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In the current era, the majority of children born with congenital heart disease (CHD) will survive well into adulthood because of major advances in surgical techniques, as well as in critical and medical care. However, reoperation and palliative surgical interventions are increasingly common in the adults with CHD. Tools to risk stratify patients effectively and therapies to improve outcomes are required to optimize the management of adult patients with CHD during the preoperative and postoperative periods and beyond. Exercise testing is an invaluable tool to guide risk stratification. In addition, exercise training in patients with CHD may decrease postoperative complications by enhancing physiological reserve and also has an important role in physical rehabilitation. This review aims to provide individualized recommendations on exercise prescription in patients with CHD in the preoperative and postoperative settings. The response to exercise testing and prognostic implications is also discussed.

Atrial septal defects (ASDs) represent the most common congenital heart defect diagnosed in adulthood. Although considered a simple defect, challenges in optimal diagnostic and treatment options still exist due to great heterogeneity in terms of anatomy and time-related complications primarily arrhythmias, thromboembolism, right heart failure and, in a subset of patients, pulmonary arterial hypertension (PAH). Atrial septal defects call for tertiary expertise where all options may be considered, namely catheter vs. surgical closure, consideration of pre-closure ablation for patients with atrial tachycardia and suitability for closure or/and targeted therapy for patients with PAH. This review serves to update the clinician on the latest evidence, the nuances of optimal diagnostics, treatment options, and long-term follow-up care for patients with an ASD.

Background In people with a Fontan circulation, serial cardiopulmonary exercise testing (CPET) to evaluate change in peak exercise capacity has been increasingly recognised as a useful prognostic tool; a decline is associated with adverse clinical outcomes. The aim of this study is to describe the “natural” history of exercise capacity in the Australian and New Zealand (ANZ) Fontan cohort and to identify factors associated with a decline. Methods The ANZ Fontan registry was retrospectively reviewed for adolescent and adult patients (≥16 years) with serial CPET results performed on a cycle ergometer ≥6 months apart. Patients were excluded if they underwent a surgical procedure or fenestration closure in-between tests or if the tests were considered as submaximal effort. Exercise capacity trajectory was defined as the change in percentage of predicted peak oxygen uptake (% pred VO2peak) points per year. Results Thirty-seven (37) patients (59.5% male, mean age 24±7 years) were eligible. Average duration between CPET was 5.3±3.9 years. At baseline, % pred VO2peak was 61.3±14.5%. Thirteen (13) (35%) had a systemic right ventricle, and 14 (38%) had an atriopulmonary type Fontan circulation. Average change in % pred VO2peak overall was +1.3±6.4 percentage points per year. Sixteen (16) had a negative exercise capacity trajectory, and the average decline in that group was −2.7±3.4 percentage points per year. There was no association between exercise capacity trajectory and clinical characteristics. Of the 18 patients with physical activity levels recorded, 12 (67%) were physically active and % pred VO2peak in that group increased by 2.7±4.0 percentage points per year compared with the physically inactive group who fell by 0.5±0.8 percentage points per year. Conclusions In this ANZ series of Fontan patients, over half of our cohort had stable, or an increase, in peak exercise capacity. Regular participation in physical activity was common in patients with a positive exercise capacity trajectory. Clinical characteristics were not associated with exercise capacity trajectory.

Rheumatic heart disease (RHD) is the result of episodes of acute rheumatic fever with valvular (and other cardiac) damage caused by an abnormal immune response to group A streptococcal infections, usually during childhood and adolescence. As a result of improved living conditions and the introduction of penicillin, RHD was almost eradicated in the developed world by the 1980s. However, being a disease of poverty, its burden remains disproportionately high in the developing world, despite being a fundamentally preventable disease. Rheumatic heart disease generates relatively little attention from the medical and science communities, in contrast to other common infectious problems (such as malaria, HIV, tuberculosis), despite the major cardiovascular morbidity/mortality burden imposed by RHD. This relative neglect and paucity of funding have probably contributed to limited fundamental medical advances in this field for over 50 years. Given the importance of prevention before the onset of major valvular damage, the main challenges for RHD prevention are improving social circumstances, early diagnosis, and effective delivery of antibiotic prophylaxis. Early identification through ultrasound of silent, subclinical rheumatic valve lesions could provide an opportunity for early intervention. Simple echocardiographic diagnostic criteria and appropriately trained personnel can be valuable aids in large-scale public health efforts. In addition, a better understanding of the immunogenic determinants of the disease may provide potential routes to vaccine development and other novel therapies.

BACKGROUND: To assess the association between body composition and the risk of adverse outcomes in Fontan patients. METHODS: Participants from the Australian and New Zealand Fontan Registry with dual-energy X-ray absorptiometry scans were included. Appendicular lean mass (ALM), appendicular lean mass index (ALM divided by height squared; ALMI) and total body fat mass percentage (%BF) were calculated. ALMI and %BF z-scores were derived using age- and sex-matched reference ranges. The primary outcome was Fontan failure (death, transplantation, New York Heart Association functional class III/IV, protein-losing enteropathy, and plastic bronchitis) or moderate-or-severe ventricular dysfunction. RESULTS: 144 patients were included. Mean %BF was 29% (SD 10) with 50% having increased adiposity. Mean ALMI z-score was -1.4 (SD 1.1); one third of patients had skeletal muscle deficiency (ALMI z-score

Aims We investigated long-term mortality associated with changes in left ventricular ejection fraction (LVEF) in a large, real-world patient cohort. Methods and results A total of 117 275 adults (63 ± 16 years, 46% women) had LVEF quantified by the same method ≥6 months apart. This included 17 343 cases (66 ± 15 years, 48% women) being initially investigated for heart failure (HF). During 3.3 [interquartile range (IQR) 1.7–6.0] years from first to last echocardiogram, median change in LVEF was −1 (IQR −8 to +5) units from a baseline of 62% (IQR 54–69%). During subsequent 7.6 (IQR 4.3–10.1) years of follow-up, 11 397 (9.7%) and 34 101 (29.1%) cases died from cardiovascular disease and all causes, respectively. Actual 5-year, all-cause mortality increased from 12% to 29% among those with the smallest to the largest decrease in LVEF (from 30 units); the adjusted risk of cardiovascular-related mortality increased two- to eightfold beyond a >10-unit decline in LVEF (vs. minimal change; P 30-unit increase to >30-unit decline in LVEF (vs. minimal change; P

Background Exercise intolerance is present even in the early stages of pulmonary arterial hypertension (PAH) and is associated with poorer prognosis. Respiratory muscle dysfunction is common and may contribute to exercise limitation. We sought to investigate the effects of inspiratory muscle training (IMT) to improve exercise capacity in PAH. Methods Adults with PAH were prospectively recruited and randomly assigned to either IMT or a control group. At baseline and after 8 weeks, assessment of respiratory muscle function, pulmonary function, neurohormonal activation, 6-minute walk distance and cardiopulmonary exercise testing variables were conducted. Inspiratory muscle strength was assessed by maximal static inspiratory pressure (PImax). The IMT group performed two cycles of 30 breaths at 30–40% of their PImax 5 days a week for 8 weeks. Results Twelve (12) PAH patients (60±14 years, 10 females) were recruited and randomised (six in the IMT group and six in the control group). After 8 weeks, the IMT group improved PImax by 31 cmH2O compared with 10 cmH2O in controls, p=0.02. Following IMT, 6-minute walk distance improved by 24.5 m in the IMT group and declined by 12 m in the controls (mean difference 36.5 m, 95% CI 3.5–69.5, p=0.03). There was no difference in peak oxygen uptake between-groups (mean difference 0.4 mL/kg/min, 95% CI −2.6 to 3.4, p=0.77). There was no difference in the mean change between-groups in neurohormonal activation or pulmonary function. Conclusion In this pilot randomised controlled study, IMT improved PImax and 6-minute walk distance in PAH patients.

