Skip to main content

Lund University Publications

LUND UNIVERSITY LIBRARIES

Protein-losing enteropathy following Fontan completion : A 30-year national cohort study in Sweden

Dolk, Mikaela LU orcid ; Schütz, Indra LU ; Herou, Erik LU orcid ; Nordenstam, Felicia ; Bergman, Gunnar ; Synnergren, Mats ; Rydberg, Annika ; Liuba, Petru LU ; Tran, Kiet and Sunnegårdh, Jan , et al. (2026) In Journal of Thoracic and Cardiovascular Surgery 171(3). p.3-731
Abstract

Objective: To characterize patients with protein-losing enteropathy (PLE) diagnosed before age 18 years within a nationwide Swedish Fontan cohort. Methods: Surgical records and medical charts were reviewed for all patients born after January 1, 1993, who underwent Fontan completion before January 1, 2021. Results: Among 573 reviewed patients, 28 (4.8%) developed PLE. The median time to onset of PLE after Fontan was 1.5 years (interquartile range, 0.5-5.5 years). Right ventricular morphology was associated with PLE (odds ratio, 2.3; 95% confidence interval, 1.04-5.0). Twenty-three patients (82%) received PLE-directed therapies, including pulmonary vasodilators (n = 17; 61%), subcutaneous heparin (n = 14; 50%), and/or long-term... (More)

Objective: To characterize patients with protein-losing enteropathy (PLE) diagnosed before age 18 years within a nationwide Swedish Fontan cohort. Methods: Surgical records and medical charts were reviewed for all patients born after January 1, 1993, who underwent Fontan completion before January 1, 2021. Results: Among 573 reviewed patients, 28 (4.8%) developed PLE. The median time to onset of PLE after Fontan was 1.5 years (interquartile range, 0.5-5.5 years). Right ventricular morphology was associated with PLE (odds ratio, 2.3; 95% confidence interval, 1.04-5.0). Twenty-three patients (82%) received PLE-directed therapies, including pulmonary vasodilators (n = 17; 61%), subcutaneous heparin (n = 14; 50%), and/or long-term corticosteroids (n = 13; 46%). Eleven patients (39%) underwent a total of 18 catheter interventions, including 1 fenestration and 1 lymphatic intervention. Three pacemaker procedures were performed after a PLE diagnosis. Eight patients underwent heart transplant (26%). Patients with an early onset of PLE (<2 years from Fontan completion) frequently had a history of pulmonary venous congestion. Five-year overall survival was 88%, and 5-year transplant-free survival was 79%. Conclusions: PLE remains a serious complication following Fontan, with notable mortality. Patients with right ventricular morphology of the systemic ventricle had a higher risk of PLE. Previous pulmonary venous congestion was common when PLE was diagnosed early after Fontan. Catheter-based interventions were frequently used to optimize hemodynamics; lymphatic interventions are likely to become more common. In therapy-refractory patients, heart transplantation remains an option.

(Less)
Please use this url to cite or link to this publication:
author
; ; ; ; ; ; ; ; and , et al. (More)
; ; ; ; ; ; ; ; ; ; and (Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Fontan, protein-losing enteropathy, single ventricle, total cavopulmonary connection
in
Journal of Thoracic and Cardiovascular Surgery
volume
171
issue
3
pages
3 - 731
publisher
Mosby-Elsevier
external identifiers
  • pmid:41260410
  • scopus:105024854613
ISSN
0022-5223
DOI
10.1016/j.jtcvs.2025.11.008
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2025 The Authors
id
33eb8e93-4ec5-4316-8433-848de314f50a
date added to LUP
2026-03-03 15:33:11
date last changed
2026-05-13 03:15:09
@article{33eb8e93-4ec5-4316-8433-848de314f50a,
  abstract     = {{<p>Objective: To characterize patients with protein-losing enteropathy (PLE) diagnosed before age 18 years within a nationwide Swedish Fontan cohort. Methods: Surgical records and medical charts were reviewed for all patients born after January 1, 1993, who underwent Fontan completion before January 1, 2021. Results: Among 573 reviewed patients, 28 (4.8%) developed PLE. The median time to onset of PLE after Fontan was 1.5 years (interquartile range, 0.5-5.5 years). Right ventricular morphology was associated with PLE (odds ratio, 2.3; 95% confidence interval, 1.04-5.0). Twenty-three patients (82%) received PLE-directed therapies, including pulmonary vasodilators (n = 17; 61%), subcutaneous heparin (n = 14; 50%), and/or long-term corticosteroids (n = 13; 46%). Eleven patients (39%) underwent a total of 18 catheter interventions, including 1 fenestration and 1 lymphatic intervention. Three pacemaker procedures were performed after a PLE diagnosis. Eight patients underwent heart transplant (26%). Patients with an early onset of PLE (&lt;2 years from Fontan completion) frequently had a history of pulmonary venous congestion. Five-year overall survival was 88%, and 5-year transplant-free survival was 79%. Conclusions: PLE remains a serious complication following Fontan, with notable mortality. Patients with right ventricular morphology of the systemic ventricle had a higher risk of PLE. Previous pulmonary venous congestion was common when PLE was diagnosed early after Fontan. Catheter-based interventions were frequently used to optimize hemodynamics; lymphatic interventions are likely to become more common. In therapy-refractory patients, heart transplantation remains an option.</p>}},
  author       = {{Dolk, Mikaela and Schütz, Indra and Herou, Erik and Nordenstam, Felicia and Bergman, Gunnar and Synnergren, Mats and Rydberg, Annika and Liuba, Petru and Tran, Kiet and Sunnegårdh, Jan and Hanséus, Katarina and Tran-Lundmark, Karin}},
  issn         = {{0022-5223}},
  keywords     = {{Fontan; protein-losing enteropathy; single ventricle; total cavopulmonary connection}},
  language     = {{eng}},
  number       = {{3}},
  pages        = {{3--731}},
  publisher    = {{Mosby-Elsevier}},
  series       = {{Journal of Thoracic and Cardiovascular Surgery}},
  title        = {{Protein-losing enteropathy following Fontan completion : A 30-year national cohort study in Sweden}},
  url          = {{http://dx.doi.org/10.1016/j.jtcvs.2025.11.008}},
  doi          = {{10.1016/j.jtcvs.2025.11.008}},
  volume       = {{171}},
  year         = {{2026}},
}