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Background: Although several National Data Registries for Congenital Heart Disease (CHD) exist, few are comprehensive and contemporary. A National Australian CHD Registry has been developed that aims to redress this by creating the first comprehensive data collection for CHD children and adults, initially across Australia. Methods: We defined and collected a minimum dataset of demographics, diagnoses, and procedures from people with CHD presenting at participating quaternary CHD services Australia-wide. Data were collected from a range of clinical data sources. Diagnoses and procedures were standardised to the European Paediatric Congenital Code – Short List. Methodological limitations were carefully documented. Results: From 8 participating institutions, an initial 359,084 patient records were assessed for eligibility and 68,234 unique individuals with structural CHD have been included in the current dataset. There were 20,395 (30 %) people with mild CHD, 25,157 (37 %) with moderate CHD, and 13,530 (20 %) with severe CHD (6 % unknown complexity). The most common diagnoses were Ventricular Septal Defect (16,781, 25 %), Atrial Septal Defect (6,607, 10 %), Aortic Valve Disorders (5516 8 %), Coarctation of the Aorta (5,321, 8 %), Tetralogy of Fallot (4,489, 7 %), Transposition of the Great Arteries (4,009, 6 %). Conclusion: The data presented here represents the most comprehensive cohort collected for the Australian CHD population thus far and is comparable with the largest contemporary CHD registries around the world. This Registry represents a key resource for improved understanding of the CHD population and will drive better care and outcomes for people living with CHD.

Background Peak oxygen consumption (peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$) is routinely measured in people who have congenital heart disease and is reported as a percentage of predicted value, based upon age‐ and sex‐matched normative reference values (NRVs). This study aimed to identify which NRVs are being used, assess whether NRVs are being applied appropriately, and evaluate if recommended NRVs are valid when applied to people with congenital heart disease. Methods and Results A systematic scoping review identified studies that reported peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ percentage of predicted value in people with congenital heart disease. A modified risk of bias tool evaluated the included studies. Forty‐five studies reported peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ percentage of predicted value, and only 21 (47%) studies described or provided a reference on how their percentage of predicted value was calculated. The most cited NRVs were from Wasserman (n=12) and Cooper and Weiler‐Ravell (n=7). Risk of bias analysis judged 63% of studies as having some concerns. The NRVs recommended by the American Heart Association were applied to participants with a Fontan circulation (n=70; aged 26.5±6.4 years; 59% women) to examine validity. Predicted peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ values from the Wasserman NRV was not significantly associated to measured peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ values (men: b=0.31, R2≤0.01; women: b=0.07, R2=0.02). Conclusions Numerous NRVs have been applied to individuals with congenital heart disease and are often poorly reported and inappropriately matched to participants. The Wasserman NRV was the most cited but showed poor validity when applied to a Fontan cohort.

Background: Exercise capacity is reduced in patients with a Fontan circulation compared to their healthy peers secondary to altered haemodynamics of the Fontan circulation. Cardiopulmonary exercise testing is routinely used in Fontan patients to assess their cardiopulmonary response to exercise and help guide management decisions, however their results are routinely compared to normative data derived from healthy individuals. Method: Using data available from the Pediatric Heart Network, we performed a retrospective study to derive Fontan-specific normative values in a subgroup of well-functioning adolescent patients. We utilised results from the Congenital Heart Adolescent and Teenager (CHAT) Questionnaire to classify patients into well-functioning and lower-functioning groups. Results: We generated Fontan-specific regression equations in adolescent male and females for normative values of peak oxygen consumption (VO2), maximal workload and VO2 at anaerobic threshold. Conclusion: We propose that Fontan-specific normative values are a more useful comparison than healthy normative data due to the known circulatory limitations of the Fontan circulation and will assist in management decision and prognostication.

