RT Journal Article
SR Electronic
T1 Outcome after heart–lung or lung transplantation in patients with Eisenmenger syndrome
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP 127
OP 132
DO 10.1136/heartjnl-2019-315345
VO 106
IS 2
A1 Cristel S Hjortshøj
A1 Thomas Gilljam
A1 Göran Dellgren
A1 Markku O Pentikäinen
A1 Thomas Möller
A1 Annette Schophuus Jensen
A1 Maila Turanlahti
A1 Ulf Thilén
A1 Finn Gustafsson
A1 Lars Søndergaard
YR 2020
UL http://heart.bmj.com/content/106/2/127.abstract
AB 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.