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Fontan-associated protein-losing enteropathy and post‒heart transplant outcomes: A multicenter study
- Source :
- The Journal of Heart and Lung Transplantation. 38:17-25
- Publication Year :
- 2019
- Publisher :
- Elsevier BV, 2019.
-
Abstract
- BACKGROUND The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.
- Subjects :
- Heart Defects, Congenital
Male
Pulmonary and Respiratory Medicine
medicine.medical_specialty
Adolescent
Protein-Losing Enteropathies
Disease
030204 cardiovascular system & hematology
Fontan Procedure
Young Adult
03 medical and health sciences
Postoperative Complications
0302 clinical medicine
Risk Factors
Interquartile range
Internal medicine
medicine
Humans
Enteropathy
Child
Retrospective Studies
Transplantation
business.industry
Protein losing enteropathy
Infant
Retrospective cohort study
Immunosuppressive regimen
Prognosis
medicine.disease
United States
Survival Rate
Regimen
030228 respiratory system
Multicenter study
Child, Preschool
Heart Transplantation
Female
Surgery
Cardiology and Cardiovascular Medicine
business
Follow-Up Studies
Subjects
Details
- ISSN :
- 10532498
- Volume :
- 38
- Database :
- OpenAIRE
- Journal :
- The Journal of Heart and Lung Transplantation
- Accession number :
- edsair.doi.dedup.....edf74e3bcef05c57800a0a7c9044fba7