Structured Abstract: Introduction: Rates of morbidity and mortality are high in the setting of Fontan physiology and effective medical therapies are not well-established. Clinical trials assessing phosophodiesterase-5-inhibitors, such as sildenafil, have not demonstrated major benefit in patients with a Fontan-type circulation but have only included stable, well-functioning people. Methods: We sought to retrospectively characterize the people followed by the ANZ Fontan Registry prescribed sildenafil >30 days post Fontan-surgery. Results: Of 1867 individuals, 19 (1 %) were prescribed sildenafil and met inclusion criteria; 12 (63 %) were female. Age at commencement was 9 years (IQR 13.5). Reasons for commencement were Fontan failure with elevated Fontan pressure (n = 4), failure without elevated pressure (n = 4), elevated pressure without failure (n = 6), failure symptoms without invasive assessment (n = 3) and pulmonary arterio-venous malformations (n = 2). At baseline hemodynamic study (n = 16) median Fontan pressure was 15.5 mmHg (IQR 3.1) and PVRi was 2.3 WU*m2 (IQR 1.6). Improvement in the clinical indication was noted in 11/19 (58 %). In the subset in whom sildenafil was commenced with PVRi >2 WU*m2 6/7 (86 %) improved clinically. PVRi decreased by 1.8 WU*m2 (IQR 1.0, n = 5, p = 0.03) on treatment in this group, compared with the

Objectives: Antegrade pulmonary blood flow (APBF) may be left or eliminated at the time of the superior cavopulmonary connection (SCPC). Our aim was to assess the impact of leaving native APBF at the SCPC on long-term Fontan outcomes. Methods: In the Australia and New Zealand Fontan Registry (1985-2021), 587 patients had pre-existing native APBF at the SCPC. At the SCPC, 302 patients had APBF eliminated (APBF−) and 285 patients had APBF maintained (APBF+). The incidence of Fontan failure (composite end point of Fontan takedown, transplant, plastic bronchitis, protein losing enteropathy and death) and atrioventricular (AV) valve repair/replacement post SCPC was compared between the 2 groups. Results: Sex, predominant-ventricle morphology, isomerism, primary diagnosis, and age/type of Fontan were similar between groups. APBF− versus APBF+ had a higher incidence of arch obstruction/coarctation (17% vs 7%) and previous pulmonary artery band (54% vs 45%) and a lower rate of Fontan fenestration (27% vs 41%). The risk of Fontan failure was similar between the 2 groups (hazard ratio [HR], 1.01; 95% confidence interval [CI], 0.58-1.78; P = .96). The risk of AV-valve repair/replacement was greater in APBF+ versus APBF– (HR, 2.32; CI, 1.13-4.75; P = .022). The risk of AV-valve repair/replacement remained after adjustment for arch obstruction/coarctation, previous pulmonary artery band and Fontan fenestration (HR, 2.27; CI, 1.07-4.81; P = .033). Conclusions: Maintaining APBF at the time of the SCPC does not impact the risk of Fontan failure but may increase the incidence of AV-valve repair and/or replacement post-SCPC.

Abstract Background Modern oncological therapies together with chemotherapy and radiotherapy have broadened the agents that can cause cardiac sequelae, which can manifest for pediatric oncology patients while on active treatment. Recommendations for high-risk patients who should be monitored in a pediatric cardio-oncology clinic have previously been developed by expert Delphi consensus by our group. In 2022 we opened our first multidisciplinary pediatric cardio-oncology clinic adhering to these recommendations in surveillance and management. Objectives Our pediatric cardio-oncology clinic aimed to: (i) Document cardiovascular toxicities observed within a pediatric cardio-oncology clinic and. (ii) Evaluate the applicability of the Australian and New Zealand Pediatric Cardio-Oncology recommendations. Methods Monthly multidisciplinary cardio-oncology clinics were conducted in an Australian tertiary pediatric hospital. Structured standardised approaches to assessment were built into the electronic medical record (EMR). All patients underwent baseline echocardiogram and electrocardiogram assessment together with vital signs in conjunction with standard history and examination. Results Nineteen (54%) individuals had a documented cardiovascular toxicity or pre-existing risk factor prior to referral. The two most common cardiovascular toxicities documented during clinic review included Left Ventricular Dysfunction (LVD) and hypertension. Of note 3 (8.1%) patients had CTCAE grade III LVD. An additional 10 (27%) patients reviewed in clinic had CTCAE grade I hypertension. None of these patients had hypertension noted within their referral. Cascade testing for cardiac history was warranted in 2 (5.4%) of patients. Conclusions Pediatric cardio-oncology clinics are likely beneficial to documenting previously unrecognised cardiotoxicity and relevant cardiac family histories, whilst providing an opportunity to address lifestyle risk factors.

