Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977–2012

Hjortshøj, Cristel Sørensen; Jensen, Annette Schophuus; Sørensen, Kasper; Nagy, Edit; Johansson, Bengt; Kronvall, Thomas; Dellborg, Mikael; Estensen, Mette E; Holmstrøm, Henrik; Turanlahti, Maila; Thilén, Ulf; Søndergaard, Lars

doi:10.1136/heartjnl-2016-310979

Cited by 26 publications

(18 citation statements)

References 29 publications

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“…This was a retrospective, descriptive, multicentre study of patients transplanted because of ES. From the population-based Nordic Eisenmenger database, data on all transplantations in patients with ES were available 16. ES was defined as pulmonary vascular disease in the presence of a large non-restrictive shunt accompanied by cyanosis 1 16–19.…”

Section: Methodsmentioning

confidence: 99%

“…From the population-based Nordic Eisenmenger database, data on all transplantations in patients with ES were available 16. ES was defined as pulmonary vascular disease in the presence of a large non-restrictive shunt accompanied by cyanosis 1 16–19. The patients were divided into simple pre-tricuspid (atrial septal defects (ASD)), simple post-tricuspid (ventricular septal defects (VSD), patent ductus arteriosus (PDA) and aortopulmonary window) and complex lesions including ASD, univentricular hearts, transposition of the great arteries and truncus arteriosus.…”

Section: Methodsmentioning

confidence: 99%

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Outcome after heart–lung or lung transplantation in patients with Eisenmenger syndrome

Hjortshøj

Gilljam

Dellgren³

et al. 2019

Heart

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ObjectiveThe optimal timing for transplantation is unclear in patients with Eisenmenger syndrome (ES). We investigated post-transplantation survival and transplantation-specific morbidity after heart–lung transplantation (HLTx) or lung transplantation (LTx) in a cohort of Nordic patients with ES to aid decision-making for scheduling transplantation.MethodsWe performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012.ResultsAmong 714 patients with ES in the Nordic region, 63 (9%) underwent transplantation. The median age at transplantation was 31.9 (IQR 21.1–42.3) years. Within 30 days after transplantation, seven patients (11%) died. The median survival was 12.0 (95% CI 7.6 to 16.4) years and the overall 1-year, 5-year, 10-year and 15-year survival rates were 84.1%, 69.7%, 55.8% and 40.6%, respectively. For patients alive 1 year post-transplantation, the median conditional survival was 14.8 years (95% CI 8.0 to 21.8), with 5-year, 10-year and 15-year survival rates of 83.3%, 67.2% and 50.0%, respectively. There was no difference in median survival after HLTx (n=57) and LTx (n=6) (14.9 vs 10.6 years, p=0.718). Median cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis/kidney transplantation-free survival rates were 11.2 (95% CI 7.8 to 14.6), 6.9 (95% CI 2.6 to 11.1) and 11.2 (95% CI 8.8 to 13.7) years, respectively. The leading causes of death after the perioperative period were infection (36.7%), bronchiolitis obliterans syndrome (23.3%) and heart failure (13.3%).ConclusionsThis study shows that satisfactory post-transplantation survival, comparable with contemporary HTx and LTx data, without severe comorbidities such as cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis, is achievable in patients with ES, with a conditional survival of nearly 15 years.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Outcome after heart–lung or lung transplantation in patients with Eisenmenger syndrome

Hjortshøj

Gilljam

Dellgren³

et al. 2019

Heart

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“…Indeed, Hjortshøj et al 9 report a high mortality in childhood cases of ES, while survival is better in adults, although far from optimal, with a morality rate of approximately 12% per decade.…”

Section: Management Of Patients With Significant Shunts and Esmentioning

confidence: 99%

Declining incidence and prevalence of Eisenmenger syndrome in the developed world: a triumph of modern medicine

Kempny

Dimopoulos

2017

Heart

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“…2 Furthermore, PAH-targeted therapies prolong ES survival, and these patients reach elderhood and present with more complicated conditions. [3][4][5] Due to reverse shunt from pulmonary to systemic circulation through defects at cardiac or great arterial level, ES presents with cyanosis and secondary erythrocytosis. 6 This condition could cause multiple organ dysfunction, including chronic heart failure (HF) -diastolic and systolic left (not only right -sub-pulmonary) ventricular dysfunction.…”

Section: Introductionmentioning

confidence: 99%

Insights to correlations and discrepancies between impaired lung function and heart failure in Eisenmenger patients

et al. 2020

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Impaired lung function and spirometric signs of airway obstruction without common risk factors for chronic obstructive pulmonary disease could be found in patients with Eisenmenger syndrome. This study aimed to analyse the association between lung function parameters and disease severity (including heart failure markers, associated congenital heart defect) as well as the possible reasons for airflow obstruction in Eisenmenger syndrome. The data of 25 patients with Eisenmenger syndrome were retrospectively evaluated. The patients were divided into groups according to airflow obstruction and a type of congenital heart defect. Airflow obstruction was found in nearly third (32%) of our cases and was associated with older age and worse survival. No relation was found between airway obstruction, B-type natriuretic peptide level, complexity of congenital heart defect and bronchial compression. Most of the patients (88%) had gas diffusion abnormalities. A weak negative correlation was noticed between gas diffusion (diffusing capacity of the lung for carbon monoxide) and B-type natriuretic peptide level (r = −0.437, p = 0.033). Increased residual volume was associated with higher mortality (p = 0.047 and p = 0.021, respectively). A link between B-type natriuretic peptide and lung diffusion, but not airway obstruction, was found. Further research and larger multicentre studies are needed to evaluate the importance of pulmonary function parameters and mechanisms of airflow obstruction in Eisenmenger syndrome.

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Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977–2012

Cited by 26 publications

References 29 publications

Outcome after heart–lung or lung transplantation in patients with Eisenmenger syndrome

Outcome after heart–lung or lung transplantation in patients with Eisenmenger syndrome

Declining incidence and prevalence of Eisenmenger syndrome in the developed world: a triumph of modern medicine

Insights to correlations and discrepancies between impaired lung function and heart failure in Eisenmenger patients

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