Cancer therapy related cardiac dysfunction (CTRCD) is an area of increasing focus, particularly during the survivorship period, for paediatric, adolescent and adult cancer survivors. With the advent of immunotherapy and targeted therapy, there is a new set of mechanisms from which paediatric and young adult patients with cancer may suffer cardiovascular injury. Furthermore, cardiovascular disease is the leading cause of morbidity and mortality in the survivorship period. The recently established Australian Cardio-Oncology Registry is the largest and only population-based cardiotoxicity database of paediatric and adolescent and young adult oncology patients in the world, and the first paediatric registry that will document cardiotoxicity caused by chemotherapy and novel targeted therapies using a prospective approach. The database is designed for comprehensive data collection and evaluation of the Australian practice in terms of diagnosis and management of CTRCD. Using the Australian Cardio-Oncology Registry critical clinical information will be collected regarding predisposing factors for the development of CTRCD, the rate of subclinical left ventricular dysfunction and transition to overt heart failure, further research into protectant molecules against cardiac dysfunction and aid in the discovery of which genetic variants predispose to CTRCD. A health economic arm of the study will assess the cost/benefit of both the registry and cardio-oncology clinical implementation. Finally, an imaging arm will establish if exercise cardiac magnetic resonance imaging and VO2 max testing is a more sensitive predictor of cardiac reserve in paediatric and adolescent and young adult oncology patients exposed to cardiac toxic therapies.

AIMS We investigated the sex-based risk of mortality across the spectrum of left ventricular ejection fraction (LVEF) in a large cohort of patients in Australia. METHODS AND RESULTS Quantified levels of LVEF from 237 046 women (48.1%) and 256 109 men undergoing first-time, routine echocardiography (2000-2019) were linked to 119 232 deaths (median 5.6 years of follow-up). Overall, 17.6% of men vs. 8.3% of women had an LVEF

ObjectiveA high risk of morbidity and mortality is well documented in adults with a Fontan circulation. The difference in outcomes between those with and without significant morbidity at the time of transition to adult care has not been well characterised.MethodsWe analysed clinical outcomes in patients enrolled in the Australian and New Zealand Fontan Registry ≥16 years of age. Low risk (LR) Fontan patients were defined as those without history of sustained arrhythmia, thromboembolic event, transplantation, Fontan conversion, protein-losing enteropathy, plastic bronchitis, New York Heart Association class III/IV and/or moderate/severe atrioventricular valve regurgitation or ventricular dysfunction. Increased risk (IR) patients had one or more risk factor.ResultsInclusion criteria were met in 822 patients; mean age 26±8 years, median follow-up from age 16 was 9 years, 203 had atriopulmonary connection (APC) and 619 had total cavopulmonary connection (TCPC). Survival at 30 years was higher in the LR versus IR; 94% versus 82% (p=0.005), 89% versus 77% (p=0.07) for APC and 96% versus 89% (p=0.05) for TCPC. LR patients experienced less Fontan failure (HR 0.34, 95% CI 0.23 to 0.49, pConclusionsClinical outcomes for adolescents LR at transition to adult care are markedly superior to those who have established risk factors for Fontan failure, which is an important consideration when formulating individualised long-term risk estimates and counselling patients.

Combination drug therapy for pulmonary arterial hypertension (PAH) is the international standard of care for most patients, however in Australia there are barriers to drug access. This study evaluates current treatment of PAH patients in Australia and the consistency of therapy with international guidelines.Cross-sectional analysis of patients with Group 1 PAH enrolled in the Pulmonary Hypertension Society of Australia and New Zealand Registry (PHSANZ) at 31 December 2017. Drug treatment was classified as monotherapy or combination therapy and adequacy of treatment was determined by risk status assessment using the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk calculator. Predictors of monotherapy were assessed using a generalised linear model with Poisson distribution and logarithmic link function.1,046 patients met the criteria for analysis. Treatment was classified as monotherapy in 536 (51%) and combination therapy in 510 (49%) cases. Based on REVEAL 2.0, 184 (34%) patients on monotherapy failed to meet low-risk criteria and should be considered inadequately treated. Independent predictors of monotherapy included age greater than 60 years (risk ratio [RR] 1.23, 95% confidence interval [CI] 1.09-1.38; p=0.001), prevalent enrolment in the registry (RR 1.21 [95%CI 1.08-1.36]; p=0.001) and comorbid systemic hypertension (RR 1.17 [95%CI 1.03-1.32]; p=0.014), while idiopathic/heritable/drug-induced PAH subtype (RR 0.85 [95%CI 0.76-0.96]; p=0.006), functional class IV (RR 0.50 [95%CI 0.29-0.86]; p=0.012), increased right ventricular systolic pressure (RR 0.99 [95%CI 0.99-1.00]; p0.001) and increased pulmonary vascular resistance (RR 0.96 [95%CI 0.95-0.98]; p0.001) were less likely to be associated with monotherapy.Most Australian PAH patients are treated with monotherapy and a significant proportion remain at risk of poor outcomes. This is below the standard of care recommended by international guidelines and at risk patients should be escalated to combination therapy.

Background Prognosis in pulmonary arterial hypertension (PAH) is largely dependent on right ventricular (RV) function. However, recent studies have suggested the presence of left ventricular (LV) dysfunction in PAH patients. The potential role of LV ischemia, as a contributor to progressive LV dysfunction, has not been systematically studied in PAH. We aim to assess the presence and extent of LV myocardial ischemia in patients with known PH and without obstructive coronary artery disease (CAD), using oxygen-sensitive (OS) cardiovascular magnetic resonance (CMR) and stress/rest CMR T1 mapping. Methods We prospectively recruited 28 patients with right heart catheter-proven PH and no significant CAD, 8 patients with known CAD and 11 normal age-matched controls (NC). OS-CMR images were acquired using a T2* sequence and T1 maps were acquired using Shortened Modified Look-Locker Inversion recovery (ShMOLLI) at rest and adenosine-induced stress vasodilatation; ΔOS-CMR signal intensity (SI) index (stress/rest SI) and ΔT1 reactivity (stress-rest/rest T1 mapping) were calculated. Results Global LV ΔOS SI index was significantly lower in PH patients compared with controls (11.1%±6.7% vs. 20.5%±10.5%, P=0.016), as was ΔT1 reactivity (5.2%±4.5% vs. 8.0%±2.9%, P=0.047). The ischemic segments of CAD patients had comparable ΔOS SI (10.3%±6.4% vs. 11.1%±6.7%, P=0.773) to PH patients, but lower ΔT1 reactivity (1.1%±4.2% vs. 5.2%±4.5%, P=0.036). Conclusions Decreased OS-CMR SI and T1 reactivity signify the presence of impaired myocardial oxygenation and vasodilatory response in PH patients. Given their unobstructed epicardial coronary arteries, this is likely secondary to coronary microvascular dysfunction (CMD).

Aims Sudden cardiac death (SCD) accounts for up to 25% of deaths in the adult congenital heart disease (ACHD) population. Current guidelines for defibrillator implantation are either extrapolated from acquired cardiac disease or are based upon single lesion studies, predominantly Tetralogy of Fallot (TOF). Defibrillator-related morbidity appears to be substantially higher in ACHD patients. Methods We retrospectively evaluated all patients in our ACHD database who received an implantable cardioverter-defibrillator (ICD) between 2000 and 2019, and who were ≥16 years old at time of implant. Patients were followed for appropriate shocks, inappropriate shocks, and complications. Results Of 4748 patients in our database, 59 patients (1.2%) underwent ICD implantation. ICDs were for primary prevention in 63% and secondary prevention in 37%. Over a median follow-up of 6.6 years, 24% received an appropriate shock, 27% an inappropriate shock, and 42% suffered a device-related complication (annualized risks of 3.2%, 3.6%, and 5.7%, respectively). There were no significant predictors of appropriate shocks or inappropriate shocks. All appropriate shocks in primary prevention patients occurred in TOF or transposition of the great arteries (TGA) with atrial switch, typically in the presence of multiple SCD risk factors. The majority of inappropriate shocks were due to supraventricular arrhythmias. Device-related mortality was 1.7% (0.3% per annum). Conclusions Appropriate shocks were relatively common in an ACHD ICD population followed in the long term. Device-related morbidity was significant. Although risk factors have been established for TOF, and to a lesser extent TGA, risk stratification for ICD implantation in ACHD remains challenging.