Background Peak oxygen pulse (O2pulse=oxygen consumption/heart rate) is calculated by the product of stroke volume (SV) and oxygen extraction. It has been shown to be reduced in patients with a Fontan circulation. However, in the Fontan population, it may be a poor marker of SV. We propose that the slope of the O2 pulse curve may be more reflective of SV during exercise.Methods We analysed cardiopulmonary exercise test data in 22 subjects with a Fontan circulation (cohort A) and examined the association between peak SV during exercise (aortic flow measured on exercise cardiac MRI), and O2 pulse parameters (absolute O2 pulse and O2 pulse slopes up to anaerobic threshold (AT) and peak exercise). In a separate Fontan cohort (cohort B, n=131), associations between clinical characteristics and O2 pulse kinetics were examined.Results In cohort A, peak aortic flow was moderately and significantly associated with O2pulseslopePEAK (r=0.47, p=0.02). However, neither absolute O2pulseAT nor O2pulsePEAK was significantly associated with peak aortic flow. In cohort B, O2pulseslopePEAK and O2pulseslopeAT were not significantly associated with clinical parameters, apart from a weak association with forced vital capacity.Conclusion The slope of the O2 pulse curve to peak exercise may be more reflective of peak SV in the Fontan population than a single peak O2 pulse value.

Introduction Despite growing awareness of neurodevelopmental impairments in children with congenital heart disease (CHD), there is a lack of large, longitudinal, population-based cohorts. Little is known about the contemporary neurodevelopmental profile and the emergence of specific impairments in children with CHD entering school. The performance of standardised screening tools to predict neurodevelopmental outcomes at school age in this high-risk population remains poorly understood. The NITric oxide during cardiopulmonary bypass to improve Recovery in Infants with Congenital heart defects (NITRIC) trial randomised 1371 children

People with a Fontan circulation are at risk of neurodevelopmental delay and disability, and cognitive dysfunction, that has significant implications for academic and occupational attainment, psychosocial functioning, and overall quality of life. Interventions for improving these outcomes are lacking. This review article discusses current intervention practices and explores the evidence supporting exercise as a potential intervention for improving cognitive functioning in people living with a Fontan circulation. Proposed pathophysiological mechanisms underpinning these associations are discussed in the context of Fontan physiology and avenues for future research are recommended.

Background Peak oxygen consumption (peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$) is traditionally divided (“ratio‐scaled”) by body mass (BM) for clinical interpretation. Yet, it is unknown whether ratio‐scaling to BM can produce a valid size‐independent expression of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ in people with a Fontan circulation. Furthermore, people with a Fontan circulation have deficits in lean mass, and it is unexplored whether using different measures of body composition may improve scaling validity. The objective was to assess the validity of different scaling denominators (BM, stature, body surface area, fat‐free mass, lean mass, and appendicular lean mass using ratio and allometric scaling). Methods and Results Eighty‐nine participants (age: 23.3±6.7 years; 53% female) with a Fontan circulation had their cardiorespiratory fitness and body composition measured by cardiopulmonary exercise testing and dual‐energy x‐ray absorptiometry. Ratio and allometric (log‐linear regression) scaling was performed and Pearson correlations assessed scaling validity. Scaling denominators BM (r=−0.25, P=0.02), stature (r=0.46, P

