Fontan-associated protein-losing enteropathy and post‒heart transplant outcomes: A multicenter study

Schumacher, K.; Yu, Sunkyung; Butts, Ryan J.; Castleberry, Chesney; Chen, Sharon; Edens, Erik; Godown, Justin; Johnson, Jonathan N.; Kemna, Mariska; Lin, Kimberly Y.; Lowery, Ray; Simpson, Kathleen E.; West, Shawn C.; Wilmot, Ivan; Gossett, Jeffrey G.

doi:10.1016/j.healun.2018.09.024

Cited by 50 publications

(36 citation statements)

References 13 publications

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“…Various studies have accumulated evidence that protein-losing enteropathy resolves in most cases after cardiac transplantation and is not associated with increased transplantation mortality. 16,38 Conclusions Protein-losing enteropathy is a serious end-organ dysfunction limiting survival of Fontan patients. Since no universally successful treatment exists, individualised multimodal treatment strategies combining medical, interventional, and surgical procedures are mandatory.…”

Section: Mortality and Cardiac Transplantationmentioning

confidence: 99%

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients

et al. 2020

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Objective:Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality.Material and Methods:We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986–2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed.Results:Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001–0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival.Conclusions:Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.

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Section: Mortality and Cardiac Transplantationmentioning

confidence: 99%

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients

et al. 2020

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“…Complicating the discussion of risk attribution is the fact that descriptors such as “congenital heart disease” may be confounders for a variety of other pretransplant factors that could increase post‐transplant morbidity and mortality. These include increased incidence of sensitization, complications of single ventricle palliations such as protein losing enteropathy and plastic bronchitis, pulmonary vascular disease, and the potential need for longer bypass times (as a result of extensive adhesions, presence of collaterals, and need for complex reconstructions at time of transplant) as compared to recipients without CHD …”

Section: Discussionmentioning

confidence: 99%

Review of interactions between high‐risk pediatric heart transplant recipients and marginal donors including utilization of risk score models

Gossett

Khulbey

Punnoose

et al. 2020

Pediatric Transplantation

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Background Donor organ acceptance practices vary among pediatric heart transplant professionals. We sought to understand what is known about the interactions between the “high‐risk” recipient and the “marginal donor,” and how donor risk scores can impact this discussion. Methods A systematic review of published literature on pediatric HTx was undertaken with the assistance of a medical librarian. Two authors independently assessed search results, and papers were reviewed for inclusion. Results We found that there are a large number of individual factors, and clusters of factors, that have been used to label individual recipients “high‐risk” and individual donors “marginal.” The terms “high‐risk recipient” and “marginal donor” have been used broadly in the literature making it virtually impossible to make comparisons between publications. In general, the data support that patients who could be easily agreed to be “sicker recipients” are at more risk compared to those who are clearly “healthier,” albeit still “sick enough” to need transplantation. Given this variability in the literature, we were unable to define how being a “high‐risk” recipient interplays with accepting a “marginal donor.” Existing risk scores are described, but none were felt to adequately predict outcomes from factors available at the time of offer acceptance. Conclusions We could not determine what makes a donor “marginal,” a recipient “high‐risk,” or how these factors interplay within the specific recipient‐donor pair to determine outcomes. Until there are better risk scores predicting outcomes at the time of organ acceptance, programs should continue to evaluate each organ and recipient individually.

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“…24 Several studies suggested that higher albumin level may improve intravascular volume and symptoms including edema, pleural effusion, chylothorax, and failure to thrive. 7,10,[24][25][26] Besides high-protein diet, other dietary recommendations for patients with PLE also include low-fat diet with medium-chain triglyceride supplementation. 24,27 Several medications are being used to decrease enteric protein loss, such as subcutaneous unfractionated heparin, octreotide, and systemic steroids.…”

Section: Discussionmentioning

confidence: 99%

Budesonide for Protein Losing Enteropathy in Patients with Fontan Circulation: A Systematic Review and Meta-Analysis

Kewcharoen

Mekraksakit

Limpruttidham

et al. 2019

World J Pediatr Congenit Heart Surg

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Background: Recent studies have shown that oral budesonide can be used to improve albumin level in patients with protein-losing enteropathy (PLE) following Fontan procedure. However, there has never been a systematic review and meta-analysis to confirm this finding. We performed a systematic review and meta-analysis to explore the therapeutic effect of budesonide in patients with PLE post-Fontan procedure. Methods: We searched the databases of MEDLINE and EMBASE from inception to January 2019. Included studies were published studies that evaluate albumin level before and after budesonide therapy in patients with PLE following Fontan procedure. Data from each study were combined using the random-effects model. Results: Five studies with 36 post-Fontan operation patients with PLE were included. In random-effects model, there was a statistically significant difference in albumin level between before and after budesonide treatment (weighted mean difference = 1.28, 95% confidence interval: 0.76-1.79). No publication bias was observed on a funnel plot and Egger test with a P value of .676. Conclusions: The results of this systematic review and meta-analysis show that budesonide can be used to increase albumin level in patients with PLE following Fontan operation. Further studies may focus on the impact of outcome of budesonide in this population.

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Fontan-associated protein-losing enteropathy and post‒heart transplant outcomes: A multicenter study

Cited by 50 publications

References 13 publications

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients

Review of interactions between high‐risk pediatric heart transplant recipients and marginal donors including utilization of risk score models

Budesonide for Protein Losing Enteropathy in Patients with Fontan Circulation: A Systematic Review and Meta-Analysis

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