Article Text

Download PDFPDF
Original research article
Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977–2012
  1. Cristel Sørensen Hjortshøj1,
  2. Annette Schophuus Jensen1,
  3. Keld Sørensen2,
  4. Edit Nagy3,
  5. Bengt Johansson4,
  6. Thomas Kronvall5,
  7. Mikael Dellborg6,
  8. Mette-Elise Estensen7,
  9. Henrik Holmstrøm8,
  10. Maila Turanlahti9,
  11. Ulf Thilén10,
  12. Lars Søndergaard1
  1. 1 Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
  2. 2 Department of Internal Medicine, Aalborg University Hospital, Farsoe, Denmark
  3. 3 Department of Cardiology, Karolinska University Hospital, Stockholm, Sweden
  4. 4 Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden
  5. 5 Department of Cardiology, Örebro University Hospital, Örebro, Sweden
  6. 6 Department of Cardiology, Sahlgrenska University Hospital, Göteborg, Sweden
  7. 7 Department of Cardiology, Rikshospitalet, Oslo, Norway
  8. 8 Department of Paediatric Cardiology, Rikshospitalet, Oslo, Norway
  9. 9 Department of Pediatric Cardiology, Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland
  10. 10 Department of Cardiology, Lund University Hospital, Lund, Sweden
  1. Correspondence to Dr Cristel Sørensen Hjortshøj, Department of Cardiology 2013, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark; Cristel.maria.soerensen.hjortshoej{at}


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.

  • Eisenmenger syndrome
  • pulmonary arterial hypertension
  • adult congenital heart disease
  • epidemiology
  • survival

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Contributors CSH, ASJ and LS designed the study and are responsible for the overall content. CSH, KS, EN, BJ, TK, MD, ME, HH, MT and UT collected the data. CSH analysed data and wrote the manuscript. ASJ, KS, EN, BJ, TK, MD, ME, HH, MT, UT and LS critically reviewed the manuscript and provided scientific input.

  • Funding This study was financed by an educational grant from Actelion Denmark, a branch of Actelion Pharmaceuticals in Sweden.

  • Disclaimer Actelion Pharmaceuticals was not involved in the writing or editing of the report or analysis of the data.

  • Competing interests CSH received an educational grant from Actelion Pharmaceuticals. ASJ received a research grant and speaker’s fees from Actelion Pharmaceuticals. BJ received a speaker fee from Actelion Pharmaceuticals. UT received fees for lectures and being a member of advisory board from Actelion Pharmaceuticals. LS received research grant, as well as fee for lectures and being a member of advisory board from Actelion Pharmaceuticals.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Linked Articles