Background Aortic intima‐media thickness (aIMT) measurement is an established indicator of preclinical atherosclerosis. We aimed to describe the aIMT in infants with congenital heart disease undergoing cardiac surgery over the first year of life and explore its association with cardiopulmonary bypass, growth velocity, and a diagnosis of left heart obstruction. Methods and Results A prospective cohort study measuring mean and maximum aIMT preoperatively, at 3 months, and 1 year of age in neonates with congenital heart disease undergoing cardiac surgery. Twenty‐four infants with a median gestation of 39 weeks and a median birth weight of 3184 g were included. Sixteen (67%) infants had left outflow tract obstruction. Gestation correlated inversely with baseline mean aIMT (β=−0.027, P=0.018) and positively with the percentage of increase in mean and maximum aIMT between baseline and 3 months (β=17%, P=0.027 and β=15%, P=0.023). The presence of left outflow obstruction was significantly associated with increasing mean and maximum aIMT between baseline and 1 year (mean aIMT change: β=34%, P=0.017 and maximum aIMT change β=43%, P=0.001). Both subgroups of left heart obstruction and non‐left heart obstruction significantly changed over time (P=0.001 and P

Background Patients with a single ventricle who experience early life growth failure suffer high morbidity and mortality in the perisurgical period. However, long‐term implications of poor infant growth, as well as associations between body mass index (BMI) and outcome in adulthood, remain unclear. We aimed to model BMI trajectories of patients with a single ventricle undergoing a Fontan procedure to determine trajectory‐based differences in baseline characteristics and long‐term clinical outcomes. Methods and Results We performed a retrospective analysis of medical records from patients in the Australia and New Zealand Fontan Registry receiving treatment at the Royal Children's Hospital, The Children's Hospital at Westmead, Royal Melbourne Hospital, and Royal Prince Alfred Hospital from 1981 to 2018. BMI trajectories were modeled in 496 patients using latent class growth analysis from 0 to 6 months, 6 to 60 months, and 5 to 16 years. Trajectories were compared regarding long‐term incidence of severe Fontan failure (defined as mortality, heart transplantation, Fontan takedown, or New York Heart Association class III/IV heart failure). Three trajectories were found for male and female subjects at each age group—lower, middle, higher. Subjects in the lower trajectory at 0 to 6 months were more likely to have an atriopulmonary Fontan and experienced increased mortality long term. No association was found between higher BMI trajectory, current BMI, and long‐term outcome. Conclusions Poor growth in early life correlates with increased long‐term severe Fontan failure. Delineation of distinct BMI trajectories can be used in larger and older cohorts to find optimal BMI targets for patient outcome.

Background The long‐term impact of fenestration at the time of Fontan operation remains unclear. We aimed to review the early and long‐term impact of Fontan fenestration in the Australia and New Zealand cohort. Methods and Results We reviewed 1443 patients (621 fenestrated, 822 nonfenestrated) from the Australia and New Zealand Fontan registry. Data were collected on preoperative demographics, operative details, and follow‐up. Propensity‐score matching was performed to account for the various preoperative and operative differences and risk factors. Primary outcomes were survival and freedom from failure. Median follow‐up was 10.6 years. After propensity‐score matching (407 matched pairs), there was no difference in survival (87% versus 90% at 20 years; P=0.16) or freedom from failure (73% versus 80% at 20 years; P=0.10) between patients with and without fenestration, respectively. Although patients with fenestration had longer bypass and cross‐clamp times (P

Background: Branch pulmonary artery (PA) Stenting is an established strategy for PA stenosis in older children and adults. Its use in infants is less well established. We describe our experience of branch PA Stenting in infants 50% increase in PA diameter. Procedural complications and need for PA re-intervention were ascertained. Results: Forty-one children (age 7.6 months, IQR 2.4–9.8 months, weight 5.9 ± 2.2 kg) had 43 primary Stent implantations (10/43 intraoperative versus 33/43 transcatheter; left pulmonary artery [LPA] 25/43, right pulmonary artery [RPA] 8/43 and bilateral 10/43). Diagnoses were tetralogy of Fallot (27%), hypoplastic left heart syndrome (24%), truncus arteriosus (12%), transposition of the great arteries (7%), other single ventricle (10%) and other biventricular (20%). 40/41 (98%) had undergone a cardiac intervention in the preceding 31 days, [IQR 1–181], with 17/41 having prior branch PA intervention. 14/41 patients had urgent Stenting. There were 2 minor and 1 major complications with no procedural mortality. LPA (LPApre 2.3 ± 1.0 × 2.2 ± 1.2 mm versus LPApost 5.2 ± 1.3 × 5.0 ± 1.7 mm, p

