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Aims: We set out to explore associations between a 'mitral-specific' cardiac damage score (m-CDS) and survival outcomes in mitral regurgitation (MR) and compare the performance of the m-CDS and an 'aortic-specific' CDS (a-CDS) in patients with MR within the large National Echo Database of Australia., Methods: Among 620 831 unique adults investigated with echocardiography, there were 17 658 individuals (3.1%) with moderate or greater functional MR (aged 76±13 years, 51% female) who met inclusion criteria. A randomly selected cohort of 5000 of these patients was used to test seven different CDS models for prediction of subsequent all-cause mortality during an average 3.8-year follow-up. The best-performing CDS model in the derivation cohort was then applied to a validation cohort of the remaining 12 658 individuals (aged 76±13 years, 51% female)., Results: The best-performing m-CDS model stratified the full cohort into Stage 0: control (1046 patients, 8%); Stage 1: left atrial damage (3416 patients, 27%); Stage 2: left ventricular damage (3352 patients, 26%); Stage 3: right ventricular damage (1551 patients, 12%) and Stage 4: pulmonary hypertension (3293 patients, 26%). Increasing m-CDS stage was consistently and incrementally associated with both all-cause and cardiovascular mortality at 1 year, 5 years and all-time and remained so after adjustment for increasing age and severity of MR, with a ~35% increase in mortality for each increase in CDS stage (p<0.001)., Conclusion: A m-CDS was robustly and incrementally associated with short-, medium- and long-term risk of all-cause and cardiovascular mortality in patients with functional MR in this large registry study., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Aims: Increasing aortic dilation increases the risk of aortic dissection. Nevertheless, dissection occurs at dimensions below guideline-directed cut-offs for prophylactic surgery. Currently, there are no large-scale population imaging data assessing aortic dimensions before dissection., Methods and Results: Patients within the National Echo Database of Australia were stratified according to absolute, height-indexed, and body surface area (BSA)-indexed aortic dimensions. Fatal thoracic aortic dissections (ICD-10-AM Code I71) were identified via linkage with the National Death Index. A total of 524 994 individuals were assessed, comprising patients with normal aortic dimensions (n = 460 992), mild dilation (n = 53 402), moderate dilation (n = 10 029), and severe dilation (n = 572). A total of 274 992 (52.4%) were males, with a median age of 64 years and a median follow-up time of 6.9 years. Eight hundred and ninety-nine fatal aortic dissections occurred (normal diameter = 610, mildly dilated aorta = 215, moderately dilated = 53, and severely dilated = 21). Using normal aortas as the reference population, odds of fatal dissection increased with aortic diameter [mild = odds ratio (OR) 3.05, 95% confidence interval (CI) 2.61-3.56; moderate = OR 4.0, 95% CI 3.02-5.30; severe = OR 28.72, 95% CI 18.44-44.72]. Due to the much larger number of patients without severe aortic dilation, 97.7% of fatal aortic dissections occurred in non-severely dilated aortas. Following sensitivity analysis, severe aortic dilation was responsible for at most 24.4% of fatal aortic dissections. The results were robust for absolute, height-indexed, or BSA-indexed aortic measurements., Conclusion: Although severe aortic dilatation is associated with a near-30-fold increase in fatal dissections, severely dilated aortas are implicated in only 2.3-24.4% of fatal dissections. This highlights the 'aortic paradox' and limitations of current guidelines. Future studies should seek to refine risk predictors in patients without severe aortic dilation., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: The independent effect of pulmonary hypertension (PHT) severity on mortality in those with reduced left ventricular ejection fraction (LVEF) is not