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Heart Resaearch Insittue
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Biomedical Engineering Not Elsewhere Classified | Biomechanics | Human Movement and Sports Science | Cardiology (Incl. Cardiovascular Diseases)
Cardiovascular system and diseases | Medical instrumentation | Occupational health (excl. economic development aspects) |
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 07-2021
Publisher: Elsevier BV
Date: 10-2018
DOI: 10.1016/J.AHJ.2018.07.001
Abstract: 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 in iduals (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.
Publisher: BMJ
Date: 03-07-2012
Publisher: Elsevier BV
Date: 05-2021
Publisher: MDPI AG
Date: 29-09-2021
DOI: 10.3390/JCM10194503
Abstract: There is a paucity of epidemiologic data examining the relationship between pulmonary hypertension (PH) and diabetes. The aim of this study was to determine prevalence, incidence and associates of PH complicating type 2 diabetes. Data from 1430 participants (mean age 65.5 years, 51.5% males) in the Fremantle Diabetes Study Phase 2 (FDS2) were linked with the National Echocardiographic Database of Australia (NEDA) to ascertain the prevalence and incidence of PH (estimated right ventricular systolic pressure (eRVSP) mmHg as a new suggested threshold or the conventional mmHg) over a 12-year period. PH prevalence in FDS2 was compared with that in NEDA overall and a geographically close sub-population. Multivariable analyses identified associates of prevalent/incident PH in the FDS2 cohort. Of 275 FDS2 patients (19.2%) with pre-entry echocardiography, 90 had eRVSP mmHg and 35 had eRVSP mmHg (prevalences 32.7% (95% CI 27.3–38.7%) and 12.7% (9.1–17.4%), respectively), rates that are 35–50% greater than national/local NEDA general population estimates. Moreover, 70 (5.0%) and 123 (9.2%) FDS2 participants were identified with incident PH at the respective eRVSP thresholds (incidence (95% CI) 7.6 (6.0–9.7) and 14.2 (11.8–17.0)/1000 person-years), paralleling data from recognised high-risk conditions such as systemic sclerosis. The baseline plasma N-terminal pro-brain natriuretic peptide concentration was the strongest independent associate of prevalent/incident PH. Approximately 1 in 8 people with type 2 diabetes have PH using the eRVSP mmHg threshold. Its presence should be considered as part of regular clinical assessment of in iduals with type 2 diabetes.
Publisher: Elsevier BV
Date: 11-2017
DOI: 10.1016/J.HLC.2017.08.018
Abstract: 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. Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry. Between January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57.2±18.7years, 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.
Publisher: Wiley
Date: 10-2009
DOI: 10.1111/J.1445-5994.2008.01823.X
Abstract: We sought to determine the prevalence of pulmonary complications and especially pulmonary arterial hypertension (PAH) in an Australian scleroderma population. Between July 2005 and June 2007, physicians in Western Australia were asked to refer patients with scleroderma specifically for pulmonary hypertension screening. All patients were assessed for PAH and other respiratory conditions using echocardiography, lung function testing and clinical assessments. Right heart catheterization was carried out in patients with evidence of increased right ventricular systolic pressure. Of the 184 patients analysed, 44 had possible PAH on echocardiography. Right heart catheterization confirmed the diagnosis in 24 (13%). Diffuse interstitial lung disease was found in 32 patients representing a point prevalence of 17.4%. The severity of PAH at diagnosis varied according to whether the patients were referred for screening (group A) or for diagnostic (group B) purposes. The 6-min-walk test distance and median pulmonary vascular resistance were significantly worse in group B versus group A (324 vs 402 m P= 0.02 and 884 dynes/s per cm(-5) vs 486 dynes/s per cm(-5) P < 0.01, respectively). Screening may result in earlier diagnosis of PAH with, in general more mild disease. This is important, given that early treatment for PAH while patients are less symptomatic is associated with improved exercise tolerance and pulmonary haemodynamics: indices indicative of disease progression and clinical worsening.
Publisher: Public Library of Science (PLoS)
Date: 11-07-2022
DOI: 10.1371/JOURNAL.PONE.0268580
Abstract: 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. 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). 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 in iduals with moderate AS.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 07-09-2021
Abstract: 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. Using the National Echo Database of Australia, patients in whom BAV was identified were studied. Those with moderate‐to‐severe AS (mean gradient 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 .001). Patients with BAV‐AS were younger (aged 55.3±16.7 years versus 77.3±11.0 years P .001), and where measured had larger ascending aortic diameters (37±8 mm versus 35±5 mm P .001). Age and sex‐adjusted mortality risk was significantly lower in patients with BAV‐AS (hazard ratio, 0.53 95% CI, 0.45–0.63 P .001). 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. URL: www.anzctr.org.au/ Unique identifier: ACTRN12617001387314.
Publisher: Elsevier BV
Date: 03-2018
DOI: 10.1016/J.HLC.2017.09.015
Abstract: Pulmonary hypertension (PH) is common, under diagnosed and associated with a high mortality. There are significant delays in the diagnosis of pulmonary hypertension leading to increased morbidity and delays in the initiation of treatment. Once PH is diagnosed, establishing the degree of pulmonary vascular resistance (PVR) enables clinicians to broadly ide the underlying pathology into pre-capillary or post-capillary causes, a crucial step in tailoring management. Pulmonary hypertension is most commonly due to left heart disease (PH-LHD) and echocardiography (echo) is the most widely accessible investigation in its diagnosis. Regardless of the underlying pathophysiology of LHD, the sequelae lead to pressure overload on the left heart and a reactive increase in pulmonary pressures. In this review article, we will discuss the prevalence of PH, examine the pathophysiology of PH-LHD, establish how echo can be used to identify patients with PH-LHD and discuss surrogate echo markers of PVR.