Patients with a Fontan circulation lack a sub-pulmonary ventricle with pulmonary blood flow passively redirected to the lungs. In the Fontan circulation, ventilation has a significant influence on pulmonary blood flow and cardiac output both at rest and with exercise. Children and adults with a Fontan circulation have abnormalities in lung function. In particular, restrictive ventilatory patterns, as measured by spirometry, and impaired gas transfer, as measured by the diffusing capacity of carbon monoxide, have been frequently observed. These abnormalities in lung function are associated with reduced exercise capacity and quality of life. Moderate to severe impairment in lung volumes is independently associated with reduced survival in adults with congenital heart disease. Skeletal and inspiratory muscle weakness has also been reported in patients with a Fontan circulation, with the prospect of improving respiratory muscle function through exercise training programs. In this review, we will present data on cardiopulmonary interactions in the Fontan circulation, the prevalence and severity of impaired lung function, and respiratory muscle function in this population. We will discuss potential causes for and consequence of respiratory impairments, and their impact on exercise capacity and longer-term Fontan outcome. We aim to shed light on possible strategies to reduce morbidity by improving respiratory function in this growing population of patients.

Objectives: Right ventricle to pulmonary artery (RV-PA) conduits are required for the surgical management of pulmonary atresia with ventricular septal defect and truncus arteriosus. Bioengineered RV-PA connections may address some of the shortcomings of homografts and xenografts, such as lack of growth potential and structural deterioration and may be manufactured to accommodate patient-specific anatomy. The aim of this study was to develop a methodology for in silico patient-specific design and analysis of RV-PA conduits. Methods: Cross-sectional imaging was obtained from patients with truncus arteriosus (n = 5) and pulmonary atresia with ventricular septal defect (n = 5) who underwent complete repair with a RV-PA conduit. Three-dimensional models of the heart were constructed by segmentation of the right ventricle, existing conduit, branch pulmonary arteries, and surrounding structures. A customized conduit design for each patient was proposed. Computational fluid dynamics analysis was performed and outputs, including wall shear stress and energy loss, were used to compare the performance of the existing conduits and the customized geometries. Results: In this study, a methodology for patient-specific analysis of RV-PA conduit in silico was developed. The results of simulations for 10 patients showed between 23% and 56% decrease in the average wall shear stress and between 24% and 87% reduction in average power requirements in customized designs compared with the stenosed conduits, translating into better hemodynamic performance. Conclusions: Creation of an optimal conduit for an individual patient can be achieved using surgeon-guided design and computational fluid dynamics analysis. Manufacture of personalized RV-PA conduits may obviate the need for surgical customization to accommodate existing materials and provide superior long-term outcomes.