well known., Objectives: The authors aimed to examine the prognostic impact of increasingly elevated pulmonary pressures in a large clinical cohort of adults with reduced LVEF., Methods: The authors analyzed data from the National Echocardiography Database of Australia, a large clinical registry linking routine echocardiographic investigations to mortality. In 23,675 adults with a recorded tricuspid regurgitation peak velocity (TRV) and reduced LVEF (<50%), the authors evaluated the relationship between conventional thresholds of increasing risk of PHT and mortality during median follow-up of 2.9 years (Q1-Q3: 1.0-5.4 years)., Results: Mean age was 70 ± 15 years, and 7,498 (31.7%) individuals were female. Overall, 8,801 (37.2%) had normal (TRV <2.5 m/s), 7,061 (29.8%) had borderline (2.5-2.8 m/s), 5,676 (24.0%) intermediate (2.9-3.4 m/s), and 2,137 (9.0%) individuals had high-risk PHT (>3.4 m/s). With increasing risk of PHT, 1- and 5-year actuarial mortality increased from 13.3% and 43.8% to 41.5% and 81.4%, respectively (P < 0.0001) from normal to severely elevated TRV. The adjusted HR of mortality increased by 1.31-fold (95% CI: 1.23-1.38), 1.82-fold (95% CI: 1.72-1.93), and 2.38-fold (95% CI: 2.21-2.56) in those with borderline, intermediate, and high risk of PHT respectively, compared with normal TRV. Further analyses suggested a distinctive threshold with a TRV reached >2.41 m/s (adjusted HR: 1.18 [95% CI: 1.04-1.33])., Conclusions: The authors demonstrate the prevalence and negative prognostic impact of increasingly elevated TRV levels in individuals with reduced LVEF, with a threshold for mortality lying within the range of "borderline risk" PHT., Competing Interests: Funding Support and Author Disclosures This research did not receive any specific grants from funding agencies in the public, commercial, or not for profit sectors. However, the National Echo Database of Australia has received investigator-initiated funding support from Janssen, Novartis Pharmaceuticals, and Edwards Lifesciences in the past 3 years. Both the National Echo Database of Australia (grant 1055214) and Dr Stewart (grant 11358940) are supported by the National Health and Medical Research Council of Australia. Dr Ratwatte is supported by the National Heart Foundation of Australia (grant 106796). Drs Stewart, Strange, and Playford have previously received consultancy/speaker fees from Edwards Lifesciences. Drs Stewart and Playford have received consultancy fees from Medtronic, Edwards Lifesciences, Abbott Laboratories, and ECHO IQ Pty Ltd. Dr Celermajer has reported that he has no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective Data linkage is a very powerful research tool in epidemiology, however, establishing this can be a lengthy and intensive process. This paper reports on the complex landscape of conducting data linkage projects in Australia. Methods We reviewed the processes, required documentation, and applications required to conduct multi-jurisdictional data linkage across Australia, in 2023. Results Obtaining the necessary approvals to conduct linkage will likely take nearly 2 years (estimated 730 days, including 605 days from initial submission to obtaining all ethical approvals and an estimated further 125 days for the issuance of unexpected additionally required approvals). Ethical review for linkage projects ranged from 51 to 128 days from submission to ethical approval, and applications consisted of 9-25 documents. Conclusions Major obstacles to conducting multi-jurisdictional data linkage included the complexity of the process, and substantial time and financial costs. The process was characterised by inefficiencies at several levels, reduplication, and a lack of any key accountabilities for timely performance of processes. Data linkage is an invaluable resource for epidemiological research. Further streamlining, establishing accountability, and greater collaboration between jurisdictions is needed to ensure data linkage is both accessible and feasible to researchers.