Publisher: BMJ
Date: 03-04-2023
DOI: 10.1136/HEARTJNL-2022-322184
Abstract: The significance of pulmonary hypertension (PHT) complicating aortic stenosis (AS) is poorly characterised. In a large cohort of adults with at least moderate AS, we aimed to describe the prevalence and prognostic importance of PHT in such patients. In this retrospective study, we analysed the National Echocardiography Database of Australia (data from 2000 to 2019). Adults with an estimated right ventricular systolic pressure (eRVSP), left ventricular ejection fraction (LVEF) % and with moderate or greater AS were included (n=14 980). These subjects were then categorised according to their eRVSP. The relationship between PHT severity and mortality outcomes were evaluated (median follow-up of 2.6 years, IQR 1.0–4.6 years). Subjects were aged 77±13 years and 57.4% were female. Overall, 2049 (13.7%), 5085 (33.9%), 4380 (29.3%), 1956 (13.1%) and 1510 (10.1%) patients had no (eRVSP .00 mm Hg), borderline (30.00–39.99 mm Hg), mild (40.00–49.99 mm Hg), moderate (50.00–59.99 mm Hg) and severe PHT ( .00 mm Hg), respectively. An echocardiographic phenotype was evident with worsening PHT, showing rising E:e’ ratio and right and left atrial sizes(p .0001, for all). Adjusted analyses showed that the risk of long-term mortality progressively rose as eRVSP level increased (HR 1.14–2.94, borderline to severe PHT, p .0001 for all). A mortality threshold was identified in the 4th decile of eRVSP categories (35.01–38.00 mm Hg HR 1.19, 95% CI 1.04 to 1.35), with risk progressively increasing through to the 10th decile (HR 2.86, 95% CI 2.54 to 3.21). In this large cohort study, we find that PHT is common in ≥moderate AS and mortality increases as PHT becomes more severe. A threshold for higher mortality lies within the range of ‘borderline-mild’ PHT. ACTRN12617001387314.
Publisher: Informa UK Limited
Date: 28-11-2014
DOI: 10.1586/17434440.2015.985651
Abstract: Tissue engineers have been seeking the 'Holy Grail' solution to calcification and cytotoxicity of implanted tissue for decades. Tissues with all of the desired qualities for surgical repair of congenital heart disease (CHD) are lacking. An anti-calcification tissue engineering process (ADAPT TEP) has been developed and applied to bovine pericardium (BP) tissue (CardioCel, AdmedusRegen Pty Ltd, Perth, WA, Australia) to eliminate cytotoxicity, improve resistance to acute and chronic inflammation, reduce calcification and facilitate controlled tissue remodeling. Clinical data in pediatric patients, and additional pre-market authorized prescriber data demonstrate that CardioCel performs extremely well in the short term and is safe and effective for a range of congenital heart deformations. These data are supported by animal studies which have shown no more than normal physiologic levels of calcification, with good durability, biocompatibility and controlled healing.
Publisher: BMJ
Date: 02-2023
DOI: 10.1136/OPENHRT-2022-002180
Abstract: Atrial functional mitral regurgitation (AFMR) is characterised by left atrial and consequent mitral annular dilatation causing mitral regurgitation. AFMR is likely to become more common with population ageing, alongside increases in atrial fibrillation and heart failure with preserved ejection fraction conditions causing atrial dilatation. Here, we aim to define the prevalence and characterise the patient and survival characteristics of AFMR in the National Echocardiographic Database of Australia (NEDA). 14 004 adults with moderate or severe FMR were identified from NEDA. AFMR or ventricular FMR (VFMR) was classified by LA size, LV size and LVEF. AFMR was found in 40% (n=5562) and VFMR in 60% (n=8442). Compared with VFMR, the AFMR subgroup were significantly older (mean age 78±11 years), with a higher proportion of females and of AF. Participants were followed up for a median of 65 months (IQR 36–116 months). After adjustment for age, sex, AF, and pulmonary hypertension, the prognosis for VFMR was significantly worse than for AFMR (HR 1.57, 95% CI 1.47 to 1.68 for all-cause and 1.73, 95% CI 1.60 to 1.88, p .001 for both). After further adjustment for LVEF, mortality rates were similar in VFMR and AFMR patients (HR 0.93, p=NS), though advancing age and pulmonary hypertension remained independently associated with prognosis. AFMR is a common cause of significant functional MR that predominantly affects elderly female patients with AF. Advancing age and pulmonary hypertension independently associated with survival in FMR. Prognosis was better in AFMR compared with VFMR however, this difference was accounted for by LV systolic impairment and not by MR severity.
Publisher: BMJ
Date: 03-04-2023
DOI: 10.1136/HEARTJNL-2022-322187
Abstract: Aortic regurgitation (AR) can lead to pulmonary hypertension (PHT). There is a paucity of data on the prognostic importance of PHT in these patients. We therefore aimed to describe the prevalence and prognostic importance of PHT in such patients. In this retrospective study, we analysed the National Echocardiography Database of Australia (data from 2000 to 2019). Adults with an estimated right ventricular systolic pressure (eRVSP), left ventricular ejection fraction (LVEF) % and with moderate or greater AR were included (n=8392). These subjects were then categorised according to their eRVSP. The relationship between PHT severity and mortality outcomes were evaluated (median follow-up of 3.1 years, IQR 1.5–5.7 years). Subjects were aged 74±14 years and 58.4% (4901) were female. Overall, 1417 (16.9%) had no PHT, and 3253 (38.8%), 2249 (26.9%), 893 (10.6%) and 580 (6.9%) patients had borderline, mild, moderate and severe PHT, respectively. Mean eRVSP was slightly higher in females than males (41±13 vs 39±12 mm Hg, p .0001) and increased with age in both sexes. After adjustment for age and sex, the risk of long-term mortality increased as eRVSP increased (adjusted HR (aHR) 1.20, 95% CI 1.06 to 1.36 in borderline PHT, to aHR 3.32, 95% CI 2.85 to 3.86 in severe PHT, p .0001). There was a mortality threshold seen from mild PHT onwards (eRVSP 41.36–44.15 mm Hg aHR 1.41, 95%CI 1.17 to 1.68). In this large cohort study, we characterise the relationship between AR and PHT in adults. In patients with ≥moderate AR, PHT is associated with a progressive risk of mortality, even at mildly elevated levels.
Publisher: Elsevier BV
Date: 04-2020
Publisher: Wiley
Date: 09-2015
DOI: 10.1111/IMJ.12821
Abstract: 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. We aimed to understand the characteristics and outcomes of this emerging population. 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. 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). 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.
