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Past and current cause-specific mortality in Eisenmenger syndrome

Hjortshoj, Cristel M.Sorensen; Kempny, Aleksander; Jensen, Annette Schophuus; Sorensen, Keld; Nagy, Edit; Dellborg, Mikael; Johansson, Bengt; Rudiene, Virginija; Hong, Gu and Opotowsky, Alexander R., et al. (2017) In European Heart Journal 38(26). p.2060-2067
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... (More)

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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publication status
published
subject
keywords
Cause of death, Eisenmenger syndrome, Heart failure, Mortality, Pulmonary arterial hypertension
in
European Heart Journal
volume
38
issue
26
pages
8 pages
publisher
Oxford University Press
external identifiers
  • scopus:85026506781
  • wos:000404987200011
ISSN
0195-668X
DOI
10.1093/eurheartj/ehx201
language
English
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yes
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ab4431c6-f896-44c3-9f5d-c1c8f1ce1e10
date added to LUP
2017-08-25 13:42:09
date last changed
2017-09-18 11:43:10
@article{ab4431c6-f896-44c3-9f5d-c1c8f1ce1e10,
  abstract     = {<p>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 &lt; 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.</p>},
  author       = {Hjortshoj, Cristel M.Sorensen and Kempny, Aleksander and Jensen, Annette Schophuus and Sorensen, Keld and Nagy, Edit and Dellborg, Mikael and Johansson, Bengt and Rudiene, Virginija and Hong, Gu and Opotowsky, Alexander R. and Budts, Werner and Mulder, Barbara J. and Tomkiewicz-Pająk, Lidia and D'Alto, Michele and Prokselj, Katja and Diller, Gerhard Paul and Dimopoulos, Konstantinos and Estensen, Mette Elise and Holmstrom, Henrik and Turanlahti, Maila and Thilén, Ulf and Gatzoulis, Michael A. and Sondergaard, And Lars},
  issn         = {0195-668X},
  keyword      = {Cause of death,Eisenmenger syndrome,Heart failure,Mortality,Pulmonary arterial hypertension},
  language     = {eng},
  month        = {07},
  number       = {26},
  pages        = {2060--2067},
  publisher    = {Oxford University Press},
  series       = {European Heart Journal},
  title        = {Past and current cause-specific mortality in Eisenmenger syndrome},
  url          = {http://dx.doi.org/10.1093/eurheartj/ehx201},
  volume       = {38},
  year         = {2017},
}