The population of adults living with congenital heart disease (CHD) has grown rapidly in recent decades due to major advances in surgical and medical care. Although the benefits of physical activity (PA) and exercise are well recognised in the management of chronic diseases, the therapeutic role of exercise for the CHD population has been under-studied and under-recognised. In fact, people living with complex CHD have traditionally been advised against participation in moderate or vigorous PA due to safety concerns. However, a small but growing body of literature has demonstrated that PA and exercise are safe and beneficial for the vast majority of people with CHD following appropriate screening. Exercise training (ET) is a potent therapy to improve well-being and cardiorespiratory fitness, which likely has important implications for prognosis. This has led to ET becoming an increasingly important adjunct to medical therapy in the management of this group who frequently have significant exercise limitation and complex medical issues. However, there is currently limited evidence-based guidance that has been published regarding appropriate exercise prescription in adults with CHD. This article aims to provide an overview of different congenital heart conditions, their impact on the exercise response, and to provide considerations and recommendations for ET in adolescents and adults living with CHD based on the available literature.

Background Nephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown. Methods The Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up. The Modification of Diet in Renal Disease (MDRD) equation was used to calculate eGFR. Patient outcomes were obtained from the Registry. Fontan failure was defined as death, transplantation, plastic bronchitis, protein losing enteropathy, Fontan takedown and NYHA class III-IV. Results Serum creatinine measurements were available for 328 patients. Renal dysfunction was defined as eGFR Conclusion By the time they reach adulthood, 20% of patients with a Fontan circulation have renal dysfunction by eGFR calculation. Over the course of one decade, Fontan-associated nephropathy appears well tolerated.

Aims To examine the metabolic adaptation to an 80-day exercise intervention in healthy young male adults where lifestyle factors such as diet, sleep, and physical activities are controlled. Methods and results This study involved cross-sectional analysis before and after an 80-day aerobic and strength exercise intervention in 52 young, adult, male, newly enlisted soldiers in 2015. Plasma metabolomic analyses were performed using liquid chromatography, tandem mass spectrometry. Data analyses were performed between March and August 2019. We analysed changes in metabolomic profiles at the end of an 80-day exercise intervention compared to baseline, and the association of metabolite changes with changes in clinical parameters. Global metabolism was dramatically shifted after the exercise training programme. Fatty acids and ketone body substrates, key fuels used by exercising muscle, were dramatically decreased in plasma in response to increased aerobic fitness. There were highly significant changes across many classes of metabolic substrates including lipids, ketone bodies, arginine metabolites, endocannabinoids, nucleotides, markers of proteolysis, products of fatty acid oxidation, microbiome-derived metabolites, markers of redox stress, and substrates of coagulation. For statistical analyses, a paired t-test was used and Bonferroni-adjusted P-value of Conclusion For the first time, the true magnitude and extent of metabolic adaptation to chronic exercise training are revealed in this carefully designed study, which can be leveraged for novel therapeutic strategies in cardiometabolic disease. Extending the recent report of DMGV’s predictive utility in sedentary, overweight individuals, we found that it is also a useful marker of poor metabolic response to exercise in young, healthy, fit males.

Acute limb ischemia in young adults warrants thorough investigation to determine the underlying cause. Here, we present a case of acute upper limb ischemia in a marathon runner secondary to paradoxical embolism. The patient had associated deep venous thrombosis of the lower limb with multiple pulmonary emboli and patent foramen ovale. This case report emphasizes the under-recognition of intense endurance exercise as a risk factor for venous thromboembolism and highlights the potentially debilitating embolic sequelae of venous thromboembolism in patients with patent foramen ovale. Keywords: Venous thromboembolism, Marathon runner, Paradoxical embolism, Patent foramen ovale

Background: Modernization of Chinese adults is associated with increased atherosclerotic diseases. Over 1 million farmers have been resettled upland since 1996 in the Three Gorges dam project of th...

Prompted by a cluster of observations concerning ascending aortic pathology in elite rugby players, we assessed over 150 asymptomatic predominantly retired players with echocardiography, aiming to document the prevalence and severity of ascending aortic dilatation and/or anterior aortic effacement, both 'risk factors' for potentially catastrophic aortic complications.Rugby players (at least 5 years of high level competitive rugby) were classified as elite (national, state or first grade representatives) or non-elite. A total of 152 asymptomatic players with a mean age of 45 ± 13 years (range 21-65) underwent transthoracic echocardiography. Z-scores (number of standard deviations from a population mean) were calculated for aortic root and ascending aortic size.Regarding the aortic root, a Z-score of2 was seen in 24% (expected prevalence 2.3%, p 0.001) and a Z-score3 was seen in 4% (expected prevalence 0..1%, p 0.001). Sixty-two (62) players (41%) had an aortic root greater than 40 mm diameter. Ascending aortic Z-scores were2 in 53% of players and3 in 22% (p 0.001). Abnormal anterior aortic effacement at the sinotubular junction (STJ) was seen in 88 players (58%). Abnormal aortic dilatation and effacement were associated with a longer duration of competitive rugby participation and elite status, respectively.Ascending aortic dilatation with abnormal anterior effacement is exceedingly common in asymptomatic retired elite rugby players. This warrants increased surveillance in retired players until the clinical significance of these findings can be further investigated.

Background and objective Early diagnosis of PAH is clinically challenging. Patterns of diagnostic delay in Australian and New Zealand PAH populations have not been explored in large-scale studies. We aimed to evaluate the magnitude, risk factors and survival impact of diagnostic delay in Australian and New Zealand PAH patients. Methods A cohort study of PAH patients from the PHSANZ Registry diagnosed from 2004 to 2017 was performed. Diagnostic interval was the time from symptom onset to diagnostic right heart catheterization as recorded in the registry. Factors associated with diagnostic delay were analysed in a multivariate logistic regression model. Survival rates were compared across patients based on the time to diagnosis using Kaplan-Meier method and Cox regression. Results A total of 2044 patients were included in analysis. At diagnosis, median age was 58 years (IQR: 43-69), female-to-male ratio was 2.8:1 and majority of patients were in NYHA FC III-IV (82%). Median diagnostic interval was 1.2 years (IQR: 0.6-2.7). Age, CHD-PAH, obstructive sleep apnoea and peripheral vascular disease were independently associated with diagnostic interval of ≥1 year. No improvement in diagnostic interval was seen during the study period. Longer diagnostic interval was associated with decreased 5-year survival. Conclusion PAH patients experience significant diagnostic interval, which has not improved despite increased community awareness. Age, cardiovascular and respiratory comorbidities are significantly associated with longer time to diagnosis. Mortality rates appear higher in patients who experience longer diagnostic interval.

Objectives: This study sought to evaluate the effects of the Carillon device on mitral regurgitation severity and left ventricular remodeling.Background: Functional mitral regurgitation (FM...

Many parts of the developing world, especially Sub-Saharan Africa, completely lack access to cardiac pacing. The authors initiated a multinational program to implement cardiac pacing in 14 countries in Sub-Saharan Africa (1996 to 2018), aiming to eventually build self-sustainable capacity in each country. This was based on an "on-site training" approach of performing procedures locally and educating local health care teams to work within resource-limited settings, with prospective evaluation of the program. In 64 missions, a total of 542 permanent pacemakers were implanted. In 11 of these countries, the first pacemaker implant in the country was through the mission. More than one-half of those initially listed as suitable died before the mission(s) arrived. The proportion of implantations that were completely handled by local teams increased from 3% in 1996 to 98% in 2018. These findings demonstrate the feasibility and effectiveness of a proctorship-based approach to the development of local cardiac pacing capabilities in Sub-Saharan African nations.

