Cristel M Sørensen Hjortshøj1, Aleksander Kempny2, Annette Schophuus Jensen1, Keld Sørensen3, Edit Nagy4,5, Mikael Dellborg6, Bengt Johansson7, Virginija Rudiene8, Gu Hong9, Alexander R Opotowsky10, Werner Budts11, Barbara J Mulder12, Lidia Tomkiewicz-Pajak13, Michele D'Alto14, Katja Prokšelj15, Gerhard-Paul Diller2, Konstantinos Dimopoulos2, Mette-Elise Estensen16, Henrik Holmstrøm17, Maila Turanlahti18, Ulf Thilén19, Michael A Gatzoulis2, Lars Søndergaard1. 1. Department of Cardiology, Rigshospitalet, Copenhagen, Denmark. 2. Biomedical Research Unit, Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Royal Brompton Hospital, Imperial College London, London, UK. 3. Department of Internal Medicine, Aalborg University Hospital, Farsoe, Denmark. 4. Department of Medicine, Karolinska Institutet, Stockholm, Sweden. 5. Department of Cardiology, Karolinska University Hospital, Stockholm, Sweden. 6. Department of Cardiology, Sahlgrenska Academy, University of Göteborg, Sweden. 7. Department of Cardiology, Norrland University Hospital, Umeå, Sweden. 8. Department of Cardiology, Vilnius University Hospital, Vilnius, Lithuania. 9. Department of Paediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China. 10. Boston Adult Congenital Heart (BACH), Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA. 11. Department of Cardiology, University Hospitals Leuven, Belgium. 12. Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands. 13. Department of Cardiac and Vascular Diseases, Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland. 14. Department of Cardiology, Second University of Naples, Italy. 15. Department of Cardiology, University Medical Center Ljubljana, Slovenia. 16. Department of Cardiology, Rikshospitalet, Oslo, Norway. 17. Department of Paediatric Cardiology, Rikshospitalet, Oslo, Norway. 18. Pediatric Cardiology, Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland. 19. Department of Cardiology, Lund University Hospital, Lund, Sweden.
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. Published on behalf of the European Society of Cardiology. All rights reserved.
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. Published on behalf of the European Society of Cardiology. All rights reserved.
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