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

Hjortshøj, Cristel Sørensen; Jensen, Annette Schophuus; Sørensen, Keld; Nagy, Edit LU ; Johansson, Bengt; Kronvall, Thomas; Dellborg, Mikael; Estensen, Mette Elise; Holmstrøm, Henrik and Turanlahti, Maila, et al. (2017) In Heart 103(17). p.1353-1358
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... (More)

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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publication status
published
subject
keywords
adult congenital heart disease, Eisenmenger syndrome, epidemiology, pulmonary arterial hypertension, survival
in
Heart
volume
103
issue
17
pages
6 pages
publisher
BMJ Publishing Group
external identifiers
  • scopus:85027546882
ISSN
1355-6037
DOI
10.1136/heartjnl-2016-310979
language
English
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no
id
d02cec5c-e46d-4896-841a-4bf8453914cf
date added to LUP
2017-09-04 16:52:19
date last changed
2017-09-05 03:00:03
@article{d02cec5c-e46d-4896-841a-4bf8453914cf,
  abstract     = {<p>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&lt;0.001 and HR 1.8, p&lt;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&lt;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.</p>},
  author       = {Hjortshøj, Cristel Sørensen and Jensen, Annette Schophuus and Sørensen, Keld and Nagy, Edit and Johansson, Bengt and Kronvall, Thomas and Dellborg, Mikael and Estensen, Mette Elise and Holmstrøm, Henrik and Turanlahti, Maila and Thilén, Ulf and Søndergaard, Lars},
  issn         = {1355-6037},
  keyword      = {adult congenital heart disease,Eisenmenger syndrome,epidemiology,pulmonary arterial hypertension,survival},
  language     = {eng},
  number       = {17},
  pages        = {1353--1358},
  publisher    = {BMJ Publishing Group},
  series       = {Heart},
  title        = {Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012},
  url          = {http://dx.doi.org/10.1136/heartjnl-2016-310979},
  volume       = {103},
  year         = {2017},
}