended as secondary prophylaxis to prevent recurrence of acute rheumatic fever and subsequent rheumatic heart disease (RHD). Following intramuscular injection, BPG is hydrolysed to benzylpenicillin. Little is known of the pharmacokinetics of benzylpenicillin following BPG in populations at risk of RHD. METHODS: We conducted a longitudinal pharmacokinetic study of children and adolescents receiving secondary prophylaxis throughout six monthly cycles of BPG. Dried blood spot samples were assayed with LC-MS/MS. Benzylpenicillin concentrations were analysed using non-linear mixed-effects modelling with subsequent simulations based on published BMI-for-age and weight-for-age data. RESULTS: Eighteen participants contributed 256 concentrations for analysis. None had benzylpenicillin concentrations >0.02 mg/L for the full time between doses. The median duration above this target was 9.8 days for those with a lower BMI (<25 kg/m2), who also had lower weights, and 0 days for those with a higher BMI (≥25 kg/m2). Although fat-free mass was a key determinant of benzylpenicillin exposure after a standard dose of BPG, having a higher BMI influenced absorption and almost doubled (increase of 86%) the observed t½. CONCLUSIONS: Few children and adolescents receiving BPG as secondary prophylaxis will achieve concentrations >0.02 mg/L for the majority of the time between injections. The discordance of this observation with reported efficacy of BPG to prevent rheumatic fever implies a major knowledge gap relating to pharmacokinetic/pharmacodynamic relationships between benzylpenicillin exposure and clinical outcomes.

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.

Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy.

BACKGROUND: Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy. METHODS AND RESULTS: The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to <10 years of age at the time of diagnosis. Survival free from death or transplantation was 74% (95% confidence interval, 67-80) 1 year after diagnosis, 62% (95% confidence interval, 55-69) at 10 years, and 56% (95% confidence interval, 46-65) at 20 years. In multivariable analysis, age at diagnosis <4 weeks or >5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy. CONCLUSIONS: Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication.

Detection of congenital heart disease by neonatologist performed cardiac ultrasound in preterm infants.

OBJECTIVE: We aimed to assess the frequency of de novo congenital heart disease (CHD) detection via neonatologist-performed cardiac ultrasounds (NPCU) in premature infants born at <30 weeks of gestation. STUDY DESIGN: In this cross-sectional study (2004-2023) clinicians completing NPCU flagged de novo suspected CHD. All flagged NPCUs were cross-checked with cardiologists to confirm CHD diagnosis. RESULTS: There were 2088 out of 3739 infants (56%) with at least one NPCU; 294 (14%) with cardiology referral. CHD diagnosis was confirmed in 109 of the 2088 (5.2%) infants. All major and critical CHD on NPCU imaging were suspected during NPCU and had prompt referral to the cardiology department. CONCLUSION: De novo presentation of significant CHD continues to occur in the preterm population, emphasizing the need for recognizing CHD during NPCU. Optimizing NPCU training may benefit patients with early cardiology referral and review.

SmartTots: a public-private partnership between the United States Food and Drug Administration (FDA) and the International Anesthesia Research Society (IARS).

A history of the public-private partnership 'SmartTots' between the IARS and FDA is presented. In order to raise money for research to better understand the relationship between sedative and anesthetic agents and neurotoxicity in the developing brain, the FDA approached the IARS in 2008. A partnership was developed over the following 2 years, then a Scientific Advisory Board was created to develop a research agenda. The IARS contributed $200 000 in 2011 to provide initial funding; 33 proposals were submitted in response to a request for proposals in late 2011 and resulted in the awarding of two, $100 000 grants in 2012. An Executive Board was appointed under the leadership of Michael Roizen to spearhead additional fund-raising efforts, and a director of development is working with Dr. Roizen and the Board to raise funds from individuals and organizations. Dr. Roizen has personally committed to a matching grant for anesthesiologists, up to $50 000 per year for 20 years ($1 million). Readers of the journal are encouraged to go to the website www.smarttots.org in order to better understand the issue, to contribute to the research fund themselves, and to encourage their own professional organizations to partner with SmartTots in fund-raising.

Maternal blood pressure trajectories and associations with gestational age at birth: a functional data analytic approach.

BACKGROUND: Research has revealed group-level differences in maternal blood pressure trajectories across pregnancy. These trajectories are typically constructed using clinical blood pressure data and multivariate statistical methods that are prone to bias and ignore the functional, dynamic process underlying a single blood pressure observation. The aim of this study was to use functional data analysis to explore blood pressure variation across pregnancy, and multivariate methods to examine whether trajectories are related to gestational age at birth. METHODS: Clinical blood pressure observations were available from 370 women who participated in a longitudinal pregnancy cohort study conducted in Montreal, Quebec, Canada. Functional data analysis was used to smooth blood pressure data and then to conduct a functional principal component analysis to examine predominant modes of variation. RESULTS: Three eigenfunctions explained greater than 95% of the total variance in blood pressure. The first accounted for approximately 80% of the variance and was characterized by a prolonged-decrease trajectory in blood pressure; the second explained 10% of the variance and captured a late-increase trajectory; and the third accounted for approximately 7% of the variance and captured a mid-decrease trajectory. The prolonged-decrease trajectory of blood pressure was associated with older, and late-increase with younger gestational age at birth. CONCLUSION: Functional data analysis is a useful method to model repeated maternal blood pressure observations and many other time-related cardiovascular processes. Results add to previous research investigating blood pressure trajectories across pregnancy through identification of additional, potentially clinically important modes of variation that are associated with gestational age at birth.

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.