Publisher: Informa UK Limited
Date: 06-2011
DOI: 10.1586/ERP.11.26
Abstract: In this article, we review randomized controlled trials, open-label trials and pharmacoeconomic models of bosentan for the management of patients with pulmonary arterial hypertension. Bosentan consistently improves WHO functional class and quality of life, slows clinical worsening and is associated with improved survival compared with historical treatment. Although head-to-head trials are scarce, data directly comparing bosentan with sildenafil indicate no clinically significant differences between treatments as measured by the 6-min walk distance alone. Compared with historical care, bosentan treatment, over a 15-30-year period, increases the number of quality-adjusted life years (3.49 years). Economic modeling suggests that the cost-effectiveness of bosentan is similar to that of ambrisentan (US$43,725-57,778 per quality-adjusted life year), not as cost effective as sildenafil (at 20 mg three-times daily) and more cost effective than iloprost. More randomized controlled trials of longer duration are required to confirm the results from these economic models.
Publisher: Elsevier BV
Date: 06-2019
DOI: 10.1016/J.JACC.2019.03.482
Abstract: 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 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).
Publisher: Wiley
Date: 07-2008
DOI: 10.1111/J.1440-1843.2008.01326.X
Abstract: Bosentan, an oral, dual endothelin receptor antagonist, significantly improves functional status, haemodynamic measures and survival in patients with pulmonary arterial hypertension (PAH). However, there are limited data on the effect of bosentan on quality of life (QOL) and its relationship to changes in functional status, as measured by the 6 minute walk distance (6MWD). A retrospective analysis was performed of a large, open-label, multicentre trial (VITAL) of bosentan in patients with PAH. Data for 6MWD were collected at baseline, 3 or 6 months and these results were correlated with QOL measurements collected as part of the assessment of patients enrolled in the trial. Sixty-nine patients with PAH (mean age 52 years) who were enrolled in the trial had valid QOL (SF-36) measurements and 6MWD data that could be retrieved from clinical notes. At 3 and 6 months, bosentan therapy improved 6MWD compared with baseline (49.5 m and 47.2 m, respectively, P < 0.001) as well as QOL domains, with a significant correlation between these two markers on cross-sectional analysis. However, there was a poor relationship when comparing changes in 6MWD with changes in QOL, in response to therapy. Bosentan therapy was associated with improvements in QOL and 6MWD for at least 6 months. At all measured time points, there was a close correlation between 6MWD and most QOL domains. QOL is an important parameter and should be considered as part of the standard assessment for any trial investigating therapy in PAH.
Publisher: Wiley
Date: 03-2011
DOI: 10.1111/J.1445-5994.2009.02139.X
Abstract: The Bosentan Patient Registry (BPR) was a prospective, multicentre, Australian registry funded by Actelion Pharmaceuticals. The primary aim of the registry was to collect survival data in patients with pulmonary arterial hypertension (PAH) treated with bosentan. The BPR was initiated in 15 specialized PAH centres. All patients on or starting bosentan were invited to enrol. Treating physicians notified the registry if patients discontinued bosentan, because of either a change in therapy, transplantation, intervention or death. Survival data were validated against the Australian Institute of Health and Welfare National Death Index. Between 2004 and 2007, a total of 528 patients (mean age 59 ± 17 years) were enrolled representing 69% of patients either previously taking or initiated on bosentan during that time. The BPR population was generally older with more advanced functional deficit than patients enrolled in randomized, placebo-controlled trials. Aetiology was idiopathic (iPAH) in 58% and connective tissue disease related (scleroderma (SSc)-PAH) in 42%. For iPAH patients, World Health Organisation functional classes II, III and IV at enrolment was 8.2%, 66.4% and 20.5%, and for the SSc-PAH cohort, 3.2%, 75.8% and 17.9% respectively. The observed annual mortality was 11.8% in patients with iPAH and 16.6% in patients SSc-PAH. This large Australian registry provides 'real life' information on the characteristics and management of PAH in clinical practice. Treatment with bosentan improved survival outcomes in both iPAH and SSc-PAH compared with historical controls. Age, disease severity and aetiology were critical factors in determining clinical outcomes.
Publisher: Elsevier BV
Date: 09-2020
Publisher: Wiley
Date: 30-01-2020
DOI: 10.1111/RESP.13768
Abstract: 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. 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. 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. 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.
Publisher: Informa UK Limited
Date: 05-2009
DOI: 10.2147/JMDH.S3085
Publisher: European Respiratory Society (ERS)
Date: 27-04-2020
DOI: 10.1183/13993003.01654-2019
Abstract: Pulmonary vascular resistance (PVR) 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 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.
Publisher: Elsevier BV
Date: 02-2020
Publisher: Wiley
Date: 13-08-2009
Publisher: BMJ
Date: 2022
DOI: 10.1136/OPENHRT-2021-001743
Abstract: Non-rheumatic aortic stenosis (AS) is among the most common valvular diseases in the developed world. Current guidelines support aortic valve replacement (AVR) for severe symptomatic AS, which carries high morbidity and mortality when left untreated. In contrast, moderate AS has historically been thought to be a benign diagnosis for which the potential benefits of AVR are outweighed by the procedural risks. However, emerging data demonstrating the substantial mortality risk in untreated moderate AS and substantial improvements in periprocedural and perioperative mortality with AVR have challenged the traditional risk/benefit paradigm. As such, an appraisal of the contemporary data on morbidity and mortality associated with moderate AS and appropriate timing of valvular intervention in AS is warranted. In this review, we discuss the current understanding of moderate AS, including the epidemiology, current surveillance and management guidelines, clinical outcomes, and future studies.
Publisher: Elsevier BV
Date: 02-2010
DOI: 10.1016/J.HEALUN.2009.09.020
Abstract: Bosentan has an established role in the management of pulmonary arterial hypertension (PAH). This clinical trial assessed the benefits of bosentan in the Chinese population. We investigated the efficacy and safety of bosentan in 92 Chinese citizens (mean +/- standard deviation age, 29.0 +/- 3.8 years) with PAH for a minimum of 12 weeks. All received bosentan (62.5 mg twice daily) for 4 weeks then, patients who weighed 40 kg received 125 mg twice daily. All patients were eligible to continue bosentan beyond 12 weeks. The primary end point was a change in exercise capacity from baseline to 12 and 24 weeks. Secondary end points included a change in World Health Organization (WHO) functional class and changes in cardiopulmonary hemodynamics. At baseline, 66 patients (72%) were in WHO functional class III presentation was 37 (40%) with idiopathic PAH (iPAH), 34 (37%) with PAH related to congenital heart disease (CHD), and 21 (23%) with PAH related to connective tissue disease (CTD). Exercise capacity increased to 67.8 m after 12 weeks and 92.6 m after 24 weeks (p < 0.001). After 24 weeks, WHO functional class decreased (-0.8 +/- 0.6 p < 0.001), mean pulmonary artery pressure and pulmonary vascular resistance decreased (p < 0.01), and cardiac output increased (p < 0.001). Twelve patients (13%) experienced at least 1 adverse event. Bosentan improved exercise capacity, functional class, and cardiopulmonary hemodynamics in this patient cohort and was well tolerated.