Background Historical data suggesting poor survival in patients with aortic stenosis (AS) who do not undergo treatment are largely confined to patients with severe AS. Objectives This study sought to determine the prognostic impact of all levels of native valvular AS. Methods Severity of AS was characterized by convention and by statistical distribution in 122,809 male patients (mean age 61 ± 17 years) and 118,494 female patients (mean age 62 ± 19 years), with measured aortic valve (AV) mean gradient, peak velocity, and/or area. The relationship between AS severity and survival was then examined during median 1,208 days (interquartile range: 598 to 2,177 days) of follow-up. Patients with previous aortic valve intervention were excluded. Results Overall, 16,129 (6.7%), 3,315 (1.4%), and 6,383 (2.6%) patients had mild, moderate, and severe AS, respectively. On an adjusted basis (vs. no AS; 5-year mortality 19%), patients with mild to severe AS had an increasing risk of long-term mortality (adjusted hazard ratio: 1.44 to 2.09; p 50%) and cardiovascular disease was evident from a mean AV gradient >20.0 mm Hg (moderate AS) after adjusting for age, sex, left ventricular systolic or diastolic dysfunction, and aortic regurgitation. Conclusions These data confirm that when left untreated, severe AS is associated with poor long-term survival. Moreover, they also suggest poor survival rates in patients with moderate AS. (National Echocardiographic Database of Australia [NEDA]; ACTRN12617001387314)

Background: Progressive right ventricular (RV) dysfunction in pulmonary arterial hypertension (PAH) which is contributed by RV ischemia leads to adverse clinical outcomes. Oxygen-sensitive (OS) cardiovascular magnetic resonance (CMR) has been used to determine the in vivo myocardial oxygenation of the left ventricle (LV). The aims of the present study were therefore to determine the feasibility of RV targeted rest/stress OS-CMR imaging in PAH patients and healthy volunteers. Methods: We prospectively recruited 20 patients with right heart catheter proven PAH and 9 healthy age matched controls (NC). The CMR examination involved standard functional imaging and OS-CMR imaging. An OS-CMR signal intensity (SI) index (stress/rest SI) was acquired at RV anterior, RV free-wall and RV inferior segments. In the LV, the OS-CMR SI index was acquired globally. Results: Reliable OS SI changes were only obtained from the RV inferior segment. As RV dysfunction in PAH is a global process, hence this segment was used in both patients and NC for further comparison. RV OS-CMR SI change between rest and stress in the NC was 17%±5% (mean ± SD). Nine of 20 (45%) of the PAH patients had a mean OS SI change of less than 9% (or ?2 SD different from the mean values in NC). Overall, RV OS SI index between the PAH patients and NC was 11%±9% vs. 17%±5% (P=0.045) in the RV inferior segment. In the LV, the global OS-CMR SI index between the PAH patients and NC was 11%±7% vs. 21%±9% (P=0.019). There was a strong correlation between RV Inf OS-CMR SI and LV OS-CMR SI (r=0.86, P

To evaluate the impact of PM2.5 air pollution on atherogenic processes in modernizing Southern versus Northern China, we studied 1323 asymptomatic Chinese in Southern and Northern China in 1996–2007. PM2.5 exposure and metabolic syndrome (MS) were noted. Brachial flow-mediated dilation (endothelial function FMD) and carotid intima-media thickness (IMT) were measured by ultrasound. Although age and gender were similar, PM2.5 was higher in Northern China than in Southern China. The Northern Chinese were characterized by lower lipids, folate and vitamin B12, but higher age, blood pressures, MS and homocysteine (HC) (p = 0.0015). Brachial FMD was significantly lower and carotid IMT was significantly greater (0.68 ± 0.13) in Northern Chinese, compared with FMD and IMT (0.57 ± 0.13, p < 0.0001) in Southern Chinese. On multivariate regression, for the overall cohort, carotid IMT was significantly related to PM2.5, independent of location and traditional risk factors (Model R2 = 0.352, F = 27.1, p < 0.0001), while FMD was inversely related to gender, age, and northern location, but not to PM2.5. In Southern Chinese, brachial FMD was inversely correlated to PM2.5, independent of age, whereas carotid IMT was significantly related to PM2.5, independent of age and gender. In Northern Chinese, brachial FMD was inversely related to gender only, but not to PM2.5, while carotid IMT was related to traditional risk factors. Despite a higher PM2.5 pollution in Northern China, PM2.5 pollution was more significantly associated with atherogenic surrogates in Southern compared to Northern Chinese. This has potential implications for atherosclerosis prevention.

Background The prevalence and outcomes of the different subtypes of severe low‐gradient aortic stenosis (AS) in routine clinical cardiology practice have not been well characterized. Methods and Results Data were derived from the National Echocardiography Database of Australia. Of 192 060 adults (aged 62.8±17.8 [mean±SD] years) with native aortic valve profiling between 2000 and 2019, 12 013 (6.3%) had severe AS. Of these, 5601 patients (47%) had high‐gradient and 6412 patients (53%) had low‐gradient severe AS. The stroke volume index was documented in 2741 (42.7%) patients with low gradient; 1750 patients (64%) with low flow, low gradient (LFLG); and 991 patients with normal flow, low gradient. Of the patients with LFLG, 1570 (89.7%) had left ventricular ejection fraction recorded; 959 (61%) had paradoxical LFLG (preserved left ventricular ejection fraction), and 611 (39%) had classical LFLG (reduced left ventricular ejection fraction). All‐cause and cardiovascular‐related mortality were assessed in the 8162 patients with classifiable severe AS subtype during a mean±SD follow‐up of 88±45 months. Actual 1‐year and 5‐year all‐cause mortality rates varied across these groups and were 15.8% and 49.2% among patients with high‐gradient severe AS, 11.6% and 53.6% in patients with normal‐flow, low‐gradient severe AS, 16.9% and 58.8% in patients with paradoxical LFLG severe AS, and 30.5% and 72.9% in patients with classical LFLG severe AS. Compared with patients with high‐gradient severe AS, the 5‐year age‐adjusted and sex‐adjusted mortality risk hazard ratios were 0.94 (95% CI, 0.85–1.03) in patients with normal‐flow, low‐gradient severe AS; 1.01 (95% CI, 0.92–1.12) in patients with paradoxical LFLG severe AS; and 1.65 (95% CI, 1.48–1.84) in patients with classical LFLG severe AS. Conclusions Approximately half of those patients with echocardiographic features of severe AS in routine clinical practice have low‐gradient hemodynamics, which is associated with long‐term mortality comparable with or worse than high‐gradient severe AS. The poorest survival was associated with classical LFLG severe AS.

Background Conventionally, stroke volume index (SVI) ≤35ml/m2 is considered as “low-flow” in severe aortic stenosis (AS). Recent evidence suggests a lower threshold SVI (30ml/m2) may be more prognostically informative. Objectives To assess the association between SVI and survival in patients with low-gradient severe AS, with either preserved (LVEF ≥50%) or reduced (LVEF Methods Data were derived from the National Echocardiography Database of Australia (NEDA). Of 192,060 adults (aged 62.8±17.8 years) with comprehensive aortic valve profiling between 2000–2019, 12,013 (6.3%) had severe AS. Based on standard echocardiographic parameters, 1,623 patients had severe low-gradient AS with preserved LVEF and 744 patients had severe low-gradient AS with reduced LVEF. All-cause and cardiovascular-related mortality were assessed for each low-gradient group on an adjusted basis (age, sex, BMI, AVA indexed and RVSP), according to SVI. Mean follow-up was 81±42 months. Results Compared to patients with “normal” flow (SVI >35ml/m2), those with “low-flow” (SVI ≤35ml/m2) had significantly higher BMI, smaller AVA index and lower AV mean gradient and peak velocity (p≤0.001). In low-gradient with preserved LVEF patients, adjusted survival at 1- and 3-years was significantly lower only for SVI ≤30ml/m2 compared with SVI>35ml/m2 (p35ml/m2 (p=0.015 and p=0.018, respectively). Conclusions Taken together with previous data, our results suggest that a SVI threshold of ≤30ml/m2 (rather than ≤35 ml/m2) is prognostically significant in severe low-gradient AS with preserved LVEF. Funding Acknowledgement Type of funding sources: None. Survival plots LGAS with preserved EFSummary of results

