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Congenital heart disease
Original research
Outcome after heart–lung or lung transplantation in patients with Eisenmenger syndrome
  1. Cristel S Hjortshøj1,
  2. Thomas Gilljam2,
  3. Göran Dellgren3,
  4. Markku O Pentikäinen4,
  5. Thomas Möller5,
  6. Annette Schophuus Jensen1,
  7. Maila Turanlahti4,
  8. Ulf Thilén6,
  9. Finn Gustafsson1,
  10. Lars Søndergaard1
  1. 1 Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
  2. 2 Department of Cardiology, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
  3. 3 Transplant Institute, Sahlgrenska Academy, University of Gothenburg, Gothenburg, UK
  4. 4 Department of Paediatric Cardiology, Heart and Lung Center, Helsinki University Hospital, University of Helsinki, Helsinki, Finland
  5. 5 Department of Paediatric Cardiology, Division of Paediatric and Adolescent Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway
  6. 6 Department of Cardiology, Lund University Hospital, Lund, Sweden
  1. Correspondence to Dr Cristel S Hjortshøj, Dept. of Cardiology, Rigshospitalet, Copenhagen DK-2100, Denmark; cristel.maria.soerensen.hjortshoej{at}regionh.dk

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 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.

  • Eisenmenger syndrome
  • lung transplantation
  • heart-lung transplantation

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/heartjnl-2019-315345

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    Footnotes

    • Contributors CSH, TG, MOP, TM, MT, UT and FG made substantial contributions to the conception or design of the work or the acquisition, analysis or interpretation of data. CSH drafted the work. TG, GD, MOP, ASJ, TM, MT, UT, FG and LS revised the work critically for important intellectual content. All authors contributed to the final approval of the version published.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests CSH received an educational grant from Actelion Pharmaceuticals. ASJ received a research grant and speaker’s fees from Actelion Pharmaceuticals. UT received fees for lectures and being member of advisory board from Actelion Pharmaceuticals. LS received research grant, as well as fee for lectures and being member of advisory board from Actelion Pharmaceuticals. TG, GD, MOP, TM, MT and FG reports no relationships that could be construed as a conflict of interest.

    • Patient consent for publication Not required.

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

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