Introduction: Adolescents and adults with a Fontan circulation are at risk of cognitive dysfunction; Attention and processing speed are notable areas of concern. Underlying mechanisms and brain alterations associated with worse long-term cognitive outcomes are not well determined. This study investigated brain white matter microstructure in adolescents and adults with a Fontan circulation and associations with resting and peak exercise oxygen saturations (SaO2), predicted maximal oxygen uptake during exercise (% pred VO2), and attention and processing speed. Methods: Ninety-two participants with a Fontan circulation (aged 13–49 years, ≥5 years post-Fontan completion) had diffusion MRI. Averaged tract-wise diffusion tensor imaging (DTI) metrics were generated for 34 white matter tracts of interest. Resting and peak exercise SaO2 and % pred VO2 were measured during cardiopulmonary exercise testing (CPET; N = 81). Attention and processing speed were assessed using Cogstate (N = 67 and 70, respectively). Linear regression analyses adjusted for age, sex, and intracranial volume were performed to investigate associations between i) tract-specific DTI metrics and CPET variables, and ii) tract-specific DTI metrics and attention and processing speed z-scores. Results: Forty-nine participants were male (53%), mean age was 23.1 years (standard deviation (SD) = 7.8 years). Mean resting and peak exercise SaO2 were 93.1% (SD = 3.6) and 90.1% (SD = 4.7), respectively. Mean attention and processing speed z-scores were −0.63 (SD = 1.07) and −0.72 (SD = 1.44), respectively. Resting SaO2 were positively associated with mean fractional anisotropy (FA) of the left corticospinal tract (CST) and right superior longitudinal fasciculus I (SLF-I) and negatively associated with mean diffusivity (MD) and radial diffusivity (RD) of the right SLF-I (p ≤ 0.01). Peak exercise SaO2 were positively associated with mean FA of the left CST and were negatively associated with mean RD of the left CST, MD of the left frontopontine tract, MD, RD and axial diffusivity (AD) of the right SLF-I, RD of the left SLF-II, MD, RD and AD of the right SLF-II, and MD and RD of the right SLF–III (p ≤ 0.01). Percent predicted VO2 was positively associated with FA of the left uncinate fasciculus (p

Background: Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to optimize the circulation have not been established. Exercise intolerance affects the majority of the population and is associated with worse prognosis. Historically, people living with a Fontan circulation were advised to avoid physical activity, but a small number of heterogenous, predominantly uncontrolled studies have shown that exercise training is safe—and for unique reasons, may even be of heightened importance in the setting of Fontan physiology. The mechanisms underlying improvements in aerobic exercise capacity and the effects of exercise training on circulatory and end-organ function remain incompletely understood. Furthermore, the optimal methods of exercise prescription are poorly characterized. This highlights the need for large, well-designed, multi-center, randomized, controlled trials.Aims and Methods: The Fontan Fitness Intervention Trial (F-FIT)—a phase III clinical trial—aims to optimize exercise prescription and delivery in people with a Fontan circulation. In this multi-center, randomized, controlled study, eligible Fontan participants will be randomized to either a 4-month supervised aerobic and resistance exercise training program of moderate-to-vigorous intensity followed by an 8-month maintenance phase; or usual care (control group). Adolescent and adult (≥16 years) Fontan participants will be randomized to either traditional face-to-face exercise training, telehealth exercise training, or usual care in a three-arm trial with an allocation of 2:2:1 (traditional:telehealth:control). Children (

Background: People with a Fontan circulation usually have moderately impaired exercise performance, although a subset have high physical performance (“Super-Fontan”), which may represent a low-risk phenotype.Methods: People with a “Super-Fontan” phenotype were defined as achieving normal exercise performance [≥80% predicted peak oxygen uptake (VO2) and work rate] during cardiopulmonary exercise testing (CPET) and were identified from the Australian and New Zealand Fontan Registry. A Fontan control group that included people with impaired exercise performance (

Background: This review identifies the predictors of late mortality and heart transplantation that remain relevant in the contemporary population of patients with a Fontan circulation, focusing on the potential impact of post-Fontan morbidities on the late outlook of these patients. Methods and Results: A total of 1561 patients who had survived the Fontan operation in Australia or New Zealand from 1975 to 2018 were included in this review. Over a median duration of 11.4 years, there was a total of 117 deaths (7%) and 32 heart transplantations (2%). Freedom from death and heart transplantation at 10, 20 and 35 years post Fontan surgery were 94% (95% CI 93–95%), 87% (95 %CI 85–90%) and 66% (95 %CI 57–78%) respectively. Being male, having an atriopulmonary Fontan, pre-Fontan atrioventricular valve intervention, or prolonged pleural effusions post Fontan were predictive of late death or heart transplantation. However, time-dependent variables such as the development of atrial arrhythmia, protein/losing enteropathy or late ventricular dysfunction were stronger predictors of the same outcome. Patients who developed a time-dependent risk factor had a freedom from death and heart transplantation rate of 54% (95 %CI 43–66) at 15 years and 44% (95 %CI 33–57) at 25 years post Fontan. However, 95% (95 %CI 91–99) of patients without any of the identified risk factors were free from death or heart transplantation rate at 25 years post Fontan. Conclusion: In conclusion, the occurrence of post-operative complications such as PLE, arrhythmia and ventricular dysfunction will likely precede the late demise of these patients.

