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

Hjortshøj, Cristel S. ; Gilljam, Thomas ; Dellgren, Göran ; Pentikäinen, Markku O. ; Möller, Thomas ; Jensen, Annette Schophuus ; Turanlahti, Maila ; Thilén, Ulf LU ; Gustafsson, Finn and Søndergaard, Lars (2019) In Heart
Abstract

Objective: The 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. Methods: We performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012. Results: Among 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... (More)

Objective: The 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. Methods: We performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012. Results: Among 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%). Conclusions: This 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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author
organization
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type
Contribution to journal
publication status
epub
subject
keywords
Eisenmenger syndrome, heart-lung transplantation, lung transplantation
in
Heart
publisher
BMJ Publishing Group
external identifiers
  • scopus:85071090588
ISSN
1355-6037
DOI
10.1136/heartjnl-2019-315345
language
English
LU publication?
yes
id
6df874dd-d16d-4df4-873b-4823d8dd11e8
date added to LUP
2019-09-12 11:58:11
date last changed
2019-11-20 05:52:02
@article{6df874dd-d16d-4df4-873b-4823d8dd11e8,
  abstract     = {<p>Objective: The 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. Methods: We performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012. Results: Among 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%). Conclusions: This 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.</p>},
  author       = {Hjortshøj, Cristel S. and Gilljam, Thomas and Dellgren, Göran and Pentikäinen, Markku O. and Möller, Thomas and Jensen, Annette Schophuus and Turanlahti, Maila and Thilén, Ulf and Gustafsson, Finn and Søndergaard, Lars},
  issn         = {1355-6037},
  language     = {eng},
  publisher    = {BMJ Publishing Group},
  series       = {Heart},
  title        = {Outcome after heart-lung or lung transplantation in patients with Eisenmenger syndrome},
  url          = {http://dx.doi.org/10.1136/heartjnl-2019-315345},
  doi          = {10.1136/heartjnl-2019-315345},
  year         = {2019},
}