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Introduction: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac lesion with significant anatomical heterogeneity. Surgical planning of borderline cases remains challenging and is primarily based on echocardiography. The aim was to identify echocardiographic parameters that correlate with surgical outcome and to develop a discriminatory calculator., Methods: Retrospective review of all pulmonary atresia with intact ventricular septum cases at a statewide tertiary paediatric cardiac centre was performed between 2004 and 2020. Demographic, clinical, and echocardiographic data were collected. Logistic regression was used to develop a discriminatory tool for prediction of biventricular repair., Results: Forty patients were included. Overall mortality was 27.5% ( n = 11) and confined to patients managed as univentricular (11 vs 0, p = 0.027). Patients who underwent univentricular palliation were more likely to have an associated coronary artery abnormality (17 vs 3, p = 0.001). Fifteen surviving patients (51.7%) achieved biventricular circulation while 14 (48.3%) required one-and-a-half or univentricular palliation. Nineteen patients (47.5%) underwent percutaneous pulmonary valve perforation. No patients without tricuspid regurgitation achieved biventricular repair. The combination of tricuspid valve/mitral valve annulus dimension ratio and right ventricle/left ventricle length ratio identified biventricular management with a sensitivity of 93% and specificity of 96%. An online calculator has been made available., Conclusion: Pulmonary atresia with intact ventricular septum is a challenging condition with significant early and interstage morbidity and mortality risk. Patient outcomes were comparable to internationally reported data. Right ventricle/left ventricle length and tricuspid valve/mitral valve annulus dimension ratios identified a biventricular pathway with a high level of sensitivity and specificity. Absent tricuspid regurgitation was associated with a univentricular outcome.

Aim: Rheumatic heart disease (RHD), a consequence of acute rheumatic fever (ARF), continues to cause significant morbidity and mortality in susceptible young people. Secondary antibiotic prophylaxis is an effective strategy to prevent ARF recurrence and RHD progression. However, the proportion of patients receiving the recommended minimum benzathine penicillin injections (>80%) remains low. This retrospective cohort study reviews the utility of the Hospital in the Home nursing service in providing secondary prophylaxis for outpatients in a metropolitan state capital., Methods: Fifty-eight patients with ARF/RHD receiving secondary prophylaxis through the Hospital in the Home service between 1 July 2012 and 30 June 2020 were included. Compliance of secondary prophylaxis, specialist review and echocardiogram frequency were compared against the recommendations from the Australian RHD guidelines., Results: Forty-six (79%) patients received >80% of recommended doses, with 36% (n = 21) receiving 100% and a further 29% (n = 17) achieving compliance of 90%-99%. We found that 35% of patients attended all specialist reviews (including echocardiography) at the recommended frequency, with 75% of those not attending all reviews, missing only one or two appointments. Compliance was greater in those with more severe disease. In the 12 (21%) patients who did not meet the >80% target, compliance ranged between 33% and 79% (mean 64.5%) and all had priority 3 disease., Conclusion: Community-based administration of secondary prophylaxis through a registry-based system is an effective strategy of improving compliance within a metropolitan area and should be utilised in a culturally safe and collaborative manner to increase uptake., (© 2024 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

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., Competing Interests: Statement of Competing Interests The authors declare no known competing interests., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Competing Interests: Conflicts of Interest There are no conflicts of interest to disclose.

Objective: This study sought to investigate the characteristics, morbidity (including the rate of infective endocarditis and valve replacement) and mortality of individuals undergoing percutaneous pulmonary valve implantation in Australia and New Zealand since the procedure has been performed., Background: The outcomes of percutaneous pulmonary valve implantation in Australia and New Zealand have not been evaluated. Recent international data, including patients from New Zealand, suggests the rate of infective endocarditis is not insignificant., Methods: A retrospective multi-site cohort study was undertaken via medical record review at the centres where percutaneous pulmonary valve implantation has been performed. All procedures performed from 2009-March 2018 were included. Individuals were identified from local institution databases. Data was collected and analysed including demographics, details at the time of intervention, haemodynamic outcome, post procedure morbidity and mortality. Multi-site ethics approval was obtained., Results: One hundred and seventy-nine (179) patients attended the cardiac catheter laboratory for planned percutaneous pulmonary valve implantation. Of these patients, 172 underwent successful implantation. Tetralogy of Fallot and pulmonary atresia were the most common diagnoses. The median age at procedure was 19 years (range 3-60 yrs). There was a significant improvement in the acute haemodynamics in patients undergoing percutaneous pulmonary valve implantation for stenosis. Seven (7) patients (3.9%) experienced a major procedural/early post procedure complication (death, conversion to open procedure, cardiac arrest), including two deaths. The annualised rates of infective endocarditis and valve replacement were 4.6% and 3.8% respectively. There was one death related to infective endocarditis in follow-up., Conclusions: Percutaneous pulmonary valve replacement is a relatively safe method of rehabilitating the right ventricular outflow tract., (Crown Copyright © 2022. Published by Elsevier B.V. All rights reserved.)