Cardiac rhabdomyomas in neonates may cause significant cardiac risk. Recently, sirolimus has been used to treat these lesions. The dose, duration, and monitoring for therapy are unknown. A case of sirolimus use in a premature neonate is presented. No significant adverse effects were seen. Review of published cases is included. (Level of Difficulty: Advanced.)

Background We sought to characterize body composition abnormalities in young patients living with a Fontan circulation and explore potential pathophysiologic associations. Methods and Results Twenty‐eight patients with a Fontan circulation were prospectively recruited in this cross‐sectional study. Participants underwent cardiopulmonary exercise testing, dual‐energy X‐ray absorptiometry, echocardiography, and biochemical assessment. Mean age was 26±7 years. Skeletal muscle mass, estimated by appendicular lean mass index Z score, was reduced compared with reference data (−1.49±1.10, P

Abstract Background Arterial stiffness is a significant cardiovascular risk factor. Carotid-femoral pulse wave velocity (cfPWV) is the standard for measuring arterial stiffness, but indices such as central augmentation index (cAIx) and carotid artery elasticity (cE[p]) also correlate to arterial stiffness. Early-life determinants of arterial stiffening are not well understood. We assessed the relationship between population-indexed anthropometric and hemodynamic parameters, and arterial stiffness in healthy children. Methods 97 healthy children between 2 to 20 years old were recruited. Z-scores for height, BMI, and brachial systolic (bSBP) and diastolic (bDBP) blood pressure were calculated [1,2]. cfPWV and central arterial waveforms were measured using the Sphygmocor XCEL and CvMS devices (AtCor, Sydney) respectively. Carotid artery maximum and minimum diameters were measured by high-resolution ultrasonography. cfPWV, cE[p], and cAIx were calculated. Results In bivariate analysis, cfPWV was associated with age (b = 0.52, p < 0.01) and bSBP z-score (b = 0.24, p = 0.03). These associations were independent of sex, height z-score, BMI z-score, and bDBP z-score in a multivariable model (R2 = 0.23). cE[p] was significantly associated with age (b = 0.42, p < 0.01), bSBP z-score (b = 0.45, p < 0.01), and height z-score (b = -0.33, p = 0.01), but only bSBP z-score remained statistically significant in the multivariable model (b = 0.45, p < 0.01; R2 = 0.20). cAIx was not associated with anthropometric or hemodynamic z-scores. Conclusion cfPWV is associated with age and bSBP z-score, while cE[p] is associated with bSBP z-score only, suggesting a potentially key association between systolic blood pressure and indices of arterial stiffness independent of age and other parameters of growth during healthy early life.

Background: Central aortic pressure waveforms can be modelled non-invasively in adults using general transfer functions. These techniques have been applied to, but not formally validated in paediatric populations. We recently developed and validated two age-appropriate paediatric transfer functions and sought to determine their effectiveness in children and adolescents. Methods: We recruited 97 healthy children between 2 and 20 years of age in five pre-specified age groups. Central waveforms were estimated by applying two previously developed paediatric transfer functions developed in 8 year and 14 year-old children (8TF, 14TF respectively), and a proprietary adult transfer function (aTF) (Sphygmocor CvMS, AtCor) to radial waveforms measured by tonometry [1]. Estimated central arterial parameters were measured from direct carotid tonometry. Results: 8TF estimates higher cSBP than 14TF, which estimates higher cSBP than aTF across all ages. In contrast, 8TF estimates lower cAIx than 14TF, which estimates lower cAIx than aTF across all ages (Figure 1). 8TF most accurately modelled central arterial waveform features, specifically by cAIx when comparing to carotid tonometry, in early childhood (age groups: 2–6.5 years, 0 ± 14%; 6.6–9.5 years, − 4 ± 19%; 9.6–12.5 years, −6 ± 13%), while 14TF most accurately estimated cAIx in later childhood and adolescence (12.6–15.5 years, −3 ± 11%; 15.6–20 years, 4 ± 13%). Figure 1 Conclusion: Effectiveness of transfer functions for modelling central arterial waveforms in childhood appear to be age-dependent, with evidence of better accuracy when used in populations similar to that in which they were developed. These results support the use of multiple age-appropriate paediatric transfer functions over a generalized paediatric transfer function.