Background: Regional wall motion abnormalities (WMAs) after myocardial infarction are associated with adverse remodeling and increased mortality in the short to medium term. Their long-term prognostic impact is less well understood., Methods and Results: Via the National Echo Database of Australia (2000-2019), we identified normal wall motion versus WMA for each left ventricular wall among 492 338 individuals aged 61.9±17.9 years. The wall motion score index was also calculated. We then examined actual 1- and 5-year mortality, plus adjusted risk of long-term mortality according to WMA status. Overall, 39 346/255 697 men (15.4%) and 17 834/236 641 women (7.5%) had a WMA. The likelihood of a WMA was associated with increasing age and greater systolic/diastolic dysfunction. A defect in the inferior versus anterior wall was the most and least common WMA in men (8.0% and 2.5%) and women (3.3% and 1.1%), respectively. Any WMA increased 5-year mortality from 17.5% to 29.7% in men and from 14.9% to 30.8% in women. Known myocardial infarction (hazard ratio [HR], 0.86 [95% CI, 0.80-0.93]) or revascularization (HR, 0.87 [95% CI, 0.82-0.92]) was independently associated with a better prognosis, whereas men (1.22-fold increase) and those with greater systolic/diastolic dysfunction had a worse prognosis. Among those with any WMA, apical (HR, 1.08 [95% CI, 1.02-1.13]) or inferior (HR, 1.09 [95% CI, 1.04-1.15]) akinesis, dyskinesis or aneurysm, or a wall motion score index >3.0 conveyed the worst prognosis., Conclusions: In a large real-world clinical cohort, twice as many men as women have a WMA, with inferior WMA the most common. Any WMA confers a poor prognosis, especially inferoapical akinesis/dyskinesis/aneurysm.

Objectives To provide insights into the obstacles which pose challenges to the set-up of any National Registry in Australia. Methods An analysis of our experience in executing a Multi-Institutional Agreement (MIA) and obtaining ethics and governance approvals, post-award of a large Medical Research Futures Fund grant in June 2020. Results From July 2020, our timeline to an executed MIA was 283 days, despite full-time staff working towards this goal. Subsequently, after lead site ethics approval, time to site governance approvals ranged from 9 to 291 days. A total of 214 emails were sent during the MIA development and signing. There were 11-71 emails sent to individual governance offices and the number of requested points of additional information ranged from 0 to 31 queries. Conclusions There were considerable time delays in executing the initial (pre-research) stages of a National Federal Government funded Registry project which required substantial time and resources. We report a wide variation in requirements between different states and institutions. We propose several strategies which could be implemented to facilitate a more streamlined approach to research ethics and governance. This centralised approach would allow for better use of funding and facilitate better progress in medical research.

Background: Aortic stenosis (AS) is the most common valvular heart disease in high-income countries. Adjusted for clinical confounders, the risk associated with increasing AS severity across the spectrum of AS severity remains uncertain., Methods: The authors conducted an international, multicenter, parallel-cohort study of 217,599 Australian (mean age, 76.0 ± 7.3 years; 49.3% women) and 30,865 US (mean age, 77.4 ± 7.3 years; 52.2% women) patients aged ≥65 years who underwent echocardiography. Patients with previous aortic valve replacement were excluded. The risk of increasing AS severity, quantified by peak aortic velocity (Vmax), was assessed through linkage to 97,576 and 14,481 all-cause deaths in Australia and the United States, respectively., Results: The distribution of AS severity (mean Vmax, 1.7 ± 0.7 m/sec) was similar in both cohorts. Compared with those with Vmax of 1.0 to 1.49 m/sec, those with Vmax of 2.50 to 2.99 m/sec (US cohort) or Vmax of 3.0 to 3.49 m/sec (Australian cohort) had a 1.5-fold increase in mortality risk within 10 years, adjusting for age, sex, presence of left heart disease, and left ventricular ejection fraction. Overall, the adjusted risk for mortality plateaued (1.75- to 2.25-fold increased risk) above a Vmax of 3.5 m/sec. This pattern of mortality persisted despite adjustment for a comprehensive list of comorbidities and treatments within the US cohort., Conclusions: Within large, parallel patient cohorts managed in different health systems, similar patterns of mortality linked to increasingly severe AS were observed. These findings support ongoing clinical trials of aortic valve replacement in patients with nonsevere AS and suggest the need to develop and apply more proactive surveillance strategies in this high-risk population., (Copyright © 2022 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: While large scientific and medical evidence has demonstrated the increased risk of death and cardiovascular mortality in patients with severe AS, the independent contribution of moderate AS to an increased risk of