Background The optimal method for balloon-expandable transcatheter heart valve (THV) deployment remains unknown. Current implantation protocols are volume-dependent and rely on ad-hoc filling of the deployment apparatus without accounting for annular wall tension during prosthesis expansion, predisposing patients to inconsistent clinical outcomes. During THV deployment, the annular wall tension exerted by the expanding prosthesis is determined by prosthesis diameter and balloon pressure (Laplace's Law). Objective We proposed and tested a novel method for balloon-expandable THV deployment, aimed at controlling balloon pressure and the resulting annular wall tension to allow optimal prosthesis-annulus apposition while preventing significant tissue injury. Methods 330 consecutive patients with severe native aortic stenosis who underwent balloon-expandable THV implantation between 2015–2020 were included. 106 patients were considered high-risk for annular rupture. THVs were deployed until reaching a pre-determined balloon pressure; 4–4.5atm in earlier cases to establish experience and safety, later increasing to 5–6.5atm in most cases. Post-dilatation was performed to reduce >mild angiographic regurgitation (PVR). Using a biomechanical model, annular wall stress (tension) was estimated for each case and assessed against recorded rates of post-dilatation, ≥mild paravalvular regurgitation (PVR) on TTE, new PPM or LBBB and annular rupture. Results Patients with wall stress >3MPa (n=184) had reduced post-dilatation rate (p3.5MPa; no rupture occurred in 102 high-risk cases with wall stress ≤3.5MPa. Based on these results, we defined target deployment wall stress levels (3–3.5MPa) and associated deployment pressure per THV size. Patients within this target range (n=136) had 8.1% new PPM, 12.5% new LBBB, 12.7% mild PVR with no cases of ≥moderate PVR. Importantly, there was an inconsistent relationship between deployment balloon volume and resulting annular wall stress. Conclusion Pressure-regulated THV deployment is a simple, easily reproducible, safe and effective method, regardless of high-risk anatomical complexities. Funding Acknowledgement Type of funding sources: None. Annular wall stress and PVRModel, stress vs volume and new strategy

Background Contemporary registries have documented a change in the epidemiology of PAH patients displaying increasing co-morbidities associated with left heart disease (LHD). These patients are often excluded from randomized clinical trials. It is unclear whether the presence of LHD comorbidities may adversely impact the accuracy of risk stratification and response to PAH therapy. Method Data was extracted from the Pulmonary Hypertension Society of Australia and New Zealand registry for incident patients with a diagnosis with idiopathic/heritable/toxin-induced (I/H/D)-PAH and connective tissue disease (CTD) associated PAH from 2011 - 2020. Patients without available medication and follow up data were excluded. We used the AMBITION trial exclusion criteria to define the subpopulation with LHD risk factors and haemodynamic phenotype (PAH-rLHD). Results 489 patients (I/H/D-PAH=251, CTD-PAH=238) were included in our analysis, with 103 (21.0%) fulfilling the definition of PAH-rLHD (34 had ≥3 risk factors for left heart disease (rLHD-hypertension, diabetes, obesity or ischaemic heart disease) and 76 had borderline haemodynamics (mean capillary wedge pressure 13–15 with pulmonary vascular resistance Conclusion Despite lower PVR at diagnosis, PAH-rLHD patients and “classical” PAH demonstrate similar response to first-line therapy with similar long term survival. The REVEAL 2.0 risk score can be effectively applied to the subpopulation of PAH-rLHD in real life clinical practice. Funding Acknowledgement Type of funding sources: None.

Introduction There are currently no established prognostic models for low-flow, low-Gradient (LFLG) severe aortic stenosis (AS). The Cardiac Damage Staging Classification, has been validated as a clinically useful prognostic tool in high-gradient severe AS, but not specifically in LFLG severe AS. Objective To assess and confirm the prognostic utility of the Cardiac Damage Staging Classification, in LFLG severe AS patients. Methods We analysed data from the National Echocardiography Database of Australia (NEDA), a national multicentre registry with individual data linkage to mortality. Of 192,060 adults (aged 62.8±17.8 years) with comprehensive ultrasound profiling of the native aortic valve studied between 2000–2019, 12,013 (6.3%) had severe AS. Based on standard echocardiographic parameters, 611 classical and 959 paradoxical LFLG patients were identified. Mean follow-up was 70±41 months. All-cause and cardiovascular-related mortality were assessed for each LFLG group on an adjusted basis (age and sex), according to cardiac damage classification staging. Results Paradoxical LFLG patients were younger (mean age 74.3 vs 76.2, p=0.006) and more often female (62.8% vs 36.3%, p Conclusion In patients with LFLG severe AS identified by echocardiography, increasing severity denoted by the cardiac damage staging classification is strongly associated with increasing medium- to long-term mortality. Funding Acknowledgement Type of funding sources: None. Stages + Classical LFLG 5 year mortalityParadoxical LFLG 5 year mortality curves

Background Bicuspid aortic valve (BAV) is the most common congenital heart disease in adults but is clinically heterogeneous. We aimed to describe the echocardiographic characteristics of BAV and compare patients with BAV with moderate‐to‐severe aortic stenosis (AS) with those with tricuspid aortic valve (TAV) stenosis. Methods and Results Using the National Echo Database of Australia, patients in whom BAV was identified were studied. Those with moderate‐to‐severe AS (mean gradient >20 mm Hg [BAV‐AS]) were compared with those with TAV and moderate‐to‐severe AS (TAV‐AS). Of 264 159 adults whose aortic valve morphology was specified, 4783 (1.8%) had confirmed BAV (aged 49.6±17.4 years, 69% men). Of these, 42% had no AS, and 46% had no aortic regurgitation. Moderate‐to‐severe AS was detected in a greater proportion of patients with BAV with a recorded mean gradient (n=1112, 34%) compared with those with TAV (n=4377, 4%; P P P P Conclusions In this large study of patients across the spectrum of BAV disease, the largest proportion had no significant valvulopathy or aortopathy. Compared with those with TAV‐AS, patients with BAV were more likely to have moderate‐to‐severe AS, have larger ascending aortas, and were over 2 decades younger at the time of AS diagnosis. Despite this, patients with BAV appear to have a more favorable prognosis when AS develops, compared with those with TAV‐AS. Registration URL: www.anzctr.org.au/ ; Unique identifier: ACTRN12617001387314.

ObjectivePatients with previously repaired aortic coarctation (CoA) are at risk of developing late surgical complications. Many patients with CoA also have a bicuspid aortic valve (BAV). We sought to determine in patients with repaired CoA whether the presence of BAV is associated with more cardiovascular reinterventions during follow-up.MethodsAdults with previously repaired simple CoA were recruited from our Adult Congenital Heart Disease database (Sydney, Australia). The incidence of complications relating to the ‘CoA-site’ (descending aortic aneurysm or dissection, or recoarctation) and the ‘AV/AscAo’ (aortic valve or ascending aortic pathology) that required intervention was compared between those with BAV (‘CoA-BAV’) and without BAV (‘CoA-only’).ResultsOf 146 patients with repaired CoA, 101 (69%) had BAV. Age at CoA repair was similar (median 6.0 (IQR 0.5–14.0) years vs 5.0 (IQR 0.5–11.0) years, p=0.44), as was the distribution of repair types, with end-to-end repair the most common in both groups (45.9% vs 45.6%). At a median of 28 years following initial repair, a significantly higher proportion of patients with CoA-BAV required cardiovascular reintervention (45.5% vs 20.0%, p=0.003). Whereas ‘CoA-site’ complications were more common than ‘AV/AscAo’ complications in patients with CoA only (13.3% and 0%, respectively), patients with CoA-BAV had a high prevalence of both ‘CoA-site’ as well as ‘AV/AscAo’ complications (19.8% and 21.8%, respectively). Overall survival was similar (p=0.42).ConclusionIn adults with repaired CoA, patients with CoA-BAV are more than twice as likely to require cardiovascular reintervention by early-to-mid-adult life compared with those with CoA alone. Despite this, no difference in survival outcomes was observed.