Publisher: Elsevier BV
Date: 02-2020
DOI: 10.1016/J.HLC.2018.12.009
Abstract: 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. 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. 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. 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.
Publisher: Elsevier BV
Date: 02-2008
DOI: 10.1016/J.IJCARD.2006.12.045
Abstract: Although pulmonary arterial hypertension (PAH) is widely accepted as deadly, if not a rare disease, its prognostic impact beyond reports from specialist centres is unknown. Using the unique Scottish Morbidity Record Scheme and linked survival data, we tracked the survival of all Scottish adults aged < or =65 years admitted for the first time during the period of 1986 to 2001 with a probable diagnosis of Idiopathic PAH and a PAH related to connective tissue disorders (Connective PAH) and congenital abnormalities (Congenital PAH) - the three most common forms of PAH. Overall, 374 Scottish men and women were discharged from the hospital with incident PAH during the period 1986 to 2001. On an unadjusted basis, Congenital PAH (40-45%) was associated with the lowest case fatality at 5 years in both men and women. In both sexes, Idiopathic PAH and Connective PAH were associated with high initial one-year case fatality (20-30%) with a steady accumulation of fatal events in the four years thereafter (60-75% case fatality at 5 years). Overall, the adjusted risk of dying within one year in the period 1986 to 1989 was 2.22-fold greater (OR 95% CI, 1.27 to 3.85) than in 1998 to 2001 (P<0.001). The greatest falls in one year case fatality were seen in those with Connective PAH (18-fold increased risk of dying in 1986 to 1989 versus 1998 to 2001: P=0.013). Similarly, women (adjusted OR 1.38, 95% CI 1.16 to 1.63: P<0.001) and the most deprived in iduals (OR 2.38, 95% CI 1.17 to 4.82: P<0.05) were at greater risk of dying within 5 years. Alternatively, those patients discharged in 1997 were less likely to die during this period compared to their 1986 counterparts, although this difference did not quite reach statistical significance (OR 0.45, 95% CI 0.22 to 1.06: P=0.056). This population-based study has confirmed the deadly impact of the three most common forms of PAH. Overall, there are encouraging trends in relation to one and five year adjusted survival rates particularly in relation to PAH related to connective tissue disorders.
Publisher: Elsevier BV
Date: 10-2020
Publisher: Wiley
Date: 22-12-2021
DOI: 10.1002/EHF2.13149
Abstract: 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). This study aimed to identify the proportion and characteristics of patients presenting with possible or confirmed HFpEF within the National Echo Database of Australia. 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. 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.
Publisher: BMJ
Date: 2022
DOI: 10.1136/OPENHRT-2021-001783
Abstract: To estimate the population prevalence and treatable burden of severe aortic stenosis (AS) in the UK. We adapted a contemporary model of the population profile of symptomatic and asymptomatic severe AS in Europe and North America to estimate the number of people aged ≥55 years in the UK who might benefit from surgical aortic valve replacement (SAVR) or transcatheter aortic valve implantation (TAVI). With a point prevalence of 1.48%, we estimate that 291 448 men and women aged ≥55 years in the UK had severe AS in 2019. Of these, 68.3% (199 059, 95% CI 1 77 201 to 221 355 people) would have been symptomatic and, therefore, more readily treated according to their surgical risk profile the remaining 31.7% of cases (92 389, 95% CI 70 093 to 144 247) being asymptomatic. Based on historical patterns of intervention, 58.4% (116 251, 95% CI 106 895 to 1 25 606) of the 199 059 symptomatic cases would qualify for SAVR, with 7208 (95% CI 7091 to 7234) being assessed as being in a high, preoperative surgical risk category. Among the remaining 41.6% (82 809, 95% CI 73 453 to 92 164) of cases potentially unsuitable for SAVR, an estimated 61.7% (51 093, 95% CI 34 780 to 67 655) might be suitable for TAVI. We estimate that 172 859 out of 291 448 prevalent cases of severe AS (59.3%) will subsequently die within 5 years without proactive management. These data suggest a high burden of severe AS in the UK requiring surgical or transcatheter intervention that challenges the ongoing capacity of the National Health Service to meet the needs of those affected.
Publisher: Elsevier BV
Date: 04-2019
DOI: 10.1016/J.HLC.2018.10.015
Abstract: 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. 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. 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. 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.
Publisher: Wiley
Date: 03-2011
DOI: 10.1111/J.1445-5994.2010.02403.X
Abstract: Several cellular pathways are implicated in the pathogenesis of pulmonary arterial hypertension (PAH) and attempts to arrest disease progression with a single drug would not be expected to succeed in the medium term. In clinical practice, combination therapy is often used in patients deteriorating on monotherapy, despite the absence of firm evidence from randomized controlled controls. From January 2005 to August 2009, 112 patients with World Health Organisation Functional Class (FC) II-IV PAH deteriorating on monotherapy received non-parenteral combination therapy at six Australian PAH expert hospitals. Combination therapy included bosentan, sitaxentan, ambrisentan, iloprost and sildenafil. Data were prospectively collected for survival status, 6-min walk distance, FC and echocardiographic parameters at the start of monotherapy through to commencement of combination therapy and at 6-monthly intervals thereafter. After varying periods of monotherapy (18.7±13.4onths), survival estimates on combination therapy were 88%, 71% and 61% for the additional 1, 2 and 3years respectively. Survival on dual therapy in patients with idiopathic PAH/familial PAH was 93% at 1year and 79% at 2years, and for scleroderma-related PAH, 72% at 1 year and 48% at year 2 after initiation of combination therapy. In survivors, dual therapy reversed the deterioration in FC, from 3.1±0.6 on monotherapy to 2.2±0.6 at 12months. Similarly, dual therapy improved 6-min walk distance from 316±119m to 406±129m at 12months, and sequential echocardiography demonstrated a fall in pulmonary artery systolic pressure and improved right ventricular function. Dual non-parenteral therapy appears safe and effective and should be considered for PAH patients who are deteriorating on monotherapy to improve long-term outcomes.