death remains uncertain., Methods and Findings: We conducted a multicenter study including a cohort of 30,865 US patients and another cohort of 217,599 Australian patients with equivalent echocardiographic and aortic valve profiling over the same period (2003-2017). During a median 5.2 years (US) and 4.4 years (Australian) follow-up, the risk of death (hazard ratio) of patients with moderate AS as compared to those without AS was 1.66 (95%CI 1.52-1.80) and 1.37 (95%CI 1.34-1.41) in the US and Australian cohorts, even after adjusting this analysis for age and sex. This increased risk of death and cardiovascular mortality (odds ratio) in patients with moderate AS was consistent also across subgroups of left ventricular ejection fraction (LVEF) (subgroups of LVEF < 40%, 40-49%, 50-59%, and ≥ 60%: OR of moderate AS for CV mortality 2.0 [95%CI 1.4-2.7], 1.7 [95%CI 1.2-2.4], 1.5 [95%CI 1.1-1.9], and 1.4 [95%CI 1.2-1.6], respectively)., Conclusions: The findings of this study suggest that patients with moderate AS have a potential increased risk of death and cardiovascular mortality, regardless of age, sex, and LVEF. Hence, these data suggest the need to develop specific strategies to detect and treat individuals with moderate AS., Competing Interests: Dr. Strom additionally reports grant funding from the NIH/NHLBI, Ultromics, HeartSciences, and Anumana, consulting for Bracco Diagnostics, and speaker honoraria from Northwest Imaging Forums. Dr. Strange reports consulting for Edwards Lifesciences. Changyu Shen is currently an employee of Biogen. All other authors report no relevant disclosures.

Background: We aim to establish a new and informative bi-national Registry for Congenital Heart Disease (CHD) patients in Australia and New Zealand, to document the burden of disease and clinical outcomes for patients with CHDs across the lifespan. When planning for the implementation of this Registry, we sought to evaluate the strengths and weaknesses of existing national and large regional CHD databases., Methods: We characterised 15 large multi-institutional databases of pediatric and/or adult patients with CHD, documenting the richness of their datasets, the ease of linkage to other databases, the coverage of the target cohort and the strategies utilised for quality control., Results: The best databases contained demographic, clinical, physical, laboratory and patient-reported data, and were linked at least to the national/regional death registry. They also employed automatic data verification and regular manual audits. Coverage ranged from around 25% of all eligible CHD cases for larger databases to near 100% for some smaller registries of patients with specific CHD lesions, such as the Australia and New Zealand Fontan Registry., Conclusions: Existing national and regional CHD databases have strengths and weaknesses; few combine complete coverage with high quality and regularly audited data, across the broad range of CHDs., Competing Interests: Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: We aimed to address the paucity of information describing the treatable burden of disease associated with severe aortic stenosis (AS) within Australia's ageing population., Methods: A contemporary model of the population prevalence of symptomatic, severe AS and treatment pathways in Europe and North America was applied to the 2019 Australian population aged ≥ 55 years (7 million people) on an age-specific basis. Applying Australian-specific data, these estimates were used to further calculate the total number of associated deaths and incident cases of severe AS per annum., Results: Based on an overall point prevalence of 1.48 % among those aged ≥ 55 years, we estimate that a minimum of 97,000 Australians are living with severe AS. With a 2-fold increased risk of mortality without undergoing aortic valve replacement (AVR), more than half of these individuals (∼56,000) will die within 5-years. From a clinical management perspective, among those with concurrent symptoms (68.3 %, 66,500 [95 % CI 59,000-74,000] cases) more than half (58.4 %, 38,800 [95 % CI 35,700 - 42,000] cases) would be potentially considered for surgical AVR (SAVR) - comprising 2,400, 5,400 and 31,000 cases assessed as high-, medium- or low peri-operative mortality risk, respectively. A further 17,000/27,700 (41.6 % [95 % CI 11,600 - 22,600]) of such individuals would be potentially considered to a transthoracic AVR (TAVR). During the subsequent 5-year period (2020-2024), each year, we estimate an additional 9,300 Australians aged ≥ 60 years will subsequently develop severe AS (6,300 of whom will experience concurrent symptoms). Of these symptomatic cases, an estimated 3,700 and 1,600 cases/annum, will be potentially suitable for SAVR and TAVR, respectively., Conclusions: These data suggest there is likely to be a substantive burden of individuals living with severe AS in Australia. Many of these cases may not have been diagnosed and/or received appropriate treatment (based on the evidence-based application of SAVR and TAVR) to reduce their high-risk of subsequent mortality., (© 2021. The Author(s).)