The authors propose a novel pressure-regulated method for balloon-expandable transcatheter heart valve (THV) deployment, aimed at optimizing prosthesis-annulus apposition while preventing significant tissue injury.The optimal method for balloon-expandable THV deployment remains debated. Current protocols are volume dependent, relying on under- and overfilling of the deployment apparatus. During deployment, the annular wall tension exerted by the expanding prosthesis is determined by maximal THV diameter and balloon pressure (Laplace's law).Three hundred thirty consecutive patients with severe native aortic stenosis who underwent TAVR with SAPIEN 3 THVs were included. One hundred and six patients were considered at high risk for annular rupture. THVs were deployed until reaching a predetermined balloon pressure. Postdilatation was performed to reduce mild or greater angiographic paravalvular regurgitation (PVR). Using a biomechanical model, annular wall stress was estimated for each case and assessed against rates of postdilatation, mild or greater PVR on transthoracic echocardiography, new permanent pacemaker placement or left bundle branch block, and annular rupture.Patients with wall stress3 MPa had reduced postdilatation rate (P 0.001) and reduced final PVR (P = 0.014). Annular rupture occurred in 2 of 3 high-risk patients with wall stress3.5 MPa (3.69 and 3.84 MPa); no rupture occurred in 95 high-risk patients with wall stress ≤3.5 MPa. We defined a single target deployment pressure per THV size to ensure deployment within target wall stress levels of 3 to 3.5 MPa: 6.25 atm for 23-mm THVs, 5.5 atm for 26-mm THVs, and 5 atm for 29-mm THVs. Patients within this target range (n = 136) had a 10.0% postdilatation rate, 12.7% mild PVR, and no moderate to severe PVR. The relationship between balloon filling volume and associated pressure and wall stress was inconsistent.Pressure-regulated THV deployment is a simple, reproducible, safe, and effective method, regardless of high-risk anatomical complexities.

Objectives:Concerns exist over the long-term consequences of subclavian artery ligation in subclavian flap repair for coarctation of the aorta. We sought to analyse upper limb structural and functional performance in adults who have had surgery in childhood for coarctation of the aorta, using either subclavian flap repair or end to end aortic anastomosis.Methods:Two-group observational design using anatomical and upper limb functional performance measures. Purposive sampling from our specialist adult congenital heart disease database of patients who received subclavian flap repair or end to end anastomosis for coarctation of the aorta as children. Upper limb measurements were completed using MRI and blood flow velocity with ultrasound imaging. Bilateral standardised upper limb functional testing of assessment of strength, dexterity and a standardised self-report of upper limb disability was completed.Results:Eighteen right-handed patients, 9 with subclavian repair, (38 ± 12 years, 78% males) were studied. Age at repair was 4.7 ± 5.9 years; mean time from initial repair 32 ± 9 years. The subclavian group had a larger difference between right and left when compared the end to end anastomosis group in: lower arm muscle mass (94.5 ± 42.3 mls versus 37.8 ± 94.5 mls, p = 0.008), lower arm maximal cross-sectional area, (5.9 ± 2.8 cm2 versus 2.9 ± 2.6 cm2, p = 0.038) and grip strength (14.7 ± 8.3 lbs versus 5.9 ± 5.3 lbs, p = 0.016) There were no significant functional differences between groups.Conclusions:In adults with repaired coarctation of the aorta, those with subclavian flap repair had a greater right to left arm muscle mass and grip strength differential when compared to those with end to end anastomosis repair.

There is increasing evidence that current thresholds for diagnosing pulmonary hypertension (PHT) underestimate the prognostic impact of PHT.The aim of this study was to determine the prognostic impact of increasing pulmonary pressures within the National Echocardiography Database of Australia cohort (n = 313,492).The distribution of estimated right ventricular systolic pressure (eRVSP) was examined in 157,842 men and women. All had data linkage to long-term survival during median follow-up of 4.2 years (interquartile range: 2.2 to 7.5 years).The cohort comprised 74,405 men and 83,437 women 65.6 ± 17.7 years of age. Overall, 17,955 (11.4%), 7,016 (4.4%), and 4,515 (2.9%) subjects had eRVSP levels indicative of mild (40 to 49 mm Hg), moderate (50 to 59 mm Hg), or severe (≥60 mm Hg) PHT, respectively, assuming a right atrial pressure of 5 mm Hg. These subjects were more likely to die during long-term follow up (for severe PHT, adjusted hazard ratio: 9.73; 95% confidence interval: 8.60 to 11.0; p 0.001). After adjustment for age, sex, and evidence of left heart disease, those subjects with eRVSP levels within the third (28.05 to 32.0 mm Hg; hazard ratio: 1.410; 95% confidence interval: 1.310 to 1.517) and fourth (32.05 to 38.83 mm Hg; hazard ratio: 1.979; 95% confidence interval: 1.853 to 2.114) quintiles had significantly higher mortality (p 0.001) than those in the lowest quintile. Accordingly, a clear and consistent threshold of increased mortality (including 1- and 5-year actuarial mortality) around an eRVSP of 30.0 mm Hg was evident.In this large and unique cohort, the prognostic impact of clinically accepted levels of PHT was confirmed. Moreover, a distinctly lower threshold for increased risk for mortality (eRVSP30.0 mm Hg) indicative of PHT was identified. (A Longitudinal Cohort Study of Echocardiograms From Public and Private Echocardiography Laboratories From Around Australia, Linked With the National Deaths Index; ACTRN12617001387314).

The need for permanent pacing has been identified as a predictor of poor outcomes in the late survivors of Fontan surgery. However, it is not clear if the need for a pacemaker is a surrogate marker of a declining Fontan state, or if pacing is deleterious to the Fontan circulation.We sought to compare the long-term outcomes of propensity-matched Fontan patients with and without a permanent pacemaker.Patients who have survived Fontan completion with a documented history of cardiac arrhythmia were identified from the Australia and New Zealand Fontan Registry. Pacemaker insertion details, cardiac function and electrophysiological data were obtained for the patients with a permanent pacemaker. Survival analysis was performed with propensity score matching to compare late survival and outcomes in patients with versus without a pacemaker.There was a total of 310 patients with a history of cardiac arrhythmia, of which 126 (41%) had a permanent pacemaker. After propensity-score matching, 99 pairs were generated (n = 198). Patients with a permanent pacemaker had a higher risk of death (HR 3.32 95% CI 1.60-6.90, p = 0.001) and death or transplantation (HR 3.55 95% CI 1.87-6.73, p 0.001). Patients who were only paced atrially were not at a significantly increased risk of death or transplantation. However, patients who were ventricular paced50% of the time were much more likely to encounter late death or transplantation (HR 3.82 95% CI 1.64-8.95, p = 0.002).Having a permanent pacemaker and needing ventricular pacing is likely associated with an increased risk of death and transplantation in patients with a Fontan circulation.

AIMS: Patients with cyanotic congenital heart disease (CCHD) have been suggested to develop less atherosclerosis than the general population. This study aimed to evaluate the extent of coronary atherosclerosis in patients with CCHD using intravascular ultrasound (IVUS) and near-infrared spectroscopy (NIRS).METHODS AND RESULTS: Fifteen patients with CCHD (women, 9; median age, 53 years) and 14 acyanotic controls (women, 6; median age, 53 years) were examined with IVUS-NIRS of the right coronary artery (RCA). The patients with CCHD presented with a larger RCA diameter than the controls (external elastic membrane diameter, 6.1 [4.8-6.7] vs. 4.7 [4.1-5.1] mm, respectively; p=0.01). No difference in area stenosis was found between the patients and the controls (15.8% [12.3-19.7] vs. 15.2% [9.5-18.8]; p=0.87). The presence of lipid by NIRS was noted in 43% of patients with CCHD and in 92% of the controls; however, no differences in total or max 4 mm lipid core burden index (LCBI) or in plasma lipid profile were found.CONCLUSIONS: Patients with CCHD presented with larger coronary arteries than acyanotic controls. No difference in the degree of area stenosis in the coronary arteries was found between the cyanotic and acyanotic patients; however, a lower proportion of patients with CCHD showed a positive LCBI.