Publisher: Cold Spring Harbor Laboratory
Date: 12-03-2023
DOI: 10.1101/2023.03.08.23287015
Abstract: Evidence of improved risk assessment in aortic stenosis (AS) by using energy-loss index (ELI) instead of aortic valve area indexed to body surface area (AVAi) is scarce, and positive results have been driven by aortic valve replacement. We aimed to evaluate the prognostic performance of ELI and AVAi in a head-to-head comparison using large-scale, real-world data. In the multi-center, mortality-data linked National Echocardiography Database of Australia (NEDA), patients with AS and requisite ascending aortic area measurements were identified. The prognostic value of AVAi and ELI, respectively, was analyzed using Cox regression and the C statistic. In patients with mild AS (n=3,179), moderate AS (n=4,194), and severe AS (n=3,120), there were 4,229 deaths of which 2,359 were reported as cardiovascular deaths (median [interquartile range] follow-up 2.5 [1.1–4.5] years]. Decreasing AVAi was associated with increased cardiovascular mortality (hazard ratio [95% confidence interval] 1.18 [1.16– 1.20] per 0.1 cm 2 /m 2 downward increment]. Prognostic performance for 5-year mortality did not improve by using ELI instead of AVAi (identical C statistics 0.626 [0.612–0.640]), and the relative performance did not change when analyzing 1-year cardiovascular mortality, or all-cause mortality. ELI was not associated with improved prognostic performance compared to AVAi in echocardiographic assessment of AS using large-scale, real-world clinical data. AVAi remains a relevant measure for risk prediction in AS, providing information on incremental risk with decreasing area.
Publisher: Wiley
Date: 04-2021
DOI: 10.1002/EJHF.2161
Abstract: We investigated long‐term mortality associated with changes in left ventricular ejection fraction (LVEF) in a large, real‐world patient cohort. 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 units to units) the adjusted risk of cardiovascular‐related mortality increased two‐ to eightfold beyond a ‐unit decline in LVEF (vs. minimal change P 0.001 for all comparisons). Among those initially investigated for HF (32% with initial LVEF %), the adjusted hazard ratio for cardiovascular‐related mortality ranged from 0.35 [95% confidence interval (CI) 0.28–0.49] to 4.21 (95% CI 3.30–5.22) for a ‐unit increase to ‐unit decline in LVEF (vs. minimal change P 0.001 for both comparisons). A distinctive, bi‐directional plateau of improved vs. worsening mortality was evident around a final LVEF of 50% to 55%. These data, derived from a large, heterogeneous cohort of adults being followed up with echocardiography, suggest that modest LVEF changes (particularly around an LVEF of 50–55%) may be of clinical significance.
Publisher: Wiley
Date: 26-11-2021
DOI: 10.1002/EJHF.2047
Abstract: 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. 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 %. An LVEF % 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 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%. 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 .0–69.9%. Specifically, they suggest a greater risk of mortality at higher LVEF levels among women.
Publisher: Elsevier BV
Date: 10-2016
DOI: 10.1016/J.JTCVS.2016.05.024
Abstract: The purpose of this study was to provide a preliminary assessment of the performance of a decellularized pericardial patch in a trileaflet aortic valve reconstruction in a long-term juvenile sheep model. A sheep surgical model was used to perform a complete trileaflet reconstruction (Ozaki technique) of the aortic valve with 3 separate pericardial patches. Valve function was assessed 1 week, 3 months, and 6 months after surgery via transthoracic echocardiography. Calcification resistance and host cell infiltration of the pericardial material were assessed at 6 months after surgery. Three of 6 sheep with implanted pericardial neo-cusps survived until the planned time of sacrifice after surgery, whereas 3 animals had a successful implant but died shortly after the procedure as the result of a bad recovery from cardiopulmonary bypass. Echocardiography at 6 months revealed a high coaptation area with only minimal regurgitation. In all explanted leaflets, cusp tissue was soft. There was only minimal calcification in 8 of 9 leaflets. Aortic valves reconstructed with a decellularized pericardial patch demonstrated adequate diastolic function with minimal regurgitation and resistance to calcification. Combining the Ozaki technique with this decellularized pericardial scaffold showed adequate hemodynamics, sustained mechanical integrity of the patch and limited calcification of the material. These results, together with earlier experimental and clinical data, indicate the potential of this material for aortic valve repair.
Publisher: Oxford University Press (OUP)
Date: 06-02-2022
Abstract: The interplay between aortic stenosis (AS), cardiovascular events, and mortality is poorly understood. In addition, how echocardiographic indices compare for predicting outcomes remains unexplored for the full range of AS severity. We prospectively calculated peak jet velocity (Vmax) and aortic valve area (AVA) in 5994 adult subjects with and without AS. We linked ultrasound data to 5-year mortality and clinical events obtained from electronic medical records. Proportional-hazard and negative binomial regression models were adjusted for relevant covariables such as age, sex, comorbidities, stroke-volume, LV ejection fraction, left valve regurgitation, aortic valve sclerosis or calcification, and valve replacement. We observed a strong linear relationship between Vmax and all-cause mortality (hazard ratio: 1.26, 95% confidence interval: 1.19–1.33 per 100 cm/s), cardiovascular events, as well as incidental and recurrent heart failure (HF). Adjusted risks were highly significant even at Vmax values in the range of 150–200 cm/s, risk curves separating very early after the index exam. Vmax was not associated with coronary, arrhythmic, cerebrovascular, or non-cardiovascular events. Although risks were confirmed when AVA was entered in place of Vmax, the risks estimated for categories based on the two indices were mismatched, even in patients with normal flow. An external cohort comprising 112 690 patients confirmed augmented risks of all-cause and cardiovascular mortality starting at values of Vmax and AVA in the range of mild AS. Aortic stenosis is strongly associated to all-cause mortality, cardiovascular mortality, and cardiac events, specifically HF. Risks increase in parallel to the degree of outflow obstruction but are apparent very early in patients with mild disease. Criteria for grading AS based on Vmax and AVA are mismatched in terms of outcomes.