Aims: To examine the characteristics/prognostic impact of diastolic dysfunction (DD) according to 2016 American Society of Echocardiography (ASE) and European Society of Cardiovascular Imaging (ESCVI) guidelines, and individual parameters of DD., Methods and Results: Data were derived from a large multicentre mortality-linked echocardiographic registry comprising 436 360 adults with ≥1 diastolic function measurement linked to 100 597 deaths during 2.2 million person-years follow-up. ASE/European Association of Cardiovascular Imaging (EACVI) algorithms could be applied in 392 009 (89.8%) cases; comprising 11.4% of cases with 'reduced' left ventricular ejection fraction (LVEF < 50%) and 88.6% with 'preserved' LVEF (≥50%). Diastolic function was indeterminate in 21.5% and 62.2% of 'preserved' and 'reduced' LVEF cases, respectively. Among preserved LVEF cases, the risk of adjusted 5-year cardiovascular-related mortality was elevated in both DD [odds ratio (OR) 1.31, 95% confidence interval (CI) 1.22-1.42; P < 0.001] and indeterminate status cases (OR 1.11, 95% CI 1.04-1.18; P < 0.001) vs. no DD. Among impaired LVEF cases, the equivalent risk of cardiovascular-related mortality was 1.51 (95% CI 1.15-1.98, P < 0.001) for increased filling pressure vs. 1.25 (95% CI 0.96-1.64, P = 0.06) for indeterminate status. Mitral E velocity, septal e' velocity, E:e' ratio, and LAVi all correlated with mortality. On adjusted basis, pivot-points of increased risk for cardiovascular-related mortality occurred at 90 cm/s for E wave velocity, 9 cm/s for septal e' velocity, an E:e' ratio of 9, and an LAVi of 32 mL/m2., Conclusion: ASE/EACVI-classified DD is correlated with increased mortality. However, many cases remain 'indeterminate'. Importantly, when analysed individually, mitral E velocity, septal e' velocity, E:e' ratio, and LAVi revealed clear pivot-points of increased risk of cardiovascular-related mortality., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Sex-specific differences in left ventricular ejection fraction (LVEF) and responses to neurohormonal modulating therapies are relevant to clinical trials of treatment for heart failure with preserved ejection fraction (HFpEF)., Aims: This study aimed to identify the proportion and characteristics of patients presenting with possible or confirmed HFpEF within the National Echo Database of Australia., Results: A total of 237 046 women (48.1%) and 256 019 men (aged 61.0 ± 18.3 vs. 60.6 ± 17.1 years, respectively) had sex-specific distributions of LVEF: 94.3% of women had LVEF ≥ 45% (mean LVEF 66.0 ± 8.6%), compared with 87.2% of men (mean LVEF 63.4 ± 8.7%). The presence of structural heart disease (SHD) according to the PARAGON-HF criteria could be calculated in 93.8% of women and 93.4% of men with an LVEF ≥ 45%. Of these, 64 502 (30.8%) women and 104 344 (50.0%) of men had left ventricular hypertrophy, and 78 948 (35.3%) and 95 846 (42.9%), respectively, had left atrial enlargement. As a result, the proportion of women vs. men fulfilling echocardiographic criteria for HFpEF was very different: 111 497 (53.2%) vs. 146 359 (70.1%). SHD markedly increased with age, associated with a greater increase in women than men. The same signal was observed in those referred for suspected or previously confirmed HFpEF., Conclusions: Double the number of men than women had LVEF < 45%, and the distribution of SHD had was highly sex specific. Left ventricular hypertrophy and left atrial enlargement were more common in men and becoming more frequent in women with advancing age. The echocardiographic SHD distribution was similar in those referred with suspected or confirmed HFpEF. The findings are relevant to sex-specific recruitment criteria for future clinical trials., (© 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