Background Adult congenital heart disease (ACHD) is a relatively new subspecialty in the cardiology field. The prevalence of ACHD is estimated at ∼ 3,000 per million adult population. The ACHD patient group is estimated to grow at ∼ 5% per year and in the next decade it is forecast that 1 in 150 young adults will carry some form of ACHD diagnosis. These estimates translate to ∼ 72,000 ACHD patients in Australia and ∼ 14,000 in New Zealand, although no current numbers are available. The Cardiac Society of Australia and New Zealand (CSANZ) has recently published Recommendations for Standards of Care for Adult Congenital Heart Disease (ACHD) in 2016. There is currently no long-term plan or proposal to address this huge health care burden within the federal government. This document details the size of the problem insofar as it is known and recommends solutions to be implemented. Methods This document was developed by the Adult Congenital Heart Disease Working Group of the Paediatric and Congenital Council (the Congenital Heart Alliance of Australia and New Zealand) as a response to the chronic under resourcing in this area, the risk this poses to patients and clinicians, and the clear need for long-term planning to develop safe care pathways. Results These issues were raised with the CSANZ Board in December 2015 and the document was developed in response to the Board’s request for more information. The current iteration was finalised on 14 November 2017. Conclusions The authorship group comprised participants in the CSANZ adult CHD standards of care recommendations from 2013 with the inclusion of some newly trained ACHD cardiologists, who represented most states and territories across ANZ. None of the authors has any academic or professional conflict of interest.

Carotid-femoral pulse wave velocity is associated with arterial stiffness in major elastic arteries, and predicts future cardiovascular events. However, little is known about carotid-femoral pulse wave velocity as a marker of vascular health in children. Semi-automated cuff-based devices for assessing pulse wave velocity are increasingly popular, although these utilize an algorithm developed and validated in adults. Physiological differences between adults and children may, however, reduce the accuracy of cuff-based methods. We sought to determine the accuracy of a cuff-based pulse wave velocity device in healthy children, and determine whether a novel age-appropriate algorithm increases accuracy. We recruited 29 healthy children between the ages of 2 and 20 years. Pulse wave velocity was measured both by using a tonometer on the carotid artery and an inflated cuff on the thigh, and using a tonometer on both the carotid artery and femoral artery as a reference standard. Accuracy of the cuff-based device with its standard algorithm developed in adults, and a novel age-appropriate algorithm corrected for physiological differences in leg pulse wave velocity was assessed with Regression analysis and Bland-Altman plots. Cuff-based device estimates of pulse wave velocity had excellent agreement to the reference standard (Δ = -0.26 ms-1 [SD 0.44]). The novel age-appropriate algorithm improved the accuracy of the cuff-based method (Δ = 0.02 ms-1 [SD 0.44]). The cuff-based semi-automatic approach estimates carotid-femoral pulse wave velocity with excellent agreement to the reference standard. However, adjusting the algorithm for known differences in leg pulse wave velocity further improves the accuracy of cuff-based measurement in children and adolescents.

Introduction Adults with congenital heart disease (CHD) are predisposed to arrhythmias, which can often be refractory to medical therapy. Sotalol is an attractive alternative antiarrhythmic to amiodarone in this younger patient population, given the latter's toxicity profile, but it may have proarrhythmic effects. We therefore aimed to assess the efficacy and safety of sotalol in adults with CHD. Methods We retrospectively assessed our adult CHD database for all patients ≥16 years old, with moderate to highly complex defects, who were prescribed sotalol between 2000 and 2017. Efficacy in treating the clinical arrhythmia was assessed as complete, partial or failure. Adverse effects, including proarrhythmia, were documented. Results Sotalol was prescribed in 82 of 902 adult CHD patients reviewed (9%). The mean age at sotalol commencement was 31.8 ± 13.1 years, with a median time on sotalol of 2.8 years. The average prescribed dose was 122 ± 51 mg/daily. Sotalol was completely effective in 48% (n = 39), partially effective in 46% (n = 38) and failed to control the clinical arrhythmia in 6% (n = 5). Fifteen patients (18%) discontinued sotalol due to a side effect, most commonly fatigue or dyspnoea. No episodes of torsades de pointes or sudden cardiac death were observed. Significant bradycardia related to sotalol occurred in 13% (n = 11, with permanent pacing implemented in 4), and was associated with Fontan anatomy. Conclusions In moderate to highly complex adult CHD, sotalol was reasonably effective and safe in low doses. Side effects limiting treatment were typically non-life-threatening, with significant bradycardia related to sotalol more likely in Fontan patients.

Background Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. Methods A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). Results Mean age and time since Fontan were 19.8 ± 9.3 and 14.1 ± 7.6 years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (r = 0.24, p = 0.015) and ACR (r = 0.29, p = 0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, p = 0.002). Renal impairment was mild (mGFR 60–89 ml/min/1.73 m2) in 46/131 (35%) and moderate (mGFR 30–59 ml/min/1.73 m2) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (β = 0.89, 95% CI 0.54–1.25, p = 0.002) and decreased mGFR (β = −0.77, 95% CI −1.29–0.24, p = 0.005). Conclusions In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion.

Background Pulmonary hypertension complicating left heart disease (PH-LHD) is the most common cause of PH. Off-label use of pulmonary arterial hypertension (PAH) medications for PH-LHD is prevalent, despite a lack of clinical data supporting their use. Methods A systematic review and meta-analysis was performed. Comprehensive search of all available literature to date identified ten randomised, placebo controlled trials comprising 439 treated (Phosphodiesterase 5 inhibitors: n = 206; guanylate cyclase stimulators: n = 132; endothelin receptor antagonists: n = 101) and 338 placebo patients. Random effects model was employed to assess outcomes in the treatment compared to the placebo control arm. Results The risks of all-cause mortality, cardiovascular mortality and worsening heart failure were numerically higher in the treated compared to the control group, although not statistically (all-cause mortality: RR = 1.97, 95% CI: 0.64–6.05, p = 0.24; cardiovascular mortality: RR = 2.01, 95% CI: 0.39–10.34, p = 0.4; worsening heart failure: RR = 1.23, 95% CI: 0.68–2.25, p = 0.49). Conversely, right heart hemodynamics improved numerically in the treated group, also without being significant (mean pulmonary artery pressure: MWD = −5.13 mm Hg, 95% CI: −13.2–2.9, p = 0.21; pulmonary vascular resistance: MWD = −0.87 WU, 95% CI: −1.75–0.1, p = 0.053). Conclusions The current meta-analysis demonstrated that there is no current evidence to support the widespread use of PAH therapy in PH-LHD. On the basis of a numerically increased risk of clinical harm, these agents should not be prescribed in this setting, unless further evidence of benefit arises in the future.

OBJECTIVES: Long-term data on the impact of pulmonary artery (PA) augmentation in patients who underwent the Fontan procedure are lacking. The aim of this study was to examine whether surgical or transcatheter PA augmentation at or prior to the Fontan procedure adversely affects the outcomes. METHODS: Data of 1436 patients from the Australia-New Zealand Fontan Registry (1975-2015) were analysed. Primary end point was death or Fontan failure. Cox regression with propensity score matching was used to determine risk or benefit conferred by PA augmentation. RESULTS: Forty-eight (3.3%) patients underwent PA augmentation following cavopulmonary shunt (surgical n = 14, balloon dilatation n = 16 and stent n = 18) and 105 (7.3%) patients underwent PA augmentation at the time of the Fontan procedure (surgical n = 104, stent n = 1). Median follow-up was 6.4 years with 10 deaths (6.5%) in the augmentation group and 10.5 years with 95 deaths (7.4%) in the non-augmentation group. The unadjusted Kaplan-Meier, log-rank test and Cox regression analysis demonstrated no significant difference in both end points between the 2 groups [death: hazard ratio (HR) 1.35, 95% confidence interval (CI) 0.70-2.60; P = 0.37 death or failure: HR 1.39, 95% CI 0.83-2.34; P = 0.21]. The propensity score matching yielded 131 matched pairs, with adequate balance for all covariates (the median residual bias = 0.05). The subsequent Cox regression demonstrated no significant difference in the risks of death (HR 1.30, 95% CI 0.49-3.41; P = 0.60) and death or Fontan failure (HR 0.92, 95% CI 0.46-1.85; P = 0.82). CONCLUSIONS: PA augmentation prior to or at the Fontan procedure does not confer worse long-term outcomes.