Objectives: Carotid-femoral pulse wave velocity (cfPWV) is a validated non-invasive measure of aortic stiffness. Risk factors for cfPWV are well described in adulthood, and furthermore cfPWV is associated with incident cardiovascular disease in adults (1). However, risk factors for arterial stiffness in childhood are poorly described (2). Accordingly, we sought to determine the risk factors for cfPWV in childhood and adolescence and hypothesized that cfPWV would be higher amongst those with greater adiposity. Methods: We prospectively recruited 88 healthy children (mean age = 11.0 ± 5.3 years old). Age, weight, height, and blood pressure were measured. cfPWV was assessed using a semi-automated cuff-based device (Sphygmocor XCEL; AtCor Medical, Australia), and body composition using air displacement plethysmography (BOD POD; Cosmed, Italy) (3). Associations with cfPWV were determined by multivariable linear regression, with subsequent mediation analyses to inform likely causal pathways. Results: After adjusting for age and sex, cfPWV was significantly associated with weight, body mass index (BMI), systolic blood pressure, mean blood pressure, heart rate, and lean body mass (LBM), while LBM was significantly associated with height, weight, BMI and fat mass (Table 1). After further adjusting for weight, mean blood pressure and heart rate, LBM remained significantly associated with cfPWV (ß = 0.68; p = 0.007). Mediation analyses indicate that weight mediates the association between age and cfPWV (PM = 76%), and that LBM mediates the relationship between weight and cfPWV (Figure 1). Conclusion: Higher cfPWV in healthy children and adolescents is a function of growth, and this association may be in turn mediated by higher LBM rather than adiposity.

The anomalous origin of the left brachiocephalic artery in a right sided aortic arch is a rare vascular ring which might lead to esophageal compression. The exact embryological origin of this anomaly is still widely debated. We present an infant who presented with esophageal compression symptoms and review the various hypotheses about the embryological origin of this anomaly.

BackgroundPatients with a Fontan circulation have reduced exercise capacity and respiratory muscle strength. Inspiratory muscle training (IMT) improves exercise capacity and quality of life in adults with heart failure. We assessed whether 6 weeks of a home‐based program of IMT improves inspiratory muscle strength and the ventilatory efficiency of exercise in adolescent patients with a Fontan circulation. Methods and ResultsTwenty‐three adolescent participants (aged 16±2 years) with a Fontan circulation underwent 6 weeks of IMT for 30 minutes daily. Respiratory muscle strength (maximal inspiratory pressure and expiratory pressure), lung function, and exercise capacity (cardiopulmonary exercise testing) were assessed. Fourteen of 23 participants also underwent exercise cardiac magnetic resonance imaging to examine the effects of IMT on cardiac output and systemic and pulmonary blood flow. Six weeks of IMT improved maximal inspiratory pressure by 36±24 cm H2O (61±46%) with no change in maximal expiratory pressure. Ventilatory efficiency of exercise improved after 6 weeks of IMT (from 34.2±7.8 to 32.2±5.6, P=0.04). In those who underwent exercise cardiac magnetic resonance imaging, IMT increased resting cardiac output (from 4.2±1.2 to 4.5±1.0 L/min, P=0.03) and ejection fraction (from 50.1±4.3 to 52.8±6.1%, P=0.03). ConclusionsSix weeks of IMT is associated with improved inspiratory muscle strength, ventilatory efficiency of exercise, and resting cardiac output in young Fontan patients. IMT may be a simple beneficial addition to the current management of Fontan patients, potentially reducing exercise intolerance and long‐term morbidity and mortality.