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- Hjortshøj, Cristel M Sørensen, et al.
(author)
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Past and current cause-specific mortality in Eisenmenger syndrome.
- 2017
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In: European Heart Journal. - : Oxford University Press (OUP). - 0195-668X .- 1522-9645. ; 38:26, s. 2060-2067
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Journal article (peer-reviewed)abstract
- Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015.Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001).Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.
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| 2. |
- Hjortshøj, Cristel Sørensen, et al.
(author)
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Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012
- 2017
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In: Heart. - : BMJ Publishing Group Ltd. - 1355-6037 .- 1468-201X. ; 103:17, s. 1353-1358
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Journal article (peer-reviewed)abstract
- OBJECTIVE: Improved diagnostic tools, timely closure of the shunt and a better understanding of the complexity of Eisenmenger syndrome (ES) have led to improved care and treatment in tertiary centres. These may have decreased the incidence of ES and improved survival of patients with ES, although evidence is still lacking. The aim of this study was to investigate temporal changes in incidence, prevalence and mortality in patients with ES for 35 years in the Nordic region.METHODS: This was a retrospective population-based study including 714 patients with ES. Survival analysis was performed based on all-cause mortality and accounting for immortal time bias.RESULTS: The incidence of ES decreased from 2.5/million inhabitants/year in 1977 to 0.2/million inhabitants/year in 2012. Correspondingly, prevalence decreased from 24.6 to 11.9/million inhabitants. The median survival was 38.4 years, with 20-year, 40-year and 60-year survival of 72.5%, 48.4%, and 21.3%, respectively. Complex lesions and Down syndrome were independently associated with worse survival (HR 2.2, p<0.001 and HR 1.8, p<0.001, respectively). Age at death increased from 27.7 years in the period from 1977 to 1992, to 46.3 years from July 2006 to 2012 (p<0.001).CONCLUSIONS: The incidence and prevalence of ES in the Nordic region have decreased markedly during the last decades. Furthermore, the median age at death increased throughout the study period, indicating prolonged life expectancy in the ES population. However, increasing age represents decreased incidence, rather than improved survival. Nonetheless, longevity with ES is still shorter than in the background population.
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| 3. |
- Hebert, Anders, et al.
(author)
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Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation: The TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) Study.
- 2014
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In: Circulation. - 1524-4539. ; 130:23, s. 2021-2030
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Journal article (peer-reviewed)abstract
- The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients.
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| 4. |
- Hebert, Anders, et al.
(author)
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Hemodynamic causes of exercise intolerance in Fontan patients.
- 2014
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In: International Journal of Cardiology. - : Elsevier BV. - 0167-5273. ; 175:3, s. 478-483
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Journal article (peer-reviewed)abstract
- Exercise intolerance is frequent among Fontan patients and an important determinant for quality of life. This study investigated the hemodynamic causes of impaired exercise capacity in Fontan patients with particular focus on the influence of stroke volume index (SVI) and heart rate (HR).
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| 5. |
- Rysgaard, Søren, et al.
(author)
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A mobile observatory powered by sun and wind for near real time measurements of atmospheric, glacial, terrestrial, limnic and coastal oceanic conditions in remote off-grid areas
- 2022
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In: HardwareX. - : Elsevier BV. - 2468-0672. ; 12
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Journal article (peer-reviewed)abstract
- Climate change is rapidly altering the Arctic environment. Although long-term environmental observations have been made at a few locations in the Arctic, the incomplete coverage from ground stations is a main limitation to observations in these remote areas. Here we present a wind and sun powered multi-purpose mobile observatory (ARC-MO) that enables near real time measurements of air, ice, land, rivers, and marine parameters in remote off-grid areas. Two test units were constructed and placed in Northeast Greenland where they have collected data from cabled and wireless instruments deployed in the environment since late summer 2021. The two units can communicate locally via WiFi (units placed 25 km apart) and transmit near-real time data globally over satellite. Data are streamed live and accessible from (https://gios.org). The cost of one mobile observatory unit is c. 304.000€. These test units demonstrate the possibility for integrative and automated environmental data collection in remote coastal areas and could serve as models for a proposed global observatory system.
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| 6. |
- Tarp, Julie Bjerre, et al.
(author)
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Vascular function in adults with cyanotic congenital heart disease
- 2020
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In: IJC Heart and Vasculature. - : Elsevier BV. - 2352-9067. ; 30
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Journal article (peer-reviewed)abstract
- Background: Patients with cyanotic congenital heart disease (CCHD) may have a low burden of atherosclerosis. Endothelial dysfunction is an early stage of atherosclerosis and endothelial function is previously studied in smaller CCHD groups with different techniques and variable results. We aimed to examine endothelial function and carotid atherosclerosis in a larger group of CCHD patients. Methods: This multicentre study assessed endothelial function in adults with CCHD and controls by measuring the dilatory response of the brachial artery to post-ischemic hyperaemia (endothelium-dependent flow-mediated-vasodilatation (FMD)), and to nitroglycerin (endothelium-independent nitroglycerin-induced dilatation (NID)). Flow was measured at baseline and after ischaemia (reactive hyperaemia). Carotid-intima-media-thickness (CIMT), prevalence of carotid plaque and plaque thickness (cPT-max) were evaluated ultrasonographically. Lipoproteins, inflammatory and vascular markers, including sphingosine-1-phosphate (S1P) were measured. Results: Forty-five patients with CCHD (median age 50 years) and 45 matched controls (median age 52 years) were included. The patients presented with lower reactive hyperaemia (409 ± 114% vs. 611 ± 248%, p < 0.0001), however preserved FMD response compared to controls (106.5 ± 8.3% vs. 106.4 ± 6.1%, p = 0.95). In contrast, NID was lower in the patients (110.5 ± 6.1% vs. 115.1 ± 7.4%, p = 0.053). There was no difference in CIMT, carotid plaque or cPT-max. The patients presented with lower high-density-lipoprotein cholesterol, and higher level of inflammatory markers and S1P. Conclusion: Adults with CCHD had preserved FMD in the brachial artery, but impaired NID response and lower reactive hyperaemia than controls. The preserved FMD and the comparable prevalence of carotid atherosclerosis indicate that CCHD patients have the same risk of atherosclerosis as controls.
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