The Nordic Experience of Pediatric Heart-Lung Transplantation

Pediatric heart-lung transplantation (pHLTx) remains the only treatment option for children with significant and fixed elevation of pulmonary vascular resistance (PVR) in the setting of uncorrectable end-stage heart disease. We aimed to determine the characteristics, waiting list course and long-term outcomes for children listed for pHLTx within the Scandiatransplant region.

This was a multinational, retrospective observational cohort study comprising all children <18 years of age residing in Denmark, Estonia, Finland, Iceland, Norway and Sweden that were listed for pHLTx from January first 1987 until December 31^st 2024. Data were extracted from the Scandiatransplant registry database.

Thirty-two children were listed for pHLTx during the study period. Seventeen (53.1%) reached transplantation with a peak in listing and transplantation during the 1990ies. Median age at listing was 12.1 years (range 1.5-17.6, IQR 7.6-15.8). No infants <1 year were listed for pHLTx and there was no re-listing for re-transplantation during the study period. Congenital heart disease (CHD) with Eisenmenger´s syndrome was the cause of listing and transplantation in most cases (60.7% and 58.8%, respectively). Median waiting list duration was 268 days (range 3-3409, IQR 109-822). All-cause posttransplant 30-day mortality was 17.6% (n = 3). Kaplan-Meier estimated survival was 76.5% at 1 year, 35.3% at 5 years and 23.5% at 10 years posttransplant.

Low numbers of pHLTx has been performed in Scandinavia. Long-term outcomes were unfavorable, but comparable to other larger international and North American registries. The declining number of performed pHLTx may result from a combination of poor prognosis, declining incidence of Eisenmenger´s syndrome, improved surgical techniques for cardiac repair combined with lung transplantation, and increasing use of lung transplantation for isolated pulmonary vascular disease.