Related Experiment Video
Updated: Feb 25, 2026
Donor Posterior Atrial Flap Rotation for Left Atrial Cuff Reconstruction in Lung Transplantation
Published on: October 11, 2024
Post-transplant lymphoproliferative disease is associated with early sternotomy and left ventricular hypoplasia
Britt-Marie Ekman-Joelsson1, Håkan Wåhlander1, Mats Synnergren1
11Department of Pediatrics,Institution for Clinical Sciences,Sahlgrenska Academy,University of Gothenburg,Gothenburg,Sweden.
Insights
Pediatric heart transplant recipients with congenital heart disease, especially those with infant sternotomy or multiple surgeries, face higher risks of post-transplant lymphoproliferative disorder. Epstein-Barr virus mismatch and tacrolimus also increase this risk.
Area of Science:
- Pediatric Cardiology
- Immunology
- Transplantation Medicine
Background:
- Heart transplantation in children has been available in Sweden since 1989.
- An increased incidence of post-transplant lymphoproliferative disorder (PTLD) was observed, necessitating risk factor identification.
Purpose of the Study:
- To identify risk factors for PTLD in pediatric heart transplant recipients.
- To analyze the correlation between specific clinical and immunological factors and PTLD development.
Main Methods:
- Retrospective analysis of pediatric heart transplant cases (0-18 years) from 1989 to 2014.
- Evaluation of patient demographics, surgical history, viral status, and immunosuppressive regimens.
Main Results:
- PTLD occurred in 14% of pediatric heart transplant recipients.
- Significant risk factors for PTLD included surgically palliated congenital heart disease (CHD), infant sternotomy, hypoplastic left ventricle, multiple surgical events, Epstein-Barr virus (EBV) donor-positive/recipient-negative mismatch, and tacrolimus-based immunosuppression.
- PTLD was exclusively observed in patients with pre-existing CHD.
Conclusions:
- Congenital heart disease, particularly complex forms requiring early surgical intervention, is a primary risk factor for PTLD post-pediatric heart transplantation.
- Infant sternotomy and a higher number of surgical procedures are strongly associated with PTLD development.
- EBV seromismatch and specific immunosuppressive agents like tacrolimus warrant careful consideration in managing PTLD risk.
Background:
Heart transplantation has been an option for children in Sweden since 1989. As our unit faced an increased rate of post-transplant lymphoproliferative disorder, the objective of the study was to identify possible risk factors.
Methods:
This is a retrospective study of all children aged 0-18 years who underwent heart transplantation in Gothenburg from 1989 to 2014.
Results:
A total of 71 children underwent heart transplantation. The overall incidence of post-transplant lymphoproliferative disorder was 14% (10/71); however, 17% (6/36) of those undergoing transplantation after 2007 developed lymphoma, compared with only 10% (4/35) of transplantation cases before 2007 (p=0.85). The mean age at transplantation was 9 years (0-17). The mean post-transplant follow-up time was 5.5 years (0.5-21.9) in the group that developed post-transplant lymphoproliferative disorder, compared with 10.2 years (0.02-25.2) in those who did not. In our study group, risk factors for post-transplant lymphoproliferative disorder were surgically palliated CHD (p=0.0005), sternotomy during infancy (p⩽0.0001), hypoplastic left ventricle (p=0.0001), number of surgical events (p=0.0022), mismatch concerning Epstein-Barr virus infection - that is, a positive donor-negative recipient (p⩽0.0001) - and immunosuppressive treatment with tacrolimus compared with ciclosporine (p=0.028). Discussion This study has three major findings. First, post-transplant lymphoproliferative disorder only developed in subjects born with CHD. Second, the vast majority (9/10) of the subjects developing the disorder had undergone sternotomy as infants. Third, the number of surgical events correlated with a higher risk for developing post-transplant lymphoproliferative disorder.