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 <50%. An LVEF <40% was associated with the highest crude cardiovascular-related and all-cause mortality at 5 years (∼20-30% and ∼ 40-50%, respectively). Thereafter, actual cardiovascular-related and all-cause mortality at 5 years in both sexes steeply improved to a nadir LVEF of 65.0-69.9% (reference group). Below this LVEF level, the adjusted hazard ratio (HR) for cardiovascular-related mortality for a LVEF of 55.0-59.9% was 1.36 [95% confidence interval (CI) 1.16-1.59; P < 0.001] in women and 1.21 (95% CI 1.05-1.39; P = 0.008) in men. In women, an LVEF of 60.0-64.9% was also associated with a HR 1.33 (95% CI 1.16-1.52; P < 0.001) for cardiovascular-related mortality. These associations were most striking in women and men aged <65 years and were replicated in those with suspected heart failure (32 403 cases aged 65.2 ± 16.1 years, 57.0% women). For pre-existing heart failure (33 738 cases aged 67.6 ± 16.9 years, 46.5% women), the specific threshold of increased mortality was at and below 50.0-54.9%., Conclusions: Among patients investigated for suspected or established cardiovascular disease, we found clinically relevant sex-based differences in the distribution and mortality associated with an LVEF <65.0-69.9%. Specifically, they suggest a greater risk of mortality at higher LVEF levels among women., (© 2020 European Society of Cardiology.)

Background: 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., Methods: 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., Results: 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]; p<0.001) and increased pulmonary vascular resistance (RR 0.96 [95%CI 0.95-0.98]; p<0.001) were less likely to be associated with monotherapy., Conclusions: 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., (Crown Copyright © 2020. Published by Elsevier B.V. All rights reserved.)

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., (© 2020 Asian Pacific Society of Respirology.)

Pulmonary vascular resistance (PVR) >3 Wood units is a criterion of the haemodynamic definition of pulmonary arterial hypertension (PAH). However, this cut-off is conservative and arbitrarily defined. Data is lacking on the natural history, response to therapy and survival of patients diagnosed with precapillary pulmonary hypertension (PH) with mild or borderline elevation of PVR.In Australia, PAH therapy could be prescribed solely on mean pulmonary arterial pressure (PAP) and pulmonary arterial wedge pressure (PAWP) criteria. Using the Australian and New Zealand Pulmonary Hypertension Registry, we aimed to study a population diagnosed with PAH between January 2004 and December 2017 with the pre-defined haemodynamic characteristics of mean PAP ≥25 mmHg, PAWP ≤15 mmHg and PVR <3 Wood units.Eighty-two patients met the pre-defined haemodynamic inclusion criteria (mean age 63±11 years; 67 females). Underlying aetiologies included idiopathic disease (n=39), connective tissue disease (CTD; n=42) and HIV infection (n=1). At diagnosis, mean PAP was 27 mmHg (interquartile range (IQR) 25-30 mmHg), PAWP 13 mmHg (IQR 11-14 mmHg) and PVR 2.2 Wood units (IQR 1.9-2.7 Wood units). Baseline 6-min walk distance (6MWD) was 352 m (IQR 280-416 m) and 77% of subjects were in New York Heart Association (NYHA) functional class 3 or 4. All patients were commenced on initial monotherapy with an endothelin receptor antagonist (ERA; n=66) or phosphodiesterase type-5 inhibitor (PDE5i; n=16). At first re-evaluation, 6MWD increased by 46 m (IQR 7-96 m) and 35% of subjects demonstrated improvement in NYHA functional class. After a median follow-up of 65 months (IQR 32-101 months), 18 out of 82 subjects (22.0%) had died, with estimated 1-year and 5-year survival rates of 98% and 84%, respectively. Death attributed to PAH occurred in six out of these 18 patients (33.3%, 7% of total cohort).Patients with precapillary PH and "borderline" PVR falling outside the current definition have adverse outcomes. Such patients appear to respond to PAH therapy; however, this requires further study in randomised trials., Competing Interests: Conflict of interest: J. Anderson reports research support from GlaxoSmithKline and travel grants from Actelion and Bayer. Conflict of interest: G. Strange reports grants from Actelion, Bayer, Pfizer Pharmaceuticals and GlaxoSmithKline, and personal fees from Actelion, Arena Pharmaceuticals, Bayer and GlaxoSmithKline, outside the submitted work. Conflict of interest: C. Corrigan has nothing to disclose. Conflict of interest: N. Collins reports fees for lectures from Actelion, outside the submitted work. Conflict of interest: D.S. Celermajer has nothing to disclose. Conflict of interest: N. Dwyer has participated in advisory board work for Actelion, GlaxoSmithKline