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

Published online by Cambridge University Press:  04 May 2020

Anastasia Schleiger ,
Stanislav Ovroutski ,
Björn Peters Open the ORCID record for Björn Peters [Opens in a new window] ,
Stephan Schubert ,
Joachim Photiadis ,
Felix Berger  and
Peter Kramer Open the ORCID record for Peter Kramer [Opens in a new window]
Anastasia Schleiger
Affiliation:
Department of Congenital Heart Disease/Pediatric Cardiology, Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany
Stanislav Ovroutski
Affiliation:
Department of Congenital Heart Disease/Pediatric Cardiology, Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany
Björn Peters
Affiliation:
Department of Congenital Heart Disease/Pediatric Cardiology, Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany
Stephan Schubert
Affiliation:
Department of Congenital Heart Disease/Pediatric Cardiology, Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany
Joachim Photiadis
Affiliation:
Department of Congenital Heart Surgery, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany
Felix Berger
Affiliation:
Department of Congenital Heart Disease/Pediatric Cardiology, Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany Department of Pediatric Cardiology, Charité – Universitaetsmedizin Berlin, Campus Virchow-Klinikum, Augustenburger Platz 1, 13353Berlin, Germany Partner Site Berlin, DZHK (German Center for Cardiovascular Research), Oudenarder Straße 16, 13347Berlin, Germany
Peter Kramer*
Affiliation:
Department of Congenital Heart Disease/Pediatric Cardiology, Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany
*
Author for correspondence: Peter Kramer, MD, Department of Congenital Heart Disease/Pediatric Cardiology Berlin, German Heart Center Berlin, Augustenburger Platz 1, 13353Berlin, Germany. Tel: +49 304 593 2800; Fax: +49 304 593 2900. E-mail: kramerpeter@dhzb.de
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Abstract

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.

Keywords

Protein-losing enteropathyFontan operationtreatment strategieslate mortality
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 5 , May 2020 , pp. 698 - 709
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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