Objective The number of adults with congenital heart disease has increased with improvements in surgical and medical management, posing a challenge for regional and rural settings, which might have difficulties accessing specialised professionals with congenital heart disease services. This study aims to ascertain the demographics and management of adults with congenital heart disease seen by a cardiology practice in regional Australia to better understand the needs of regional adults with congenital heart disease. Design A descriptive study using data from clinical notes collected between April 2013 and April 2016. Setting A private cardiology practice in Coffs Harbour, New South Wales. The practice has a treating cardiologist, senior sonographer, visiting cardiothoracic surgeon and visiting paediatric cardiologist. Participants One-hundred-and-one adults with congenital heart disease (age: 16-88 years; 55 women). Main outcome measures Congenital heart disease defect, lesion severity, referral reason, past surgery, pulmonary hypertension, cardiac surgery during the study, mortality, adherence with follow-up plans and specialist referral. Results Sixty-six patients had simple congenital heart disease, 24 had congenital heart disease of moderate complexity and 11 had congenital heart disease of great complexity. Most were referred for surveillance, seven were referred due to pregnancy and eight were new diagnoses. Six patients died, nine had cardiac operations and five were treated for pulmonary arterial hypertension. Overall adherence to the treating cardiologist's follow-up plans was 84%. All patients with congenital heart disease of great complexity were referred to congenital heart disease specialists. Conclusion A substantial number of patients had complex pathology, new diagnoses or required surgery, highlighting the importance of developing integrated services with the close support of outside specialists in managing adults with congenital heart disease in regional settings.

Background Epidemiology and treatment strategies continue to evolve in pulmonary arterial hypertension (PAH). We sought to define the characteristics and survival of patients with idiopathic, heritable and drug-induced PAH in the current management era. Methods Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry. Results Between January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57.2 ± 18.7 years, female 69.5%) and followed for a median duration of 26 months (IQR17–39). Co-morbidities were common such as obesity (34.1%), systemic hypertension (30.5%), coronary artery disease (16.4%) and diabetes mellitus (19.5%). Initial combination therapy was used in 54 patients (dual, n = 50; triple, n = 4). Estimated survival rates at 1-year, 2-years and 3-years were 95.6% (CI 92.8–98.5%), 87.3% (CI 82.5–92.4%) and 77.0% (CI 70.3–84.3%), respectively. Multivariate analysis showed that male sex and lower 6-minute distance at diagnosis independently predicted worse survival, whereas obesity was associated with improved survival. Co-morbidities other than obesity did not impact survival. Initial dual oral combination therapy was associated with a trend towards better survival compared with initial oral monotherapy (adjusted HR = 0.27, CI 0.06-1.18, p = 0.082) Conclusions The epidemiology and survival of patients with idiopathic PAH in Australia and New Zealand are similar to contemporary registries reported in Europe and North America. Male sex and poorer exercise capacity are predictive of mortality whereas obesity appears to exert a protective effect. Despite current therapies, PAH remains a life-threatening disease associated with significant early mortality.

The National Echocardiography Database Australia (NEDA) is a new echocardiography database collecting digital measurements on both a retrospective and prospective basis. To date, echocardiographic data from 435,133 individuals (aged 61.6 ± 17.9 years) with linkage to 59,725 all-cause deaths during a median of 40 months follow-up have been collected. These data will inform a number of initial analyses focusing on pulmonary hypertension, aortic stenosis and the role of artificial intelligence to facilitate accurate diagnoses of cardiac abnormalities.

Objectives This study sought to describe atrial arrhythmia mechanisms, acute outcomes, and long-term arrhythmia burdens following catheter ablation in adult atriopulmonary (AP) Fontan patients. Background Atrial arrhythmias are a significant cause of morbidity and mortality in the AP Fontan population. Methods Sixty consecutive atrial arrhythmia ablations were reviewed in 42 AP Fontan patients (31 ± 8 years of age), performed between 1998 and 2017. The number of induced and ablated tachycardias was recorded for each case, as well as the ability to ablate the suspected clinical tachycardia. Longer-term arrhythmia burden was assessed by using a 12-point clinical arrhythmia severity score. Results Intra-atrial re-entrant tachycardia (IART) was induced in 93% of cases (n = 56), atrioventricular re-entrant tachycardia in 2 (3%) and atrioventricular nodal re-entrant tachycardia in a single case. The mean number of tachycardias induced per case was 2.3. The critical isthmus for IART was mapped to the lateral (n = 10), inferolateral (n = 8), posterior/posterolateral (n = 16), or septal (n = 10) systemic venous atrium, or to the pulmonary venous atrium (n = 4). Ablation of all inducible tachycardias was achieved in 62%, ablation of at least one (but not all) inducible tachycardias in 25%, with failure to ablate any tachycardias in 13%. The suspected clinical arrhythmia was ablated in 50 cases (83%). Catheter ablation resulted in a significant reduction in arrhythmia score at 3 to 6, 12, and 24 months, irrespective of whether all inducible tachycardias were ablated, or the suspected clinical arrhythmia only. Twelve patients (29%) underwent at least one repeat ablation procedure, with a mean time between ablations of 2.7 ± 3.0 years. There were no cases of periprocedural death, stroke or cardiac tamponade. Conclusions Catheter ablation can be a safe and effective intervention that will significantly reduce arrhythmia burden in the AP Fontan patient.

Adults who were born preterm are at increased risk of hypertension and cardiovascular disease in later life. Infants born late preterm are the majority of preterm births; however, the effect of late preterm on risk of cardiovascular disease is unclear. The objective of this study was to assess whether vascular health and cardiac autonomic control differ in a group of late preterm newborn infants compared to a group of term-born infants.A total of 35 healthy late preterm newborn infants, with normal growth (34–36 completed weeks’ gestation) and 139 term-born infants (37–42 weeks’ gestation) were compared in this study. Aortic wall thickening, assessed as aortic intima–media thickness (IMT) by high-resolution ultrasound, and cardiac autonomic control, assessed by heart rate variability, were measured during the first week of life. Postnatal age of full-term and late preterm infants at the time of the study was 5 days (standard deviation [SD] 5) and 4 days (SD 3), respectively.Infants born late preterm show reduced aortic IMT (574 μm [SD 51] vs. 612 μm [SD 73]) and reduced heart rate variability [log total power 622.3 (606.5) ms2 vs. 1180. 6 (1114.3) ms2], compared to term infants. These associations remained even after adjustment for sex and birth weight.Infants born late preterm show selective differences in markers of cardiovascular risk, with potentially beneficial differences in aortic wall thickness in contrast to potentially detrimental differences in autonomic control, when compared with term-born control infants. These findings provide pathophysiologic evidence to support an increased risk of hypertension and sudden cardiac death in individuals born late preterm.

Background In pulmonary arterial hypertension (PAH), progressive right ventricular (RV) dysfunction is believed to be largely secondary to RV ischaemia. A recent pilot study has demonstrated the feasibility of Oxygen-sensitive (OS) cardiovascular magnetic resonance (CMR) to detect in-vivo RV myocardial oxygenation. The aims of the present study therefore, were to assess the prevalence of RV myocardial ischaemia and relationship with RV myocardial interstitial changes in PAH patients with non-obstructive coronaries, and corelate with functional and haemodynamic parameters. Methods We prospectively recruited 42 patients with right heart catheter (RHC) proven PAH and 11 healthy age matched controls. The CMR examination involved standard functional imaging, OS-CMR imaging and native T1 mapping. An ΔOS-CMR signal intensity (SI) index (stress/rest signal intensity) was acquired at RV anterior, RV free-wall and RV inferior segments. T1 maps were acquired using Shortened Modified Look-Locker Inversion recovery (ShMOLLI) at the inferior RV segment. Results The inferior RV ΔOS-CMR SI index was significantly lower in PAH patients compared with healthy controls (9.5 (– 7.4–42.8) vs 12.5 (9–24.6)%, p = 0.02). The inferior RV ΔOS-CMR SI had a significant correlation to RV inferior wall thickness (r = – 0.7, p p = 0.02). Compared to healthy controls, patients with PAH had higher native T1 in the inferior RV wall: 1303 (1107–1612) vs 1232 (1159–1288)ms, p = 0.049. In addition, there was a significant difference in the inferior RV T1 values between the idiopathic PAH and systemic sclerosis associated PAH patients: 1242 (1107–1612) vs 1386 (1219–1552)ms, p = 0.007. Conclusion Blunted OS-CMR SI suggests the presence of in-vivo microvascular RV dysfunction in PAH patients. The native T1 in the inferior RV segments is significantly increased in the PAH patients, particularly among the systemic sclerosis associated PAH group.