and Bayer, outside the submitted work. Conflict of interest: J. Feenstra has nothing to disclose. Conflict of interest: D. Keating reports personal fees for lectures from GlaxoSmithKline, Actelion and Novartis, outside the submitted work. Conflict of interest: E. Kotlyar reports personal fees for lectures from Actelion, Bayer, Novartis, Servier and GlaxoSmithKline, as well as advisory board work for GlaxoSmithKline, Actelion and Bayer, outside the submitted work. Conflict of interest: M. Lavender has been consultant to and received grants from GlaxoSmithKline, Actelion and Bayer, outside the submitted work. Conflict of interest: H. Whitford reports fees for lectures, advisory board work and travel grants from Actelion, GlaxoSmithKline and Bayer, outside the submitted work. Conflict of interest: K. Whyte has nothing to disclose. Conflict of interest: T. Williams reports advisory board work for Actelion, GlaxoSmithKline and Bayer, as well as grants from Actelion, outside the submitted work. Conflict of interest: J.P. Wrobel reports fees for lectures, advisory board work and travel grants from Actelion, Boehringer Ingelheim, GlaxoSmithKline and Roche, outside the submitted work. Conflict of interest: A. Keogh reports advisory board work for Actelion, GlaxoSmithKline and Bayer, as well as grants from Actelion, Bayer, Pfizer, Gilead, Arena, Respira, Acceleron, Pfizer and Bellerophon, outside the submitted work. Conflict of interest: E.M. Lau reports speaking and consultancy fees from Actelion and Menarini Pharmaceuticals, as well as research support from Actelion and GlaxoSmithKline. Conflict of interest: S. Ratwatte has nothing to disclose., (Copyright ©ERS 2020.)

Background: There is a paucity of data describing the day-to-day experiences of adult Australians personally living with or caring for a child born with congenital heart disease (CHD). Such data would be of great practical importance to inform health care initiatives to improve outcomes., Methods: 588 men (38.3 ± 11.9 years) and women (39.6 ± 12.6 years, 78% of respondent patients) living with CHD and 1,091 adult carers (93% mothers) of children with CHD (median age 7.3 [IQR 3.5-13.3 years], 54% male), representing all Australian states and territories, responded to a comprehensive online survey designed and hosted by the Congenital Heart Alliance of Australia and New Zealand. Data on demographic factors, the nature of underlying CHD, interactions with health care services, psychological wellbeing and wider impacts of CHD were collected., Results: Most respondents were able to identify the type of CHD they (29% with a simple lesion such atrial septal defect, 17% tetralogy of Fallot) or their child had (21% with a simple lesion, 15% tetralogy of Fallot), whilst 90% cases of CHD had undergone cardiac surgery. Patients with CHD were mostly employed (70%) or studying (8.8%), whilst 9.1% were receiving disability benefits. In terms of transition care, 52% of adult patients had been referred by a paediatric to adult cardiologist with 84% still actively managed by a specialist. Overall, 31% of patients with CHD sought emergency care and required >10 days sick leave in the past 12 months. Moreover, 71% and 55% of patients, respectively, reported recent feelings of anxiety/worry or depressive thoughts related to their CHD (61% sought professional assistance). Consistent with high levels of disruption to daily living, 59% of carer respondents (24%>10 days) had taken carer's leave in the past 12 months., Conclusions: These contemporary, self-reported, Australian data reveal the burden of living and caring for CHD from an adult's perspective. Survey respondents highlighted the potential disconnect between paediatric and adult CHD services and suggest an important, unmet need for dedicated health services/community care to cost-effectively manage high levels of health care utilisation coupled with associated psychological distress., (Copyright © 2019 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Background: Pulmonary arterial hypertension (PAH) prognosis has improved with targeted therapies; however, the long-term outlook remains poor. Objective multiparametric risk assessment is recommended to identify patients at risk of early morbidity and mortality, and for optimization of