Publisher: Elsevier BV
Date: 02-2021
Publisher: SAGE Publications
Date: 2011
DOI: 10.4137/CMT.S2689
Abstract: Pulmonary arterial hypertension (PAH) is defined as a group of diseases characterized by a progressive increase in pulmonary vascular resistance (PVR) leading to right ventricular failure and premature death. Untreated, it is a potentially devastating disease. However, the past decade has seen remarkable improvements in our understanding of the pathology associated with the condition and the development of multiple PAH-specific therapies with the ability to alter the natural history of the disease. These new advances provide a significant opportunity for practitioners to detect and treat patients with PAH in a timely and effective manner, thereby improving overall mortality, morbidity, and quality of life associated with this disease. The aim of this review is two-fold: firstly to review the evidence for efficacy and safety of non-parenteral PAH therapies and to discuss treatment selection based on clinically meaningful differences among the approved therapies, such as the potential for serious drug-drug interactions, convenience of dosing schedules, and rates of limiting side effects. Secondly, the central role of the PAH clinical nurse in the multidisciplinary care of patients with PAH will be discussed, together with issues relating to adherence and interventions to enhance patient compliance.
Publisher: Springer Science and Business Media LLC
Date: 22-02-2010
Abstract: Pulmonary arterial hypertension (PAH) has witnessed dramatic treatment advances over the past decade. However, with the exception of epoprostenol, data from short-term randomized controlled trials (RCTs) have not shown a benefit of these drugs on survival. There remains a need to differentiate between available therapies and current endpoint responses which in turn, could be used to guide treatment selection and provide long-term prognostic information for patients. We performed a systematic literature search of MEDLINE and EMBASE databases for RCTs of PAH-specific therapy published between January 1980 and May 2009. Articles were selected if they contained a placebo comparator and described hemodynamic changes from baseline. We applied the weighted mean change in hemodynamic variables to the equation developed by the National Institutes of Health (NIH) Registry to estimate long-term survival with each therapy. Ten RCTs involving 1,635 patients met the inclusion criteria. Suitable hemodynamic data were identified for bosentan, sitaxentan, sildenafil, epoprostenol, beraprost and treprostinil. 77.6% of patients were female and the mean (SD) age was 46.5 ± 4.9 years. 55.5% of patients had idiopathic PAH (iPAH), 23.9% PAH related to connective tissue disease, and 18.2% PAH related to congenital heart disease. Based on the effects observed in short-term trials and, relative to placebo, all analyzed therapies improved survival. The estimated 1-year survival was 78.4%, 77.8%, 76.1%, 75.8%, 75.2%, and 74.1% for epoprostenol, bosentan, treprostinil, sitaxentan, sildenafil, and beraprost, respectively. These estimates are considerably lower than the 1-year observed survival reported in several open-label and registry studies with PAH-specific therapies: 88% - 97%. When applied to the NIH Registry equation, hemodynamic changes from baseline appear to underestimate the survival benefits observed with long-term PAH therapy.
Publisher: Oxford University Press (OUP)
Date: 03-11-2020
Abstract: To examine the characteristics rognostic impact of diastolic dysfunction (DD) according to 2016 American Society of Echocardiography (ASE) and European Society of Cardiovascular Imaging (ESCVI) guidelines, and in idual parameters of DD. 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. ASE/EACVI-classified DD is correlated with increased mortality. However, many cases remain ‘indeterminate’. Importantly, when analysed in idually, mitral E velocity, septal e’ velocity, E:e’ ratio, and LAVi revealed clear pivot-points of increased risk of cardiovascular-related mortality.
Publisher: Elsevier BV
Date: 10-2019
DOI: 10.1016/J.JACC.2019.08.004
Abstract: Historical data suggesting poor survival in patients with aortic stenosis (AS) who do not undergo treatment are largely confined to patients with severe AS. This study sought to determine the prognostic impact of all levels of native valvular AS. 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. 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 eak 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 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).
Publisher: European Respiratory Society (ERS)
Date: 06-07-2023
DOI: 10.1183/23120541.00082-2023
Abstract: We addressed the paucity of data describing the characteristics and natural history of incident pulmonary hypertension (PHT). Adults (n=13 448) undergoing routine echocardiography without initial evidence of PHT (estimated right ventricular systolic pressure, eRVSP .0 mmHg) or left heart disease (LHD) were studied. Incident PHT (eRVSP ≥30.0 mmHg) was detected on repeat echocardiogram a median of 4.1 years apart. Mortality was examined according to increasing eRVSP levels (30.0–39.9, 40.0–49.9 and ≥50.0 mmHg) indicative of mild-to-severe PHT. A total of 6169 men (45.9%, aged 61.4±16.7 years) and 7279 women (60.8±16.9 years) without evidence of PHT were identified (first echo). Subsequently, 5412 (40.2%) developed evidence of PHT, comprising 4125 (30.7%), 928 (6.9%), and 359 (2.7%) cases with an eRVSP of 30.0–39.9 mmHg, 40.0–49.9 mmHg, and ≥50.0 mmHg, respectively (incidence being 94.0 and 90.9 cases per 1000 men and women/year). Median eRVSP increased by +0.0 (IQR −2.27 to +2.67) mmHg and +30.68 (+26.03 to +37.31) mmHg among those with eRVSP .0 mmHg versus ≥50.0 mmHg. During median 8.1 years follow-up, 2776 (20.6%) died from all-causes. Compared to those with eRVSP .0 mmHg, the adjusted risk of all-cause mortality was 1.30-fold higher in 30.0–39.9 mmHg, 1.82-fold higher in 40.0–49.9 mmHg and 2.11-fold higher in ≥50.0 mmHg groups (all p .001 ). New onset PHT, as indicated by elevated eRVSP, is a common finding among older patients without LHD followed-up with echocardiography. This phenomenon is associated with an increased morality risk even among those with mildly elevated eRVSP.