A 14-year-old boy presented to us with a diagnosis of severe asthma and oxygen desaturation of 76% on a 6-minute-walk test. A contrast echocardiogram revealed echocontrast in the left and right atria simultaneously. A secundum atrial septal defect and partial cor triatriatum dexter were diagnosed, and the atrial defect was closed by cardiac catheterisation.

Intraoperative near-infrared spectroscopy cerebral oxygenation monitoring assists intraoperative decision-making in environments without extracorporeal membrane oxygenation (ECMO), left ventricular assist device (LVAD) or access to cardiac transplantation. We report a case of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA), undergoing cardiac surgery. A 4-month-old infant presented in extremis with cardiac failure. We discuss the pathophysiology and challenging intraoperative management of ALCAPA with extensive ischaemia and myocardial infarction.

The presence of a haemodynamically significant patent ductus arteriosis (PDA) in the premature newborn may contribute to the development of brochopulmonary dysplasia, intraventricular haemorrhage and necrotising enterocolitis. It is therefore essential that the diagnosis of such a PDA be made in time in order to intervene medically or surgically. Echocardiography is at present the prime diagnostic tool, but it is mainly available in tertiary hospitals. Chest roentgenography is of little diagnostic value especially in the presence of respiratory disease, which is often present in premature babies. Very little is known about the diagnostic value of the electrocardiogram (ECG) in premature babies. The aim of this study was to describe the standard 12-lead ECG findings in low-birth-weight babies with haemodynamically significant PDAs. Thirty-two babies with haemodynamically significant PDAs, as established by echocardiography, were included in the study. Standard 12-lead ECGs were done in all these babies. In 15 patients ECG abnormalities were found; only 22% had left atrial and left ventricular enlargement indicative of significant left-to-right shunting, while 78% had no ECG changes indicative of left-to-right shunting. In conclusion, the ECG cannot be used to identify haemodynamically significant PDAs.

Complete atrioventricular septal defect (CAVSD) is known to be associated with Down syndrome (DS) and to be a major cause of infantile death in these patients. Recently several leading articles reported that complete surgical repair before 6 months of age is a relatively low-risk procedure leading to a dramatic improvement in outcome. Most surgical reviews, however, fail to describe their selection criteria and to include patients who die early in infancy or who are unfit for surgery for some or other reason. A retrospective descriptive study was done of all children with CAVSD treated over a period of 15 years from 1980 to 1994 at our institution. The aims were to describe the profile of children with CAVSD in our population, and to evaluate screening procedures and treatment received versus outcome. Thirty-three patients were included in the study, 25 with DS and 8 without. The basic skills of careful auscultation, interpretation of chest radiograph and electrocardiograms (ECGs) are all 100% sensitive in alerting the physician to this condition. Careful screening, especially of DS babies, is essential to ensure early optimal intervention. Echocardiography is the least invasive, most informative investigation to establish a definite diagnosis. The Rastelli anatomical classification is highly indicative of outcome. Children in the Rastelli C group showed a universally dismal prognosis. Digitalis and furosemide are still the mainstay of medical treatment. No additional benefit was observed with the limited introduction of angiotensin-converting enzyme (ACE) inhibitors. Complete repairs were undertaken earlier and more frequently in recent years, but limited resources had led to the majority of patients not receiving surgery at all. There was insufficient urgency to aim for early complete repair, and more than 20% of children died before even being considered for surgery. This study is the first in South Africa to describe our own patient population and to evaluate local practice. Adequate screening of babies (especially those with DS), with early referral for complete repair in the first 6 months of life, remains the goal of optimal management.