treatment. The US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk score is a new model proposed for the follow-up of patients with PAH but has not been externally validated., Methods: The REVEAL 2.0 risk score was applied to a mixed prevalent and incident cohort of patients with PAH (n = 1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier survival was estimated for each REVEAL 2.0 risk score strata and for a simplified three-category (low, intermediate, and high risk) model. Sensitivity analysis was performed on an incident-only cohort., Results: The REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ Registry cohort, with a C statistic of 0.74 (both for full eight-tier and three-category models). When applied to incident cases only, the C statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12- and 60-month survival estimates (all risk category comparisons P < .001). Although the full eight-tier REVEAL 2.0 model separated patients at low, intermediate, and high risk, survival estimates overlapped within some of the intermediate- and high-risk strata., Conclusions: The REVEAL 2.0 risk score was validated in a large external cohort from the PHSANZ Registry. The REVEAL 2.0 model can be applied for risk assessment of patients with PAH at follow-up. The simplified three-category model may be preferred for clinical use and for future comparison with other prognostic models., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

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 < 0.001 for all comparisons). The 5-year mortality was 56% and 67%, respectively, in those with moderate AS (mean gradient 20.0 to 39.0 mm Hg/peak velocity 3.0 to 3.9 m/s) and severe AS (≥40.0 mm Hg, ≥4.0 m/s, or AV area <1.0 cm 2 in low-flow, low-gradient severe AS). A markedly increased risk of death from all causes (5-year mortality >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)., (Copyright © 2019 American College of Cardiology Foundation. All rights reserved.)

Background: There is increasing evidence that current thresholds for diagnosing pulmonary hypertension (PHT) underestimate the prognostic impact of PHT., Objectives: 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)., Methods: 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)., Results: 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., Conclusions: 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 (eRVSP >30.0 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)., (Copyright © 2019 American College of Cardiology Foundation. All rights reserved.)

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., (Copyright © 2018 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Background: The management of children with congenital heart disease (CHD) has improved over recent decades and several patients surviving with CHD into adulthood are increasing. In developed countries, there are now as many adults as there are children living with CHD. Pulmonary arterial hypertension (PAH) occurs in ∼ 5% of patients with CHD., Aim: We aimed to understand the characteristics and outcomes of this emerging population., Methods: We collected data retrospectively and prospectively from 12 contributing centres across Australia and New Zealand (2010-2013). Patients were included if they had been diagnosed with PAH and CHD and had been seen once in an adult centre after 1 January 2000., Results: Of 360 patients with CHD-PAH, 60% were female and 90% were New York Heart Association functional class II or III at the time of adult diagnosis of PAH. Mean age at diagnosis of PAH in adulthood was 31.2 ± 14 years, and on average, patients were diagnosed with PAH 6 years after symptom onset. All-cause mortality was 12% at 5 years, 21% at 10 years and 31% at 15 years. One hundred and six patients (30%) experienced 247 hospitalisations during 2936 patient years of follow up. Eighty-nine per cent of patients were prescribed PAH specific therapy (mean exposure of 4.0 years)., Conclusions: Adults with PAH and CHD often have this diagnosis made after significant delay, and have substantial medium-term morbidity and mortality. This suggests a need for children transitioning to adult care with CHD to be closely monitored for this complication., (© 2015 Royal Australasian College of Physicians.)