Publisher: BMJ
Date: 07-2023
DOI: 10.1136/OPENHRT-2023-002265
Abstract: We developed an artificial intelligence decision support algorithm (AI-DSA) that uses routine echocardiographic measurements to identify severe aortic stenosis (AS) phenotypes associated with high mortality. 631 824 in iduals with 1.08 million echocardiograms were randomly spilt into two groups. Data from 442 276 in iduals (70%) entered a Mixture Density Network (MDN) model to train an AI-DSA to predict an aortic valve area cm 2 , excluding all left ventricular outflow tract velocity or dimension measurements and then using the remainder of echocardiographic measurement data. The optimal probability threshold for severe AS detection was identified at the f1 score probability of 0.235. An automated feature also ensured detection of guideline-defined severe AS. The AI-DSA’s performance was independently evaluated in 184 301 (30%) in iduals. The area under receiver operating characteristic curve for the AI-DSA to detect severe AS was 0.986 (95% CI 0.985 to 0.987) with 4622/88 199 (5.2%) in iduals (79.0±11.9 years, 52.4% women) categorised as ‘high-probability’ severe AS. Of these, 3566 (77.2%) met guideline-defined severe AS. Compared with the AI-derived low-probability AS group (19.2% mortality), the age-adjusted and sex-adjusted OR for actual 5-year mortality was 2.41 (95% CI 2.13 to 2.73) in the high probability AS group (67.9% mortality)—5-year mortality being slightly higher in those with guideline-defined severe AS (69.1% vs 64.4% age-adjusted and sex-adjusted OR 1.26 (95% CI 1.04 to 1.53), p=0.021). An AI-DSA can identify the echocardiographic measurement characteristics of AS associated with poor survival (with not all cases guideline defined). Deployment of this tool in routine clinical practice could improve expedited identification of severe AS cases and more timely referral for therapy.
Publisher: Elsevier BV
Date: 08-2016
Publisher: Springer Science and Business Media LLC
Date: 16-01-2020
Publisher: Oxford University Press (OUP)
Date: 06-07-2013
DOI: 10.1093/ICVTS/IVT268
Publisher: Cold Spring Harbor Laboratory
Date: 22-09-2020
DOI: 10.1101/2020.09.19.20197988
Abstract: The Top End of Australia has a high proportion of Indigenous people with a high burden of chronic cardiac and pulmonary diseases likely to contribute to pulmonary hypertension (PH). The epidemiology of PH has not been previously studied in this region. Patients with PH were identified from the Northern Territory echocardiography database from January 2010 to December 2015 and followed to the end of 2019 or death. PH was defined as a tricuspid regurgitation velocity ≥2.75 m/s measured by Doppler echocardiography. The etiology of PH, as categorized by published guidelines, was determined by reviewing electronic health records. 1764 patients were identified comprising 49% males and 45% Indigenous people. The prevalence of PH was 955 per 100,000 population (with corresponding prevalence of 1587 for Indigenous people). Hypertension, atrial fibrillation, diabetes and respiratory disease were present in 85%, 45%, 41% and 39%, respectively. Left heart disease was the leading cause for PH (58%), the majority suffering from valvular disease (predominantly rheumatic). Pulmonary arterial hypertension (PAH), respiratory disease related PH, chronic thromboembolic PH (CTEPH) and unclear multifactorial PH represented 4%, 16%, 2% and 3%, respectively. Underlying causes were not identifiable in 17% of the patients. Only 31% of potentially eligible patients were on PAH-specific therapy. At census, there was 40% mortality, with major predictors being age, ePASP and Indigenous ethnicity. PH is prevalent in Northern Australia, with a high frequency of modifiable risk factors and other treatable conditions. Whether earlier diagnosis, interpretation and intervention improves outcomes merits further assessment.
Publisher: Wiley
Date: 02-2014
DOI: 10.1111/IMJ.12321
Abstract: On 5 May 2013 it was World Pulmonary Hypertension (PHT) Day marking three decades on from the first reported deaths in an epidemic because of toxic rapeseed (canola) oil. This epidemic provided the impetus to the first World Health Organization to set up a world symposia. World leaders of PHT met for the fifth time in Nice, France in February 2013. Although we wait the official proceedings, this meeting provides us opportunity to reflect on the current situation in Australia and New Zealand, and examine the implications for our two countries. PHT remains difficult to identify, delays in patient diagnosis persist, and breathlessness remains dominant in the diagnosis of all causes of PHT. This review examines some of the recent changes in diagnosis, our understanding of the emerging expanding epidemiology data and the patient's journeys through the healthcare system. We also review the current treatment options on monotherapy and in poly-pharmacy or combination therapy, along with the strategic management implications of the lack of funded combination therapy associated with prognosis.
Publisher: European Respiratory Society (ERS)
Date: 04-05-2020
DOI: 10.1183/13993003.00008-2020
Abstract: Current risk stratification tools in pulmonary arterial hypertension (PAH) are limited in their discriminatory abilities, partly due to the assumption that prognostic clinical variables have an independent and linear relationship to clinical outcomes. We sought to demonstrate the utility of Bayesian network-based machine learning in enhancing the predictive ability of an existing state-of-the-art risk stratification tool, REVEAL 2.0. We derived a tree-augmented naïve Bayes model (titled PHORA) to predict 1-year survival in PAH patients included in the REVEAL registry, using the same variables and cut-points found in REVEAL 2.0. PHORA models were validated internally (within the REVEAL registry) and externally (in the COMPERA and PHSANZ registries). Patients were classified as low-, intermediate- and high-risk ( %, 5–20% and % 12-month mortality, respectively) based on the 2015 European Society of Cardiology/European Respiratory Society guidelines. PHORA had an area under the curve (AUC) of 0.80 for predicting 1-year survival, which was an improvement over REVEAL 2.0 (AUC 0.76). When validated in the COMPERA and PHSANZ registries, PHORA demonstrated an AUC of 0.74 and 0.80, respectively. 1-year survival rates predicted by PHORA were greater for patients with lower risk scores and poorer for those with higher risk scores (p .001), with excellent separation between low-, intermediate- and high-risk groups in all three registries. Our Bayesian network-derived risk prediction model, PHORA, demonstrated an improvement in discrimination over existing models. This is reflective of the ability of Bayesian network-based models to account for the interrelationships between clinical variables on outcome, and tolerance to missing data elements when calculating predictions.
Publisher: Elsevier BV
Date: 09-2021
Publisher: Wiley
Date: 2013
Abstract: Survival rates for patients with idiopathic pulmonary arterial hypertension (IPAH) have improved with the introduction of PAH‐specific therapies. However, the time between patient‐reported onset of symptoms and a definitive diagnosis of IPAH is consistently delayed. We conducted a retrospective, multi‐center, descriptive investigation in order to (a) understand what factors contribute to persistent diagnostic delays, and (b) examine the time from initial symptom onset to a definitive diagnosis of IPAH. Between January 2007 and December 2008, we enrolled consecutively diagnosed adults with IPAH from four tertiary referral centers in Australia. Screening of patient records and “one‐on‐one” interviews were used to determine the time from patient‐described initial symptoms to a diagnosis of IPAH, confirmed by right heart catheterization (RHC). Thirty‐two participants (69% female) were studied. Mean age at symptom onset was 56 ± 16.4 years and 96% reported exertional dyspnea. Mean time from symptom onset to diagnosis was 47 ± 34 months with patients subsequently aged 60 ± 17.3 years. Patients reported 5.3 ± 3.8 GP visits and 3.0 ± 2.1 specialist reviews before being seen at a pulmonary hypertension (PH) center. Advanced age, number of general practitioner (GP) visits, heart rate, and systolic blood pressure at the time of diagnosis were significantly associated with the observed delay. We found a significant delay of 3.9 years from symptom onset to a diagnosis of IPAH in Australia. Exertional dyspnea is the most common presenting symptom. Current practice within Australia does not appear to have the specific capacity for timely, multi‐factorial evaluation of breathlessness and potential IPAH.
Publisher: Wiley
Date: 08-2012
DOI: 10.1111/J.1445-5994.2011.02708.X
Abstract: Pulmonary arterial hypertension (PAH) frequently accompanies childhood congenital heart disease (CHD) and may persist into adult life. The advent of specific PAH therapies for PAH prompted formation of a national Australian and New Zealand registry in 2010 to document the incidence, demographics, presentation and outcomes for these patients. This multicentre, prospective, web-based registry enrols patients with CHD-associated PAH being followed in a tertiary centre. The inclusion criteria stipulated patient age ≥16 years, a measured mean pulmonary arterial pressure >25 mmHg at rest or echocardiographical evidence of PAH or a diagnosis of Eisenmenger syndrome, and followed since 1 January 2000. A single observer collected standardised data during a series of site visits. Of the first 50 patients enrolled, 30 (60%) were female. The mean age (standard deviation (SD)) at the time of PAH diagnosis or confirmation in an adult centre was 27.23 (10.07) years, and 32 (64%) patients are currently aged >30 years. Fourteen (28%) patients were in World Health Organization Functional Class II and 36 (72%) in Class III at the time of diagnosis. Forty-seven of 50 (94%) had congenital systemic-pulmonary shunts, and 36 (72%) never underwent intervention. Thirteen (26%) had Down syndrome. Confirmation of PAH by recent cardiac catheterisation was available in 30 (60%) subjects. During follow up, a total of 32 (64%) patients received a PAH-specific therapy. CHD associated with PAH in adult life has resulted in a new population with unique needs. This registry will allow documentation of clinical course and long-term outcomes for these patients.
Publisher: Elsevier BV
Date: 2020
DOI: 10.1016/J.CHEST.2019.08.2203
Abstract: 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. 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. 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. 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.
Publisher: Springer Science and Business Media LLC
Date: 11-08-2021
DOI: 10.1186/S12913-021-06843-0
Abstract: 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. 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. 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 in iduals (∼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 in iduals 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. These data suggest there is likely to be a substantive burden of in iduals 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.
Publisher: Oxford University Press (OUP)
Date: 17-08-2023
Publisher: BMJ
Date: 09-08-2018
DOI: 10.1136/HEARTJNL-2017-311876
Abstract: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively. The patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having ‘complex’ anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariabl e analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45 p .001). WHO ≥3 at presentation was associated with a worse prognosis (mortality HR 1.82, 95% CI 1.19 to 2.78 p=0.006). Treatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.
Publisher: Elsevier BV
Date: 11-2021
DOI: 10.1016/J.ECHO.2021.05.017
Abstract: There are currently no established prognostic models for "low-gradient" severe aortic stenosis (AS), including those with low-flow, low-gradient (LFLG) or normal-flow, low-gradient (NFLG) severe AS. The "cardiac damage staging classification" has been validated as a clinically useful prognostic tool for high-gradient severe AS but not yet for these other common subtypes of severe AS, LFLG and NFLG. The authors analyzed data from the National Echo Database of Australia, a large national, multicenter registry with in idual data linkage to mortality. Of 192,060 adults (mean age, 62.8 ± 17.8 years) with comprehensive ultrasound profiling of the native aortic valve studied between 2000 and 2019, 12,013 (6.3%) had severe AS. On the basis of standard echocardiographic parameters, 5,601 patients with high-gradient, 611 with classical and 959 with paradoxical LFLG, and 911 with NFLG severe AS were identified. Mean follow-up was 88 ± 45 months. All-cause and cardiovascular-related mortality were assessed for each group on an adjusted basis (age and sex) and analyzed by cardiac damage stage. Patients with LFLG AS had greater associated cardiac damage at diagnosis (stages 3 and 4 in 34% of those with classical LFLG, 22.5% of those with paradoxical LFLG, 15.5% of those with NFLG, and 14% of those with high-gradient AS P < .001). For all four major subtypes of severe AS, there was a progressive increase in 1- and 5-year mortality with increasing cardiac damage score. For ex le, for paradoxical LFLG severe AS, compared with stage 0 patients, adjusted 1-year all-cause mortality was 22% higher in stage 1 patients, 55% higher in stage 2 patients (P = .095), and 155% higher in stage 3 and 4 patients (P < .001). Among patients with classical LFLG severe AS, compared with stage 1 patients, adjusted 1-year all-cause mortality was 55% higher in stage 2 patients (P = .018) and 100% higher in stage 3 and 4 patients (P < .001). Regardless of severe AS subtype, increasing severity denoted by the cardiac damage staging classification is strongly associated with increasing mortality risk.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 16-11-2021
Abstract: 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. 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. 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.
Start Date: 12-2005
End Date: 06-2008
Amount: $130,000.00
Funder: Australian Research Council
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